Achalasia Cardia in Children

Gastroenterology

Clinicals - History

Fact Explanation
Dysphagia Liquids are more difficult to swallow than the solids. Some patients may have dysphagia for both. This is due to the poor relaxation of the lower esophageal sphincter and aperistalsis. Dysphagia
Liquids are more difficult to swallow than the solids. Some patients may have dysphagia for both. This is due to the poor relaxation of the lower esophageal sphincter and aperistalsis.
Recurrent vomiting or regurgitation of undigested food The dilated segment of the esophagus and the poor relaxation of the lower esophageal sphincter tend to store swallowed food. This results in recurrent vomiting and regurgitation. Recurrent vomiting or regurgitation of undigested food
The dilated segment of the esophagus and the poor relaxation of the lower esophageal sphincter tend to store swallowed food. This results in recurrent vomiting and regurgitation.
Failure to thrive Recurrent vomiting, and respiratory tract infections cause failure to thrive. Failure to thrive
Recurrent vomiting, and respiratory tract infections cause failure to thrive.
Chronic cough Due to recurrent aspiration of regurgitated food particles. The trachea is compressed by the dilated esophagus. This also may contribute to recurrent cough. Chronic cough
Due to recurrent aspiration of regurgitated food particles. The trachea is compressed by the dilated esophagus. This also may contribute to recurrent cough.
Chest pain Chest pain is localized to the sub-sternal area. Pain may be burning type.
Chest pain may mimic an ischemic chest pain (severe tightening type of chest pain over the retrosternal area) and it is due to the compression of the left atrium from the dilated esophagus.
Chest pain
Chest pain is localized to the sub-sternal area. Pain may be burning type.
Chest pain may mimic an ischemic chest pain (severe tightening type of chest pain over the retrosternal area) and it is due to the compression of the left atrium from the dilated esophagus.
Choking Food particles accumulate in the dilated part of the esophagus. When the child lies supine the food particles enter the trachea and results in chocking. Choking
Food particles accumulate in the dilated part of the esophagus. When the child lies supine the food particles enter the trachea and results in chocking.
Recurrent pulmonary infections Aspiration of the food particles to the respiratory tract causes recurrent aspiration pneumonia. Recurrent pulmonary infections
Aspiration of the food particles to the respiratory tract causes recurrent aspiration pneumonia.
Wheezing May be due to the recurrent respiratory tract infections. Wheezing
May be due to the recurrent respiratory tract infections.
Hoarseness of the voice Dilated esophagus compresses recurrent laryngeal nerve. Hoarseness of the voice
Dilated esophagus compresses recurrent laryngeal nerve.

Clinicals - Examination

Fact Explanation
Loss of weight and wasting This is due to poor nutrition and recurrent respiratory tract infections. Loss of weight and wasting
This is due to poor nutrition and recurrent respiratory tract infections.
Clinical signs of pneumonia If the patient has developed aspiration pneumonia fever and lung signs of pneumonia (reduced chest expansion, dullness to percussion, increased vocal fremitus, reduced air entry and bronchial breathing over the affected segment of the chest) Clinical signs of pneumonia
If the patient has developed aspiration pneumonia fever and lung signs of pneumonia (reduced chest expansion, dullness to percussion, increased vocal fremitus, reduced air entry and bronchial breathing over the affected segment of the chest)
Pulmonary atelectasis Aspirated food particles obstruct the airways and causes collapse of the lung segments. Pulmonary atelectasis
Aspirated food particles obstruct the airways and causes collapse of the lung segments.

Investigations - Diagnosis

Fact Explanation
Esophageal manometry This is the gold standard in diagnosing achalasia. Esophageal manometry is helpful in detecting aperistalsis and failed relaxation of the lower esophageal sphincter. Increased resting esophageal body pressure and simultaneous non-propagating esophageal contractions are in favor of achalasia. Esophageal manometry
This is the gold standard in diagnosing achalasia. Esophageal manometry is helpful in detecting aperistalsis and failed relaxation of the lower esophageal sphincter. Increased resting esophageal body pressure and simultaneous non-propagating esophageal contractions are in favor of achalasia.
Barium swallow study Marked dilatation of the esophagus is visible. Air bubbles and food particles are visible in the dilated part. The distal part of the esophagus is markedly narrowed. The radiological sign is referred to “bird’s beak”. The contrast is poorly emptied from the esophagus to the stomach in most cases. However the sensitivity of this investigation is comparatively less. Dilated, tortuous esophagus and esophageal angulation are late findings. Barium swallow study
Marked dilatation of the esophagus is visible. Air bubbles and food particles are visible in the dilated part. The distal part of the esophagus is markedly narrowed. The radiological sign is referred to “bird’s beak”. The contrast is poorly emptied from the esophagus to the stomach in most cases. However the sensitivity of this investigation is comparatively less. Dilated, tortuous esophagus and esophageal angulation are late findings.
Computer Tomography (CT) of chest This shows dilated esophagus, food particles and thickening of the esophagus. Lung collapse can also be detected if present. CT scan is also able to detect malignant changes and lung secondary if present. Computer Tomography (CT) of chest
This shows dilated esophagus, food particles and thickening of the esophagus. Lung collapse can also be detected if present. CT scan is also able to detect malignant changes and lung secondary if present.
Esophagogastroduodenoscopy Retained saliva, food particles, dilated upper esophageal segment and narrowed distal part of the esophagus are suggestive of achalasia. This also helps in excluding the possibility of a mechanical obstruction of the esophageal lumen. