Category Archive Eurozone

Swallowing problems for liquids and solids

Swallowing problems for liquids and solids/Achalasia is a rare neurodegenerative motor smooth muscle motility disorder of the esophagus resulting in deranged oesophageal peristalsis and loss of lower oesophageal sphincter function that makes it difficult for food and liquid to pass into your stomach. Achalasia occurs when nerves in the tube connecting your mouth and stomach (esophagus) become damaged. As a result, the esophagus loses the ability to squeeze food down, and the muscular valve between the esophagus and stomach (lower esophageal sphincter) doesn’t fully relax — making it difficult for food to pass into your stomach.

Achalasia is a rare disorder that makes it difficult for food and liquid to pass from the swallowing tube connecting your mouth and stomach (esophagus) into your stomach.

Synonyms of Achalasia

  • Cardiospasm
  • Dyssynergia esophagus
  • Esophageal peristalsis
  • Megaesophagus
  • Esophageal achalasia;
  • Swallowing problems for liquids and solids;
  • lower esophageal sphincter spasm

Types of Achalasia

  • Achalasia Type 1 (Classic Achalasia)
    • No contractility or peristalsis
    • The lower esophageal sphincter fails to relax (all Achalasia types)
    • Responds to Laparoscopic Heller Myotomy
  • Achalasia Type 2 (with esophageal compression)
    • No normal peristalsis (but some pressurizations)
    • The lower esophageal sphincter fails to relax (all Achalasia types)
    • Responds to all treatment options
  • Achalasia Type 3 (Spastic Achalasia)
    • No normal peristalsis
    • Spastic contractions in distal esophagus (>20% of swallows)
    • The lower esophageal sphincter fails to relax (all Achalasia types)
    • Responds poorly to treatment

Causes of Achalasia

Dysphagia could be during the oropharyngeal or pharyngeal phases of swallowing.

A. Oropharyngeal dysphagia

It is a delay in the transit of liquid or solid bolus during the oropharyngeal phase of swallowing. It could be due to three main subgroups – (1) neurological, (2) muscular, or (3) anatomical.

  • Neurological causes include cerebrovascular accidents (post-stroke dysphagia), brainstem infarctions with cranial nerve involvement. Other causes include basal ganglia lesions as in Parkinson’s disease. Also, head and neck injuries and surgery, multiple sclerosis, central nervous tumor, botulism, amyotrophic lateral sclerosis, supranuclear palsy, and degenerative cervical spine disease.
  • Muscular causes include polymyositis, muscular dystrophy, and myasthenia gravis (a lesion at the neuromuscular junction).
  • Anatomical causes include Zenker diverticulum, enlarged thyroid, esophageal web, tumors, abscess, external compression by an aortic aneurysm (known as dysphagia aortic). Also, cervical discectomy and fusion may be associated with postoperative dysphagia.

B. Esophageal dysphagia- could be due to mechanical obstruction, or motility disorders. 

  • Mechanical obstruction causes include Schatzki ring, esophageal stricture, esophageal carcinoma, eosinophilic esophagitis.
  • Motility disorder causes include esophageal spasm, achalasia, ineffective esophageal motility, and scleroderma.

Mechanical obstruction is associated with dysphagia only to solid food, while the motility disorder causes are usually associated with solid and liquid dysphagia. The dysphagia may be intermittent (e.g., Schatzki ring, esophageal spasm) or permanent (as in esophageal stricture, carcinoma, achalasia, scleroderma, ineffective esophageal motility).

C. Rheumatological disorders

  • Sjogren syndrome (occurs in one-third of patients and caused by both xerostomia and abnormal esophageal motility, mainly of the proximal esophagus.
  • Systemic lupus erythematosus
  • Mixed connective tissue disease
  • Rheumatoid arthritis.
  • Systemic sclerosis (as part of the CREST syndrome)

D. Medications

Several drugs may contribute to the severity of dysphagia. The mechanisms by which these drugs may cause dysphagia include xerostomia and changes in esophageal motility. Also, the dysphagia may be secondary to the development of drug-induced esophagitis or the development of gastroesophageal reflux disease. Examples of these drugs are:

  • Antipsychotic (e.g., olanzapine, clozapine)
  • Tricyclic antidepressant
  • Potassium supplements
  • NSAIDs
  • Bisphosphonates
  • Calcium channel blockers
  • Nitrates
  • Theophylline
  • Alcohol
  • Medications with immunosuppressant effects (e.g., cyclosporin) can predispose to infective esophagitis and dysphagia
  • Opioids

It is important to note here that narcotic sedatives such as opioids can lead to compromise of airway due to central effects and could increase the risk of aspiration in patients with dysphagia. The use of opiates, even in low disease, in patients with psychiatric disorders or Parkinson’s disease, can develop hypercontractile or hypertensive esophageal consequences mimicking type III achalasia.

Others

  • Diffuse esophageal spasm.
  • Esophageal cancer.
  • Eosinophilic esophagitis
  • Hiatal hernia.
  • Parkinson disease.
  • Zenker diverticulum.
  • Multiple sclerosis.
  • Paterson-Kelly syndrome.
  • Dysphagia lusoria is a type of dysphagia that develops in childhood, due to compression of the esophagus by vascular abnormality. Usually, there is an aberrant right subclavian artery arising from the left side of the aortic arch, or a double aortic arch, or other rare anomalies.
  • Benign strictures.
  • Esophageal webs and rings
  • Esophageal reflux

Symptoms of Achalasia

Achalasia symptoms generally appear gradually and worsen over time. Signs and symptoms may include:

  • Inability to swallow (dysphagia), which may feel like food or drink is stuck in your throat
  • Regurgitating food or saliva
  • Heartburn
  • Belching
  • Chest pain that comes and goes
  • Coughing at night
  • Pneumonia (from aspiration of food into the lungs)
  • Weight loss
  • Vomiting
  • Trouble swallowing (dysphagia). This is the most common early symptom.
  • Regurgitation of undigested food.
  • Chest pain that comes and goes; pain can be severe.
  • Cough at night
  • Weight loss/malnutrition from difficulty eating. This is a late symptom.
  • Hiccups, difficulty belching (less common symptoms)

Diagnosis of Achalasia

Achalasia can be overlooked or misdiagnosed because it has symptoms similar to other digestive disorders. To test for achalasia, your doctor is likely to recommend:

  • Endoscopy – Approximately 2% to 4% of patients with suspected achalasia have pseudoachalasia from infiltrating malignancy or stricture. Potential risk factors for malignancy-associated pseudoachalasia include older age at the time of diagnosis, shorter duration of symptoms, and more weight loss (12 vs 5 kg) on presentation. Patients with 2 or more of these risk factors on presentation should undergo a careful investigation to rule out malignancy.,
  • Barium esophagram – A barium esophagram is a noninvasive radiologic study that can assist with initial diagnosis or response to treatment with graded PD. A barium swallow evaluates the morphology of the esophagus and classically shows a dilated or tortuous esophagus with a narrowed LES and “bird’s beak” appearance.
  • Manometry – HRM is the gold standard test for the diagnosis of achalasia. Conventional manometry tracings in patients with achalasia show the absence of esophageal peristalsis and incomplete LES relaxation with residual pressures of over 10 mm Hg. HRM with esophageal pressure topography is more sensitive and specific than conventional manometry and is able to classify achalasia into 3 distinct subtypes, which can have treatment implications. Type II achalasia has the best response to treatment, followed by type I achalasia, whereas type III achalasia is the most difficult to treat.,
  • Esophageal manometry. This test measures the rhythmic muscle contractions in your esophagus when you swallow, the coordination and force exerted by the esophagus muscles, and how well your lower esophageal sphincter relaxes or opens during a swallow. This test is the most helpful when determining which type of motility problem you might have.
  • X-rays of your upper digestive system (esophagram). X-rays are taken after you drink a chalky liquid that coats and fills the inside lining of your digestive tract. The coating allows your doctor to see a silhouette of your esophagus, stomach, and upper intestine. You may also be asked to swallow a barium pill that can help to show a blockage of the esophagus.
  • Upper endoscopy. Your doctor inserts a thin, flexible tube equipped with a light and camera (endoscope) down your throat, to examine the inside of your esophagus and stomach. Endoscopy can be used to define a partial blockage of the esophagus if your symptoms or results of a barium study indicate that possibility. Endoscopy can also be used to collect a sample of tissue (biopsy) to be tested for complications of reflux such as Barrett’s esophagus.

Treatment of Achalasia

Achalasia treatment focuses on relaxing or stretching open the lower esophageal sphincter so that food and liquid can move more easily through your digestive tract.

Specific treatment depends on your age, health condition and the severity of the achalasia.

Nonsurgical treatment

Nonsurgical options include:

  • The management plan. may include (i) elimination of certain food consistencies from the diet. (ii) adjustment of meal bolus seizes and (iii) use of techniques such as chin-tuck, head-turn, and supraglottic maneuvers to help in minimizing/preventing aspiration. Also, strengthening and coordinating muscles involved in swallowing. Gastroscopy tubes may be indicated in patients who fail to respond to the above-stated measures.
  • Pneumatic dilation. A balloon is inserted by endoscopy into the center of the esophageal sphincter and inflated to enlarge the opening. This outpatient procedure may need to be repeated if the esophageal sphincter doesn’t stay open. Nearly one-third of people treated with balloon dilation need repeat treatment within five years. This procedure requires sedation.
  • Botox (botulinum toxin type A). This muscle relaxant can be injected directly into the esophageal sphincter with an endoscopic needle. The injections may need to be repeated, and repeat injections may make it more difficult to perform surgery later if needed. Botox is generally recommended only for people who aren’t good candidates for pneumatic dilation or surgery due to age or overall health. Botox injections typically do not last more than six months. A strong improvement from the injection of Botox may help confirm a diagnosis of achalasia.
  • BT injection. for achalasia is an effective short-term therapy. BT injection into the LES locally inhibits the release of acetylcholine, causing relaxation of the smooth muscle, which allows for easier passage of food bolus into the gastric body.
  • Balloon dilation. In this non-surgical procedure, you’ll be put under light sedation while a specifically designed balloon is inserted through the LES and then inflated. The procedure relaxes the muscle sphincter, which allows food to enter your stomach. Balloon dilation is usually the first treatment option in people in whom surgery fails. You may have to undergo several dilation treatments to relieve your symptoms, and every few years to maintain relief.
  • Stretching the esophagus (pneumatic dilation). The doctor inserts a balloon in the valve between the esophagus and stomach and blows it up to stretch the tight muscles. You might need this procedure several times before it helps.


Medication

  • Muscle relaxants – such as nitroglycerin (Nitrostat) or nifedipine (Procardia) before eating. These medications have limited treatment effects and severe side effects. Medications are generally considered only if you’re not a candidate for pneumatic dilation or surgery, and Botox hasn’t helped. This type of therapy is rarely indicated.
  • Sublingual nifedipine – significantly improves outcomes in 75% of people with mild or moderate disease. It was classically considered that surgical myotomy provided greater benefit than either botulinum toxin or dilation in those who fail medical management.[rx] However, a recent randomized controlled trial found pneumatic dilation to be non-inferior to laparoscopic Heller myotomy.[rx]
  • Pharmacotherapy-nitrates, calcium-channel blockers – (e.g., nifedipine 10 to 20 mg sublingual 15 to 30 minutes before meals). It acts by lowering the lower esophageal sphincter resting pressure. Nitrates, calcium channel blockers, and phosphodiesterase-5 inhibitors to reduce the lower esophageal sphincter (LES) pressure.
  • Calcium channel blockers  – inhibit the entry of calcium into the cells blocking smooth muscle contraction, leading to a decrease in LES pressure. Hypotension, pedal edema, headache, the rapid development of tolerance, and incomplete symptom improvement are limiting factors to its use. Nitrates increase nitric oxide concentrations in smooth muscles, causing an increase in cyclic adenosine monophosphate levels, which leads to smooth muscle relaxation. These treatments are less effective, provide only short-term relief of symptoms, and are primarily reserved for patients who are waiting for or who refused more definitive therapy, such as pneumatic dilatation or surgery.
  • Scopolamine – also known as hyoscine Devil’s Breath, is a natural or synthetically produced tropane alkaloid and anticholinergic drug that is formally used as a medication for treating motion and sickness, achalasia, and postoperative nausea and vomiting. It is also sometimes used before surgery to decrease saliva.[rx] When used by injection, effects begin after about 20 minutes and last for up to 8 hours.[rx] It may also be used orally and as a transdermal patch.[rx]


Surgery

Surgical options for treating achalasia include:

  • Peroral endoscopic myotomy (POEM) – is an effective minimally invasive alternative to laparoscopic Heller myotomy to treat achalasia at limited centers. Dissection of the circular fibers of the LES is achieved endoscopically, leading to relaxation of the LES; however, the risk of gastroesophageal reflux is high because it does not include an antireflux procedure. Esophagectomy is the last resort.
  • Heller myotomy. The surgeon cuts the muscle at the lower end of the esophageal sphincter to allow food to pass more easily into the stomach. The procedure can be done noninvasively (laparoscopic Heller myotomy). Some people who have a Heller myotomy may later develop gastroesophageal reflux disease (GERD). To avoid future problems with GERD, a procedure known as fundoplication might be performed at the same time as a Heller myotomy. In fundoplication, the surgeon wraps the top of your stomach around the lower esophagus to create an anti-reflux valve, preventing acid from coming back (GERD) into the esophagus. Fundoplication is usually done with a minimally invasive (laparoscopic) procedure.
  • Peroral endoscopic myotomy (POEM). In the POEM procedure, the surgeon uses an endoscope inserted through your mouth and down your throat to create an incision in the inside lining of your esophagus. Then, as in a Heller myotomy, the surgeon cuts the muscle at the lower end of the esophageal sphincter. POEM may also be combined with or followed by later fundoplication to help prevent GERD. Some patients who have a POEM and develop GERD after the procedure are treated with daily oral medication.

References

Lower Esophageal Sphincter Spasm – Symptoms, Treatment

Lower Esophageal Sphincter Spasm/Achalasia is a rare neurodegenerative motor smooth muscle motility disorder of the esophagus resulting in deranged oesophageal peristalsis and loss of lower oesophageal sphincter function that makes it difficult for food and liquid to pass into your stomach. Achalasia occurs when nerves in the tube connecting your mouth and stomach (esophagus) become damaged. As a result, the esophagus loses the ability to squeeze food down, and the muscular valve between the esophagus and stomach (lower esophageal sphincter) doesn’t fully relax — making it difficult for food to pass into your stomach.

Achalasia is a rare disorder that makes it difficult for food and liquid to pass from the swallowing tube connecting your mouth and stomach (esophagus) into your stomach.

Synonyms of Achalasia

  • Cardiospasm
  • Dyssynergia esophagus
  • Esophageal peristalsis
  • Megaesophagus
  • Esophageal achalasia;
  • Swallowing problems for liquids and solids;
  • lower esophageal sphincter spasm

Types of Achalasia

  • Achalasia Type 1 (Classic Achalasia)
    • No contractility or peristalsis
    • The lower esophageal sphincter fails to relax (all Achalasia types)
    • Responds to Laparoscopic Heller Myotomy
  • Achalasia Type 2 (with esophageal compression)
    • No normal peristalsis (but some pressurizations)
    • The lower esophageal sphincter fails to relax (all Achalasia types)
    • Responds to all treatment options
  • Achalasia Type 3 (Spastic Achalasia)
    • No normal peristalsis
    • Spastic contractions in distal esophagus (>20% of swallows)
    • The lower esophageal sphincter fails to relax (all Achalasia types)
    • Responds poorly to treatment

Causes of Achalasia

Dysphagia could be during the oropharyngeal or pharyngeal phases of swallowing.

A. Oropharyngeal dysphagia

It is a delay in the transit of liquid or solid bolus during the oropharyngeal phase of swallowing. It could be due to three main subgroups – (1) neurological, (2) muscular, or (3) anatomical.

  • Neurological causes include cerebrovascular accidents (post-stroke dysphagia), brainstem infarctions with cranial nerve involvement. Other causes include basal ganglia lesions as in Parkinson’s disease. Also, head and neck injuries and surgery, multiple sclerosis, central nervous tumor, botulism, amyotrophic lateral sclerosis, supranuclear palsy, and degenerative cervical spine disease.
  • Muscular causes include polymyositis, muscular dystrophy, and myasthenia gravis (a lesion at the neuromuscular junction).
  • Anatomical causes include Zenker diverticulum, enlarged thyroid, esophageal web, tumors, abscess, external compression by an aortic aneurysm (known as dysphagia aortic). Also, cervical discectomy and fusion may be associated with postoperative dysphagia.

B. Esophageal dysphagia- could be due to mechanical obstruction, or motility disorders. 

  • Mechanical obstruction causes include Schatzki ring, esophageal stricture, esophageal carcinoma, eosinophilic esophagitis.
  • Motility disorder causes include esophageal spasm, achalasia, ineffective esophageal motility, and scleroderma.

Mechanical obstruction is associated with dysphagia only to solid food, while the motility disorder causes are usually associated with solid and liquid dysphagia. The dysphagia may be intermittent (e.g., Schatzki ring, esophageal spasm) or permanent (as in esophageal stricture, carcinoma, achalasia, scleroderma, ineffective esophageal motility).

C. Rheumatological disorders

  • Sjogren syndrome (occurs in one-third of patients and caused by both xerostomia and abnormal esophageal motility, mainly of the proximal esophagus.
  • Systemic lupus erythematosus
  • Mixed connective tissue disease
  • Rheumatoid arthritis.
  • Systemic sclerosis (as part of the CREST syndrome)

D. Medications

Several drugs may contribute to the severity of dysphagia. The mechanisms by which these drugs may cause dysphagia include xerostomia and changes in esophageal motility. Also, the dysphagia may be secondary to the development of drug-induced esophagitis or the development of gastroesophageal reflux disease. Examples of these drugs are:

  • Antipsychotic (e.g., olanzapine, clozapine)
  • Tricyclic antidepressant
  • Potassium supplements
  • NSAIDs
  • Bisphosphonates
  • Calcium channel blockers
  • Nitrates
  • Theophylline
  • Alcohol
  • Medications with immunosuppressant effects (e.g., cyclosporin) can predispose to infective esophagitis and dysphagia
  • Opioids

It is important to note here that narcotic sedatives such as opioids can lead to compromise of airway due to central effects and could increase the risk of aspiration in patients with dysphagia. The use of opiates, even in low disease, in patients with psychiatric disorders or Parkinson’s disease, can develop hypercontractile or hypertensive esophageal consequences mimicking type III achalasia.

Others

  • Diffuse esophageal spasm.
  • Esophageal cancer.
  • Eosinophilic esophagitis
  • Hiatal hernia.
  • Parkinson disease.
  • Zenker diverticulum.
  • Multiple sclerosis.
  • Paterson-Kelly syndrome.
  • Dysphagia lusoria is a type of dysphagia that develops in childhood, due to compression of the esophagus by vascular abnormality. Usually, there is an aberrant right subclavian artery arising from the left side of the aortic arch, or a double aortic arch, or other rare anomalies.
  • Benign strictures.
  • Esophageal webs and rings
  • Esophageal reflux

Symptoms of Achalasia

Achalasia symptoms generally appear gradually and worsen over time. Signs and symptoms may include:

  • Inability to swallow (dysphagia), which may feel like food or drink is stuck in your throat
  • Regurgitating food or saliva
  • Heartburn
  • Belching
  • Chest pain that comes and goes
  • Coughing at night
  • Pneumonia (from aspiration of food into the lungs)
  • Weight loss
  • Vomiting
  • Trouble swallowing (dysphagia). This is the most common early symptom.
  • Regurgitation of undigested food.
  • Chest pain that comes and goes; pain can be severe.
  • Cough at night
  • Weight loss/malnutrition from difficulty eating. This is a late symptom.
  • Hiccups, difficulty belching (less common symptoms)

Diagnosis of Achalasia

Achalasia can be overlooked or misdiagnosed because it has symptoms similar to other digestive disorders. To test for achalasia, your doctor is likely to recommend:

  • Endoscopy – Approximately 2% to 4% of patients with suspected achalasia have pseudoachalasia from infiltrating malignancy or stricture. Potential risk factors for malignancy-associated pseudoachalasia include older age at the time of diagnosis, shorter duration of symptoms, and more weight loss (12 vs 5 kg) on presentation. Patients with 2 or more of these risk factors on presentation should undergo a careful investigation to rule out malignancy.,
  • Barium esophagram – A barium esophagram is a noninvasive radiologic study that can assist with initial diagnosis or response to treatment with graded PD. A barium swallow evaluates the morphology of the esophagus and classically shows a dilated or tortuous esophagus with a narrowed LES and “bird’s beak” appearance (Figure 5).
  • Manometry – HRM is the gold standard test for the diagnosis of achalasia. Conventional manometry tracings in patients with achalasia show the absence of esophageal peristalsis and incomplete LES relaxation with residual pressures of over 10 mm Hg. HRM with esophageal pressure topography is more sensitive and specific than conventional manometry and is able to classify achalasia into 3 distinct subtypes, which can have treatment implications (Table 3). Type II achalasia has the best response to treatment, followed by type I achalasia, whereas type III achalasia is the most difficult to treat.,
  • Esophageal manometry. This test measures the rhythmic muscle contractions in your esophagus when you swallow, the coordination and force exerted by the esophagus muscles, and how well your lower esophageal sphincter relaxes or opens during a swallow. This test is the most helpful when determining which type of motility problem you might have.
  • X-rays of your upper digestive system (esophagram). X-rays are taken after you drink a chalky liquid that coats and fills the inside lining of your digestive tract. The coating allows your doctor to see a silhouette of your esophagus, stomach, and upper intestine. You may also be asked to swallow a barium pill that can help to show a blockage of the esophagus.
  • Upper endoscopy. Your doctor inserts a thin, flexible tube equipped with a light and camera (endoscope) down your throat, to examine the inside of your esophagus and stomach. Endoscopy can be used to define a partial blockage of the esophagus if your symptoms or results of a barium study indicate that possibility. Endoscopy can also be used to collect a sample of tissue (biopsy) to be tested for complications of reflux such as Barrett’s esophagus.

Treatment of Achalasia

Achalasia treatment focuses on relaxing or stretching open the lower esophageal sphincter so that food and liquid can move more easily through your digestive tract.

Specific treatment depends on your age, health condition and the severity of the achalasia.

Nonsurgical treatment

Nonsurgical options include:

  • The management plan. may include (i) elimination of certain food consistencies from the diet. (ii) adjustment of meal bolus seizes and (iii) use of techniques such as chin-tuck, head-turn, and supraglottic maneuvers to help in minimizing/preventing aspiration. Also, strengthening and coordinating muscles involved in swallowing. Gastroscopy tubes may be indicated in patients who fail to respond to the above-stated measures.
  • Pneumatic dilation. A balloon is inserted by endoscopy into the center of the esophageal sphincter and inflated to enlarge the opening. This outpatient procedure may need to be repeated if the esophageal sphincter doesn’t stay open. Nearly one-third of people treated with balloon dilation need repeat treatment within five years. This procedure requires sedation.
  • Botox (botulinum toxin type A). This muscle relaxant can be injected directly into the esophageal sphincter with an endoscopic needle. The injections may need to be repeated, and repeat injections may make it more difficult to perform surgery later if needed. Botox is generally recommended only for people who aren’t good candidates for pneumatic dilation or surgery due to age or overall health. Botox injections typically do not last more than six months. A strong improvement from the injection of Botox may help confirm a diagnosis of achalasia.
  • BT injection. for achalasia is an effective short-term therapy. BT injection into the LES locally inhibits the release of acetylcholine, causing relaxation of the smooth muscle, which allows for easier passage of food bolus into the gastric body.
  • Balloon dilation. In this non-surgical procedure, you’ll be put under light sedation while a specifically designed balloon is inserted through the LES and then inflated. The procedure relaxes the muscle sphincter, which allows food to enter your stomach. Balloon dilation is usually the first treatment option in people in whom surgery fails. You may have to undergo several dilation treatments to relieve your symptoms, and every few years to maintain relief.
  • Stretching the esophagus (pneumatic dilation). The doctor inserts a balloon in the valve between the esophagus and stomach and blows it up to stretch the tight muscles. You might need this procedure several times before it helps.


Medication.

  • Muscle relaxants – such as nitroglycerin (Nitrostat) or nifedipine (Procardia) before eating. These medications have limited treatment effects and severe side effects. Medications are generally considered only if you’re not a candidate for pneumatic dilation or surgery, and Botox hasn’t helped. This type of therapy is rarely indicated.
  • Sublingual nifedipine – significantly improves outcomes in 75% of people with mild or moderate disease. It was classically considered that surgical myotomy provided greater benefit than either botulinum toxin or dilation in those who fail medical management.[rx] However, a recent randomized controlled trial found pneumatic dilation to be non-inferior to laparoscopic Heller myotomy.[rx]
  • Pharmacotherapy-nitrates, calcium-channel blockers – (e.g., nifedipine 10 to 20 mg sublingual 15 to 30 minutes before meals). It acts by lowering the lower esophageal sphincter resting pressure. Nitrates, calcium channel blockers, and phosphodiesterase-5 inhibitors to reduce the lower esophageal sphincter (LES) pressure.
  • Calcium channel blockers  – inhibit the entry of calcium into the cells blocking smooth muscle contraction, leading to a decrease in LES pressure. Hypotension, pedal edema, headache, the rapid development of tolerance, and incomplete symptom improvement are limiting factors to its use. Nitrates increase nitric oxide concentrations in smooth muscles, causing an increase in cyclic adenosine monophosphate levels, which leads to smooth muscle relaxation. These treatments are less effective, provide only short-term relief of symptoms, and are primarily reserved for patients who are waiting for or who refused more definitive therapy, such as pneumatic dilatation or surgery.
  • Scopolamine – also known as hyoscine Devil’s Breath, is a natural or synthetically produced tropane alkaloid and anticholinergic drug that is formally used as a medication for treating motion and sickness, achalasia, and postoperative nausea and vomiting. It is also sometimes used before surgery to decrease saliva.[rx] When used by injection, effects begin after about 20 minutes and last for up to 8 hours.[rx] It may also be used orally and as a transdermal patch.[rx]


Surgery

Surgical options for treating achalasia include:

  • Peroral endoscopic myotomy (POEM) – is an effective minimally invasive alternative to laparoscopic Heller myotomy to treat achalasia at limited centers. Dissection of the circular fibers of the LES is achieved endoscopically, leading to relaxation of the LES; however, the risk of gastroesophageal reflux is high because it does not include an antireflux procedure. Esophagectomy is the last resort.
  • Heller myotomy. The surgeon cuts the muscle at the lower end of the esophageal sphincter to allow food to pass more easily into the stomach. The procedure can be done noninvasively (laparoscopic Heller myotomy). Some people who have a Heller myotomy may later develop gastroesophageal reflux disease (GERD). To avoid future problems with GERD, a procedure known as fundoplication might be performed at the same time as a Heller myotomy. In fundoplication, the surgeon wraps the top of your stomach around the lower esophagus to create an anti-reflux valve, preventing acid from coming back (GERD) into the esophagus. Fundoplication is usually done with a minimally invasive (laparoscopic) procedure.
  • Peroral endoscopic myotomy (POEM). In the POEM procedure, the surgeon uses an endoscope inserted through your mouth and down your throat to create an incision in the inside lining of your esophagus. Then, as in a Heller myotomy, the surgeon cuts the muscle at the lower end of the esophageal sphincter. POEM may also be combined with or followed by later fundoplication to help prevent GERD. Some patients who have a POEM and develop GERD after the procedure are treated with daily oral medication.

References

Altitude-Related Disorders – Causes, Symptoms, Treatment

Altitude-Related Disorders/Altitude sickness occurs when you cannot get enough oxygen from the air at high altitudes. This causes symptoms such as a headache, loss of appetite, and trouble sleeping. It happens most often when people who are not used to high altitudes go quickly from lower altitudes to 8000 ft (2500 m) or higher. For example, you may get a headache when you drive over a high mountain pass, hike to a high altitude, or arrive at a mountain resort.

Acute mountain sickness (AMS) is a syndrome that arises in non-acclimatized individuals who ascend to high altitudes. It is a form of acute altitude illness that occurs due to a decrease in the atmospheric partial pressure of oxygen as the altitude increases, inducing hypoxia. This includes acute mountain sickness (AMS), high-altitude cerebral oedema (HACE), and high-altitude pulmonary edema (HAPE).  This condition typically occurs at an altitude of >2500 meters; however, it can occur at lower elevations in high-risk individuals.

Types of High Altitude Sickness

High altitude oxygenation is improving oxygenation or enriching the body with additional oxygen at high altitudes.

According to the Society of Mountain Medicine (Effects of high altitude on humans), there are three altitude regions:

  • High Altitude  – 1500 to 3500 meters above sea level (4900-11500 ft.)
  • Very high altitude – 3500 to 5500 meters above sea level (11500 to 18000 ft.)
  • Extreme altitude –  above 5500 meters above sea level (18000 ft.)

High altitude (1500 to 3500 m)

  • The onset of physiologic effects of diminished inspiratory oxygen pressure (PIO2) includes decreased exercise performance and increased ventilation (lowering of arterial PaCO2).
  • Minor impairment exists in arterial oxygen transport (arterial oxygen saturation [SaO2] at least 90%, but arterial PO2 is significantly diminished).
  • Because of the large number of people who ascend rapidly to 2500 to 3500 m, high-altitude illness is common in this range.

Very high altitude (3500 to 5500 m)

  • Maximum SaO2 falls below 90% as the arterial PO2 falls below 60 mm Hg.
  • Extreme hypoxemia may occur during exercise, during sleep and in the presence of high-altitude pulmonary edema or other acute lung conditions.
  • Severe altitude illness commonly occurs in this range.

Extreme altitude (above 5500 m)

  • Marked hypoxemia, hypocapnia and alkalosis are characteristic of extreme altitudes.
  • Progressive impairment of physiologic function eventually outstrips acclimatization. As a result, no human habitation occurs above 5500 m.[]

Most people who get altitude sickness get AMS, acute mountain sickness. Higher than 10,000 feet, 75% of people will get mild symptoms. There are three categories of AMS:

  • Mild AMS – Symptoms, such as mild headache and fatigue, don’t interfere with your normal activity. Symptoms improve after a few days as your body acclimates. You can likely stay at your current elevation as your body adjusts.
  • Moderate AMS – Symptoms start to interfere with your activities. You may experience severe headaches, nausea, and difficulty with coordination. You’ll need to descend to start to feel better.
  • Severe AMS – You may feel short of breath, even at rest. It can be difficult to walk. You need to descend immediately to a lower altitude and seek medical care.
  • HAPE (High-altitude pulmonary edema) – HAPE produces excess fluid on the lungs, causing breathlessness, even when resting. You feel very fatigued and weak and may feel like you’re suffocating.
  • HACE (High-altitude cerebral edema) – HACE involves excess fluid on the brain, causing brain swelling. You may experience confusion, lack of coordination, and possibly violent behavior.

Causes of Altitude-Related Disorders

Acute Mountain Sickness is caused by the body’s reaction to the reduced oxygen level in respired air and resultant tissue hypoxia. At baseline metabolic levels, the brain is the most sensitive organ regarding hypoxia and oxygen stress. Thus, the symptoms of Acute Mountain Sickness (discussed below) are mediated by the central nervous system (CNS). In many travelers at altitude, respirations during sleep develop a periodic pattern that may contribute to the development of symptoms.

Along with other illnesses related to altitude, HAPE occurs above 2500 meters but can occur at altitudes as low as 2000 meters. Risk factors include individual susceptibility due to low hypoxic ventilatory response (HVR), the altitude attained, a rapid rate of ascent, male sex, use of sleep medication, excessive salt ingestion, ambient cold temperature, and heavy physical exertion. Preexisting conditions such as those leading to increased pulmonary blood flow, pulmonary hypertension, increased pulmonary vascular reactivity, or patent foramen ovale may have a higher predisposition towards the development of HAPE.

  • Asthma
  • Bronchitis
  • Mucous plugging
  • Myocardial infarction
  • Pneumonia
  • Pneumothorax
  • Pulmonary embolism
  • Upper respiratory tract infection
  • Acute psychosis
  • Brain tumour
  • Carbon monoxide poisoning
  • Central nervous system infection
  • Cerebrovascular bleed or infarct
  • Cerebrovascular spasm
  • Diabetic ketoacidosis
  • Hypoglycemia
  • Hyponatremia
  • Ingestion of drugs
  • Seizure disorder
  • Coronary artery bypass
  • Eisenmenger syndrome
  • Severe symptomatic valvular heart disease
  • Severe decompensated congestive heart disease
  • Uncontrolled ventricular and supraventricular tachycardia
  • Uncontrolled hypertension
  • Unstable angina
  • History of altitude illness
  • The rate of ascent
  • The ultimate altitude reached
  • Other medical conditions such as pulmonary hypertension, chronic obstructive pulmonary disease, restrictive lung disease, pulmonary fibrosis, and congenital heart disease
  • The degree of cold
  • The amount of physical exertion
  • Use of alcohol and sleeping pills

Symptoms of Altitude-Related Disorders

Some symptoms of low oxygen saturation levels include:

  • Shortness of breath
  • Cyanosis
  • Extreme fatigue and weakness
  • Mental confusion
  • Headaches

Symptoms of mild, short-term altitude sickness usually begin 12 to 24 hours after arriving at high altitude. They lessen in a day or two as your body adjusts. These symptoms include:

  • Dizziness.
  • Fatigue and loss of energy.
  • Shortness of breath.
  • Loss of appetite.
  • Sleep problems.

Symptoms of moderate altitude sickness are more intense and worsen instead of improving over time:

  • Worsening fatigue, weakness, and shortness of breath.
  • Coordination problems and difficulty walking.
  • Severe headache, nausea, and vomiting.
  • Chest tightness or congestion.
  • Difficulty doing regular activities, though you may still be able to walk independently.

Severe altitude sickness is an emergency. The symptoms are similar to moderate AMS but more severe and intense. If you start experiencing these symptoms, you must be taken to a lower altitude immediately for medical care:

  • Shortness of breath, even when resting.
  • Inability to walk.
  • Confusion.
  • Fluid buildup in the lungs or brain.

HAPE, when fluid builds up in the lungs, prevents oxygen from moving around your body. You need medical treatment for HAPE. Symptoms include:

  • Cyanosis, when your skin, nails or whites of your eyes start to turn blue.
  • Confusion and irrational behavior.
  • Shortness of breath even when resting.
  • Tightness in the chest.
  • Extreme fatigue and weakness.
  • Feeling like you’re suffocating at night.
  • Persistent cough, bringing up white, watery fluid.

HACE happens when the brain tissue starts to swell from the leaking fluid. You need medical treatment for HACE. Symptoms include:

  • Headache
  • Loss of coordination.
  • Weakness.
  • Disorientation, memory loss, hallucinations.
  • Psychotic behavior.
  • Coma.

Acute high altitude illness summary

Condition Symptoms and Signs Treatment Prophylaxis
Acute mountain sickness Headache, anorexia, nausea, vomiting, dizziness, fatigue, weakness, insomnia Descent, acetazolamide, dexamethasone, supplemental oxygen Slow ascent, acetazolamide, dexamethasone
High altitude pulmonary edema Dyspnea at rest, cough, decreased exercise performance, chest pain/tightness, low pulse oximetry, central cyanosis, tachypnea, tachycardia, rales, wheezing Descent, supplemental oxygen, nifedipine, phosphodiesterase-5 inhibitors, salmeterol, portable hyperbaric chambers Slow ascent, nifedipine, phosphodiesterase-5 inhibitors, salmeterol
High altitude cerebral edema Change in mental status or ataxia in a person with AMS or HAPE Descent, dexamethasone, acetazolamide, supplemental oxygen, portable hyperbaric chambers Slow ascent, dexamethasone, acetazolamide

Diagnosis of Altitude-Related Disorders

History and Physical

The hallmark of Acute Mountain Sickness is a headache, with other symptoms including nausea, vomiting, loss of appetite, fatigue/malaise (particularly at rest), sleep disturbance, and dizziness/lightheadedness.  Acute Mountain Sickness symptoms can begin after only a few hours and typically present the first day at a given altitude, resolving after one to three days, even without treatment, as the body adjusts physiologically (acclimates) to the lower oxygen levels.

The presence of facial or extremity edema can be present with or without Acute Mountain Sickness symptoms and is felt to be a marker for not yet being acclimated to the altitude. Rarely, retinal hemorrhages can occur and affect visual fields.

The onset of neurological findings such as a progressive decline in cognitive/mental function, the declining level of consciousness, impaired coordination, slurred speech, and/or lassitude signify the transition from AMS to HACE.  A typical evaluation consists of an abnormal neurological exam, with ataxia often being the earliest finding.
Early symptoms may be misinterpreted as exhaustion and it is important to exclude these, as well as other disorders such as dehydration, hypoglycemia, hypothermia, or hyponatremia which all may have signs and symptoms that overlap with that of HACE.  Though rarely available, laboratory testing may show an elevated white blood cell count in the setting of HACE, whereas any number of metabolic abnormalities may be present with the aforementioned others within the differential diagnosis.
Lumbar puncture may have an increased opening pressure with otherwise normal laboratory findings. CT may show cerebral edema, but MRI is a better study to evaluate for more subtle signs of edema and can remain abnormal for days up to weeks.  To date, there has been no direct correlation between the severity of edema with clinical outcome.

The Lake Louise Score for the diagnosis of acute mountain sickness.

Symptoms Severity Score
1. Headache None 0
Mild 1
Moderate 2
Severe/incapacitating 3
2. Gastrointestinal None 0
Poor appetite or nausea 1
Moderate nausea or vomiting 2
Severe nausea or vomiting/incapacitating 3
3. Fatigue/weakness None 0
Mild 1
Moderate 2
Severe/incapacitating 3
4. Dizziness/lightheaded None 0
Mild 1
Moderate 2
Severe/incapacitating 3
5. Difficulty sleeping None 0
Not as well as usual 1
Poor night’s sleep 2
Unable to sleep 3
A diagnosis of acute mountain sickness (AMS) requires (a) score > 3, (b) presence of headache and (c) recent ascent.
High-altitude cerebral edema With AMS Altered mental status or/and ataxia
Without AMS Altered mental status and ataxia
Acute mountain sickness (AMS)
-  In the setting of a recent gain in altitude, there is the presence of headache and at least one of the following:
-  Gastrointestinal (anorexia, nausea, or vomiting)
-  Fatigue or weakness
-  Dizziness or lightheadedness
-  Difficulty sleeping
High-altitude cerebral edema (HACE)
-  Can be considered “end-stage” or severe AMS. In the setting of a recent gain in altitude, there is either —
-  the presence of a change in mental status and/ or ataxia in a person with AMS
-  or the presence of both mental status changes and ataxia in a person without AMS.
High-altitude pulmonary edema (HAPE)
In the presence of a recent gain in altitude, the presence of the following:
At least two of the following symptoms —
-  Dyspnea at rest
-  Cough
-  Weakness or decreased exercise performance
-  Chest tightness or congestion
At least two of the following signs:
-  Crackles or wheezing in at least one lung field
-  Central cyanosis
-  Tachypnea
-  Tachycardia

Treatments of Altitude-Related Disorders

Non-pharmacological

  • Pure oxygen – Giving pure oxygen can help a person with severe breathing problems caused by altitude sickness. Physicians at mountain resorts commonly provide this treatment.
  • A Gamow bag – This portable plastic hyperbaric chamber can be inflated with a foot pump and is used when a rapid descent is not possible. It can reduce the effective altitude by up to 5,000 ft (1,500 m). It is usually used as an aid to evacuate people with severe symptoms, not to treat them at high altitudes.
  • Gradual Ascent – The recommended method for the prevention of high-altitude illness is to allow the body time to acclimatize via gradual ascent. The WMS recommends one day of travel for every 1,500 ft ascent above 10,000 ft above sea level and a day of rest every 3 to 4 days of travel.
  • Descent – Non-severe AMS: Descent is not necessary for non-severe AMS. It responds well to rest and/or pharmacological treatment. If symptoms resolve, ascent may resume. Severe AMS is AMS with incapacitating symptoms. The appropriate and definitive treatment for severe AMS is immediate descent to a lower altitude.
  • Supplemental Oxygen – If available, supplemental oxygen should be administered with oxygen saturation of above 90% as a goal for both severe AMS and HACE.  Supplemental oxygen should only be used in conjunction with evacuation or while waiting for it.
  • Portable Hyperbaric Chamber – These portable chambers are indicated for severe AMS and HACE when evacuation is delayed.  Symptoms will recur when the patient exits the chamber. However, it may temporarily improve symptoms long enough for patients to be able to assist with their evacuation.  The equipment and constant supervision make this a resource-intensive treatment, but it has the potential to save lives in remote areas where evacuation may be delayed.

Medication

Established drug treatments include acetazolamide, dexamethasone, and nifedipine. Acetazolamide is thought to be effective in treating AMS, creating an acidemia, increasing ventilation, and therefore, increasing the arterial oxygen content. Dexamethasone is effective at reducing edema and symptoms in HACE, just as it is in any other form of cerebral edema. Nifedipine is used in HAPE for its dilatory effect on the pulmonary vasculature; however, a recent study did not demonstrate any benefit over descent and supplemental oxygen in patients with HAPE. In addition to these established treatments, potential novel therapies have been suggested such as ibuprofen, nitrates, and intravenous (IV) iron supplementation.

Acetazolamide

Acetazolamide prevents AMS when taken before ascent; it can also help speed recovery if taken after symptoms have developed. The drug works by acidifying the blood and reducing the respiratory alkalosis associated with high elevations, thus increasing respiration and arterial oxygenation and speeding acclimatization. An effective dose that minimizes the common side effects of increased urination and paresthesias of the fingers and toes is 125 mg every 12 hours, beginning the day before ascent and continuing the first 2 days at elevation, or longer if ascent continues.

Acetazolamide (125mg PO every 12 hours)

  • Acetazolamide is the only medication proven to speed acclimatization. It induces metabolic acidosis by bicarbonate diuresis. This acidosis triggers compensatory hyperventilation helping acclimatization. There are two adverse effects of this medication worth considering.  First, acetazolamide increases urination frequency and therefore increases the risk of dehydration, which is a concern during high altitude travel.  Secondly, acetazolamide has a similar molecular structure to sulfa medications and should be used cautiously in patients with sulfa allergy.  Although the risk of cross-reactivity is low, travelers with sulfa allergies are recommended to undergo a trial of acetazolamide before travel.

Allergic reactions to acetazolamide are uncommon. As a nonantimicrobial sulfonamide, it does not cross-react with antimicrobial sulfonamides. However, it is best avoided by people with a history of anaphylaxis to any sulfa. People with a history of severe penicillin allergy have occasionally had allergic reactions to acetazolamide. The pediatric dose is 5 mg/kg/day in divided doses, up to 125 mg twice a day.

Dexamethasone

Dexamethasone (initial 8mg PO, IM, or IV followed in 6 hours by 4mg PO, IM, or IV every 6 hours). Dexamethasone is effective for preventing and treating AMS and HACE and prevents HAPE as well. Unlike acetazolamide, if the drug is discontinued at elevation before acclimatization, a mild rebound can occur. Acetazolamide is preferable to prevent AMS while ascending, with dexamethasone reserved as an adjunct treatment for the descent. The adult dose is 4 mg every 6 hours. An increasing trend is to use dexamethasone for “summit day” on high peaks such as Kilimanjaro and Aconcagua, in order to prevent abrupt altitude illness.

Dexamethasone (4mg PO, IM, or IV every 12 hours)

  • For those unable to take acetazolamide, dexamethasone may be used as a preventive agent.  It also may be considered for individuals involved in an unusually high-risk situation (i.e., search and rescue personnel airlifted to above 11,000 ft).  Dosages for dexamethasone is the same for PO, IM, and IV routes of administration.  If used for longer than ten days, it must be tapered slowly to prevent withdrawal symptoms.

Nifedipine

Nifedipine both prevents and ameliorates HAPE. For prevention, it is generally reserved for people who are particularly susceptible to the condition. The adult dose for prevention or treatment is 30 mg of extended-release every 12 hours or 20 mg every 8 hours.

Painkillers or Ibuprofen

Acetaminophens, such as Tylenol, can be taken for headaches. Ibuprofen, an anti-inflammatory medicine, can also help. Other than the tight-fit hypothesis previously discussed, other possible mechanisms causing high-altitude headaches include activation of the trigeminovascular system by vasodilatation or inflammatory mediators, or alteration in the blood–brain barrier by inflammatory mediators causing vasogenic edema. The central role of inflammation in these mechanisms has led to an interest in nonsteroidal anti-inflammatory medications such as ibuprofen.

Nitrates

Nitric oxide (NO) regulates physiological processes in the human body, including vasodilation, immune function, platelet aggregation, glucose homeostasis, muscle contraction, and mitochondrial function.

One possible means of eliciting this effect is via dietary nitrate supplementation (ie, nitrate-rich beetroot juice). Nitrate ingestion has been shown to increase plasma concentrations of NO metabolites (nitrate and nitrite), reduce steady-state oxygen consumption, improve arterial and tissue oxygenation, enhance exercise tolerance, and improve performance, during acute normobaric hypoxia. Interestingly, under hypoxic conditions, nitrate supplementation in the form of beetroot juice resulted in faster muscle recovery and restored maximal oxidative ATP resynthesis and exercise tolerance to normoxic values, when compared to placebo.,

Both sildenafil (a selective phosphodiesterase type 5 [PDE-5] inhibitor) and bosentan (a nonselective endothelin-receptor antagonist) have been mooted as potential treatments for AMS, due to their effect on prolonging the effect of NO. Sildenafil has been demonstrated to reduce PASP, increase oxygen delivery, and minimize the decrease in exercise capacity in both normobaric hypoxia and actual high altitude. By dilating the pulmonary vascular bed, these drugs reduce the degree of hypoxic pulmonary vasoconstriction and consequent pulmonary hypertension, and therefore, the risk of HAPE.

IV iron supplementation

In hypoxic conditions, oxygen-dependent hydroxylase enzymes are unable to degrade HIF, so iron supplementation encourages the breakdown of HIF, as in normoxic conditions. This is significant as HIF is believed to coordinate the cellular inflammatory response to hypoxia.

IV iron supplementation immediately prior to ascent to high altitude resulted in a significantly lower rise in AMS score from sea level to altitude, compared to IV saline. However, there was no significant difference in absolute AMS score at altitude between the two groups. Iron supplementation is an intriguing prospect in the prophylactic management of AMS; however, its feasibility on field expeditions is questionable. Oral iron supplemental is a possible alternative that needs more investigation.

Exercise and AMS

While the exact mechanism underlying high-altitude illness remains hotly debated, exercise has been suggested as an independent risk factor for the development of AMS. High-intensity intermittent exercise on a trekking expedition was associated with increased interstitial lung fluid at 4,090 m, suggesting that exercise increases the risk of HAPE. Corroborating this, AMS scores were significantly higher in trekkers with a higher rating of perceived exertion.

Conversely, several recent chamber studies have failed to demonstrate a statistical difference in the development of AMS between rest and exercise at simulated altitude. Of note, in comparing the change in interstitial fluid between exercise in hypoxia at 4,090 m and exercise in simulated hypoxia, there was no significant increase in simulated hypoxia, while there was an increase in actual hypoxia. This suggests that chamber studies are possibly underestimating the effect of exercise on the development of AMS and HAPE and that this may be due to the difference between the normobaric hypoxia experienced in chamber studies and hypobaric hypoxia experienced at altitude.

Nutrition

There are a few nutritional concerns for athletes at high altitudes. First, there is an association between chronic high altitude exposure and significant weight loss. This seems to be primarily due to loss of fat-free mass, which may have significant negative effects on physical performance. Factors possibly contributing to this weight loss are decreased physical activity, hypoxia, irregular sleep pattern, cold exposure, and nutritional imbalance related to protein metabolism.,

Inspiratory muscle training

In addition to IHE and IHT, there are other potential techniques such as dietary nitrate supplementation (which has been discussed earlier) and inspiratory muscle training (IMT), which may attenuate arterial oxygen desaturation and a reduction in exercise performance during high-altitude exposure.

Acute exposure to hypoxia and the associated reduction in the arterial partial pressure of oxygen increases minute ventilation, in an attempt to normalize arterial oxygen saturation. However, this hyperventilation increases the work of breathing and in turn the demand for respiratory blood flow, making the respiratory muscles more susceptible to fatigue.

Already abroad and need to see a doctor?

The following list of resources can help international travelers identify health care providers and facilities around the world. CDC does not endorse any particular provider or medical insurance company, and accreditation does not ensure a good outcome.

  • The nearest US embassy or consulate can help travelers locate medical services and notify friends, family, or employers of an emergency. They are available for emergencies 24 hours a day, 7 days a week, overseas.
  • The Department of State maintains a list of travel medical and evacuation insurance providers.
  • The International Society of Travel Medicine maintains a directory of health care professionals with expertise in travel medicine in more than 80 countries.
  • The International Association for Medical Assistance to Travelers maintains a network of physicians, hospitals, and clinics that have agreed to provide care to members.
  • Travel agencies, hotels, and credit card companies (especially those with special privileges) may also provide information.
  • A number of countries or national travel medicine societies have websites related to travel medicine that provide access to clinicians, including the following:
    • Australia: Travel Medicine Alliance
    • Canada: Health Canada (www.phac-aspc.gc.ca and https://travel.gc.ca)
    • China: International Travel Healthcare Association
    • Great Britain: National Travel Health Network and Centre and British Global and Travel Health Association
    • South Africa: South African Society of Travel Medicine

Tips for acclimatization

  • Be sure you are in good shape before you travel – If you have significant medical problems, check with your doctor before you go; even with his approval, be sure to go slowly and listen to your body for warning symptoms. Travel is usually safe for men with mild to moderate heart or lung disease and for most with well-controlled high blood pressure or diabetes, but high altitudes are very dangerous for people with sickle cell anemia.
  • Ascend gradually – You can fly to Denver or Mexico City in one hop, but if you’re going higher, a few days of acclimation are worth your time. Above 8,000 feet, don’t go up more than 1,000 feet a day.
  • Travel high, sleep low – For example, if you ski at 9,000 feet, you’ll do best if your lodge is 1,000–1,500 feet lower. If you’re hiking, ascend in stages, and sleep at altitudes below your daily peak.
  • Limit your exercise during your first days at altitude — and take it easy throughout your trip if you have medical problems or you feel sick in any way.
  • Drink plenty of fluids – Dehydration is sneaky at altitude because you will lose lots of water through your lungs even if you don’t perspire. Drink enough to keep your urine clear and copious. Avoid alcohol or minimize your consumption, particularly for the first 48 hours at altitude. Avoid sedatives.
  • Be alert for symptoms – You can manage mild mountain sickness yourself (see “Treatment” below), but you’ll need help for anything more serious. Don’t ignore symptoms; instead, return to a lower elevation and get help.
  • Ascend gradually, if possible. Avoid going directly from low elevation to more than 9,000 ft (2,750 m) sleeping elevation in 1 day. Once above 9,000 ft (2,750 m), move sleeping elevation no higher than 1,600 ft (500 m) per day, and plan an extra day for acclimatization every 3,300 ft (1,000 m).
  • Consider using acetazolamide to speed acclimatization if abrupt ascent is unavoidable.
  • Avoid alcohol for the first 48 hours; continue caffeine if a regular user.
  • Participate in only mild exercise for the first 48 hours.
  • Having a high-elevation exposure (greater than 9,000 ft [2,750 m]) for 2 nights or more, within 30 days before the trip, is useful, but closer to the trip departure is better.
  • Dress warmly.
  • Wear sunglasses.

These steps can help your body acclimate:

  • Walk-up – Start below 10,000 feet and walk to a high altitude instead of driving or flying. If you drive or fly to an elevation higher than 10,000 feet, stay at your first stop for at least 24 hours before going higher.
  • Go slow – Once above 10,000 feet, don’t increase your altitude more than 1,000 feet a day.
  • Rest – Build a rest day into your schedule for every 3,000 feet you climb.
  • “Climb high and sleep low” – If you climb more than 1,000 feet in a day, come down to sleep at a lower altitude.
  • Know your body – Recognize the signs and symptoms of altitude sickness. Move to a lower altitude (or avoid climbing higher) if you notice any symptoms.
  • Stay hydrated – Drink 3-4 quarts of water per day.
  • Avoid alcohol – Alcohol can dehydrate your body. It also has stronger effects at higher elevations, which can impair judgment.
  • Eat carbs – Eat a diet that’s more than 70% carbohydrates.
  • Know the “don’ts – Avoid tobacco and depressant drugs, such as sleeping pills and tranquilizers.

What should I ask my doctor?

If you’re planning to travel to high altitudes, ask your healthcare provider:

  • Should I take preventive medication to avoid altitude sickness?
  • Do I have any risk factors that would prevent me from visiting high elevations?
  • What other steps can I take to prevent altitude sickness?
  • What steps should I take if I start to feel symptoms during my climb?

References

Vulvovaginitis – Causes, Symptoms, Diagnosis, Treatment

Vulvovaginitis/Vulvitis is inflammation of the vulva, the external female mammalian genitalia that includes the labia majora, labia minora, clitoris, and introitus (the entrance to the vagina). It may co-occur as vulvovaginitis with vaginitis, inflammation of the vagina, and may have infectious or non-infectious causes. The warm and moist conditions of the vulva make it easily affected. Vulvitis is prone to occur in any female especially those who have certain sensitivities, infections, allergies, or diseases that make them likely to have vulvitis. Postmenopausal women and prepubescent girls are more prone to be affected by it, as compared to women in their menstruation period.[rx] It is so because they have low estrogen levels which makes their vulvar tissue-thin and dry. Women having diabetes are also prone to be affected by vulvitis due to the high sugar content in their cells, increasing their vulnerability. Vulvitis is not a disease, it is just an inflammation caused by an infection, allergy, or injury. Vulvitis may also be a symptom of any sexually transmitted disease or fungal infection.

Vulvitis, inflammation, and infection of the vulva—the external genitalia of the female. The external organs of the vulva include the labia majora and minora (folds of skin), the clitoris, and the vestibular glands. The basic symptoms of vulvitis are superficial red, swollen, and moisture-laden lesions on the skin of the vulva. Itching sensations are a particularly prominent and consistent symptom. The areas of affected vulvar skin may turn white, crack, or develop fluid-filled blisters that break open, ooze, and crust over.

Types of Vulvitis

  • Candida vulvitis – A yeast infection of female external genitals (the vulva).
  • Chronic vulvitis – A chronic inflammatory skin condition that affects external female genitals (the vulva).

Causes of Vulvovaginitis

Vulvitis may be caused by the following reasons:

  • Allergies or sensitivities towards certain products like – colored or perfumed toilet papers, vaginal sprays or douches, shampoos and hair conditioners, laundry detergents, creams or medications.
  • Reactions to – bubble baths or soaps used on genital area, spermicides, sanitary napkins.
  • Irritations caused by – a yeast infection, chlorinated water in swimming pools or hot tubs, synthetic underwear or nylon pantyhose without a breathable cotton crotch, wearing a wet bathing suit for a long time, bike or horseback riding, douching, poor personal hygiene, incontinence, by urine or stool if it remains in contact with the vulva (as may occur in women who have incontinence or are confined to bed), contact with urine and stool sometimes cause ongoing (chronic) vulvitis.
  • Infections such as – vaginitis, genital herpes, a viral and fungal infection.
  • Factors such as – Diabetes, scabies or pubic lice, eczema or dermatitis, oral sex.[rx]
  • The use of colored or perfumed toilet paper
  • An allergic reaction to bubble bath or soap used to clean the genital area
  • Use of vaginal sprays or douches
  • Irritation by a chlorinated swimming pool or hot tub water
  • Allergic reaction to spermicide
  • Allergic reaction to sanitary napkins
  • Wearing synthetic underwear or nylon pantyhose without a breathable cotton crotch
  • Wearing a wet bathing suit for extended periods of time
  • Bike or horseback riding
  • Fungal or bacterial infections including scabies or pubic lice
  • Herpes
  • Skin conditions such as eczema or dermatitis

Postmenopausal women can be particularly susceptible to vulvitis. As estrogen levels drop, the vulvar tissues become thinner, drier, and less elastic. This makes women more vulnerable to irritation and infection.

Symptoms of Vulvovaginitis

The symptoms of vulvitis are

  • Extreme and constant itching
  • A burning sensation in the vulvar area
  • Vaginal discharge
  • Small cracks on the skin of the vulva
  • Redness and swelling on the vulva and labia (lips of the vagina)
  • Clear fluid-filled Blisters on the vulva
  • Scaly, thick, whitish patches on the vulva
  • Bumps or warts
  • Soreness
  • Pain with sex
  • Increased sensitivity when wiping with toilet paper
  • Blisters on the vulva
  • Scaly, thick, whitish patches on the vulva

Symptoms of vulvitis may also be an indication of other diseases or disorders. A doctor should be consulted when one is having any of these symptoms. The symptoms may vary on depending on the cause and the time period it has been infected for.

Diagnosis of Vulvovaginitis


Adolescents with signs and symptoms of vulvitis should be referred to clinicians who have expertise in adolescent medicine. At Children’s Hospital of Philadelphia (CHOP), these young people are evaluated by our Adolescent Medicine specialists.

At CHOP, diagnosis of vulvitis begins with a series of questions about the patient’s overall medical history, menstrual cycle, symptoms, and any sexual activity. A physical examination follows, which may include a pelvic examination. We welcome parents and caregivers as key partners in supporting a young person’s care during and after treatment of vulvitis.

To help young people develop the skills needed to be responsible for their own health, clinicians typically ask to spend time alone with patients during each visit. This helps young people become comfortable talking with their healthcare providers about their concerns and allows patients to ask questions that may be more difficult to say in front of their parents and caregivers. We respect the privacy and confidentiality of our young patients while ensuring that young people are safe and connected to the appropriate services and resources they need.

To confirm a diagnosis of vulvitis — as well as rule out more serious causes for the vaginal irritation — clinicians may perform the following tests:

  • Urinalysis
  • Tests for sexually transmitted diseases (STDs)
  • Bloodwork or other tests to evaluate for other medical causes of symptoms
  • Urine tests
  • Tests for sexually transmitted infections (STIs)
  • Pap test. This test involves a microscopic exam of cells collected from the cervix. It’s used to find changes that may be cancer or may lead to cancer. It also shows other conditions, such as infection or inflammation.

Treatment of Vulvovaginitis

The treatment of inflammation of the vulva (vulvitis) depends on the underlying cause.

Prescribed medications

After your doctor identifies the type of organism causing your vulvovaginitis, they’ll likely prescribe medication.

Medications for this condition may include:

  • oral antibiotics
  • antibiotic creams (applied directly to the skin)
  • antibacterial creams (applied directly to the skin)
  • antifungal creams (applied directly to the skin)
  • oral antifungal pills
  • oral antihistamines, if an allergic reaction is a possible cause
  • estrogen creams

It is usually recommended that you do not use soap or fragranced products to wash your vulva. In addition, you should avoid contact with your vulval skin with bubble bath, shampoo, personal deodorants, wet wipes, detergents, textile dyes, fabric conditioners, and sanitary wear. These can all worsen the irritation.

You should just wash this area once a day with warm water. Ideally, you should wear cotton underwear that is not too tight. Your doctor may recommend an emollient which you can use instead of soap and this can be very soothing for many women. Emollient creams can also be very soothing to use, as they work to moisturize the skin. If the itching is particularly bad then your doctor may recommend you take an antihistamine tablet which will reduce the itching.

If you are using contraception, it is recommended that your partner should avoid using spermicidal lubricated condoms. Some women find using lubricating gels useful during sex (intercourse).

Steroid creams are often given which are usually used for short periods. These work by reducing the inflammation and also the irritation and can be really effective for many women. There are different strengths of steroid cream and usually, the weakest strength is given first. However, if this does not work effectively then stronger steroid creams are usually given. If the underlying cause is an infection then an antifungal or antibiotic treatment may be given.

Sometimes a cream, pessary, or vaginal tablet containing the female chemical (hormone) called estrogen is prescribed if the cause of your vulvitis is low estrogen levels.

Local anesthetic creams, some medications, and surgery are some treatments for women with burning, stinging but often unexplained pain affecting the skin around the vagina or vulva (vulvodynia).

Diffuse vestibulitis should be managed as outlined earlier for vulvitis, with oral antifungal treatment for candidiasis if the yeast cultures are positive, avoidance of possible allergens, and use of topical corticosteroids. Vulvar vestibulitis is initially treated symptomatically. Vulvar burning may respond to tap water or baking soda douches to temporarily reduce vaginal acidity. Tepid baths may also provide transient relief. The dyspareunia usually improves with the use of precoital lubricants and topical anesthetic agents. Many patients obtain satisfactory relief of symptoms with these nonoperative measures. For women who do not achieve a satisfactory response, surgical treatment (vestibulopathy) provides good results in about 75% of patients.


Prevention of vulvitis

Your treatment and prevention options depend on your age, health history, lifestyle, and personal risk factors. Your doctor may discuss prevention strategies with you such as:

  • Wearing underwear with a breathable cotton lining
  • Avoiding soaps and hygiene products with added colors or fragrances
  • Changing out of wet clothing as soon as possible
  • Washing the genital area with mild soap and water daily, and drying thoroughly before dressing
  • Avoiding tight-fitting pants and underwear

Treatment will depend on the severity of the irritation. Mild cases of vulvitis, or vulvar itching, may not require medical attention. Simple self-care measures, including avoidance of irritating substances and sitting in warm water, can ease discomfort and promote healing.

If your vulvitis symptoms do not improve within a day or so, visit your doctor at Dignity Health. Your doctor can help determine the cause of vulvar irritation and prescribe appropriate treatment. Medical treatment of vulvitis may include:

  • Topical creams or ointments, including hydrocortisone ointment or estrogen cream
  • Medication to treat infection, if an infection is causing your symptoms, to eliminate the underlying condition

With appropriate diagnosis and treatment, most cases of vulvitis go away within a few weeks, and possibly sooner.

Tips for vulvar hygiene

  • Removal of irritants – no baby wipes; nylons or tight-fitting clothing; wet bathing suits; bathtubs filled with soap, shampoo, or bubble bath; scented detergents; washing powders; or dryer sheets. Sleep in nightgowns or long T-shirts without underwear. All underwear should be dye-free.
  • Cleaning –  Soak the area in warm water for 10–15 minutes per day. Never scrub. Gently wipe front to back with hypoallergenic soap. Stand up in the bath to be soaped, shampooed, and rinsed. Let air-dry or gently pat dry.
  • Toileting – Lean forward when voiding to prevent pooling of urine in the lower vagina and always wipe front to back.
  • Know the reason for your visit and what you want to happen.
  • Before your visit, write down the questions you want to be answered.
  • Bring someone with you to help you ask questions and remember what your provider tells you.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also, write down any new instructions your provider gives you.
  • Know why a new medicine or treatment is prescribed, and how it will help you. Also, know what the side effects are.
  • Ask if your condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if you do not take the medicine or have the test or procedure.
  • If you have a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your provider if you have questions.

Home remedies

If you’ve had a yeast infection in the past, you may be able to treat vulvovaginitis using over-the-counter products available at any pharmacy, including:

  • vaginal creams
  • suppositories
  • topical ointments
  • oral pills


A pharmacist can likely advise you on the best product for your symptoms and how to apply the product.

Crushed garlic and coconut oil, both known for their antibacterial properties, may also help treat the condition.

You may be able to relieve some of the symptoms of your vulvovaginitis by sitting in a sitz bath — a warm, shallow bath that only covers your hip area. Adding tea tree oil or a trace amount of vinegar or sea salt to the bath may help kill some bacteria if that’s the cause of your symptoms.

Be careful not to sit in the bath for too long. Use a towel to dry the affected area completely after your bath.

Consult your doctor if the inflammation or discharge doesn’t improve after a week of home treatment.

Shop for vulvovaginitis home remedies online:

  • vaginal creams
  • suppositories
  • topical ointments
  • coconut oil
  • sitz baths
  • tea tree oil

References

What does pantothenic acid do for your body?

What does pantothenic acid do for your body?/Pantothenic Acid is a water-soluble vitamin ubiquitously found in plants and animal tissues with antioxidant properties. Vitamin B5 is a component of coenzyme A (CoA) and a part of the vitamin B2 complex. Vitamin B5 is a growth factor and is essential for various metabolic functions, including the metabolism of carbohydrates, proteins, and fatty acids. This vitamin is also involved in the synthesis of cholesterol, lipids, neurotransmitters, steroid hormones, and hemoglobin.

Vitamin B or Pantothenic acid is a water-soluble vitamin. Pantothenic acid is an essential nutrient. Animals require pantothenic acid in order to synthesize coenzyme-A (CoA), as well as to synthesize and metabolize proteins, carbohydrates, and fats. The anion is called pantothenate. Pantothenic acid is the amide between pantoic acid and β-alanine. Its name derives from the Greek pantothen, meaning from everywhere, and small quantities of pantothenic acid are found in nearly every food, with high amounts in fortified whole-grain cereals, egg yolks, liver, and dried mushrooms. It is commonly found as its alcohol analog, the provitamin panthenol (pantothenol), and calcium pantothenate.

Deficiency Symptoms of Pantothenic Acid / Vitamin B5

Pantothenic acid deficiency has only been observed in individuals who were fed diets virtually devoid of pantothenic acid or who were given a pantothenic acid metabolic antagonist, omega-methyl pantothenic acid. The subjects exhibited various degrees of signs and symptoms, including irritability and restlessness; fatigue; apathy; malaise; sleep disturbances; gastrointestinal complaints such as nausea, vomiting, and abdominal cramps; neurobiological symptoms such as numbness, paresthesias, muscle cramps, and staggering gait; and hypoglycemia and increased sensitivity to insulin.

Symptoms of a vitamin B5 deficiency may include

Recommended Intakes of Vitamin B5

Intake recommendations for pantothenic acid and other nutrients are provided in the Dietary Reference Intakes (DRIs) developed by the Food and Nutrition Board (FNB) at the National Academies of Sciences, Engineering, and Medicine. DRI is the general term for a set of reference values used for planning and assessing nutrient intakes of healthy people. These values, which vary by age and sex, include:

  • Recommended Dietary Allowance (RDA) – Average daily level of intake sufficient to meet the nutrient requirements of nearly all (97%–98%) healthy individuals; often used to plan nutritionally adequate diets for individuals.
  • Adequate Intake (AI) – Intake at this level is assumed to ensure nutritional adequacy; established when evidence is insufficient to develop an RDA.
  • Estimated Average Requirement (EAR) – Average daily level of intake estimated to meet the requirements of 50% of healthy individuals; usually used to assess the nutrient intakes of groups of people and to plan nutritionally adequate diets for them; can also be used to assess the nutrient intakes of individuals.
  • Tolerable Upper Intake Level (UL) – Maximum daily intake unlikely to cause adverse health effects.

When the FNB evaluated the available data, it found the data insufficient to derive an EAR for pantothenic acid. Consequently, the FNB established AIs for all ages based on usual pantothenic acid intakes in healthy populations.

[stextbox id=’custom’]

Adequate Intakes (AIs) for Pantothenic Acid 
Age Male Female Pregnancy Lactation
Birth to 6 months 1.7 mg 1.7 mg
7–12 months 1.8 mg 1.8 mg
1–3 years 2 mg 2 mg
4–8 years 3 mg 3 mg
9–13 years 4 mg 4 mg
14–18 years 5 mg 5 mg 6 mg 7 mg
19+ years 5 mg 5 mg 6 mg 7 mg

Dietary Reference Intakes for Pantothenic Acid by Life Stage Group (mg/day)

Adequate Intake
Life Stage Group
0-6 mo 1.7
7-12 mo 1.8
1-3 yr 2
4-8 yr 3
9-13 yr 4
14-18 yr 5
19-30 yr 5
31-50 yr 5
51-70 yr 5
> 70 yr 5
Pregnancy
< or = 18 yr 6
19-50 yr 6
Lactation
< or = 18 yr 7
19-50 yr 7

 

[/stextbox]

Food Source of Pantothenic Acid / Vitamin B5

Several food sources of pantothenic acid are listed bellow

 Selected Food Sources of Pantothenic Acid 
Food Milligrams
(mg) per
serving
Percent
DV*
Breakfast cereals, fortified with 100% of the DV 10 100
Beef liver, boiled, 3 ounces 8.3 83
Shitake mushrooms, cooked, ½ cup pieces 2.6 26
Sunflower seeds, ¼ cup 2.4 24
Chicken, breast meat, skinless, roasted, 3 ounces 1.3 13
Tuna, fresh, bluefin, cooked, 3 ounces 1.2 12
Avocados, raw, ½ avocado 1.0 10
Milk, 2% milkfat, 1 cup 0.9 9
Mushrooms, white, stir-fried, ½ cup sliced 0.8 8
Potatoes, russet, flesh, and skin, baked, 1 medium 0.7 7
Egg, hard-boiled, 1 large 0.7 7
Greek yogurt, vanilla, nonfat, 5.3-ounce container 0.6 6
Ground beef, 85% lean meat, broiled, 3 ounces 0.6 6
Peanuts, roasted in oil, ¼ cup 0.5 5
Broccoli, boiled, ½ cup 0.5 5
Whole-wheat pita, 1 large 0.5 5
Chickpeas, canned, ½ cup 0.4 4
Rice, brown, medium-grain, cooked, ½ cup 0.4 4
Oats, regular and quick, cooked with water, ½ cup 0.4 4
Cheese, cheddar, 1.5 ounces 0.2 2
Carrots, chopped, raw, ½ cup 0.2 2
Cabbage, boiled, ½ cup 0.1 1
Clementine, raw, 1 clementine 0.1 1
Tomatoes, raw, chopped or sliced, ½ cup 0.1 1
Cherry tomatoes, raw, ½ cup 0 0
Apple, raw, slices, ½ cup 0 0

*DV = Daily Value. DVs were developed by the U.S. Food and Drug Administration (FDA) to help consumers compare the nutrient contents of products within the context of a total diet. The DV for the values in Table 2 is 10 mg for adults and children age 4 years and older. This value, however, decreases to 5 mg when the updated Nutrition and Supplement Facts labels are implemented. The updated labels must appear on food products and dietary supplements beginning in January 2020, but they can be used now. The FDA does not require food labels to list pantothenic acid content unless a food has been fortified with this nutrient. Foods providing 20% or more of the DV are considered to be high sources of a nutrient.

The U.S. Department of Agriculture’s (USDA’s) National Nutrient Database for Standard Reference website lists the nutrient content of many foods and provides a comprehensive list of foods containing pantothenic acid arranged by pantothenic acid content and by food name.

Health Benefit of Pantothenic Acid / Vitamin B5

  • Pantothenic acid – has been used for a wide range of disorders such as acne, alopecia, allergies, burning feet, asthma, grey hair, dandruff, cholesterol-lowering, improving exercise performance, depression, osteoarthritis, rheumatoid arthritis, multiple sclerosis, stress, shingles, aging and Parkinson’s disease. It has been investigated in clinical trials for arthritis, cholesterol-lowering and exercise performance.[Mason P; Dietary Supplements,
  • The topical application of pantothenate –  is widely used in clinical practice for wound healing.
  • Pantothenic acid deficiency –  Taking pantothenic acid by mouth prevents and treats pantothenic acid deficiency.
  • Skin reactions from radiation therapy – Applying dexpanthenol, a chemical similar to pantothenic acid, to areas of irritated skin does not seem to help treat skin reactions from radiation therapy.
  • Some research suggests that taking pantothenic acid in combination with pantethine and thiamine does not improve muscular strength or endurance in well-trained athletes.
  • There is conflicting evidence regarding the usefulness of pantothenic acid in combination with large doses of other vitamins for the treatment of ADHD.
  • Early research suggests that taking dexpanthenol, a chemical similar to pantothenic acid, by mouth daily or receiving dexpanthenol shots can help treat constipation.
  • Early research suggests that using specific eye drops (Siccaprotect) containing dexpanthenol, a chemical similar to pantothenic acid, does not improve most symptoms of dry eyes.
  • Some evidence suggests that applying gel or drops containing dexpanthenol, a chemical similar to pantothenic acid, reduces some symptoms of eye trauma. However, not all research is consistent.
  • Early research suggests that pantothenic acid (given as calcium pantothenate) does not reduce symptoms of osteoarthritis.
  • There is inconsistent evidence on the potential benefits of taking pantothenic acid after surgery. Taking pantothenic acid or dexpanthenol, a chemical similar to pantothenic acid, does not seem to improve bowel function after stomach surgery. However, taking dexpanthenol by mouth might reduce other symptoms after surgery, such as sore throat.
  • Developing research suggests that pantothenic acid (given as calcium pantothenate) does not reduce the symptoms of arthritis in people with rheumatoid arthritis.
  • Early research suggests that using a specific spray (Nasicur) that contains dexpanthenol, a chemical similar to pantothenic acid, helps relieve nasal dryness.
  • Early research suggests that using a nasal spray containing dexpanthenol, a chemical similar to pantothenic acid, after sinus surgery reduces discharge from the nose, but not other symptoms.
  • Research on the effects of pantothenic acid for preventing skin irritations is not consistent. Some early research suggests that a specific product (Bepanthol Handbalsam) containing dexpanthenol, a chemical similar to pantothenic acid, does not prevent skin irritation when applied to the skin. However, other research suggests that dexpanthenol ointment can prevent skin irritation.
  • Early research suggests that using a specific ointment (Hepathrombin-50,000-Salbe Adenylchemie) containing dexpanthenol, a chemical similar to pantothenic acid, as well as heparin and allantoin reduces swelling related to ankle sprains.
  • Alcoholism.
  • Allergies.
  • Hair loss.
  • Asthma.
  • Heart problems.
  • Carpal tunnel syndrome.
  • Lung disorders.
  • Colitis.
  • Eye infections (conjunctivitis).
  • Convulsions.
  • Kidney disorders.
  • Dandruff.
  • Depression.
  • Diabetic problems.
  • Enhancing immune function.
  • Headache.
  • Hyperactivity.
  • Low blood pressure.
  • Inability to sleep (insomnia).
  • Irritability.
  • Multiple sclerosis.
  • Muscular dystrophy.
  • Muscle cramps.

References

What causes swimmer’s shoulder?

What causes swimmer’s shoulder?/Swimmer’s shoulder is an umbrella term covering a range of painful shoulder overuse injuries that occur in swimmers. Because there are various parts of your shoulder that can be injured from your swimming stroke, your pain can be anything from a local pain near the shoulder joint, to a spreading pain that travels up your shoulder and neck or down into your arm. Being an overuse injury, it is caused by repeated trauma rather than a specific incident.

Swimmers have a significant potential for shoulder injuries due to the unique nature of the different strokes involved in swimming as well as the high volume of repetitions needed during training. Swimmer’s shoulder is a term that can represent numerous shoulder pathologies. These include impingement syndrome, rotator cuff tendinitis, labral injuries, instability secondary to ligamentous laxity or muscle imbalance/dysfunction, neuropathy from nerve entrapment, and anatomic variants. In order for the athlete to return to the sport in an appropriate and timely manner, the clinician must be able to differentiate between these different etiologies. 

Swimmer’s shoulder is the term used to describe the problem of shoulder pain in the competitive swimmer. Swimming is an unusual sport in that the shoulders and upper extremities are used for locomotion, while at the same time requiring above average shoulder flexibility and range of motion (ROM) for maximal efficiency. This is often associated with an undesirable increase in joint laxity. Furthermore, it is performed in a fluid medium, which offers more resistance to movement than air. This combination of unnatural demands can lead to a spectrum of overuse injuries seen in the swimmer’s shoulder, the most common of which is rotator cuff tendinitis.

Pathophysiology

Swimming strokes can be broken down into pull-through and recovery phases. The latissimus dorsi and the pectoralis major are the primary contributors to the propulsive forces of the swim stroke by adduction and internal rotation. The subscapularis and serratus anterior muscles also play an integral role in the freestyle stroke.  The freestyle stroke can be divided into six distinct parts/phases:

  • Hand entry
  • Forward reach
  • Pull through
  • Middle pull through
  • Hand exit
  • Middle recovery

It is important for the athlete to have a properly balanced shoulder regarding muscle strength. Improper muscle balancing can cause the onset of shoulder pain.  An absolute or sudden increase in training yardage and poor technique can also be associated with the onset of pain. The coaching staff can observe a dropped elbow during the recovery phase of the freestyle stroke as one of the early signs of possible injury.

Causes of Swimmer’s Shoulder

  • Overtraining
  • Not enough rest periods
  • Poor stroke mechanics
  • Poor breathing technique
  • Poor flexibility or range of motion
  • Decreased rotator cuff or shoulder blade (scapular muscle) strength
  • Poor core strength or stability
  • Decreased hip muscle strength
  • Shoulder pain in swimmers includes subacromial impingement,
  • Rotator cuff tendinopathy, labral pathology,
  • Suprascapular nerve entrapment, or any combination thereof.
  • Additionally, an injury may be accompanied or exacerbated by structural abnormalities including scapular dyskinesis, glenohumeral hyperlaxity, excess subluxation, glenohumeral internal rotation deficit (GIRD), or muscle strength imbalances.

Symptoms of Swimmer’s Shoulder

The most common swimmer’s shoulder symptoms include:

  • Reduced range of motion in your shoulder compared to your other shoulder.
  • Increased joint laxity than with the other shoulder.
  • Reduced strength when compared with your other shoulder.
  • If you’re experiencing shoulder pain while swimming, you should consult with a shoulder expert as soon as possible to avoid the problem from becoming worse stop swimming and rest your shoulder to help reduce the inflammation
  • Localized pain is felt in the affected part of the shoulder
  • Pain may extend to the neck and/or down the arm in some cases
  • Pain worsens while resting on the affected shoulder
  • Tenderness of the affected area
  • Decreased range of movement
  • The decrease in shoulder strength
  • Increased joint laxity

Technically speaking, some common characteristics of swimmer’s shoulder are

  • Bicep and supraspinatus tendon inflammation within the subacromial muscles in the back leading to shoulder impingement syndrome.
  • Training errors, like overloading, overtraining and particularly poor stroke technique.
  • The onset of symptoms tends to be linked with altered shoulder joint mobility, posture, muscle performance or neuromuscular control.
  • Many swimmers have innate ligamentous laxity while also having multidirectional shoulder instability, or basically, more joint movement.
  • However, due to the nature of swimming, all swimmers can develop imbalances in the muscle where the internal rotators and adductors of the arm over-develop. This can leave a weakness of the scapular stabilizers and external rotators, unfortunately, because they’re not being used as much.

Diagnosis of Swimmer’s Shoulder

  • Physical examination is the first step in the process of diagnosis
  • X-rays and other imagining techniques may be needed to assess the changes in the structure of the joint
  • The doctor may check for any change in the pattern of swimming stroke
  • The presence of ‘lazy elbow’ where the elbow on the affected side cannot be lifted to the normal height out of the water.

Shoulder Apprehension Test

  • Place the shoulder in maximum abduction and external rotation (90-90 position) and apply an anteriorly-directed force to the shoulder from behind. To be positive, it must elicit a feeling of apprehension or instability. Generally, the only discomfort is seen in swimmers rather apprehension or sense of instability.

Load and Shift Test

  • With the patient seated, stabilize the scapula to the thorax with one hand, while the other hand is placed across the posterior glenohumeral joint line and humeral head, and the webspace across the patient’s acromion. The index finger should the over the anterior GH joint line.
  • Load and shift of the humeral head across the stabilized scapula in an anteromedial direction to assess anterior stability, and in a posterolateral direction to assess posterior instability is done. Normal motion anteriorly is half of the distance of the humeral head, more movement is considered to be a sign of glenohumeral joint laxity.

Strength Assessment of Rotator Cuff

Subscapularis

  • Internal rotation movement against resistance
  • Lift-off test
    • Shoulder in internal rotation with the back of the patient’s hand against the small of the back.
    • The patient attempts to lift the hand away from the back against the examiner’s resistance.

Infraspinatus, teres minor

  • External Rotation against resistance [shoulder in the neutral position at the side and the elbow flexed to 90°.

Supraspinatus

  • Resisted shoulder elevation with the arms extended, internally rotated, and positioned in the scapular plane (approximately 30-45° anterior to the coronal plane).
  • If weak, retest the supraspinatus in the same arm position except with the arms externally rotated (ie, thumbs pointing upwards).

Sulcus Sign [Assessment of Joint Laxity]

The arm is pulled inferiorly and gap or sulcus is looked between the humeral head and lateral edge of the acromion. This indicates the inferior subluxation of the humeral head.

  • Grade 1 – Less than 1 fingerbreadth (< 1 cm)
  • Grade 2 – One fingerbreadth (1-2 cm)
  • Grade 3 – Greater than 1 fingerbreadth (> 2 cm)

Compare to the opposite shoulder (should be similar). If not then consider unilateral traumatic injury. In addition, check for generalized ligamentous laxity.

Tests for Labral Tear

  • A labral tear is suggested by a painful click that is noted during the recovery phase of an overhand stroke. Often, this click can be reproduced during the exam.

O’Brien test for Labral Tear [SLAP Lesion]

  • A downward force is applied with the extent in the forward flexed position, adducted 15° toward the midline, with the shoulder in the maximal internal rotation (thumb pointing down). A pain that occurs in this position and relieved when external rotation is done suggests a SLAP lesion.

Positive Neer impingement test

If a subacromial injection relieves pain associated with passive forward flexion >90°

  • Positive Hawkins test – positive if internal rotation and passive forward flexion to 90° causes pain
  • Jobe test – pain with resisted pronation and forward flexion to 90° indicates supraspinatus pathology
  • Painful Arc Test – pain with the arm abducted in the scapular plane from 60° to 120°  
  • Yocum Test – positive if pain reproduced with elbow elevation while ipsilateral hand placed on the contralateral shoulder. sensitive but nonspecific
  • Internal Impingement test – positive if the pain is elicited with the abduction and external rotation of the shoulder
  • Take medical history, with emphasis on pain, sleep disturbance, loss of function and treatments.
  • Perform a physical examination, documenting the range of motion, strength and shoulder impingement signs.

X-rays

  • X-rays are performed to rule out bony causes of shoulder pain like a stress fracture or lesion or loose body. X-rays should be obtained if the pain persists after 6 weeks of rest and rehabilitation.
  • The desirable x-rays are anteroposterior (AP) y-scapular or outlet view and axillary view of the shoulder.

Magnetic Resonance Imaging

  • MRI is able to tell about rotator cuff pathology and status of the bones, ligaments, and other tendons in the shoulder.
  • But in most of cases, the MRI is normal or in some cases may demonstrate some increased signal in the substance of the supraspinatus tendon. Fluid in the subacromial bursa may signify bursitis. MRI arthrogram with intraarticular gadolinium is able to depict labral tear.

Diagnostic Subacromial Injection

  • Subacromial injection of an anesthetics agent like lignocaine can be used for diagnosis. Immediate relief of pain following the injection suggests an injury of the rotator cuff and/or the overlying bursa. Intra-articular injection providing relief, on the other hand, suggests intraarticular pathology.


Treatment of Swimmer’s Shoulder

Swimmers Shoulder Treatment Options

  • Rest is important to allow the joint to heal
  • Application of ice packs may be recommended to reduce pain and inflammation
  • Sling or shoulder tape may give adequate support
  • Physiotherapy sessions may be recommended to improve strength and flexibility
  • Early Injury Treatment
  • Avoid the HARM Factors
  • Acupuncture and Dry Needling
  • Sub-Acute Soft Tissue Injury Treatment
  • Core Exercises
  • Scapular Stabilisation Exercises
  • Shoulder Exercises
  • Biomechanical Analysis
  • Proprioception & Balance Exercises
  • Medications
  • Posture Correction
  • Soft Tissue Massage
  • Brace or Support
  • Dry Needling
  • Electrotherapy & Local Modalities
  • Heat Packs
  • Joint Mobilisation Techniques
  • Kinesiology Tape
  • Neurodynamics
  • Prehabilitation
  • Scapulohumeral Rhythm Exercises
  • Strength Exercises
  • Stretching Exercises
  • Supportive Taping & Strapping
  • TENS Machine
  • Video Analysis
  • Yoga

Immediate Treatment

Identifying the involved tendons or muscles, the severity or stage of the problem and receiving proper diagnosis will result in the most efficient treatment. A proper plan of treatment includes:

  • Applying ice – Apply directly to the shoulder after training for around 20 minutes.
  • Use anti-inflammatory medications – Use for a few days after the injury to reduce inflammation.
  • Rest – This might mean total rest with no swimming for about 24 to 48 hours depending on the severity. Or, you may be able to swim, but decrease yardage while you avoid butterfly, backstroke or kicking only with your arms at your side. Wait a few days until you’re free of pain before you resume swim training.

Medication  

Your doctor may prescribe anti-inflammation pills. These could form a very important part of the treatment.

  • Analgesics – Prescription-strength drugs that relieve pain but not inflammation.
  • Antidepressants  – A Drug that blocks pain messages from your brain and boosts the effects of exorphins (your body’s natural painkillers).
  • Medication – Common pain remedies such as aspirinacetaminophen, ibuprofen, and naproxen can offer short-term relief. All are available in low doses without a prescription. Other medications, including muscle relaxants and anti-seizure medications, treat aspects of spinal stenoses, such as muscle spasms and damaged nerves.
  • Corticosteroid injections – Your doctor will inject a steroid such as prednisone into your back or neck. Steroids make the inflammation go down. However, because of side effects, they are used sparingly.
  • Manual Therapy – Your physical therapist may use manual techniques, such as gentle joint movements, soft-tissue massage, and shoulder stretches to get your shoulder moving properly so that the tendons and bursa avoid impingement.
  • Range-of-Motion Exercises –  You will learn exercises and stretches to help your shoulder and shoulder blade move properly, so you can return to reaching and lifting without pain.
  • Mobility and stretching – Stretches can be done on a daily basis assuming there is no pain. Stretching must be done regularly and continued throughout the rehabilitation process. Below are some important stretches would be performed in order to encourage a full range of motion and improved posture.

 Get professional passive care. This might include things like

  • Interferential current
  • Ultrasound
  • Trigger point work
  • Cross friction tendon massage
  • Adjustments to your neck or shoulder
  • Post-isometric relaxation of the muscles involved

Do rehabilitation exercises

  • These will strengthen your weak muscles (particularly your external shoulder rotators). You perform these exercises with minimal weights (three to five pounds) and perform them in an extremely controlled, specific manner. Perform these exercises a few times a week if you’ve ever had problems with your shoulders. Typically, it’s said that for each day you swim on a bad shoulder, you’ll require one day of rehab.

Modify your swimming training

  • It’s often said hand paddles aren’t good for your shoulders. But, Zoomers and other types of fins keep your body elevated in the water; therefore, they might help if you begin experiencing small problems with your shoulders during your workout. Ask the coach or a friend to check your body and arm position. They may even want to videotape you so you can take a look and the coach can discuss the stroke mechanics with you.

Shoulder Stretching and Dynamic Warm-Up

  • The shoulder is the most frequently stretched joint among swimmers. To date, swimmers have adopted stretches that target the static stabilizers of the GHJ. In the past, static stretches and pre-race rituals (i.e., ballistic windmill motion) were utilized in hopes of preventing injury [].
  • Recent research on swimmer’s flexibility suggests there is no indication that extraordinary shoulder joint motion or flexibility is necessary to achieve a fast, efficient stroke [, ]. In addition, there is no evidence that static stretching immediately before activity will reduce muscle injury rates [, ].
  • Many swimmers are generally flexible and possess loose connective tissue (general joint laxity) []. Because of their inherent laxity, swimmers should emphasize preserving the overall stability of the shoulder and less time on general static stretches [].
  • Instead, a dynamic warm-up has been found to produce short-term and long-term performance enhancements in power, agility, strength, muscle endurance, and anaerobic capacity [, , , ].
  • A dynamic warm-up tends to include some form of dynamic stretching, agility, and plyometric activities and specific motor pattern movements [].  Demonstrates dynamic warm-up which is specifically designed for the swimming population. Swimmers should employ a steady pace with each activity and avoid ballistic type motions.

Focused Strengthening Exercises

  • Dryland training has been an important part of strengthening programs among swimmers at different ages and skill levels. The main focus of these training programs seems to be the spine and core strengthening []. Strength deficits can play an important role in fatigue development.
  • The majority of swimmers demonstrated signs of scapular dyskinesis in the course of a 100-min swimming session []. Through EMG analysis of the painful shoulder, discovered muscle activity of the serratus anterior is significantly depressed through the important pull-through phase in swimmers with shoulder pain [].
  • Revealed in the course of a swim season the internal rotators of competitive swimmers become proportionally stronger when compared to their antagonists, increasing muscle imbalance, and the risk of an injury process [, ].
  • Fatigue may lead to a short-term decrease in acromiohumeral distance []. As a result, a dryland program focusing on the shoulder external rotators and the scapular stabilizers would be of benefit and should be recommended as routine prophylactic measures []. Strengthening internal rotators should be de-emphasized.

Rehabilitations of Swimmer’s Shoulder

Phase 1- Pain Relief and Anti-inflammatory Tip

As with most soft tissue injuries the initial treatment is RICE – Rest, Ice, Compression and Elevation.

  • In the early phase, you’ll most likely be unable to fully lift your arm or sleep comfortably. You should stop doing the movement or activity that provoked the shoulder pain in the first place and avoid doing anything that causes pain in your shoulder.
  • You may need to wear a sling or have your shoulder taped to provide pain relief. In some cases, it may mean that you need to sleep relatively upright or with pillow support.
  • Ice is a simple and effective modality to reduce your pain and swelling. Apply for 20-30 minutes every 2 to 4 hours during the initial phase, or when you notice that your injury is warm or hot.
  • Anti-inflammatory medication (if tolerated) and natural substances (eg arnica) may help reduce your pain and swelling. However, it is best to avoid anti-inflammatory drugs during the initial 48 to 72 hours when they may encourage additional bleeding. Most people can tolerate paracetamol as pain-reducing medication. As you improve, supportive taping will help to both support the injured soft tissue and reduce excessive swelling.

Your osteopath will utilize a range of pain relief techniques including joint mobilizations and massage to assist you during this painful phase.

Phase 2- Regain Full Range of Motion

  • If you protect your injured rotator cuff structures appropriately the injured tissues will heal. Inflammed structures eg (tendonitis, bursitis) will settle when protected from additional damage.
  • Symptoms related to swimmers’ shoulders may take several weeks to improve. During this time it is important to create an environment that allows you to return to normal use quickly and prevent a recurrence.
  • It is important to lengthen and orientate your healing scar tissue via joint mobilizations, massage, shoulder muscle stretches and light active-assisted and active exercises.
  • Researchers have concluded that osteopathic treatment will improve your range of motion quicker and, in the long-term, improve your functional outcome.
  • In most cases, you will also have developed short or long-term protective tightness of your joint capsule (usually posterior) and some compensatory muscles. These structures need to be stretched to allow normal movement.
  • Signs that you have full soft tissue extensibility include being able to move your shoulder through a full range of motion. In the early stage, this may need to be passively helped (by someone else) eg. your osteopath. As you improve you will be able to do this under your own muscle power.

Phase 3- Restore Scapular Control

Your shoulder blade (scapula) is the base of your shoulder and arm movements.

  • Normal shoulder blade-shoulder movement – known as scapulohumeral rhythm is required for a pain-free and powerful shoulder function. Alteration of this movement pattern results in impingement and subsequent injury.
  • Researchers have identified poor scapulohumeral rhythm as a major cause of rotator cuff impingement. Any deficiencies will be an important component of your rehabilitation. Plus, they have identified scapular stabilization exercises as a key ingredient for successful rehabilitation.

Phase 4- Restore Normal Neck Scapulo-Thoracic Shoulder Function

It may be difficult to comprehend, but your neck and upper back (thoracic spine) are very important in the rehabilitation of shoulder pain and injury.

  • Neck or spine dysfunction can not only refer pain directly to your shoulder, but it can affect a nerve’s electrical energy, causing weakness and altered movement patterns.
  • Plus, painful spinal structures from poor posture or injury do not provide your shoulder or scapular muscles with a solid pain-free base to act upon.
  • In most cases, especially chronic shoulders, some treatment directed at your neck or upper back will be required to ease your pain, improve your shoulder movement and stop the pain or injury returning.

Phase 5- Restore Rotator Cuff Strength

  • It may seem odd that you don’t attempt to restore the strength of your rotator cuff until a later stage in the rehabilitation. However, if a structure is injured we need to provide nature with an opportunity to undertake primary healing before we load the structures with anti-gravity and resistance exercises.
  • Having said that, researchers have discovered the importance of strengthening the rotator cuff muscles with a successful rehabilitation program. These exercises need to be progressed in both load and position to accommodate for which specific rotator cuff tendons are injured and whether or not you have a secondary condition such as bursitis.

Phase 6 – Restore Technique, Speed, Power, and Agility

Swimming requires repetitive arm actions, which place enormous forces on your body (contractile and non-contractile).

  • In order to prevent a recurrence as you return to swimming, your osteopath will guide you with exercises to address these important components of rehabilitation to both prevent a recurrence and improve your sporting performance.
  • Depending on what your training or competitive program entails, a speed, agility, technique correction, and power program will be customized to prepare you for swimming-specific training.

Phase 7 – Return to Swimming

  • Depending on the demands of your swimming season, you will require individual exercises and a progressed training regime to enable a safe and injury-free return to swimming.
  • Your osteopath will discuss your goals, time frames and training schedules with you to optimize you for a complete return to swimming.
  • The perfect outcome will have you performing at full speed, power, agility and function with the added knowledge that a thorough rehabilitation program has minimized your chance of future injury.


Prevention

Tips for Improving Stroke Mechanics

It’s important to gradually increase the intensity and length of swims to avoid overtraining. Allow the body proper rest periods between competitions and training sessions.

Below we’ve outlined the swim stroke techniques for each stroke to help prevent common swimming injuries.

Freestyle stroke

  • When breathing, keep the head in line with the body to avoid neck pain or numbness and tingling in the arms.
  • Rotate the body toward the breathing side to avoid turning the neck too far and over-reaching with the arms.
  • Breathe equally to both sides to prevent excess stress on one side of the neck.

Backstroke

  • Weak muscles in the front of the neck will tire more quickly than strong ones, resulting in neck soreness with increased laps.
  • Swimmers just starting to swim this stroke should gradually increase both distance and intensity.
  • Rotating the body properly with each stroke also will help decrease stress on the neck and shoulders.

Breaststroke

  • Keep the head in line with the body to avoid increased stress on the neck.
  • Strong thigh and hip muscles will make for a stronger kick and a faster swim.
  • Leg strength will also help decrease the stress and strain placed on the knees as swimming distance increases.

Butterfly

  • The proper timing of this stroke decreases the possibility of the neck, shoulder, or back pain.
  • Focusing on a strong kick and upper body will aid in body position, as well as breathing.

References

What causes swimmer's shoulder?


Types of Fingernails Infection; Treatment, Prevention

Fingernails Infection or Paronychia is an infection of the proximal and lateral fingernails and toenails folds, including the tissue that borders the root and sides of the nail. This condition can occur spontaneously or following trauma or manipulation. Fingernails infection is among the most common infections of the hand. Fingernails infection results from the disruption of the protective barrier between the nail and the nail fold, introducing bacteria and predisposing the area to infection. Acute fingernails infection is usually limited to one nail; however, if drug-induced, it can involve many nails.

Paronychia /Fingernails infection is an inflammatory process of the nail fold. Acute fingernails infection is most often the result of bacterial infection, commonly from Staphylococcus aureus. Chronic fingernails infection is usually caused by Candida albicans. The predisposing factor in the production of chronic paronychia is trauma or maceration producing a break in the seal (cuticle) between the nail fold and the nail plate. This break produces a pocket that holds moisture and promotes the growth of microorganisms.

Test Diagnosis of Fingernails Infection

Types of Fingernails Infection

The classification of proximal fingernails infection is according to the clinical presentation

  • Acute fingernails infection – lasting less than six weeks, painful and purulent condition; most frequently caused by a bacterial infection, especially staphylococci.
  • Chronic fingernails infection – usually caused by mechanical or chemical factors and sometimes infectious etiology like a fungal infection, especially Candida species. Risk factors include occupation (dishwasher, bartender, housekeeper), certain medications, and immunosuppression (diabetes, HIV, malignancy).
  • Prosector’s fingernails infection- is a primary inoculation of tuberculosis of the skin and nails, named after its association with prosectors, who prepare specimens for dissection. Paronychia around the entire nail is sometimes referred to as runaround paronychia.
  • Painful fingernails infection – in association with a scaly, erythematous, keratotic rash (papules and plaques) of the ears, nose, fingers, and toes may be indicative of acrokeratosis paraneoplastic, which is associated with squamous cell carcinoma of the larynx.

Fingernails Infection

Classification can also be by etiology

  • Bacterial, commonly staphylococci
  • Viral, commonly Herpes simplex virus
  • Fungal, commonly Candida species

Noninfectious causes of proximal fingernails infection can include contact irritants, excessive moisture, and medication reaction.

Alternatively, proximal fingernails infection may be divided as follows

  • Candidal fingernails infection – is an inflammation of the nail fold produced by Candida albicans.[rx]
  • Pyogenic fingernails infection – is an inflammation of the folds of skin surrounding the nail caused by bacteria.[rx] Generally, acute paronychia is a pyogenic fingernail infection it is usually caused by a bacterial infection.[rx]

According to diagnostic Criteria of paronychia or fingernail infection  are

  • Cellulitis – Cellulitis is a superficial infection and will present as erythema and swelling to the affected portion of the body with no area of fluctuance. Treatment is with oral antibiotics.
  • Felon – A felon is a subcutaneous injection of the digital pulp space. The area becomes warm, red, tense, and very painful due to the confinement of the infection, creating pressure in the individual compartments created by the septa of the finger pad. These require excision and drainage, usually with a longitudinal incision and blunt dissection to ensure adequate drainage.
  • Herpetic whitlow – This is a viral infection of the distal finger caused by HSV. Patients usually develop a burning, pruritic sensation before the infection erupts. A physical exam will show vesicles, vesicles pustules, along with pain and erythema. It is important to not confuse this with a felon or a  proximal fingernails infection as incision and drainage of herpetic whitlow could result in a secondary bacterial infection and failure to heal.
  • Onychomycosis – This is a fungal infection of the nail that causes whitish-yellowish discoloration. Sometimes difficult to treat and requires oral antibiotics instead of topical.
  • Nail Psoriasis – psoriasis can also affect the fingernails and toenails. It may cause thickening of the nails with areas of pitting, ridges, irregular contour, and even raising of the nail from the nail bed.
  • Squamous cell carcinoma – Squamous cell carcinoma is mainly cancer of the skin but can also affect the nail bed. It is a rare malignant subungual tumor subject to misdiagnosis as chronic paronychia.

Causes of Fingernails Infection

  • Acute proximal fingernails infection is usually due to bacterial infection with Staphylococcus aureus (which may be multiresistant), Streptococcus pyogenes, Pseudomonas, or other bacterial pathogens. It can also be due to the cold sore virus, Herpes simplex, and the yeast, Candida albicans.
  • The cause or causes of chronic fingernails infection are not fully understood. In many cases, it is due to dermatitis of the nail fold. Often several different micro-organisms can be cultured, particularly Candida albicans and the Gram-negative bacilli, pseudomonas.
  • Infections (Bacterial, mycobacterial, or viral)
  • Raynaud’s disease
  • Metastatic cancer, subungual melanoma, squamous cell carcinoma. Benign and malignant neoplasms should always be excluded when chronic fingernails infection does not respond to conventional treatment
  • Papulosquamous disorders like psoriasis, vesiculobullous disorders-pemphigus
  • Drug toxicity from medications such as retinoids, epidermal growth factor receptor inhibitors (cetuximab), and protease inhibitors. Indinavir- induces retinoid-like effects and remains the most frequent cause of chronic in fingernails infection patients with HIV disease.
  • Retinoids also induce fingernails infection. The mechanism can be -nail fragility and minor trauma by small nail fragments.[] Fingernails infection has also been reported in patients taking cetuximab, an anti-epidermal growth factor receptor (EGFR) antibody used in the treatment of solid tumors.[]
  • Acute paronychia is usually caused by bacteria. Fingernails infection is often treated with antibiotics, either topical or oral or both. Chronic proximal fingernails infection is most often caused by a yeast infection of the soft tissues around the nail but can also be traced to a bacterial infection. If the infection is continuous, the cause is often fungal and needs antifungal cream or paint to be treated.[rx]
  • Repeatedly washing hands and trauma to the cuticle such as may occur from repeated nail-biting. In the context of bartending, it is known as bar rot.[rx]

Paronychia/ fingernails infection can occur with diabetes, drug-induced immunosuppression,[rx] or systemic diseases such as pemphigus.[rx]

Symptoms of Fingernails Infection

  • The skin typically presents as red, itchy, and hot, along with intense pain. Pus is usually present, along with gradual thickening and browning discoloration of the nail plate.
  • Red, scaly, itchy or raised patches
  • Patches may be redder on outside edges or resemble a ring
  • Patches that begin to ooze or develop a blister
  • Bald patches may develop when the scalp is affected
  • Nails may thicken, discolor or begin to crack[rx]
  • Cracking and peeling skin on your feet, most commonly between your toes and on your soles
  • Dry skin on your soles or sides of your feet
  • Raw skin on your feet
  • Discolored, thick, and crumbly toenails
  • Toenails that pull away from the nail bed
  • Itchy erosions and/or scales between the toes, especially between 4th and 5th toes
  • Scale covering the sole and sides of the feet (hyperkeratotic/moccasin type, usually caused by T. rubrum)
  • mall to medium-sized blisters, usually affecting the inner aspect of the foot (vesiculobullous type).
  • It can also uncommonly cause oozing and ulceration between the toes (ulcerative type), or pustules (these are more common in tinea pedis due to T. interdigitale than that due to T. rubrum).


Diagnosis of Fingernails Infection

  • Digital Pressure Test – Diagnostic for early fingernails infection with abscess before abscess is clearly demarcated. The patient opposes the thumb and affected finger (applying pressure to a pulp on the volar aspect at fingertip). Abscess becomes demarcated with blanching
  • CBC, ESR, Hb test – It is basically done for blood-related infection and others
  • Urine Examination It is done to investigate the infection of urine with other problems.
  • RBS ( Random blood sugar)
  • Serum creatinine – examination for the diagnosis of kidney problems.

Laboratory investigations

The following are the various laboratory tests that can be used for confirming a diagnosis of dermatophytosis.

  • Direct microscopic examination – [] Treatment of skin specimen with 10–20% potassium hydroxide (KOH) is a quick and inexpensive bedside tool to provide evidence of dermatophyte infection. Fluorescent staining with optical brighteners (diamino stilbene) is the most sensitive method to microscopically detect fungi in skin scales as well as in specimens from nails and hair.[] These substances bind to chitin, the main cell wall component of fungi
  • Culture and antifungal sensitivity – [] Sabouraud dextrose agar (SDA, 4% peptone, 1% glucose, agar, water) is the most commonly used isolation media for dermatophytosis and serves as the medium on which most morphologic descriptions are based. The development of the colony takes 7–14 days. Modified SDA, with the addition of gentamicin, chloramphenicol, and cycloheximide is more selective for dermatophytes as chloramphenicol inhibits the growth of saprophytic fungus. The dermatophyte test medium is an alternative to isolation media that contain pH indicator phenol red. It is incubated at room temperature for 5–14 days.

Antifungal Susceptibility Testing

  • Microdilution method – The broth microdilution assay for antifungal susceptibility testing of dermatophytes has been previously developed as a modification of the Clinical and Laboratory Standards Institute M38-A2 standard method. The final concentrations of terbinafine and itraconazole used is 0.06–32.0 μg/ml and for fluconazole, 0.13–64.0 μg/ml.[] A standardized inoculum is prepared by counting the microconidia microscopically. Cultures are grown on SDA slants for 7 days at 35°C to produce conidia.
  • Sterile normal saline (85%) –  is added to the agar slant, and the cultures are gently swabbed with a cotton-tipped applicator to dislodge the conidia from the hyphal mat. The suspension is transferred to a sterile centrifuge tube, and the volume is adjusted to 5 ml with sterile normal saline. The resulting suspension is counted on a hemacytometer and is diluted in RPMI 1640 medium to the desired concentration.
  • Minimum fungicidal concentration (MFC) determination – For determination of the MFC, 100-μl aliquots are removed from the assay wells showing no visible growth at the end of incubation and streaked onto SDA plates. The plates are incubated at 30°C for 7 days. The MFC is defined as the lowest drug concentration at which no visible fungal growth or colonies developed
  • Dermatophyte identification – This can be based on colony characteristics, microscopic morphology, and physiologic tests. Dermatophytes can be distinguished based upon their morphology of the macroconidia. Few physiological tests are available which help in confirmation of certain species.

Histopathology

  • Histology may be used in the diagnosis of Majocchi’s granuloma in which the KOH examination of scale on the surface may more often be negative.
  • When present, hyphae may be appreciated in stratum corneum on hematoxylin and eosin staining. Special stains most commonly used are periodic acid-Schiff and Gomori methenamine silver which helps to highlight hyphae.

Dermoscopy

  • The comma hairs, which are slightly curved, fractured hair shafts, and corkscrew hair shave been described as the dermoscopic marker of tinea capitis. Broken and dystrophic hairs are also seen. However, in tinea corporis, the involvement of vellus hair as seen on dermoscopy is an indicator of systemic therapy.[]

Polymerase chain reaction and nucleic acid sequence-based amplification

These tests not only help in the rapid and early diagnosis of infection but also help in determining drug resistance,[] and include:

  • Uniplex PCR for direct dermatophyte detection in clinical samples – A PCR for the direct detection of dermatophytes in skin scales is available as in-house PCR-ELISA assay which separately identifies numerous dermatophyte species. In a pilot study, the sensitivity and specificity of the test compared to cultures was 80.1% and 80.6%
  • Multiplex PCR for fungal detection in dermatophytes – Commercially available multiplex PCR tests enable simultaneous amplification of 21 dermatomycotic pathogens with subsequent DNA detection by means of agarose gel electrophoresis.

New molecular methods like matrix-assisted laser desorption ionization-time of flight mass spectrometry

  • It is based on the detection of biochemical characteristics, proteolytic degradation product which is a result of the activity of mycological infections or noninfectious diseases. These are represented by proteolytic degradation products of native proteins.
  • The peptide patterns of affected samples are identified by comparison with known peptide spectra from skin disorders stored in an already existing database. This procedure is immensely time-saving, as it enables simultaneous identification of up to 64 dermatophyte strains, with results coming back within 24 h.[]

Reflectance confocal microscopy

  • It provides imaging of the epidermis and superficial dermis at cellular level resolution and can be used to detect cutaneous fungi and parasitic infestations.[] Branching fungal hyphae can be detected over an erythematous annular scaly patch. The advantage of the test is noninvasive and in a retrospective analysis of the test by Friedman et al. sensitivity was found to be 100%.

Treatment for Paronychia

Non-Pharmacological

  • Keeping the hands warm and dry – Avoiding anything which might irritate the skin, such as soaps and detergents. Also avoiding injury, eg avoid manicures, finger sucking, nail-biting, etc.
  • Antiseptics or antifungal lotions – or solutions may be applied for several months. Topical antiseptic may be prescribed for a localized, minor infection.
  • Draining the pus out – If a lot of pus has collected, and your finger or toe is very swollen, the pus may need to be drained. A small cut is made to allow the pus to come out.
  • Warm bathing – It may also help to soak the affected finger in warm salted water four times a day. Soak affected digit in warm water, several times daily.
  • Wear Comfortable shoes  – Wearing very comfortable shoes if the affected nail is a toenail, to avoid any pressure on it.
  • Tea tree oil – has been used as an alternative therapy for treating fingernails infection with some success. A study from 2002 reported that a 50 percent solution of tea tree oil effectively treated an athlete’s foot in 64 percent of trial participants.
  • Treatment for paronychia which lasts more than six weeks – If the problem has dragged on for six weeks or more, it is called chronic fingernails infection. Chronic means persisting. If this is the case there may be an underlying skin condition. In other cases, there can be infected with a yeast or fungus. This is particularly common in those people mentioned above who have their hands in water a lot.


Pharmacological

  • Antibiotics – If your infection is caused by bacteria then an antibiotic may be prescribed. Antibiotics commonly used for this type of infection include flucloxacillin or erythromycin. In a minor infection, an antibiotic cream may be all that is needed – for example, fusidic acid cream.
  • NSAIDs – If pain in severe condition prescription-strength drugs that reduce both pain and inflammation. Pain medicines and anti-inflammatory drugs help to relieve pain and stiffness, allowing for increased mobility and exercise. There are many common over-the-counter medicines called non-steroidal anti-inflammatory drugs (NSAIDs). They include etodolacaceclofenacetoricoxib, ibuprofen, and naproxen.
  • Topical Medications – These prescription-strength creams, gels, ointments, patches, and sprays help relieve pain and inflammation through the skin and proximal fingernails infection.
  • Calcium & vitamin D3 – To improve bone health and healing quickly. As a general rule, men and women age 50 and older should consume 1,200 milligrams of calcium a day, and 600 international units of vitamin D a day.
  • Glucosamine & DiacereinChondroitin sulfate – can be used to tightening the loose tendon, cartilage, ligament, and cartilage, ligament regenerates cartilage or inhabits the further degeneration of cartilage, ligament.
  • Dietary supplement -to remove general weakness & improved health with strong immunity.
  • Antidepressants – A drug that blocks pain messages from your brain and boosts the effects of endorphins (your body’s natural painkillers).
  • Vitamin C, Vitamin B complex and Vitamin E It helps to heal the tissue, cell of the subcutaneous area and the infected side.

Treatment options include

Acute fingernails infection

  • Oral antibiotics may be necessary for severe or prolonged bacterial infection; often a tetracycline such as a doxycycline is prescribed.
  • Consider early treatment with aciclovir in case of severe herpes simplex infection.
  • Surgical incision and drainage may be required for abscess followed by irrigation and packing with gauze.
  • Rarely, the nail must be removed to allow pus to drain.
  • Oral antifungal agent (itraconazole or fluconazole), if C Albicans is confirmed.

Chronic fingernails infection

Attend to predisposing factors.

  • Keep the hands dry and warm.
  • Avoid wet work, or use totally waterproof gloves that are lined with cotton.
  • Keep fingernails scrupulously clean.
  • Wash after dirty work with soap and water, rinse off and dry carefully.
  • Apply emollient hand cream frequently – dimethicone barrier creams may help.

Other management

  • Patients with diabetes and vascular disease with toenail paronychia infections should be examined for signs of cellulitis.
  • Surgical excision of the proximal nail fold may be necessary.
  • Eponychial marsupialization involves surgical removal of a narrow strip of skin next to the nail, to reduce the risk of infection.
  • Swiss roll technique has the advantage of retaining the nail plate and quicker recovery.

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Treatment Options for Typical Pathogens Associated with Acute Paronychia/fingernails infection

PATHOGEN ANTIBIOTIC OPTIONS

Gram-negative aerobes

Fusobacterium

Amoxicillin/clavulanate (Augmentin), clindamycin, fluoroquinolones

Pseudomonas

Ciprofloxacin

Gram-negative anaerobe

Bacteroides

Amoxicillin/clavulanate, clindamycin, fluoroquinolones

Gram-negative facultative anaerobes

Eikenella

Cefoxitin

Enterococcus

Amoxicillin/clavulanate

Klebsiella

Trimethoprim/sulfamethoxazole, fluoroquinolones

Proteus

Amoxicillin/clavulanate, fluoroquinolones

Gram-positive aerobes

Staphylococcus

Cephalexin (Keflex)

For suspected methicillin-resistant Staphylococcus aureus infections: clindamycin, doxycycline, trimethoprim/sulfamethoxazole

Streptococcus

Cephalexin

noteLocal community resistance patterns should always be considered when choosing antibiotics.

Information from references [rx] and [rx].

[/stextbox]

Prevention

Because fungi grow particularly well in a moist environment, it’s important to make sure you keep your feet dry. You might try the following, for example:

  • Wash your feet with soap and water every day and dry them thoroughly, especially between the toes.
  • Wash socks, bedding, and towels in water that’s 140°F (60°C) or higher. Combining washing socks and application of OTC antifungal recommendations should treat most cases of athlete’s foot. You can disinfect your shoes by using disinfectant wipes (like Clorox wipes) or sprays.
  • Put antifungal powder on your feet every day.
  • Don’t share socks, shoes, or towels with others.
  • Wear sandals in public showers, around public swimming pools, and in other public places.
  • Wear socks made out of breathable fibers, such as cotton or wool, or made out of synthetic fibers that wick moisture away from your skin.
  • Thoroughly dry your feet with a towel after having a shower or bath, or after swimming
  • Wearing shoes that aren’t too tight and let your feet breathe
  • Not wearing the same pair of shoes two days in a row
  • Taking your shoes off as often as possible
  • Dry feet and toes meticulously after bathing
  • Use desiccating foot powder once or twice daily
  • Avoid wearing occlusive footwear for long periods
  • Thoroughly dry shoes and boots
  • Clean the shower and bathroom floors using a product containing bleach
  • Great shoes with antifungal powder. If treatment of tinea pedis is unsuccessful, consider reinfection, coexistent untreated fungal nail infection, reinfection due to an untreated family member, or an alternative diagnosis.
  • Wearing flip-flops when using swimming pools, communal showers and changing rooms
  • Not sharing towels, shoes, or socks
  • Washing socks, bedding, and towels at 60 degrees Celsius or more
  • Adding special anti-fungal laundry sanitizers if washing at lower temperatures

Can paronychia be prevented or avoided by

The best away to avoid acute paronychia is to take good care of your nails.

  • Avoid injuring your nails and fingertips.
  • Don’t bite or pick your nails.
  • Keep your nails trimmed and smooth.
  • Avoid cutting nails too short.
  • Don’t scrape or trim your cuticles, as this can injure the skin.
  • Use clean nail clippers or scissors.


References

Fingernails Infection

Granulomatous Inflammatory Arthritis

Arthrocutaneouveal granulomatosis/Blau syndrome (BS) is a rare autosomal dominant, autoinflammatory syndrome characterized by the clinical triad of granulomatous recurrent uveitis, dermatitis, and symmetric arthritis. The gene responsible for BS has been identified in the caspase recruitment domain gene CARD15/NOD2. In the majority of patients, the disease is characterized by early-onset, usually before 3-4years of age. The manifestations at disease onset are usually represented by articular and cutaneous involvement signs, generally followed later by ocular manifestations which are often the most relevant morbidity of BS.

Blau Syndrome is an autosomal dominant genetic inflammatory disorder which affects the skin, eyes, and joints. It is caused by a mutation in the NOD2 (CARD15) gene. Symptoms usually begin before the age of 4, and the disease manifests as early-onset cutaneous sarcoidosis, granulomatous arthritis, and uveitis.

Blau syndrome which is also known as familial or pediatric granulomatous arthritis or early-onset sarcoidosis (EOS), is a granulomatous disease with an autosomal dominant inheritance pattern. The typical sites that are affected are skin, including a maculopapular skin rash, joints, with polyarticular granulomatous arthritis, and eyes, with granulomatous uveitis. In severe cases, the granulomatous inflammation can disseminate to liver, lung and kidney. Diagnosis is made by a combination of medical history, histologic evidence of (non-caseating) granulomas at the site of inflammation and genetic testing for mutations in the NOD2/CARD15 gene.

Other Names

Arthrocutaneouveal granulomatosis; ACUG; Granulomatosis, familial, Blau type; Granulomatous inflammatory arthritis, dermatitis, and uveitis, familial; Synovitis granulomatous with uveitis and cranial neuropathies; Granulomatosis, familial juvenile systemic; Jabs syndrome, Jabs Syndrome, Early Onset Sarcoidosis
Arthrocutaneouveal granulomatosis

Pathophysiology

The NOD2 protein is part of the NOD family of proteins. These proteins form intracellular pattern recognition molecules that recognize danger signals such as toxins, bacteria or viruses and are an important part of innate immunity. NOD2 specifically recognizes muramyl dipeptide that is part of the bacterial cell wall peptidoglycan. When activated NOD2 leads to NF-kappa beta release, which in turn promotes apoptosis.

NOD2 mutations in Blau syndrome affect the nucleotide-binding domain of the protein. This leads to a gain of function that is now independent of muramyl dipeptide concentration, resulting in excessive NF kappa beta activation. Interestingly, susceptibility to Crohn’s disease has also been associated with mutations in NOD2, which shares some phenotypic similarities with Blau such as multisystem granulomatous inflammation. Polymorphisms associated with Crohn’s effect a different region of the NOD2 gene.

Recently, Mycobacterium avium ss. paratuberculosis (MAP) DNA has been identified in affected tissues from Blau patients. This organism has been implicated in the pathogenesis of Crohn’s and has also been found in patients with sarcoidosis. It is possible that NOD2 mutations in Blau could allow the persistence of MAP, leading to aberrant inflammation.

Causes of Blau Syndrome

The elucidation that the gene defect in BS involves the CARD15/NOD2 gene has stimulated many investigators, to define how this gene operates as part of the innate immune system, that responds to bacterial polysaccharides, such as muramyl dipeptide, to induce signaling pathways that induce cytokine responses and protect the organism. In BS the genetic defect seems to lead to overexpression, and poor control of the inflammatory response leading to widespread granulomatous, inflammation and tissue damage.[rx][rx]

Arthrocutaneouveal granulomatosis

Symptoms of Blau Syndrome

  • Arthritis typically affects peripheral joints mainly wrists, knees, ankles, and proximal interphalangeal (PIP) joints of the hands.
  • Tenosynovitis is equally a characteristic feature; tendon sheaths appear enlarged on examination; most often the extensor tendons of the wrist, the pes anserinus, peroneal and flexor tibialis tendon sheaths are affected.
  • Lymphocyte subpopulations, microcytic anemia (HB = 8.2 g/dl; MCV = 59.4 fl),
  • Anisocytosis and anisochromia on the manual blood smear, a slightly elevated level of C-reactive protein (9.28 mg/l), the lowered concentration of iron without other iron irregularities,
  • Deficiency of class M and G3 immunoglobulins, and vitamin D3 deficiency.
  • Symmetrical knee and ankle arthritis without erosive and destructive changes in the joints.
  • Tenosynovitis and synovitis were excluded.
  • Eczematous dermatitis decreased,
  • Multiple subcutaneous nodules emerged on both upper and lower limbs, as well as on the torso;
  • The skin lesions responded well to the escalation of the glucocorticoid dose.
  • Recurrent diarrhea and concomitant abdominal pain
  • Features included fever, sialadenitis,
  • Lymphadenopathy,
  • Erythema nodosum,
  • Leukocytoclastic vasculitis,
  • Transient neuropathies,
  • Granulomatous glomerular and interstitial nephritis, interstitial lung disease,
  • Arterial hypertension, pericarditis,
  • Pulmonary embolism,
  • Hepatic granulomas, splenic involvement, and chronic renal failure.

Cutaneous findings

  • Dermatitis in the form of a dark red, slightly scaly, maculopapular, or eczematoid-like or lichenoid-like rash may occur frequently in the first year of life.[rx ,rx]
  • Dermatitis is often symmetric, on the trunk and/or extremities and is usually intermittent with spontaneous resolution,
  • Other described cutaneous findings are represented by erythema nodosum, leukocytoclastic vasculitis, bilateral leg ulcers, ichthyosis Vulgaris and pityriasis lichenoides.[rx] ,[rx], [rx]

Ocular findings

  • Granulomatous uveitis is a frequent manifestation (70–80%), often bilateral and with a chronic recurrent course.[rx ,rx]
  • Ocular granulomatosis is often referred to as the blurred vision, painful and red eyes, floaters and photophobia. [rx]
  • Ocular involvement can potentially evolve into moderate or severe visual loss, thus representing in many patients the most severe manifestation of these diseases.[rx ,rx]

Uveitis

  • It can also affect all the ocular segments, with iridocyclitis, band keratopathy with inflammatory precipitates and vitrinite.[rx ,rx]
  • Involvement of the posterior ocular segment with chorioretinitis, vasculitis, macular oedema with retinal detachment, and optic neuritis has been described in several cases.[rx]
  • Cataracts, inflammatory glaucoma, peripheral synechiae, increased intraocular pressure and optic atrophy can represent uveitis complications.[rx]


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Comparative analysis of patient’s phenotype with the phenotype of patients with BS carrying germline NOD2 mutations
Clinical manifestation Somatic p.Arg334Gln Patients with BS with germline NOD2mutations
p.Arg334Gln All patients
n 1 7 15
Age at disease onset (mo), median (IQR) 36 20 (14.5-23) 20 (11.5-24)
Skin rash, n (%) 1 (100) 7 (100) 13 (86.7)
Articular manifestations, n (%)
 Patients with joint disease 1 (100) 7 (100) 14 (93.3)
 Age at onset of articular disease (mo), median (IQR) 120 24 (20-27) 21.5 (13.5-28.5)
 Ratio polyarthritis/oligoarthritis (%) 0/100 57.1/42.9 71.4/28.6
 Joints affected Only large joints Both large and small joints Both large and small joints
 Chronic arthritis 1 (100) 7 (100) 14 (100)
 Tenosynovitis No 7 (100) 13 (92.8)
 Deformities No 4 (57.1) 6 (42.8)
Ocular manifestations, n (%)
 Patients with bilateral uveitis 1 (100) 6 (85.7) 11 (73.3)
 Panuveitis 1 (100) 5 (71.4) 8 (53.3)
Fever, n (%) No 3 (42.9) 7 (20)
Other manifestations, n (%)
 Cranial neuropathies No 1 (14.3) 1 (6.6)
 Headache No 2 (28.6) 3 (20)
 Renal involvement No 2 (28.6) 2 (13.3)
 Heart involvement No 1 (14.3) 1 (6.6)
 Pericarditis No No 1 (6.6)
 Hepatosplenomegaly No 1 (14.3) 2 (13.3)
 Adenopathies 1 (100) 1 (14.3) 2 (13.3)
 Oral ulcers No 1 (14.3) 2 (13.3)

IQR, Interquartile range.

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Diagnosis of Blau Syndrome

On physical examination,

  • Numerous 1- to 2-mm, red-brown to pinkish-tan, flat-topped papules covered much of the face, trunk, and extremities. Individual lesions were closely grouped in oval clusters and linear arrays, with the confluence on the face.
  • The upper part of the chest knees, elbows, palms, and soles was relatively spared. No oral mucosal or conjunctival lesions were evident. No joint tenderness or swelling, lymphadenopathy, or hepatosplenomegaly was found.

The following laboratory tests produced normal results

  • Complete blood cell count – chemistry panel, serum calcium level, hepatic function panel, erythrocyte sedimentation rate, and urinalysis.
  • Blood test blood tests are important to exclude other diseases that can be associated with granulomatous inflammation (such as immune deficiency or Crohn’s disease). They are also important to see the extent of the inflammation and to evaluate the involvement of other organs (such as the kidney or liver).
  • The serum angiotensin-converting enzyme – level was 159 U/L (reference range, 13-100 U/L). A tuberculin skin test result was negative
  • Chest radiography results – were normal, and ultrasonography of the wrists, elbows, and knees revealed no synovial thickening or effusions.
  • Abdominal ultrasound scan (USS) – documented enlargement of the liver (30 mm below the right costal arch) and spleen (length 118 mm, with age-specific reference range up to 95 mm), with normal echogenicity of both organs.
  • Peripheral lymph node USS – revealed the presence of suspicious (hypoechogenic, up to 20 mm in diameter, without a marked sinus) supraclavicular, infraclavicular and axillary lymph nodes.
  • Histopathological examination – of one of the left supraclavicular lymph nodes showed reactive changes with neutrophilic infiltration.
  • Examination of the skin biopsy specimen demonstrated the presence of multiple granulomas composed of giant cells and epithelial cells at the border of the skin and subcutaneous tissue, raising suspicion of sarcoidosis or erythema annulare.
  • Clinical suspicion – It is relevant to consider Blau syndrome when a child presents a combination of symptoms (joint, skin, eye) out of the typical clinical triad. A detailed investigation into the family history should be considered because this disease is very rare and inherited in an autosomal dominant manner.
  • Demonstration of granulomas –  to make the diagnosis of Blau syndrome/EOS, the presence of typical granulomas in the affected tissue is essential. Granulomas can be seen on a biopsy of a skin lesion or of an inflamed joint. Other causes of granulomatous inflammation (such as tuberculosis, immune deficiency or other inflammatory diseases such as some vasculitides) need to be excluded by thorough clinical examination and blood tests, imaging, and other tests.
  • Genetic analysis – in the last couple of years, it has been possible to perform a genetic analysis of patients to ascertain the presence of mutations that are thought to be responsible for the development of Blau syndrome/EOS.
  • Advanced Skin biopsy – a skin biopsy involves the removal of a tiny piece of tissue from the skin and it is very easy to perform. If the skin biopsy shows granulomas, the diagnosis of Blau syndrome is made after the exclusion of all other diseases that are associated with granuloma formation.
  • The Genetic Testing Registry (GTR) – provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.
  • Genetic test – the only test that unambiguously confirms the diagnosis of Blau syndrome is a genetic test that shows the presence of a mutation in the NOD2 gene.

Treatment of Blau Syndrome

Treatment options should be guided by which organs are affected. Empiric experience has guided therapeutic choices as there have been no clinical trials owing to the rarity of Blau syndrome.

  • Consider no treatment as a rash may spontaneously resolve.
  • Superpotent (Class 1) or potent (class 2) topical corticosteroids may aid resolution of rash, although relapse may occur when discontinuing treatment.
  • Azithromycin (orally) 10mg/kg 3 times weekly was described as efficacious in one case report.

Arthritis and Occular Manifestations

  • NSAIDs may help with low-level joint inflammation.
  • Low-dose systemic corticosteroids (0.5mg/kg per day) with occasional up-titration of dose for flares has been the mainstay of treatment described historically.
  • Methotrexate has been used as a steroid-sparing agent.
  • Thalidomide at an initial dose of 2mg/kg has been used successfully in two children with refractory ocular disease.
  • Infliximab (5mg/kg IV infusion every 6 to 8 weeks) has been used in refractory cases.
  • Anakinra (100mg s.c.) once daily has been used in one patient with success, although another report of two patients showed no improvement.

Therapy

  • High-dose corticosteroids – have been shown to be efficacious when administered in concomitance with BS and EOS acute flares, as well as low-dose corticosteroids that are generally used during the quiescent stage.[rx ,rx]
  • Concomitant use of immunosuppressants – (methotrexate, or azathioprine, or mycophenolate mofetil) has been shown to be effective.[rx ,rx ,rx]
  • Further, the anti-TNF-α agent –  adalimumab, in combination with corticosteroids and/or methotrexate, has recently been shown to induce remission in case of refractory arthrocutaneous, ocular and systemic involvement.[rx]
  • Treatment with another anti-TNF-α drug – infliximab, has led to successful outcomes, also as monotherapy.[rx ,rx,rx]
  • Cyclosporine – (3 mg/kg/day) and intravenous human immunoglobulins at a supplementation dose (0.4 g/kg/month) were added to the therapeutic protocol.
  • If local treatment fails – oral prednisolone alone or in combination with MTX, azathioprine and mycophenolate mofetil, can be introduced.
  • High-dose prednisolone – can be used during attacks and low dose in more stable periods (10). Also, TNF-α inhibitors, such as infliximab and adalimumab, are used in BS/EOS (10, 11). Interleukin (IL)-1β receptor antagonists, such as anakinra and canakinumab, have been used with variable clinical outcome.
  • In cases with severe uveitis – canakinumab may be useful. In our case infliximab in combination with MTX induced remission for 8 months. However, when the MTX dose was decreased, symptoms reoccurred. Adalimumab improved uveitis and arthritis, but not a cutaneous eruption.
  • Anecdotal evidence supports – the use of steroids immunomodulation with TNF-α inhibitors and possibly other immunomodulatory therapies, such as thalidomide, methotrexate, and anti-IL-1 therapy, in the treatment of Blau syndrome.


References

Arthrocutaneouveal granulomatosis

What are the first signs of gangrene?

What are the first signs of gangrene?/Wet Gangrene is characterized by thriving bacteria and has a poor prognosis (compared to dry gangrene) due to sepsis resulting from the free communication between infected fluid and circulatory fluid. In wet gangrene, the tissue is infected by saprogenic microorganisms (Clostridium perfringens or Bacillus fusiformis, for example), which cause the tissue to swell and emit a bad smell. Wet gangrene usually develops rapidly due to blockage of venous (mainly) or arterial blood flow.[rx] The affected part is saturated with stagnant blood, which promotes the rapid growth of bacteria.

Gangrene localized death of animal soft tissue, caused by prolonged interruption of the blood supply that may result from injury or infection. Diseases in which gangrene is prone to occur include arteriosclerosis, diabetes, Raynaud’s disease, thromboangiitis obliterans (Buerger’s disease), and typhus. It also may occur after severe burns, freezing, or prolonged bed rest (bed sores).

Gangrene is a rapidly progressive life-threatening infection of skeletal muscle caused by clostridia (principally Clostridium perfringens). It is due to wound contamination in the setting of severe tissue trauma, inadequate surgical débridement, immunosuppression, and impaired blood supply. Rarely, nontraumatic gas gangrene caused by Clostridium septicum may occur in patients with occult gastrointestinal malignancies and lead to transient bacteremia.

Gangrene is a type of tissue death caused by a lack of blood supply.[rx] Symptoms may include a change in skin color to red or black, numbness, swelling, pain, skin breakdown, and coolness.[rx] The feet and hands are most commonly affected.[rx] Certain types may present with a fever or sepsis.[rx]

Types of Gangrene

What are the first signs of gangrene?

Dry gangrene

  • Dry gangrene is a form of coagulative necrosis that develops in ischemic tissue, where the blood supply is inadequate to keep tissue viable. It is not a disease itself, but a symptom of other diseases.[rx] Dry gangrene is often due to peripheral artery disease but can be due to acute limb ischemia. As a result, people with arteriosclerosis, high cholesterol, diabetes, and smoking commonly have dry gangrene.[rx] 
  • The affected area becomes cold and numb.
  • Initially, the affected area becomes red.
  • Then, it develops a brown discoloration.
  • Finally, it becomes black and shriveled.

Wet gangrene

  • Wet or infected, gangrene is characterized by thriving bacteria and has a poor prognosis (compared to dry gangrene) due to sepsis resulting from the free communication between infected fluid and circulatory fluid. In wet gangrene, the tissue is infected by saprogenic microorganisms (Clostridium perfringens or Bacillus fusiformis, for example), which cause the tissue to swell and emit a bad smell. Wet gangrene usually develops rapidly due to blockage of venous (mainly) or arterial blood flow.[rx] The affected part is saturated with stagnant blood, which promotes the rapid growth of bacteria.
  • The affected area becomes swollen and decays.
  • It is extremely painful.
  • Local oozing occurs.
  • It produces a foul-smelling odor.
  • It becomes black.
  • The affected person develops a fever.

Gas gangrene

  • Gas gangrene is a bacterial infection that produces gas within tissues. It can be caused by Clostridium, most commonly alpha toxin-producing C. perfringens, or various nonclostridial species.[rx][rx] The infection spreads rapidly as the gases produced by the bacteria expand and infiltrate healthy tissue in the vicinity. Because of its ability to quickly spread to surrounding tissues, gas gangrene should be treated as a medical emergency.
  • A brown-red or bloody discharge may ooze from the affected tissues.
  • The gas produced by Clostridia may produce a crackling sensation when the affected area is pressed.
  • It becomes swollen, and blisters may develop.
  • Pain in the affected area is severe.
  • The affected person develops fever, increased heart rate, and rapid breathing if the toxins spread into the bloodstream.

Clinical consideration when gas gangrene is present.

(I) Clostridial myonecrosis (true gas gangrene)
  •  (A) Localized – crepitant or noncrepitant
  • (B) Diffuse – crepitant or noncrepitant together with toxemia
(II) Clostridial cellulitis – anaerobic or crepitant
(III) Nonclostridial
  • (A) Bacterial – aerobic androgenic infections; Staphylococcal
    fasciitis; anaerobic streptococcal infections
  • (B) Nonbacterial – mechanical trauma; infiltration from air-hose
    injury.

Internal gangrene

  • Gangrene that affects one or more of your organs, such as your intestines, gallbladder or appendix, is called internal gangrene. This type of gangrene occurs when blood flow to an internal organ is blocked — for example, when your intestines bulge through a weakened area of muscle in your abdomen (hernia) and become twisted. Internal gangrene may cause fever and severe pain. Left untreated, internal gangrene can be fatal.

Fournier’s gangrene

  • Fournier’s gangrene involves genital organs. Men are more often affected, but women can develop this type of gangrene as well. Fournier’s gangrene usually arises due to an infection in the genital area or urinary tract and causes genital pain, tenderness, redness, and swelling.

Progressive bacterial synergistic gangrene (Meleney’s gangrene)

  • This rare type of gangrene typically occurs after an operation, with painful skin lesions developing one to two weeks after surgery.

Other Types

  • Necrotizing fasciitis, also known as hemolytic streptococcal gangrene, is an infection that spreads deep into the body along tissue planes. It is characterized by infection with S.pyogenes, a gram-positive cocci bacteria.[rx]
  • Noma is a gangrene of the face.
  • Fournier gangrene is a type of necrotizing fasciitis that usually affects the genitals and groin.[rx]
  • Venous limb gangrene may be caused by heparin-induced thrombocytopenia and thrombosis.[rx]
  • Severe mesenteric ischemia may result in gangrene of the small intestine.
  • Severe ischemic colitis may result in gangrene of the large intestine.

What are the first signs of gangrene?

Causes of Gangrene

All forms of gangrene happen because of a loss of blood supply to a certain area. This deprives tissue of oxygen and nutrients, causing the tissue to die.

Dry forms can also result from:

  • Vascular problems – Most commonly due to the poor health of arteries and veins in the legs and toes. This usually develops over time due to conditions such as diabetes, peripheral arterial disease, and high blood pressure.
  • Severe burns, scalds, and cold – Heat, chemical agents, and extreme cold, including frostbite, can all lead to dry gangrene. Wet gangrene may develop later.
  • Raynaud’s disease – There is impaired circulation to the ends of fingers and toes, especially in cold weather. Raynaud’s is implicated in some cases of gangrene.
  • Diabetes – Imbalanced blood sugar levels can damage blood vessels and nerves, reducing the oxygen supply to extremities.
  • Injury – Deep, crushing, or penetrating wounds that are sustained in conditions that allow bacterial infection can lead to gangrene. Examples are war zones and railway, machinery, and street accidents if lacerated and bruised tissues are contaminated.
  • Dry gangrene – If the area is infected with bacteria.
  • Embolism – The sudden blockage of an artery can lead to dry gangrene, but it also increases the risk of infection, and therefore wet gangrene.
  • Immune deficiency – If an immune system is weakened, for example by HIV, diabetes, long-time alcohol or drug abuse, or recent chemotherapy or radiotherapy, minor infections escalate more quickly and can become gangrenous.

Anorectal Causes

  • Trauma
  • Ischiorectal, perirectal, or perianal abscesses, appendicitis,
  • diverticulitis, colonic perforations
  • Perianal fistulotomy, perianal biopsy, rectal biopsy, hemorrhoidectomy, anal fissures excision
  • Steroid enemas for radiation proctitis
  • Rectal cancer

Genitourinary Causes

  • Trauma
  • Urethral strictures with urinary extravasation
  • Urethral catheterization or instrumentation, penile implants insertion, prostatic biopsy, vasectomy, hydrocele aspiration, genital piercing, intracavernosal cocaine injection
  • Periurethral infection; chronic urinary tract infections
  • Epididymitis or orchitis
  • Penile artificial implant, foreign body
  • Hemipelvectomy
  • Cancer invasion to the external genitalia
  • Diabetes,
  • Peripheral arterial disease,
  • Smoking, major trauma,
  • Alcoholism, HIV/AIDS, frostbite, and Raynaud’s syndrome.[rx][rx]

Symptoms of Gangrene

  • Coldness and numbness in the affected area
  • Pain in or beyond the affected area
  • Redness and swelling around a wound (this is often present when wet gangrene develops)
  • Sores that keep cropping up in the same place
  • Initial redness and swelling 
  • Either a loss of sensation or severe pain in the affected area
  • Sores or blisters that bleed or release a dirty-looking or foul-smelling discharge (if the gangrene is caused by an infection)
  • The skin becomes cold and pale
  • Persistent, unexplained fever, with a temperature higher than 100.4°F (38°C)
  • A bad-smelling wound
  • Striking discoloration of the skin, with shades of greenish-black, blue, red, or bronze
  • Pus or discharge from a wound
  • Blisters and a crackling feeling under the skin
  • Confusion, pain, fever, and low blood pressure, especially if the gangrene is internal
  • Shock

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Differences in features of dry gangrene, wet gangrene, and gas gangrene

Feature Dry gangrene Wet gangrene Gas gangrene
Site Commonly limbs More common in bowel Limbs
Mechanism Arterial occlusion More commonly venous obstruction Gases produced by Clostridium bacteria
Macroscopy Organ dry, shrunken, and black Part moist, soft, swollen, rotten, and dark Organ red, cold, pale, numb, shriveled up, and auto-amputation
Putrefaction Limited due to very little blood supply Marked due to congestion of organ with blood Marked due to bacteria and infiltration of gases produced by them in tissues
Line of demarcation Present at the junction between healthy and gangrenous parts No clear-cut line of demarcation No clear-cut line of demarcation
Bacteria Bacteria fail to survive Numerous present Major cause
Prognosis Generally better due to little septicemia Generally poor due to profound toxemia Generally poor due to quickly spread to the surrounding tissues

Note: Data from NHP.gov.in.

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Diagnosis of Gangrene

What are the first signs of gangrene?

Laboratory Studies

The following studies are indicated in patients Fournier gangrene.

  • CBC with the differential count.
  • Electrolytes, BUN, creatinine, blood glucose levels: acidosis with hyperglycemia or hypoglycemia may be present. Dehydration occurs as the disease progresses.
  • ABG sampling to provide a more accurate assessment of acid/base disturbance.
  • Blood and urine cultures.
  • Disseminated intravascular coagulation (DIC) panel (coagulation studies, fibrinogen/fibrin degradation product levels) to find evidence of severe sepsis.
  • Cultures of any open wound or abscess.
  • Fluid or tissue culture – where a small tissue or fluid sample from the affected area is tested to find out which bacteria are responsible for the condition and determine the most effective antibiotic to treat it with
  • Blood cultures – where a sample of blood is taken and put into special culture bottles and placed in a warm environment (incubated) to encourage the growth of bacteria so they can be examined further
  • Imaging tests – a range of imaging tests, such as X-rays, magnetic resonance imaging (MRI) scans or computerized tomography (CT) scans can be used to confirm the presence and spread of gangrene; these tests can also be used to study blood vessels so any blockages can be identified
  • Ankle-brachial index test – This test helps in determining arterial blood circulation and blood pressure in the lower extremities.
  • Carotid duplex test – This test is done to know the rate of blood flow through carotid arteries. It helps in exploring the presence of any plaques that may cause carotid artery disease.
  • Computed angiography and magnetic resonance angiography – These are the computerized imaging tests that are useful in studying in the blood vessels. These techniques help the vascular surgeons to know the severity of the disease.
  • Duplex ultrasound Duplex ultrasound is used to assess the blood flow status in blood vessels. With this technique, vascular surgeons easily explore the existence of an occlusion or clot and plan the treatment.
  • Skin perfusion pressure (SPP) and skin vascular resistance – SPP and skin vascular resistance measurements also help in distinguishing the patients who require vascular reconstruction or major amputation and the patients who only require foot care or require minor amputation [rx]. A prospective, double-blinded study by Castronuovo et al concluded that in ~80% cases, SPP helps in diagnosing the critical limb ischemia accurately.,
  • Ultrasonography A US finding in Fournier gangrene is a thickened wall containing hyperechoic foci that demonstrate reverberation artifacts, causing “dirty” shadowing that represents gas within the scrotal wall. Evidence of gas within the scrotal wall may be seen prior to clinical crepitus. Reactive unilateral or bilateral hydroceles may also be present. If testicular involvement occurs, there is likely an intraabdominal or retroperitoneal source of infection. US is also useful in differentiating Fournier gangrene from an inguinoscrotal incarcerated hernia; in the latter condition, gas is observed in the obstructed bowel lumen, away from the scrotal wall [].
  • Computed Tomography The CT features of Fournier gangrene include soft-tissue thickening and inflammation. CT can demonstrate asymmetric fascial thickening, any coexisting fluid collection or abscess, fat stranding around the involved structures, and subcutaneous emphysema secondary to gas-forming bacteria. The underlying cause of the Fournier gangrene, such as a perianal abscess, a fistulous tract, or an intraabdominal or retroperitoneal infectious process, may also be demonstrated at CT. In early Fournier gangrene, CT can depict progressive soft-tissue infiltration, possibly with no evidence of subcutaneous emphysema. Because the infection progresses rapidly, the early stage with lack of subcutaneous emphysema is brief and is rarely seen at CT [].

Treatment of Gangrene


Broad-Spectrum Antibiotics Coverage

  • Empiric broad-spectrum antibiotic therapy should be instituted as soon as possible until the culture results could make adjusted the therapy. The antibiotic regimen chosen must have a high degree of effectiveness against staphylococcal and streptococcal bacteria, gram-negative, coliforms, pseudomonas, Bacteroides, and clostridium.
  • Classically Triple therapy is usually recommended. Third generation cefalosporins or aminoglycosides, plus penicillin and metronidazole.
  • Clindamycin may be used as it is shown to suppress toxin production and modulate cytokine production; also use of linezolid, daptomycin, and tigecycline is warranted in cases of previous hospitalizations with prolonged antibiotic therapy which may lead to resistant Bacteroides [].

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Pharmacologic approaches for gangrene management

Pharmacologic approach Drugs class examples Mechanism of action
Pain management Opioids and opioid-like analgesics Morphine, oxycodone, dextromethorphan, tapentadol, tramadol Mimic the actions of endogenous opioid peptides by interacting with mu, delta, or kappa opioid receptors
Topical medications: capsaicin, lidocaine Provide local action on the skin to relieve pain
Circulation management Antiplatelet agents: aspirin, clopidogrel, prasugrel, ticlopidine, dipyridamole, abciximab, eptifibatide, tirofiban, ticagrelor, vorapaxar Prevent the aggregation of platelets and fibrinogenesis
Anticoagulants: heparin, fondaparinux, danaparoid, bishydroxycoumarin, warfarin, acenocoumarol, phenindione Cause activation of anticlotting factors, direct inhibition of thrombin, inhibition of synthesis of blood coagulation factor precursors (zymogens), and activation of protein C
Fibrinolytic agents: streptokinase, urokinase, alteplase, reteplase, tenecteplase Cause lysis of thrombi/clot to recanalize the occluded vessels
Antibiotics Penicillins: flucloxacillin Inhibit bacterial cell wall synthesis by binding to specific penicillin-binding proteins located inside the bacterial cell wall
Fluoroquinolones: ciprofloxacin Inhibit topoisomerase II (DNA gyrase) and topoisomerase IV, which are required for bacterial DNA replication, transcription, repair, strand supercoiling repair, and recombination
Antiprotozoals: metronidazole In reduced form, they covalently bind to DNA, disrupt its helix structure, inhibiting bacterial nucleic acid synthesis, and cause bacterial cell death
Carbapenems: ertapenem, meropenem Show bactericidal activity by inhibiting the bacterial cell wall synthesis
Glycopeptides: teicoplanin, vancomycin Inhibit the bacterial cell wall synthesis and cause cell death
Tetracyclines: doxycycline Reversibly bind to 30S ribosomal subunits and possibly to 50S subunits, block the binding of aminoacyl tRNA to mRNA, and inhibit bacterial protein synthesis
Lincosamides: clindamycin Inhibit bacterial protein synthesis by binding to 50S ribosomal subunits of the bacteria
Oxazolidinones: linezolid Selectively inhibit bacterial protein synthesis by binding to bacterial ribosomes and prevent the formation of a functional 70S initiation complex

Note: Data taken from Tripathi, NHS.org., and Drugbank.ca.

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Early IV antibiotics

  • Early IV antibiotics with early surgical debridement followed by hyperbaric oxygen therapy can salvage patients with an otherwise nearly always fatal disease. Intravenous antibiotics and early surgical debridement of the necrotic tissue reduce fatality rate to about 30%. With the addition of hyperbaric oxygen therapy, this can be reduced down to 5 to 10%.
  • Hyperbaric oxygen therapy helps by halting exotoxin production by the bacteria, helps to improve the bactericidal effect of the antibiotic, treats the tissue ischemia, improves reperfusion injury of the tissue, and promotes the activation and migration of stem cells and polymorphonuclear cells.

Pain management

  • Gangrene is usually associated with intermittent claudication in the limbs of patients. Thus, it is important to effectively manage the pain to let the patient continue exercises to improve circulation.
  • Opioid analgesics are recommended for pain associated with chronic limb ischemia. Apart from vascular factors, neuropathic pain should also be managed as a priority.

Circulation management

As the senile/dry gangrene is a consequence of ischemia, improving the blood circulation in limbs helps overcome peripheral artery disease. As per the American Diabetes Association, antiplatelet therapy or platelet aggregation inhibitors are highly recommended for preventing the vascular complications in diabetes patients. In some cases, surgery may be carried out to restore the blood flow to the affected area. The main techniques used to achieve this are:

  • Bypass surgery – where the surgeon redirects the flow of blood and bypasses the blockage by connecting (grafting) one of your veins to a healthy part of an artery
  • Angioplasty – where a tiny balloon is placed into a narrow or blocked artery and is inflated to open up the vessel; a small metal tube, known as a stent, may also be inserted into the artery to help keep it open

Research suggests that both techniques are equally effective in restoring blood flow and preventing the need for amputation in the short-term. An angioplasty has the advantage of having a faster recovery time than bypass surgery, although it may not be as effective in the long-term as bypass surgery.

Lipid-lowering agents

  • Dyslipidemia plays a major role in the progression of diabetic toe gangrene. Thus, diabetes patients should undergo a regular lipid profile check-up. In a descriptive case series study performed by Memon et al, 55.11% cases were reported to have abnormal lipid profile and 44.89% cases had lipid profile within the normal range.
  • As per Wagner’s scale, the percentage of patients with superficial ulcers (Grade I), ulcer extension (Grade II), deep ulcer with abscess (Grade III), gangrene of ore foot (Grade IV), and extensive gangrene foot (Grade V) was found to be 30.61%, 19.39%, 21.42%, 16.33%, and 12.25% respectively. A significant difference (p=0.001) was found in the gangrenous diabetic foot, grade IV and grade V.

Larval debridement therapy (biosurgery)

  • In some cases, it may be possible to use larval debridement therapy, also known as biosurgery, instead of conventional surgery to remove the dead tissue. Certain types of fly larvae are ideal for this because they feed on dead and infected tissue but leave healthy tissue alone. They also help fight infection by releasing substances that kill bacteria and stimulate the healing process.
  • Maggots used for larval therapy are specially bred in a laboratory using eggs that have been treated to remove bacteria. The maggots are placed on the wound and covered with gauze, under a firm dressing, which keeps them on the wound (and out of sight). After a few days, the dressing is cut away and the maggots are removed. Medical studies have shown larval debridement therapy can achieve more effective results than surgical debridement. However, because of the nature of this type of treatment, many people are reluctant to try it.

Exercise

  • Apart from drugs, in dry gangrene patients with peripheral arterial disease, guideline-directed management and therapy should be followed to decrease arterial occlusion or impaired perfusion, prevent the progression of chronic critical limb ischemia, and improve the functional status of affected limbs.
  • This involves exercises which are done under structured or unstructured programs. Structured exercise programs include intermittent walking followed by alternate rest time. These are organized in the health care facilities and are supervised by a trained health care professional. Unstructured home-based exercise programs also include walking as a major exercise without any supervisor’s guidance.,

Massage therapy

  • Massage Therapy is also recommended to improve the circulation in limbs. Only light pressure massage is given to the affected limb to avoid any clot formation. Massage should be cautiously done in patients with a stent, with special instructions given by the health care professional.

Fasciotomy

  • It may be necessary to relieve compartment pressures. As the infection progresses into deep tissue along and under the fascia tissue compartment pressures increase, which perpetuates further tissue ischemia and necrosis. Surgical debridement should focus on removing all the necrotic tissue, and foreign bodies such as soil, debris, and shrapnel. It is also important to irrigate the wounds with copious amounts of sterile normal saline.

Hyperbaric oxygen therapy

  • It should be added to standard therapy of antibiotics and surgical debridement to help improve survival. It is important to have coordinated care of these critically ill patients with an intensivist, general surgeon, orthopedic surgeon, urologist (in the setting of Fournier’s gangrene of the testicles and perineal structures), gynecologist (in the setting of uterine gas gangrene), infectious disease specialist, hematologist/oncologist, gastroenterologist (in the setting of spontaneous gas gangrene), and hyperbaric oxygen therapy specialist. The flow of consultation starts with usually an emergency department provider and early recognition of the disease.

Conventional Radiography

  • At radiography, hyperlucencies representing soft tissue gas may be seen in the region overlying the scrotum or perineum. Subcutaneous emphysema may be seen extending from the scrotum and perineum to the inguinal regions, anterior abdominal wall, and thighs.
  • However, the absence of subcutaneous air in the scrotum or perineum does not exclude the diagnosis of Fournier gangrene. Up to 90% of patients with Fournier gangrene have been reported to have subcutaneous emphysema so that at least 10% do not demonstrate this finding [].
  • Radiography may also demonstrate significant swelling of scrotal soft tissue. Deep fascial gas is rarely seen at radiography, which represents a significant weakness of this modality in the diagnosis and evaluation of Fournier gangrene [].

Radical Surgical Debridement

  • A debridement of the necrotic tissue as soon as possible it is widely recommended Laor et al. found no significant difference between the onset time of symptoms, early surgical treatment, and mortality, but other studies from Kabay et al. [] and Korkut et al. [] show that this time interval should be as short as possible.
  • Debridement of deep fascia and muscle is not usually required as these areas are rarely involved similar to testes. Debridement should be stopped when the separation of the skin and the subcutaneous is not performed easily because the cutaneous necrosis is not a good marker. Multiple surgical debridements is the rule rather than the exception, with an average of 3.5 procedures required per patient []

Fecal and Urinary Diversion

  • Colostomy has been used for fecal diversion in cases of severe perineal involvement. The rationale for rectal diversion includes a decrease in the number of germs in the perineal region and improved wound healing. Justifications for its construction are anal sphincter involving, fecal incontinence, or continues fecal contamination of the wound’s margins. In several papers, the percentage of patients with a colostomy is around 15% depending on the series [].

Topical Therapy

  • There have been reports of the use of honey to aid wound healing. Honey has a low pH of 3.6 and contains enzymes which digest necrotic tissues it also has antibacterial property due to phenolic acid. These changes occur within a week of applying honey to the wound. Unfortunately, there is no randomized study on the efficacy of honey in these special situations. []

Hyperbaric Oxygen Therapy

  • Hyperbaric oxygen therapy implies placing the patient in an environment of increased ambient pressure while breathing 100% oxygen, resulting in enhanced oxygenation of the arterial blood and tissues and demonstrated benefits of hyperbaric oxygen include adequate oxygenation for optimal neutrophil phagocytic function, inhibition of anaerobic growth, increased fibroblast proliferation and angiogenesis, reduction of edema by vasoconstriction, and increased intracellular antibiotics transportation [].

Vacuum-Assisted Closure

  • With the recent advent of the vacuum-assisted closure (VAC) system dressing, there seems to be a dramatic improvement with minimizing skin defects and speeding tissue healing. It simply works by exposing a wound to subatmospheric pressure for an extended period to promote debridement and healing.
  • Weinfeld et al. treated four consecutive cases using negative pressure dressings (VAC) to bolster skin grafts in male genital reconstruction.

Plastic Reconstruction

  • Various workers have used different techniques to provide skin cover including transplantation of testes, free skin grafts, axial groin flaps, and myocutaneous flaps. Split thickness skin graft seems to be the treatment of choice in treating perineal and scrotal skin defects.

Home Exercise

  • A healthy, well-balanced diet and regular exercise will keep your blood pressure and cholesterol levels at a healthy level, helping prevent your blood vessels become damaged.
  • Unless advised otherwise by your doctor, you should be aiming for at least 150 minutes (2.5 hours) of moderately intense physical exercise a week.

Moderate-intensity physical activity is any activity that increases your heart and breathing rate. It may make you sweat but you’ll still be able to hold a normal conversation. Examples include:

  • fast walking
  • cycling on level ground or with few hills
  • swimming
  • tennis

You should choose physical activities you enjoy because you’re more likely to continue doing them. It’s probably unrealistic to meet these exercise targets immediately if you haven’t exercised much in the past. Aim to start gradually and build up the amount of exercise you do overtime.

Diet

  • Eating an unhealthy diet high in fat will make any existing atherosclerosis worse and increase your risk of developing gangrene.
  • Continuing to eat high-fat foods will cause more fatty plaques to build-up in your arteries. This is because fatty foods contain cholesterol.

There are 2 types of fat-saturated and unsaturated. Avoid foods that contain saturated fats because they increase levels of “bad cholesterol” in your blood.

Foods high in saturated fat include:

  • meat pies
  • sausages and fatty cuts of meat
  • butter
  • ghee (a type of butter often used in Indian cooking)
  • lard
  • cream
  • hard cheese
  • cakes and biscuits
  • food containing coconut or palm oil


Prevention of Gangrene

  • Our emergency clinicians should be aware of this severe and potentially fatal infectious disease and should not delay treatment or prompt orthopedic surgery consultation. Gas gangrene, while rare in now peace days, can be a devastating complication of almost any small wound or surgical procedure even one as common as closed reduction of fractures. It is our experience that we should give sufficient extension of the wound to provide adequate visualization of the surgical field so as to be certain that all the necrotic or foreign material has been removed.
  • Strict aseptic techniques should be observed for even the most minor procedure. Clostridial spores are ubiquitous and can reside in hospital environments, possibly on surgeons’ hands, patients’ skin, topical application, and so on.
  • The best way to prevent gas gangrene is meticulous wound debridement and delayed closure for all potentially contaminated wounds regardless of closed or open fractures.
  • Once gas gangrene is diagnosed, careful and adequate debridement should be instituted immediately to avoid further deterioration excision of necrotic tissue still the cornerstone of treatment, which should be involved with antibiotics and all other supportive treatments.
  • Systematic resuscitative efforts should be instituted immediately in whom the diagnosis of incipient gas gangrene is even considered. This cannot be overemphasized.
  • Recognized that gas gangrene may occur spontaneously and often in an immunocompromised patient, postoperative wounds may also develop gas gangrene due to the local soft tissue damage and decreasing blood supply.
  • Keep wounds clean and sterile by cleaning all wounds thoroughly with an antiseptic solution.
  • Watch for signs of infection, such as pus, redness, swelling, or unusual pain.
  • Consult a health-care provider if any wound becomes infected.
  • People with diabetes should control their blood sugar levels with proper medication.
  • Education about proper foot care is vital for people with diabetes. They should routinely examine their feet for any signs of injury or change in skin color. Any small injury should be immediately cared for. They should keep their nails trimmed and wear comfortable well-fitting shoes.


References

What are the first signs of gangrene?

How does allopurinol prevent tumor lysis syndrome?

How does allopurinol prevent tumor lysis syndrome?/Tumor lysis syndrome (TLS) is characterized by a massive tumor cell death leading to the development of metabolic derangements and target organ dysfunction? TLS can occur as a result of cancer treatment or spontaneously. Blood cancers constitute the vast majority of TLS cases because of the sensitivity to therapy and rapid division rates.

Tumor lysis syndrome (TLS) is a potentially life-threatening condition that occurs in oncologic and hematologic patients with large tumor burden, either due to cytotoxic therapy or, less commonly, spontaneously because of massive tumor cell lysis. TLS is clinically characterized by acute renal failure, hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia. While limited options are available for treating TLS, identifying patients at high risk for developing TLS and prevention in high-risk patients remain an important aspect in the treatment of cancer patients. In general, treatment of TLS consists of intensive hydration, stimulation of diuresis, and, more specifically, in the use of allopurinol and rasburicase.

Tumor lysis syndrome (TLS) describes the pathological sequela of the rapid lysis of tumor cells. The shift of potassium, phosphorus, and nucleic acid material into the extracellular space can rapidly overcome existing homeostatic mechanisms, leading to acute kidney failure, arrhythmia, and death. TLS is the most common oncologic emergency, and although commonly seen in the context of initial chemotherapeutic treatment of hematologic malignancies, increasing recognition is being paid to the occurrence of spontaneous TLS and TLS secondary to treatment of bulky solid tumors.,

Tumor lysis syndrome is a group of metabolic abnormalities that can occur as a complication during the treatment of cancer,[rx] where large amounts of tumor cells are killed off (lysed) at the same time by the treatment, releasing their contents into the bloodstream. This occurs most commonly after the treatment of lymphomas and leukemias. In oncology and hematology, this is a potentially fatal complication, and patients at increased risk for TLS should be closely monitored before, during, and after their course of chemotherapy.

How does allopurinol prevent tumor lysis syndrome?

Pathophysiology

The pathophysiology of tumor lysis syndrome is complicated. Tumor lysis syndrome is caused by the massive release of intracellular ions such as potassium, phosphorus, and nucleic acids that have been metabolized to uric acid. The main organ is responsible for the excretions of these substances in the kidney. When the compensatory response of the kidney is exhausted as a result of the massive release of intracellular ions, uric acid obstructive uropathy develops which can then progress to acute kidney injury.

Molecules called nucleotides comprise DNA. These nucleotides are units made of a phosphate group, a sugar group, and a nitrogen base. The nitrogen base is adenine, thymine, guanine or cytosine. Adenine and guanine are purines while thymine and cytosine are pyrimidines. Ribonucleic acid, however, is made up of a ribose sugar and a nitrogen base adenine, thymine, and uracil.

The metabolism of the purines adenine and guanine in a stepwise process leads to the production of xanthine. Adenine is metabolized to hypoxanthine whereas guanine is metabolized to xanthine. Xanthine is then further metabolized into uric acid in a reaction that is catalyzed by xanthine oxidase. Most mammals have the enzyme urate oxidase that can transform uric acid to allantoin which is a more soluble substance that can be easily excreted by the kidney. Human beings lack this enzyme.

Due to the rapid turnover of tumor cells, there is an overwhelming production of uric acid which then crystallizes in the renal tubules causing obstructive uropathy from and decreased glomerular filtration rate. In rat models, urate nephropathy causes an increase in both proximal and distal tubule pressure. Peritubular capillary pressure and vascular resistance also increase. Uric acid scavenges nitric oxide which is a potent vasodilator. The scavenging of nitric oxide produces vasoconstriction and kidney ischemia. Uric acid is also a potential pro-inflammatory agent and can cause the release of other cytokine-like tumor necrosis factor-alpha, protein I. These cytokines attract white blood cells and facilitate further injury to the kidney.

Electrolyte Imbalance

Hyperkalemia

The concentration of potassium within the cell is about 120 to 130 meq/L. The lysis of tumorous cells leads to a massive release of intracellular potassium. The excess potassium is usually taken up by the liver and skeletal muscle. The rest is excreted via the gastrointestinal system or the kidney. The obstructive uropathy from uric acid salts can limit the excretion of potassium. Sometimes the hyperkalemia from the solid tumor can reach a potentially life-threatening level. The risk of hyperkalemia is cardiac arrest from arrhythmia.

Hyperphosphatemia

Hyperphosphatemia is another electrolyte imbalance associated with tumor lysis syndrome. The nucleic acid has a phosphate group, and the breakdown of the tumorous cell will lead to the release of a significant amount of phosphorus into the bloodstream. Most of the phosphorus is really excreted. This ability of the kidney to handle a high load of phosphorus is inhibited by acute kidney injury or chronic kidney disease.

Hyperphosphatemia is less common in spontaneous tumor lysis syndrome than those induced by chemotherapy. It leads to the chelation of calcium causing hypocalcemia. Deposition of calcium and phosphorus salts in the kidney and soft tissues can also occur.

Hypocalcemia

Hypocalcemia in tumor lysis syndrome is mostly secondary to the chelation of phosphorus. This condition is more potentially life-threatening than hyperphosphatemia. Possible complications from hypocalcemia include arrhythmia, tetany, seizure, and death. The calcium level might still be relatively low even after the normalization of the phosphorus level because of a deficiency of 1, 25 Vitamin D.

Causes of Tumor Lysis Syndrome

Tumor lysis syndrome is most common in patients diagnosed with leukemia who have a very high white blood cell (WBC) count. It can also be seen in high-grade lymphomas especially after the initiation of aggressive chemotherapy. Other solid tumors that can cause tumor lysis syndrome are hepatoblastoma or neuroblastoma. There are reports of tumor lysis syndrome occurring spontaneously before the initiation of chemotherapy.

An international panel of experts has stratified tumors based on the risk of developing tumor lysis syndrome.

High-Risk Tumors

  • Advanced Burkitt lymphoma
  • Advanced leukemia
  • Early-stage Burkitt lymphoma or leukemia with elevated lactate dehydrogenase
  • Acute lymphocytic leukemia with WBC count greater than 100,000/microliters, or if the baseline increase of lactate dehydrogenase is twice the upper limit of normal
  • Diffuse large B-cell lymphoma and bulky disease with an elevated baseline lactate dehydrogenase of twice the upper limit of normal
  • Acute myeloid leukemia with WBC count greater than or equal to 10,000/microliters

Intermediate-Risk Tumors

  • Acute myeloid leukemia with WBC count between 25,000 and 100,000/microliters
  • Acute lymphocytic leukemia with WBC less than 100,000/microL and lactate dehydrogenase of less than twice the upper limit of normal
  • Diffuse large B-cell lymphoma with a baseline increase in lactate dehydrogenase of twice ULN but the non-bulky disease
  • Early-stage Burkitt lymphoma or leukemia with a lactate dehydrogenase of less than twice the upper limit of normal

Low-Risk Tumors

  • Solid cancers
  • Multiple myelomas
  • Indolent lymphomas
  • Chronic lymphocytic leukemia
  • Chronic myeloid leukemia
  • Acute myelogenous leukemia with a WBC count less than 25,000/microliters and a lactate dehydrogenase elevated to less than twice the upper limit of normal

There are case reports of tumor lysis syndrome associated with the administration of steroids, biological immunomodulators, and monoclonal antibodies. Agents are associated with the development of tumor lysis syndrome include:

  • Thalidomide
  • Bortezomib
  • Hydroxyurea
  • Paclitaxel
  • Fludarabine
  • Etoposide
  • Zoledronic acid

In rare instances, tumor lysis syndrome has been observed in patients under general anesthesia undergoing surgery. Other rare occurrences of tumor lysis syndrome are seen in pregnancy or high fever.

High-Risk Tumors

  • Acute lymphocytic leukemia (5.2% to 23%)
  • Acute myeloid leukemia with a WBC count greater than 75,000 (18 %)
  • B-cell acute lymphoblastic leukemia (26.4%)
  • Burkitt lymphoma (14.9%)

Intermediate-Risk Tumors

  • Acute myeloid leukemia with WBC count between 25,000 and 50,000 (6%)
  • Diffuse large B-cell lymphoma (6%)

Low-risk Tumors

  • Acute myeloid leukemia with WBC count less than 25,000 (1%)
  • Chronic lymphocytic leukemia (0.33%)
  • Chronic myelogenous leukemia (Case reports)
  • A solid tumor (Case reports)

Tumor lysis syndrome is most commonly associated with the initiation of cytotoxic chemotherapy. However, there are case reports of tumor lysis syndrome precipitated by radiation therapy, including the use of thalidomide, dexamethasone therapy, and the use of newer chemotherapeutic agents like rituximab and bortezomib.

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Risk Factors for Tumor Lysis Syndrome.

Category of Risk Factor Risk Factor Comments
Cancer mass Bulky tumor or extensive metastasis The larger the cancer mass or the higher the number of cells that will lyse with treatment, the higher the risk of clinical tumor lysis syndrome.
Organ infiltration by cancer cells Hepatomegaly, splenomegaly, and nephromegaly generally represent tumor infiltration into these organs, and therefore a larger tumor burden than that of patients without these findings.
Bone marrow involvement Healthy adults have 1.4 kg of bone marrow. A marrow that has been replaced by leukemic cells contains a cancer mass greater than 1 kg and therefore represents bulky disease.
Renal infiltration or outflow-tract obstruction Cancers that infiltrate the kidney or obstruct urine flow predispose to nephropathy from other causes, such as the tumor lysis syndrome.
Cell lysis potential The high rate of proliferation of cancer cells Lactate dehydrogenase level is a surrogate for tumor proliferation. The higher the level, the greater the risk of the tumor lysis syndrome.
Cancer-cell sensitivity to anticancer therapy Cancers that are inherently more sensitive to therapy have a higher rate of cell lysis and a greater risk of tumor lysis syndrome than the other cancers.
The intensity of initial anticancer therapy The higher the intensity of initial therapy, the greater the rate of cancer-cell lysis and the risk of the tumor lysis syndrome. For example, some protocols for acute lymphoblastic leukemia begin with a week of prednisone monotherapy, and others begin with a combination of a glucocorticoid, vincristine, asparaginase, and daunorubicin. A patient treated on the latter protocol would have a higher risk of tumor lysis syndrome.
Features on patient presentation Nephropathy before a diagnosis of cancer A patient with preexisting nephropathy from hypertension, diabetes, gout, or other causes has a greater risk for acute kidney injury and the tumor lysis syndrome.
Dehydration or volume depletion Dehydration decreases the rate of urine flow through renal tubules and increases the level of solutes (e.g., phosphorus, uric acid) that can crystallize and cause nephropathy.
Acidic urine Uric acid has a lower solubility in acidic urine and therefore crystallizes more rapidly. A patient who presents with acidic urine and hyperuricemia usually already has uric acid crystals or microcrystals in the renal tubules.
Hypotension Hypotension decreases urine flow and increases the level of solutes that can crystallize. Hypotension can also independently cause acute kidney injury.
Exposure to nephrotoxins Vancomycin, aminoglycosides, contrast agents for diagnostic imaging and other potential nephrotoxins increase the risk of acute kidney injury from the lysis of cancer cells.
Supportive care Inadequate hydration Initial boluses of normal saline until the patient is euvolemic followed by infusion of suitable intravenous fluids at two times the maintenance rate (about 180 ml/hr in an adult who can tolerate hyperhydration) increases the rate of urine flow through renal tubules, decreases the level of solutes that can crystallize and cause acute kidney injury, and decreases the time that those solutes remain in the tubules so that even if microcrystals form they may not have time to aggregate into clinically important crystals before removal by the high flow of urine.
Exogenous potassium Unless the patient has severe hypokalemia or a dysrhythmia from hypokalemia, potassium should not be included in the intravenous fluids, and potassium (from food or medications) should be minimized until the risk period for the tumor lysis syndrome has passed.
Exogenous phosphate Restricting dietary phosphate and adding a phosphate binder reduce the exogenous load of phosphate so that the kidneys need only excrete the endogenous load of phosphate released by cancer-cell lysis.
Delayed uric acid removal Allopurinol prevents the formation of new uric acid by inhibiting xanthine oxidase and preventing the conversion of xanthine to uric acid. It does not remove existing uric acid and does increase urinary excretion of xanthine, which can crystallize and cause nephropathy. Rasburicase is an enzyme that rapidly removes uric acid by converting it to allantoin, which is highly soluble and readily excreted in the urine. The longer the uric acid level remains high, the greater the risk of crystal formation and acute kidney injury.

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Symptoms of Tumor Lysis Syndrome

Hyperkalemia – Potassium is mainly an intracellular ion. High turnover of tumor cells leads to a spill of potassium into the blood. Symptoms usually do not manifest until levels are high (> 7 mmol/L) [normal 3.5–5.0 mmol/L] and they include

  • cardiac conduction abnormalities (can be fatal)
  • severe muscle weakness or paralysis

Hyperphosphatemia – Like potassium, phosphates are also predominantly intracellular. Hyperphosphatemia causes acute kidney failure in tumor lysis syndrome, because of deposition of calcium phosphate crystals in the kidney parenchyma.

Hypocalcemia – Because of the hyperphosphatemia, calcium is precipitated to form calcium phosphate, leading to hypocalcemia. Symptoms of hypocalcemia include (but are not limited to):

  • Tetany
  • Sudden mental incapacity, including emotional lability
  • Parkinsonian (extrapyramidal) movement disorders
  • Papilledema
  • Myopathy

Hyperuricemia[rx] and hyperuricosuria. Massive cell death and nuclear breakdown generate large quantities of nucleic acids. Of these, the purines (adenine and guanine) are converted to uric acid via the purine degradation pathway and excreted in the urine. However, at the high concentrations of uric acid generated by tumor lysis, uric acid is apt to precipitate as monosodium urate crystals.

  • Acute uric acid nephropathy (AUAN) – due to hyperuricosuria has been a dominant cause of acute kidney failure but with the advent of effective treatments for hyperuricosuria, AUAN has become a less common cause than hyperphosphatemia. Two common conditions related to excess uric acid, gout and uric acid nephrolithiasis, are not features of tumor lysis syndrome.
  • Lactic acidosis.[rx][rx]
  • Pretreatment spontaneous tumor lysis syndrome. This entity is associated with acute kidney failure due to uric acid nephropathy prior to the institution of chemotherapy and is largely associated with lymphoma and leukemia. The important distinction between this syndrome and the post-chemotherapy syndrome is that spontaneous TLS is not associated with hyperphosphatemia.
  • One suggestion for the reason for this is that the high cell turnover rate leads to high uric acid levels through nucleobase turnover but the tumor reuses the released phosphate for the growth of new tumor cells. In post-chemotherapy TLS, tumor cells are destroyed and no new tumor cells are being synthesized

TLS is most common during cytotoxic treatment of hematologic neoplasms.[rx]

  • Dark urine reduced urine output or flank pain
  • Lack of appetite and fatigue
  • Numbness, seizures, or hallucinations
  • Muscle cramps and spasms
  • Heart palpitations symptoms are generally nonspecific and can include:
  • Kidney failure and death can occur, especially if TLS is left untreated.
  • TLS is diagnosed based on blood tests, along with signs and symptoms. Its onset may be subtle, with only a few abnormal laboratory values, but it can also present with frank kidney and organ failure.
  • Nausea with or without vomiting


Diagnosis of Tumor Lysis Syndrome

How does allopurinol prevent tumor lysis syndrome?

 

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Cairo-Bishop grading of clinical tumor lysis syndrome for adults

Variable Grade 0 Grade I Grade II Grade III Grade IV GradeV
Creatinine None 1.5 times ULN. The rise in creatinine is not attributable to the chemotherapeutic agent(s) > 1.5-3.0 times ULN. The rise in creatinine is not attributable to the chemotherapeutic agent(s) > 3.0-6.0 times ULN. The rise in creatinine is not attributable to the chemotherapeutic agent(s) > 6.0 times ULN. The rise in creatinine is not attributable to the chemotherapeutic agent(s) Death
Cardiac arrhythmia None Intervention not indicated Nonurgent medical intervention indicated. Cardiac arrhythmias not attributable to the chemotherapeutic agent(s) Symptomatic and incompletely controlled medically or controlled with a device (e.g., defibrillator). Cardiac arrhythmias not attributable to the chemotherapeutic agent(s) Life-threatening (e.g., arrhythmia associated with HF, hypotension, syncope, shock). Cardiac arrhythmias not attributable to the chemotherapeutic agent(s) Death
Seizures None One brief, generalized seizure; seizure(s) well controlled by anticonvulsants or infrequent focal motor seizures not interfering with ADL Seizure in which consciousness is altered; poorly controlled seizure disorder; with breakthrough generalized seizures despite medical intervention Seizure of any kind which is prolonged, repetitive or difficult to control (e.g., status epilepticus, intractable epilepsy)

TLS should be suspected in patients with large tumor burden who develop acute kidney failure along with hyperuricemia (> 15 mg/dL) or hyperphosphatemia (> 8 mg/dL). (Most other acute kidney failure occurs with uric acid < 12 mg/dL and phosphate < 6 mg/dL). Acute uric acid nephropathy is associated with little or no urine output. The urinalysis may show uric acid crystals or amorphous urates. The hypersecretion of uric acid can be detected with a high urine uric acid – creatinine ratio > 1.0, compared to a value of 0.6–0.7 for most other causes of acute kidney failure.

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Cairo-Bishop definition

In 2004, Cairo and Bishop defined a classification system for tumor lysis syndrome.[rx]

Laboratory tumor lysis syndrome: abnormality in two or more of the following, occurring within three days before or seven days after chemotherapy.

  • uric acid > 8 mg/dL or 25% increase
  • potassium > 6 meq/L or 25% increase
  • phosphate > 4.5 mg/dL or 25% increase
  • calcium < 7 mg/dL or 25% decrease

Clinical tumor lysis syndrome: laboratory tumor lysis syndrome plus one or more of the following:

  • increased serum creatinine (1.5 times upper limit of normal)
  • cardiac arrhythmia or sudden death
  • seizure

A grading scale (0–5) is used depending on the presence of lab TLS, serum creatinine, arrhythmias, or seizures.

Howard definition

In 2011, Howard proposed a refinement of the standard Cairo-Bishop definition of TLS accounting for 2 limitations:[11]

  • Two or more electrolyte laboratory abnormalities must be present simultaneously to be considered related to TLS. In fact, some patients may present with one abnormality, but later another one may develop that is unrelated to the TLS (e.g., hypocalcemia associated with sepsis).
  • A 25% change from baseline should not be considered a criterion since such increases are rarely clinically important unless the value is already outside the normal range.

Moreover, any symptomatic hypocalcemia should constitute clinical TLS.

History

The history and physical examination of patients with tumor lysis syndrome should be focused on the primary causes of the tumor lysis.

  • Time of onset of malignancy should be elicited with attention to the presence of constitutional symptoms like weight loss or anorexia. Presence of respiratory symptoms dyspnea, orthopnea, and tachypnea can be a sign of airway compression from a primary tumor.
  • Urinary symptoms such as dysuria, flank pain, and hematuria
  • Signs and symptoms that can be associated with hypocalcemia include nausea, vomiting, seizure, tetanic spasm, and change in mental status.
  • Other clinical manifestations of tumor lysis syndrome include, but are not limited to, syncopal attack, palpitation lethargy, pitting edema, facial edema, abdominal distention, and other sign of fluid overload.

Physical Examination

The physical examination should focus on the electrolyte abnormalities that are associated with tumor lysis syndrome. The physical findings associated with these abnormalities are listed below.

Hypocalcemia

Uremia for hyperuricemia and obstructive uropathy

  • Weakness
  • Lethargy
  • Malaise
  • Nausea
  • Vomiting
  • Metallic taste in the mouth
  • Irritability
  • Generalized pruritis
  • Rales and Ronchi from volume overload
  • Muffled heart sound from pericarditis secondary to uremia
  • Joint pain
  • Renal colicky pain
  • Calcium phosphate crystal deposits in the skin
  • Pruritis
  • Gangrene

The signs and symptoms of tumor lysis syndrome can develop spontaneously or about 72 hours after the initiation of chemotherapy.

Evaluation

  • Tumor lysis syndrome is diagnosed based on criteria that were developed by Cairo and Bishop. The criteria established by Cairo and Bishop have several limitations. The most crucial drawback is that the definition of tumor lysis syndrome based on this criterion requires the initiation of chemotherapy.
  • However, in clinical practice, tumor lysis syndrome can develop spontaneously without the initiation of chemotherapy. The second limitation is the use of creatinine level greater than 1.5 the upper limit for age and gender.
  • This is not standard as a patient with CKD (Chronic Kidney Disease) will have elevated creatine in the absence of AKI. The Cairo-Bishop criteria also factor the severity of tumor lysis syndrome based on the severity of illness from grade 0 (asymptomatic) to 4 (death).

Laboratory Diagnosis of Tumor Lysis Syndrome

Requires 2 or more of the following criteria achieved in the same 24-hour period from 3 days before to 7 days after chemotherapy initiation:

  • Uric acid 25% increase from baseline or greater than or equal to 8.0 mg/dL
  • Potassium 25% increase from baseline or greater than or equal to 6.0 mEq/L
  • Phosphorus 25% increase from baseline or greater than or equal to 0.5 mg/dL (greater than or equal to 6.5 mg/dL in children)
  • Calcium 25% decrease from baseline or less than or equal to 7.0 mg/dL

Clinical Diagnosis of Tumor Lysis Syndrome

Laboratory tumor lysis syndrome plus 1 or more of the following:

  • Creatinine greater than 1.5 times the upper limit of normal of an age-adjusted reference range
  • Seizure
  • Cardiac arrhythmia or sudden death

Other origins of AKI should be excluded. In the evaluation of tumor lysis syndrome, the following studies are necessary:

Imaging

  • X-Ray and CT scan of the chest to evaluate the presence of mediastinal mass and the presence of a concomitant pleural effusion
  • CT scan and an ultrasound of the abdomen and retroperitoneal structure if the mass lesion is located in the abdomen or retroperitoneum. Care must be taken with intravenous (IV) contrast because of the presence of AKI in tumor lysis syndrome.

Electrocardiography (ECG)

  • ECG is part of the workup for patients with tumor lysis syndrome to check for findings associated with hyperkalemia and hypocalcemia. Hyperkalemia is a potential cause of fatal arrhythmia in tumor lysis syndrome.

Complete Blood Count (CBC)

  • CBC helps in the diagnosis of malignancy associated with tumor lysis syndrome. The hallmark of most malignancy is leukocytosis with anemia and thrombocytopenia.

Comprehensive Metabolic Panel (CMP)

  • The metabolic derangement associated with tumor lysis syndrome is hyperkalemia, hypocalcemia, hyperphosphatemia, and hyperuricemia. Blood urea nitrogen (BUN), creatinine, and lactate dehydrogenase are also elevated in tumor lysis syndrome. CMP must be monitored between two to three times daily before and after initiation of therapy. Elevated laboratory value might be indicative of the beginning of tumor lysis syndrome.

Urine Analysis

  • Precipitation of uric acid salt can cause obstructive uropathy. In the treatment of tumor lysis syndrome, Alkalinisation of urine with sodium bicarbonate is the standard of care. Frequent urine analysis with an assessment of urine pH, specific gravity and output are mandatory.

Differential Diagnosis

Tumor lysis syndrome should be differentiated  from other clinical conditions that can cause

  • Hyperkalemia
  • Hyperphosphatemia
  • Hyperuricemia

The differential diagnosis of each electrolyte abnormalities are listed below:

Hyperkalemia

  • Hypocalcemia
  • Metabolic acidosis
  • Congenital adrenal hyperplasia
  • Toxicity from digitalis
  • Acute tubular necrosis
  • Electrical burn
  • Head trauma
  • Rhabdomyolysis
  • Thermal burns

 Hyperphosphatemia

  • Monoclonal gammopathy
  • Waldenstrom macroglobulinemia
  • Multiple Myeloma
  • Other differentials to be considered in hyperphosphatemia include:
  • Pseudohypoparathyroidism
  • Rhabdomyolysis
  • Vitamin D intoxication
  • Oral saline laxative (Phospho-soda) abuse
  • Pseudohyperphosphatemia

Hyperuricemia

  • Hyperparathyroidism
  • Hypothyroidism
  • Nephrolithiasis
  • Alcoholic ketoacidosis
  • Diabetic ketoacidosis
  • Gout
  • Pseudogout
  • Type 1 a glycogen storage disease
  • Hemolytic anemia
  • Hodgkins lymphoma
  • Uric acid nephropathy


Treatment of Tumor Lysis Syndrome

Electrolytes imbalances

Hyperkalemia – Intravenous calcium gluconate may be given to stabilize cardiac membranes for severe hyperkalemia or for electrocardiogram changes. For temporary serum reductions in potassium levels, intravenous insulin with dextrose or high dose of inhaled beta-agonists may be used. Oral sodium polystyrene resin, i.e. kayexalate, is recommended for definitive treatment of hyperkalemia. If these measures are insufficient or for severe metabolic derangements, renal replacement therapy should be considered with the consultation of nephrology.

Hyperphosphatemia – Aggressive intravenous fluid resuscitation with the maintenance of high urine output and oral phosphate binders such as sevelamer may be sufficient to improve phosphate levels. Hemodialysis may be required for severe hyperphosphatemia not controlled by these methods.

Hypocalcemia – Hypocalcemia will correct without specific intervention as phosphate levels normalize. With the exception of severe symptomatic hypocalcemia, intravenous calcium gluconate can be considered, however, it is otherwise not recommended due to elevated risk of calcium-phosphate precipitation.

Rapid Expansion of Intravascular Volume

Treatment of tumor lysis syndrome starts with rapid volume expansion. It is recommended to use crystalloids in volume expansion as this will help to increase the glomerular filtration rate (GFR) quickly. Improved GFR helps with the excretion of solutes associated with tumor lysis syndrome. The drawback to this is that the kidney functions should still be intact. Intravenous fluid should be initiated 48 hours before the start of chemotherapy and should be continued for 48 hours after chemotherapy. Hydration with about 3 to 3.5 liters/m2 per day or 4 to 5 liters per day might be needed to provide adequate hydration. This will provide a urine output of about 3 liters per day

Medications

Allopurinol

This is a structural isomer of hypoxanthine. Xanthine oxidase converts allopurinol to oxypurinol. This is the active metabolite, and it is excreted primarily by the kidney. CKD or AKI impair the elimination of oxypurinol. The level of xanthine in the urine and serum can be elevated after the administration of allopurinol because of the inhibition of the conversion of xanthine to uric acid. Xanthine by itself has limited solubility and can crystallize in the renal tubules making the obstructive uropathy associated with tumor lysis syndrome worse.

Allopurinol can decrease the production of uric acid in tumor lysis syndrome but is ineffective in the treatment of hyperuricemia associated with tumor lysis syndrome. Allopurinol is a very useful agent to prevent the development of tumor lysis syndrome.

The use of allopurinol is associated with the development of skin rash, eosinophilia, and acute hepatitis. The combination of these symptoms is called allopurinol hypersensitivity syndrome. In the treatment of tumor lysis syndrome, clinicians should be aware of a potential drug to drug interaction with azathioprine, immunosuppressive drug use in patients with solid organ transplant and autoimmune disorder.

Recombinant Urate Oxidase

A recombinant version of urate oxidase is a drug that is used to treat hyperuricemia in patients with leukemia, lymphoma, and solid tumor who are undergoing chemotherapy.

It is derived from Aspergillus by recombinant technology. The drug’s mechanism of action is the catalyzes of uric acid to allantoin, carbon dioxide, and hydrogen peroxide.

Hydrogen peroxide is a potent oxidizing agent and can cause severe methemoglobinemia or hemolytic anemia in patients with glucose 6 phosphate dehydrogenase G6PD deficiency. The Food and Drug Administration approved recombinant urate oxidase in 2009 This medication can be administered intramuscularly. It can also be given intravenously at doses of between 50 to 100 U/kg per day.

Sodium Bicarbonate for Urine Alkalinisation

The normal urine is acidic with a pH of about 5. The solubility of uric acid in urine is increased about 10-fold with the alkalinization of urine. This can be achieved by adding about 40 to 50 mEq/liter of sodium bicarbonate to the fluid use for hydration in tumor lysis syndrome.

The risk of alkalinization of the urine is a decrease in the level of ionized calcium as there is less bonding of calcium to albumin. This can worsen the hypocalcemia associated with tumor lysis syndrome leading o arrhythmia or tetany. That apart, the alkalinization of urine can favor the precipitation of calcium and phosphate salts in the kidney tubules thus making AKI in tumor lysis syndrome worse.

Therefore, alkalinization of urine with sodium bicarbonate is only advisable if rasburicase is not readily available. Even with that, the level of calcium should be serially monitored.

Calcium

Calcium chloride and calcium gluconate can be administered parenterally to treat hypocalcemia. In tumor lysis syndrome hypocalcemia is secondary to hyperphosphatemia; therefore, administration of calcium can potentiate the deposition of calcium phosphate crystals in soft tissues and the kidney making AKI worse. This might sometimes necessitate the use of hemodialysis.

Hemodialysis

This is an option that is available to use in a dire situation if the level of potassium and phosphorus is too high in the face of tumor lysis syndrome associated AKI. In tumor lysis syndrome, there is an ongoing liberation of intracellular ions. If intermittent hemodialysis is utilized for extracorporeal clearance, rebound hyperkalemia or hyperphosphatemia might develop. Because of this, continuous renal replacement therapy is the best modality for solute removal. This is done with a high flow rate for the dialysate or replacement fluid*-+. For life-threating hyperkalemia, early hemodialysis is recommended. For severe hyperphosphatemcnvmb, is, continuous renal replacement therapy might also be the best treatment modality.

Febuxostat

This medication is also a xanthine oxidase inhibitor that is relatively new to the market. It is more expensive than allopurinol. It does not cause the hypersensitivity reaction that is associated with allopurinol.

In the clinical trial, the Febuxostat for Tumor Lysis Syndrome Prevention in Hematologic Malignancies (FLORENCE), febuxostat provides better control of hyperuricemia of tumor lysis syndrome with a good safety profile and preservation of renal functions.

Alkalinization of urine

Alkalinization of urine was historically recommended in the management of TLS due to the possibility that it may increase the solubility of uric acid in urine. However, recent increasing evidence suggests that urine alkalinization is associated with increased precipitation of calcium phosphate in the renal tubules, particularly in patients with hyperphosphatemia. Therefore, alkalinization of the urine is not recommended in TLS prophylaxis and therapy anymore.

Allopurinol

Allopurinol is available as oral and intravenous formulations and prevents the conversion of hypoxanthine to xanthine and xanthine to uric acid. The renal clearance of hypoxanthine and xanthine are ten times higher than that of uric acid. Allopurinol has several drug-drug interactions, especially with 6-mercaptopurine, thiazide diuretics, azathioprine, cyclosporine, cyclophosphamide, and amoxicillin. It is necessary to adjust the dose or monitor serum levels of these drugs. The drug should be discontinued in case of skin rash due to the possibility of severe hypersensitivity reactions. Indeed, the dose of allopurinol needs to be adjusted in case of renal insufficiency.

Rasburicase

In most mammals, but not in humans, uric acid is oxidized to allantoin using the enzyme urate oxidase. In humans, uric acid is the end product of purine metabolism. Allantoin is ten times more soluble than uric acid and is easily excreted in the urine. Obtained from Aspergillus flavus, a nonrecombinant urate oxidase has been available since 1968.

Hyperkalemia may cause serious cardiac arrhythmias; therefore, potassium should be withheld from hydration fluid. Patients with potassium levels ≥6 mmol/L should be closely monitored and immediate measures should be taken (infusion of calcium gluconate, therapy with β-adrenergic agonists, and intravenous infusion of insulin and glucose).

Treating hyperphosphatemia is difficult, especially if accompanied by AKI. Oral phosphate binders are less effective, and their oral administration could be difficult in these patients. Significant hyperphosphatemia is treated best with renal replacement therapy.

Renal replacement therapy in TLS should be considered for patients with persistent hyperkalemia despite adequate therapy, severe acidosis, and volume overload unresponsive to diuretic therapy.

Volume Expansion

Once TLS has developed, efforts should be made to re-establish normal concentrations of extracellular solutes. Provided that there has not been a complete loss of kidney function, volume expansion, with a goal of increasing kidney excretion of these solutes, is the bedrock of TLS therapy.,

In addition to augmenting potassium, phosphate, and uric acid excretion, a robust urine flow rate will decrease the calcium-phosphate product in the renal tubules, decreasing the risk of crystal formation and micro-obstruction. As we discuss above, we agree with current consensus statements suggesting a target fluid intake of 3 L per day, barring contraindications.

Diuretics

Although the use of diuretics to enhance urinary flow rate may be expected to decrease the risk of tubular calcium-phosphate precipitation, this practice has not been studied. Furthermore, the hemodynamic changes associated with diuretic use may further compromise kidney function in this population. Barring clinically important volume overload, we do not routinely use diuretics in the care of patients with TLS.

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Recommendations for the prevention and treatment of tumor lysis syndrome

Low-risk disease Intermediate-risk disease High-risk disease
Diagnostic measures • No specific measures • Daily monitoring of laboratory abnormalities before and during the first 7 days of anticancer therapy • At least twice daily monitoring of laboratory abnormalities before and during the first 7 days of anticancer therapy
Preventive measures • Moderate hydration is recommended • Vigorous hydration
• Keep urinary output >100 mL/h
• Treatment with allopurinol or febuxostat should be started at least 24 hours before initiation of anticancer therapy and should be continued till normalization of uric acid levels and signs of large tumor burden are absent
• Vigorous hydration
• Keep urinary output >100 mL/h
• Single-dose 6 mg of rasburicase. Repeat doses as necessary. In the case of contraindication treatment with febuxostat
Treatment of established tumor lysis syndrome • Admission to intensive care unit with continuous cardiac monitoring and monitoring of laboratory abnormalities every 4–6 hours
• Early nephrology consultation to estimate the indications for renal replacement therapy
• Correction of electrolyte abnormalities
• Vigorous hydration, keep urinary output >100 mL/h
• Single-dose 6 mg of rasburicase. Repeat doses as necessary. In the case of contraindication, treatment with febuxostat

 

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References

How does allopurinol prevent tumor lysis syndrome?

Diet, Exercise of Clubfoot I Can Do Not Going To Doctor

Diet, Exercise of Clubfoot I Can Do Not Going To Doctor/Clubfoot (congenital talipes equinovarus) also referred to as clubfoot is one of the most common birth defects involving the musculoskeletal system. Although clubfoot is recognizable at birth, the severity of the deformity can vary from mild to an extremely rigid foot that is resistant to manipulation. Two classification systems are widely used in the initial evaluation of clubfoot deformities. It is defined as fixation of the foot in adduction, in supination and in varus, i.e. inclined inwards, axially rotated outwards and pointing downwards [rx]. The calcaneus, navicular and cuboid bones are medially rotated in relation to the talus, and are held in adduction and inversion by ligaments and tendons.

Clubfoot is a birth defect where one or both feet are rotated inward and downward.[rx][rx] The affected foot and leg may be smaller than the other.[rx] In about half of those affected, both feet are involved.[rx] Most cases are not associated with other problems.[rx] Without treatment, people walk on the sides of their feet, which causes problems with walking.[rx]

Pathoanatomy of Clubfoot

Numerous anatomical studies of clubfoot have confirmed the gross changes in the shape and position of the talus, navicular, calcaneus and cuboid [,. The tendons, tendon sheaths, ligaments, and fascia of the foot have undergone adaptive changes and became fibrotic or contractured [. The talocalcaneocuboid joints are subluxated [, , , . Nevertheless, until today, the question still remains as to whether the initial anatomical changes first occurred in the tarsal bones with subsequent soft tissue adaptation or vice versa.

Types of Clubfoot

The purpose of a classification system is to help in subsequent management and prognosis. Various classifications of clubfoot exist in the literature [, , . However, without a uniform standard, these classifications pose a major problem. Furthermore, some are too complex for practical use.

Dimeglio in 1991 divided clubfeet into 4 categories based on joint motion and ability to reduce the deformities [.

  • Soft foot – may also be called postural foot and corrected by standard casting or physiotherapy treatment.
  • Soft > Stiff foot – 33% of cases. It is usually a long foot which is more than 50% reducible and responds initially to casting. However, if the total correction has not been achieved after 7 or 8 months, surgery must be performed.
  • Stiff > Soft foot – 61% of cases. It is less than 50% reducible and after casting or physiotherapy, it is released surgically according to specific requirements,
  • Stiff foot – it is teratologic and poorly reducible. It is in severe equinus deformity, often bilateral and requires an extensive surgical correction.

This serves two purposes

  • Completely correcting the clubfoot – as the definitive treatment. Mild clubfoot may fall into this category.
  • Partially correcting a rigid clubfoot thereby – making the surgical approach less extensive [, . Casting tends to prevent further tightening of the contracted structures during the interval prior to surgery [.

Clubfoot is often broadly classified into two major groups

  • Isolated (idiopathic) clubfoot – is the most common form of the deformity and occurs in children who have no other medical problems.
  • A nonisolated clubfoot – occurs in combination with various health conditions or neuromuscular disorders, such as arthrogryposis and spina bifida. If your child’s clubfoot is associated with a neuromuscular condition, the clubfoot may be more resistant to treatment, require a longer course of nonsurgical treatment, or even multiple surgeries.

Diet, Exercise of Clubfoot I Can Do Not Going To Doctor

Causes of Clubfoot

The primary causes of the deformity. Many scholarly studies blame congenital clubfoot on:

  • Abnormal leg muscle development []
  • Connective tissue genetic defect []
  • The defective cartilaginous anlage of the anterior part of the talus []
  • Intra-uterine compression []
  • Dislocation of the talonavicular joint []
  • Defect in peroneal muscle innervation []
  • Defective anterior horn cells []
  • Abnormal tendon insertions [, ]
  • The arrest of development []
  • Tight deltoid ligament [] etc.

Genetics

  • Mutations in genes involved in muscle development are risk factors for clubfoot, specifically those encoding the muscle contractile complex (MYH3TPM2TNNT3TNNI2 and MYH8). These can cause congenital contractures, including clubfoot, in distal arthrogryposis (DA) syndromes.[rx]
  • Clubfoot can also be present in people with genetic conditions such as Loeys–Dietz syndrome. The PITX1-TBX4 transcriptional pathway has become key to the study of clubfoot. PITX1 and TBX4 are uniquely expressed in the hind limb.[rx]

Mechanical Factors in Utero

  • This is the oldest theory and was first proposed by Hippocrates [, , . He believed that the foot was held in a position of equinovarus by external uterine compression. However, Parker in 1824 and Browne in 1939 believed that diminution of amniotic fluid, as in oligohydramnios, prevents fetal movement and renders the fetus vulnerable to extrinsic pressure [.

Neuromuscular Defect

  • Some investigators still maintain the opinion that equinovarus foot is always the result of the neuromuscular defect [. On the other hand, others have shown no abnormalities in their histological studies [ and electromyographic studies of the muscles in clubfoot [. .

Primary Germ Plasma Defect

  • Irani and Sherman [ had dissected 11 equinovarus feet and 14 normal feet [. In clubfoot, they found that the neck of talus was always short, with its anterior portion rotated medially and plantarly. They suggested that the deformity probably resulted from a primary germ plasma defect.

Arrested Fetal Development

a) Intrauterine environment

  • Heuter and Von Volkman first proposed that the arrest of fetal development early in embryonic life was a cause of congenital clubfoot [. This theory was maintained by Bohm in 1929 [, . However, the opponents of this theory were Mau [ and Bessel-Hagen [.

b) Environmental influences

  • The harmful influence of teratogenic agents on the fetal environment and development are well exemplified by the effect of rubella and thalidomide in pregnancy. Many authors believe that there are various environmental factors responsible for the appearance of a clubfoot, as there are various substances capable of producing a temporary growth arrest [, , .

Hereditary

  • Clubfoot tends to be familial in a significant number of cases [, , . It is inherited as having a polygenic multifactorial trait [, , , , . Wynne-Davis stated that polygenic inheritance is more susceptible to the influence of environmental factors [.

The Mechanical Forces or Positional Hypothesis

  • Promoted the widely held hypothesis of uterine restriction, believing that restriction of fetal foot movement by the uterus caused ICTEV. He suggested that ICTEV arose from oligohydramnios sequence, i.e. believing that reduced amniotic fluid volume is in itself a cause.

The Bone/Joint Hypothesis

  • The bone/joint hypothesis postulates that positional bony abnormalities underlie the anomaly. Hippocrates wrote: ‘The deformity involves the entire combination of bones which make up the skeleton of the foot. All the changes seen in the soft part are secondary.

The Connective Tissue Hypothesis

  • The connective tissue hypothesis suggests that a primary abnormality of the connective tissue is responsible for ICTEV. This is supported by the association of ICTEV with joint laxity [. Affected children have marked plantar fibrosis at the surgery. Fetal studies give conflicting evidence. [ performed a systematic pathological study of 12 fetuses with ICTEV.

The Vascular Hypothesis

  • They documented vascular abnormalities in ‘all deformed feet of 12 fetuses’. At the level of the sinus tarsi, there was the blocking of one or more branches of the vascular tree of the foot. This was ‘most conspicuous in the early period of fetal life, and reduced to a simple knot of fatty infiltration and fibrous tissue in older specimens and the stillborn’

Support for a Neurological Hypothesis

  • Talipes equinovarus is a feature of many neurological syndromes; for example, it is often seen in association with neurological abnormalities that are secondary to spina bifida.

The Developmental Arrest Hypothesis

  • During late normal human limb development (9–38 weeks), chondrification of the foot is completed, ossification commences, joint cavitation and ligament formation is completed and the distal limb rotates medially.

Others

  • Lifestyle choices – If you smoke or use illegal drugs while you’re pregnant, you raise your baby’s chances of being born with it.
  • Other birth defects – In some cases, it’s linked with other another condition a baby is born with, such as spina bifida.
  • Too little amniotic fluid during pregnancy – This surrounds your baby in the womb. If there’s not enough, your baby’s chances of being born with clubfoot are higher.

Symptoms of Clubfoot

  • The top of the foot is usually twisted downward and inward, increasing the arch and turning the heel inward.
  • The foot may be turned so severely that it actually looks as if it’s upside down.
  • The affected leg or foot may be slightly shorter.
  • The calf muscles in the affected leg are usually underdeveloped.

In an infant born with clubfoot

  • The top of the foot twists downwards and inwards
  • The arch is more pronounced and the heel turns inward
  • In severe cases, the foot may look as if it is upside-down
  • The calf muscles tend to be underdeveloped
  • If only one foot is affected, it is usually slightly shorter than the other, especially at the heel

Diagnosis of Clubfoot

Diagnosis of clubfoot deformity is by physical examination. Typically, a newborn is examined shortly after delivery with a head to toe assessment. Examination of the lower extremity and foot reveals the deformity, which may affect one or both feet. Examination of the foot shows four components of deformity.

  • First, there is a higher arch on the inside of the foot – This component of the deformity can occur without the other aspects of clubfoot deformity. In isolation, this aspect of the deformity is called cavus deformity.
  • Second, the forefoot is curved inward or medially (toward the big toe) – This component of the deformity can occur without the other aspects of clubfoot deformity. In isolation, this aspect of the deformity is called metatarsus adductus.
  • Third, the heel is turned inward – This is a natural motion of the heel and subtalar joint, typically referred to as inversion. In clubfoot deformity, the turning in (inversion) of the heel is fixed (not passively correctable) and considered a varus deformity.
  • Fourth, and finally, the ankle is pointed downward – This is a natural motion of the ankle referred to as plantar flexion. In clubfoot deformity, this position is fixed (not correctable) and is referred to as equinus deformity.

Ultrasound

In some cases, it may be possible to detect the disease prior to birth during a prenatal ultrasound. Prenatal diagnosis by ultrasound can allow parents the opportunity to get information about this condition and make plans for treatment after their baby is born.[rx]

Clubfoot sometimes diagnosed in utero

  • 1st trimester – associated anomalies, including non-musculoskeletal ones, are very common in children diagnosed with clubfoot in the first trimester
  • 2nd trimester – these are typically true clubfeet, but associated anomalies are less common
  • 3rd trimester – if clubfoot first diagnosed in 3rd trimester, the false positive rate is higher due to a higher probability of intrauterine crowding

Treatment of Clubfoot

In general, the original correction may be recovered in four to six weeks with manipulations and plaster casts, changed every 14 days, holding the foot in marked abduction and as much dorsiflexion as possible at the ankle in the last cast. This treatment is followed by lengthening the tendon Achilles when dorsiflexion of the ankle is less than 15 degrees. A percutaneous tenotomy can be performed until one year of age. The last plaster cast is left on for three to four weeks. When the cast is removed, shoes attached in external rotation to a bar are worn at night and with naps, until the child is about four years old.

Treatment of recurrences

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Recommendations for Clubfoot Bracing Schedules (based on current knowledge)

a. Final correction in the first few months of life
  • i. Wear brace 23 hours/day for first three months
  • ii. Follow a gradual weaning schedule: one month 20-22 hours/day, one month 18-20 hours/day, one month 16-18 hours/day,
  • and one month 14-16 hours/day
  • iii. Maintain night-time wearing of the brace (12-14 hours/day) as the child grows and is walking full time for up to age 4-5 years
b. Final correction achieved after 8-9 months of age and child is ready for crawling or walking
  • i. Allow some mobility to help in the development of the weak muscles.
  • ii. Begin initial bracing with 18-20 hours/day for 2 months and then 16 hours a day for 3-4 months
  • iii. Follow standard maintenance protocol (a. iii.)
c. Final correction at age 2 to 4 years of age
  • i. Use the brace at night as per standard protocol (a.iii)
d. Final Correction after 4 years of age
  • i. Some patients may still tolerate the brace at night for 1-2 years.
  • ii. In some patients, the use of an AFO will be more acceptable.
e. Children with loose joints (approximately 2-3% of cases)
  • i. Set the shoe to 30-40% abduction (abduction of 60 to 70 degrees may lead to flat foot, usually presenting when the patient
  • starts walking at 10-16 months of age and after)
  • ii. Do not stop using the brace as there is a risk of relapse.
f.Children with has atypical/complex clubfoot
  • i. Set the shoe for the affected foot at 20-30 degrees.
  • ii. Do not bend the bar unless there is 10-15 degrees of dorsiflexion with the last cast.
  • iii. Change the angle of the shoe to 40-50 degrees as the foot becomes more normal looking and add the bend in the far to
  • allow 10-15 degrees of dorsiflexion.

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Stretching and Casting

This is the most common treatment for clubfoot. Your doctor will

  • Move your baby’s foot into a correct position and then place it in a cast to hold it there
  • Reposition and recast your baby’s foot once a week for several months
  • Perform a minor surgical procedure to lengthen the Achilles tendon (percutaneous Achilles tenotomy) toward the end of this process

After the shape of your baby’s foot is realigned, you’ll need to maintain it with one or more of the following

  • Doing stretching exercises with your baby
  • Putting your child in special shoes and braces
  • Making sure your child wears the shoes and braces as long as needed — usually full time for three months, and then at night and during naps for up to three years

Manipulation

  • Manipulation should be gentle but yet strong enough to stretch the soft tissue contractures. Forceful manipulation may result in a spurious correction producing rocker bottom foot.
  • Traditionally as suggested by Hippocrates [, the components of clubfoot deformity were corrected from distal to proximal (i.e. correction of supination, forefoot adduction and followed by equinus).
  • However, this concept is no longer popular, as equinus, varus and adduction deformities occur simultaneously and not as an isolated component. Thus, attempts are made to correct all elements of the deformities simultaneously.

Bracing

  • In Ponseti’s first case series he described the use of the foot abduction orthosis (FAO) after 3 months of full time bracing for an additional mean duration of 21 months (ranging from ten to 30 months) with a recurrence found in 56 % of cases [].
  • Due to the high rate of recurrence in this first series he recommended the FAO to be used at night for at least five or six years in his second paper on club foot treatment [].
  • Non-compliance has been recognized as a significant risk factor for the recurrence of club foot after correction with the Ponseti method with the parental educational level being an important factor [].


The Ponseti Technique

The corrective process utilizing the Ponseti technique can be divided into two phases

  • The treatment phase, during which time the deformity is corrected, and
  • The maintenance phase, during which time a brace is utilized to prevent a recurrence.

The treatment phase starts as soon as the skin condition of the child permits the use of plaster casts, till that time regular corrective manipulation of the foot by the mother is carried out. The treatment phase starts with the first cast aiming to align the forefoot with the midfoot and hindfoot. This is achieved by;

  • Stabilizing the talus by placing the thumb over the lateral part of its head.
  • Elevating the first ray to achieve supination of the forefoot in respect to the midfoot and hindfoot.
  • Putting on a well-padded plaster cast by holding this position and molding it well.

In doing so, the cavus [rx] is corrected, typically after one cast.

Diet, Exercise of Clubfoot I Can Do Not Going To Doctor

The First cast – Correction of cavus deformity

One week later, the first cast is removed and, if the cavus has been corrected, then after a short period of manipulation, the next toe-to-groin plaster cast is applied [rx] by

  • Stabilizing the talus by placing thumb over the lateral part of its head.
  • Holding the supinated foot in abduction while applying the cast.
  • Applying a well-padded plaster by holding the corrected position and molding it well.

French Method

  • Another nonsurgical method to correct clubfoot incorporates stretching, mobilization, and taping. The French method — also called the functional or physical therapy method — is typically directed by a physical therapist who has specialized training and experience.
  • Like the Ponseti method, the French method is begun soon after birth and requires family involvement. Each day, the baby’s foot must be stretched and manipulated, then taped to maintain the range of motion gained by the manipulation. After taping, a plastic splint is put on over the tape to maintain the improved range of motion.
  • This method requires approximately three visits to the physical therapist each week. Because this is a daily regimen, the therapist will teach the parents how to do it correctly at home.

After 3 months, most babies have significant improvement in foot position, and visits to the physical therapist are required less often. Like children treated with the Ponseti method, babies treated with the French method commonly require an Achilles tenotomy to improve dorsiflexion of the ankle.

Achilles Tenotomy

  • With the Ponseti method, the vast majority of patients require Achilles tenotomy to correct residual equinus after casting. Multiple techniques have been described for tenotomy, each with their own risk profile.
  • Some advocate for an open procedure performed in the operating room while others perform the procedure in recently, where Achilles tenotomy was performed in the operating room with a mini-open technique. All patients were discharged home the same day; none experienced adverse effects of anesthesia or complications from the procedure [].


New Techniques Without Tenotomy

  • New techniques that are variations of the Ponseti method may one day provide an alternative to Achilles tenotomy for correction of equinus deformity. In one study, a dynamic dorsiflexion splint was applied to correct equinus after completion of serial manipulation and casting.
  • Mean dorsiflexion improved nearly 15° at final follow-up with the use of a dynamic splint; however, 10 % of patients dropped out of the study group secondary to poor compliance and/or skin complications secondary to the splint [].
  • Another non-surgical method of equinus correction involves the injection of Botox into the gastrocsoleus complex after serial manipulations and casting.

Tibialis Anterior Tendon Transfer

  • Dynamic supination during gait and heel varus are common deformities that can occur after Ponseti casting and are usually attributed to poor brace compliance. Residual forefoot adductus after completion of casting [] and higher initial Pirani scores at the beginning of treatment [] increase the probability of recurrent deformity and subsequent surgical intervention.
  • The tibialis anterior tendon transfer (TATT) has been described as an effective procedure to address recurrent deformity. Holt et al. demonstrated the long-term effectiveness of the TATT.


Operative

Posteromedial soft tissue release and tendon lengthening

  • resistant and/or recurrent feet in young children which have failed Ponseti casting and bracing
  • “rocker bottom” feet that develop following serial casting which failed non-surgical intervention
  • syndrome-associated clubfoot if casting fails
  • when performed, it is often done at 9-10 months of age in non-syndromic feet so walking is not delayed
    • requires postoperative casting for optimal results
    • long-term stiffness and pain are relatively common
    • the extent of soft-tissue release correlates inversely with the long-term function of the foot and patient

Medial column lengthening or lateral column-shortening osteotomy, or cuboid decancellation

  • often combined with initial surgical clubfoot release in children more than 2-3 years old
  • may be performed in 3-10 years old children with recurrent deformity and “bean-shaped” foot

Talectomy

  • in severe, rigid recurrent clubfoot in children with arthrogryposis
  • age typically 6-10 years

Multiplanar supra malleolar osteotomy

  • rarely necessary
  • salvage procedure in older children with complex, rigid, multiplanar clubfoot deformities that have failed conventional operative management
  • salvage procedure in older children (8-10 yrs) with an insensate foot.

Ring fixator (Taylor Spatial Frame) application and gradual correction

  • complex deformity resistant to standard methods of treatment
  • recurrence of deformity is very high after frame removal

Triple arthrodesis

  • almost never indicated
  • contraindicated in insensate feet due to rigidity and resultant ulceration

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Recommendations for Parents (based on current knowledge)

a. Expect your child to fuss in the brace for the first 2-3 days.
i. This is not because the brace is painful, but because it is something new and different.
ii. The child may have skin sensitivity as a result of the casting
ii. If your child is completely inconsolable and you believe that they are in pain, contact your physician immediately.
b.Play with your child in the brace. This is key to getting quickly over the child’s irritability.
i. Since the child is unable to move his/her legs independent of each other in the brace, you must teach your child that he/she can
kick and swing the legs simultaneously.
ii. Gently flex and extend the knees by pushing and pulling on the bar of the brace.
iii. Try making a game of the motions by singing and/or talking to your child in an encouraging manner.
c. Make the treatment a routine.
i. Your child is less likely to fuss if you make the use of this brace as a routine, non-negotiable part of their daily activities, just like
putting on their pajamas, brushing their teeth, and reading books at night.
ii. When the child is only wearing the brace while sleeping, put the brace on any time your child goes to the “sleeping spot.”
iii. Some parents have made a brace for the child’s favorite stuffed animal or doll.
iv. Some parents call the brace “Nite-Nite shoes” or “Magic Shoes”
d. Show your child pictures of other children with clubfoot wearing their brace.
e. Use rewards and incentives to help your child understand the importance of the brace.
For older children, ask your physician to talk to the child at follow up appointments about their brace and how it helps them maintain the correction.
g. Pad the bar. This will protect your child, yourself, and your furniture from being hit by the bar when the child is wearing the brace.
i. A bicycle handlebar pad or foam pipe insulation covered with fabric or tape works well.
ii. Placing a sleep sack on the child at night will also help with padding and keep the baby from pulling at the straps and laces
with their hands.
h. If you notice any bright red spots or blistering contact your health care provider.
i. Some mild redness is normal with use.
ii. Bright red spots or blisters, especially on the back of the heel, usually indicate that the shoe was not worn tightly enough.
Make sure that the heel stays down in the shoe.
iii. Tighten the strap by one more hole or tighten the laces
iv. Remove the tongue of the shoe. Use of the brace without the tongue will not harm your child.
v. If the brake shoe has laces, lace the shoes from top to bottom, so the bow is by the toes.
vi. Check the width of the brace and widen if necessary.
vii. Never use lotion on any red spots on the skin. Lotion will make the problem worse.
viii. If persistent and the foot comes out of the shoe, it may be a sign of early relapse.

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Complications

Clubfoot typically doesn’t cause any problems until your child starts to stand and walk. If the clubfoot is treated, your child will most likely walk fairly normally. He or she may have some difficulty with:

  • Movement disorders – The affected foot may be slightly less flexible.
  • Leg length – The affected leg may be slightly shorter, but generally does not cause significant problems with mobility.
  • Shoe size – The affected foot may be up to 1 1/2 shoe sizes smaller than the unaffected foot.
  • Calf size – The muscles of the calf on the affected side may always be smaller than those on the other side.
  • Arthritis – Your child is likely to develop arthritis.
  • Poor self-image – The unusual appearance of the foot may make your child’s body image a concern during the teen years.
  • Inability to walk normally – The twist of the ankle may not allow your child to walk on the sole of the foot. To compensate, he or she may walk on the ball of the foot, the outside of the foot or even the top of the foot in severe cases.
  • Problems stemming from walking adjustments – Walking adjustments may prevent natural growth of the calf muscles, cause large sores or calluses on the foot, and result in an awkward gait.


References

Diet, Exercise of Clubfoot I Can Do Not Going To Doctor

Child Foot Deformities I Can Know Not Going To Doctor

Child Foot Deformities I Can Know Not Going To Doctor/Foot deformity is a disorder of the foot that can be congenital or acquired.  Such deformities can include hammer toe, club foot, flat feet, pes cavus, etc. A foot deformity is a disorder of the foot that can be congenital or acquired. Such deformities can include hammer toe, club foot, flat feet, pes cavus, etc. In its normal form, the structure of the human foot allows us to walk in an upright position. Our feet consist of bones, joints, muscles, tendons, and ligaments, which also keep everything in place. This makes them stable and strong, while at the same time being flexible and adaptable. Feet can become deformed as a result of external factors, certain foot postures or diseases. Foot deformities may but don’t always cause problems, such as pain and walking difficulties. There are various types of foot deformities. Some are present at birth.

Most people have slightly deformed feet. This is completely normal and usually doesn’t lead to any problems. Hardly anyone has ideal feet.

Types of Foot Deformity

Splayfoot

  • In splayfoot, the metatarsal bones spread out and the front end of the foot becomes wider. As a result, more pressure is put on the middle bones in the forefoot. This is usually painful and can make the skin hard and thick, leading to calluses. People with splayfeet are also more likely to develop bunions (hallux valgus). This is where the first metatarsal bone moves sideways (towards the other foot) and the big toe leans in towards the neighboring toes.

Fallen arch / flat foot

  • In people with fallen arches, the hollow arch under the foot is flatter than usual. When standing and walking, most of the foot – from the heel to the ball of the foot touches the floor. Fallen arches can become painful after a number of years, particularly when you put your weight on them.
  • More extreme cases of fallen arches are referred to as flat feet. This is where the entire sole of the foot touches the floor. Fallen arches and flat feet usually develop over time. People are rarely born with them. The possible causes of fallen arches include weak foot muscles, the abnormal strain on the foot, unsuitable footwear and joint inflammations.
Illustration: Healthy foot, fallen arch and flat foot – as described in the article

Pronated foot

  • In this foot deformity, the heel leans inward. Pronated feet already arise in childhood, often together with a fallen arch or flat foot. But they usually only start causing problems after several decades – at around the age of 30 or 40. In people who are overweight and/or knock-kneed, the foot often remains pronated.
  • Many parents look for medical advice because they think that their child might have a flat or pronated foot. It’s normal for the soles of children’s feet to be flatter than those of adults, though. The foot arch, midfoot and hindfoot only reach their actual normal position at around the age of ten. So “flat feet” usually don’t need to be treated in children.
Illustration: Healthy foot and pronated foot – as described in the article

High-arched feet

  • As the name suggests, people with this foot deformity have an unusually high foot arch, and the upper surface of the foot (the instep) is higher than normal too. Because of this, the ball of the foot has to carry more of the weight. This can lead to pain and calluses (areas of hard, thick skin). High-arched feet are often caused by nerve problems. They increase the likelihood of ankle injuries and claw toes.
Illustration: Healthy foot and high-arched foot – as described in the article

Equinus foot

  • In equinus foot, the footpoints down and the heel can’t be lowered onto the floor because the calf muscles are too short. People who have this deformity can only walk and stand on the front and middle part of the foot, and they can’t roll the foot in a smooth heel-to-toe movement. Equinus foot may arise following brain damage that affects the communication between nerves and muscles. It can also develop in people who are bedridden for a long period of time, or who have had an injury (e.g. an ankle injury).

Clubfoot

  • In this severe deformity, the foot points down and inwards. It’s often only possible to stand on the outer edge of the foot, or – in extreme cases – even only on the upper surface of the foot. The person has difficulties standing and walking, their foot hurts and eventually stiffens. People who have a clubfoot are usually born with it. In those children, certain muscles don’t develop properly during pregnancy. The reason for this is often not known. Genes and developmental problems in the mother’s womb are thought to play a role. Clubfoot is more common in boys. It can occur together with a developmental problem affecting the hip (hip dysplasia).

High Arch Foot or Pes Cavus

  • In a normal foot, the gait cycle (walking) begins with the arch in a flattened position, allowing the foot to be loose enough to adapt to the terrain. When the leg is perpendicular to the ground, the arch begins to rise to allow the foot to lock and support the weight of the body as it is propelled forward. In individuals with a flat foot (pes planus), the foot stays loose and unlocked.
  • In those with a high arch (pes cavus), the arch does not flatten with weight-bearing and the foot stays locked—the foot is not flexible and thus pounds the ground as the person walks. Neurologic conditions, such as cerebral palsy and Charcot-Marie-Tooth (CMT), can result in a structurally high arched foot.

Flat Foot or Pes Planus

  • The opposite of a high arched foot is a flat foot (pes planus), which, due to its structure, is “loose.” Flat foot is among the most common structural deformities of the foot, in which the medial arch is collapsed or begins to collapse at some point.[3] This deformity can be congenital or acquired if ligaments can no longer support the foot structure because they are injured (posterior tibial tendon dysfunction) or become mal-aligned later in life.
  • For a person with a congenital foot deformity of this nature, there are increased strains on ligaments and tendons, resulting in medial arch pain and overuse injuries (eg, tibial stress fractures). Treatment requires providing support to the foot in order to control the motion of the foot. These supports may include supportive shoes, inserts, or prescription orthotic devices. In severe cases, surgical interventions may also be necessary.

Hammer Toes and Claw Toes

  • Hammertoes (shown) and claw toes are also common foot deformities named for their appearance. In a normal lesser toe (toes not including the big toe), there are three phalanges connected by two joints. When the joint closest to the foot, the proximal joint, contracts, it is referred to as a hammertoe. If the joint closer to the nail, the distal joint, is contracted, the deformity is referred to as a claw toe. In addition, there may be a rotation of the toe, referred to as an adductor varus deformity, which is commonly seen in the fourth and fifth toes.

Hallux Limitus

  • In hallux limitus, changes occur to the top of the great toe joint (metatarsophalangeal [MTP] joint) (rather than to the side, as seen in a bunion), limiting its ability to bend backward. During normal walking and lifting of the heel off the ground, the great toe dorsiflexes and the MTP joint bends. If the great toe joint is compressed, there is a reduction in the range of motion (ROM), which results in increased pressure to the top of the joint.[10] Over time, osseous changes occur, which further reduces the ROM of the great toe joint. This process can continue and result in a fusion of the great toe joint (ie, stiff big toe or hallux rigidus).

Ingrown Toenail (Onychocryptosis, Unguis Incarnatus)

  • Ingrown nails (onychocryptosis, unguis incarnatus) are most commonly seen in children and young adults, as well as in the older population.[11] In young patients, ingrown nails are usually due to inconsistent growth of the toe structures, resulting in the nail border penetrating the skin; later in life, ingrown nails are commonly seen when the bone structure changes, resulting in nail deformity.
  • Other causes include cutting a nail too short along the lateral portion, such that the nail fold is irritated or penetrated, as well as external compression from shoes that are too tight.

Onychomycosis

  • Onychomycosis is a fungal infection of the nail that is caused by an interruption of the nail plate and nail bed, allowing the fungus to grow. This is commonly seen when an injury occurs to the nail, either from repetitive trauma through athletic activities or from direct trauma, such as dropping something heavy on a toe.
  • Common nail changes seen with onychomycosis include discoloration (yellow-brown), thickening of the nail, separation of the nail from the nail bed, and debris underneath the nail.

Plantar Wart or Plantar Verruca

Verrucous skin growths on the plantar surface are caused by the human papillomavirus (HPV) and have a few common characteristics, as follows

  • Black dots within the wart referred to as capillary budding
  • Interruption of the skin lines, where the wart appears to be separating the skin from the wart as it grows
  • More painful when squeezed, compared to direct pressure
    Calluses are commonly misdiagnosed as warts, but calluses generally appear near bony prominences, such as joints, have skin lines that go through them, and are more painful with direct pressure.
  • Plantar verrucas are usually self-limiting and can resolve without treatment. However, there are countless treatment options, ranging from duct tape and surgery to freezing, laser, and topical acids[18,19]; all of these are designed to irritate the wart, allowing the body to react to the irritant and recognize the viral infection. This condition is prevalent in children, in whom treatments have a high success rate. However, once patients are beyond the teenage years, treatment success rates are lower, and this condition may require medical and/or surgical intervention.

Subungual Exostosis

  • A subungual exostosis is a benign tumor composed of bone and cartilage that can grow out from the distal phalanx. This growth, which usually occurs underneath the nail bed, can result in a deformity to the toe. The center of the nail may lift up, leading to a pincher toenail.
  • A more distal growth can push the nail bed out from beneath the nail, giving the appearance of a skin growth below the nail plate left). When this occurs, it is commonly mistaken for a wart, and a radiographic examination of the toe is necessary to confirm the diagnosis (right).
  • The only treatment for the excessive bone growth, besides observation, is surgical removal of the lesion and curettage of the underlying bone. Subungual exostoses are usually found in children and young adolescents, whose feet and toes are still growing.

Spongiotic Dermatitis

  • Inflammatory skin conditions, including eczema, are referred to as spongiotic dermatitis, in which intercellular edema within the epidermis (spongiosis) leads to widening of the intercellular spaces between keratinocytes and elongation of the intercellular bridges, which may progress to intraepidermal vesiculation.[22] The inflammatory process evolves over time, causing the skin to become erythematous, scaly, and pruritic. In more severe cases, pustules may form.
  • Because there are many causes of spongiotic dermatitis, it is sometimes difficult to determine the exact etiology. Most often, it results from a hypersensitivity reaction. The precipitating factor can be an external exposure, such as a contact allergen (eg, detergents, soaps, shoe material), or an internal exposure, such as those relating to food (food allergies) and medications. Other factors can include insect bites or a viral or bacterial infection.

Gangrene

When there is an interruption of blood flow to tissues (typically via infection, vascular dysfunction, or trauma), it causes tissue death or gangrene. The three main types of gangrene are dry gangrene, followed by two infectious types, wet gangrene and gas gangrene (a subtype of wet gangrene). The foot is a common location for the occurrence of gangrene.

  • Dry gangreneDry gangrene is usually noninfectious and occurs when blood flow to a digit or limb is disrupted or suddenly stopped for example, by an arterial clot or a hypothermic injury such as frostbite (shown). Initially, the area becomes a  dusky or purplish color. Over time, the necrotic or dead tissue separates from the viable or healthy tissue, at which time a well-demarcated line divides these two types of tissue; the necrotic tissue eventually peels away, similar to a healing scab.[43] Management includes the restoration of the blood supply to the affected area. Surgical intervention is sometimes, but not always, required.
  • Wet gangrene – Wet and gas gangrene constitute medical emergencies: These rapidly spreading infections require prompt diagnosis and almost always require emergent medical and/or surgical treatment. Wet gangrene involves aggressive infection (group A beta-hemolytic streptococci, other streptococcal species, staphylococcal species[41-43]) on the surface of the skin, whereas gas gangrene involves a deeper infection within muscle fibers, in which the organism (usually, but not always, Clostridium perfringens) produces gas bubbles within the limb. Management of wet gangrene includes infection control, surgical debridement and, potentially, amputation. Hyperbaric oxygen therapy may facilitate faster-wound healing.

Diagnosis and Treatment of Foot Deformities


Diagnosis

  • Complete evaluation of feet, knees, hips, and spine
  • Flexible vs. resistant foot deformities
    • Evaluation of foot deformities, according to whether the deformity may be corrected with active (muscular contraction) or passive (manual correction by examining physician) manipulation.
      • Resistant deformity: difficult or impossible to correct → indicates a structural abnormality 
      • Flexible deformity: may be easily corrected → indicates a muscular imbalance
  • X-ray: evaluate skeletal deformities

Basic principles of treatment

  • Correctable foot deformities – foot orthotics and manipulative treatment with casting and splinting are usually successful 
  • Resistant foot deformitiessurgical correction is usually required to reposition structures or relieve muscle contractures

Prompt treatment of congenital foot deformities is vital! Surgery may often be avoided if the manipulation is implemented correctly and consistently! If muscular imbalances are not corrected at an early age, they may result in structural deformities and often require surgery!

Clubfoot (Talipes Equinovarus)

  • Definition: Clubfoot is a complex foot deformity that is comprised of five fixed deformities.
    • Hindfoot
      • Equinus foot position: short Achilles tendon fixes the foot in plantar flexion 
      • Varus position = supination of the calcaneus
    • Forefoot
      • Adductus (Pigeon toe, false clubfoot, metatarsus adductus): medial deviation of the toes (adduction of the forefoot)
      • Supinatus: inversion of the forefoot
    • Cavus (high arch): distinct arching of the foot
  • Epidemiology
    • One of the most common congenital anomalies (∼ 1/1000 births)
    • Bilateral involvement in ∼ 50% of cases
  • Etiology
    • Congenital: most common form
    • Acquired: rare (e.g., secondary to neurological conditions or trauma)
  • Pathogenesis
    • Dominant medial musculature; posterior tibial muscle is considered to be the muscle primarily responsible for the clubfoot (→ plantar flexion and supination, particularly of the hindfoot)
    • Medial deviation of the talar neck
    • Weak peroneus muscles
    • Shortened Achilles tendon
  • Diagnostics
    • Physical examination: See “Diagnosis and treatment of foot deformities” above.
    • X-ray: The long axes of the calcaneus and talus are parallel.
  • Differential diagnosispostural clubfoot 
  • Complications: pathological strain with ulceration and early onset of arthrosis
  • Treatment
    • Manipulative treatment: the Ponseti-method (manual correction with serial casting ) should be initiated within 24 hours of birth 
    • Achilles tenotomy: the equinus foot position may be corrected by surgically by lengthening the Achilles tendon with a Z-shaped suture


References

Child Foot Deformities I Can Know Not Going To Doctor

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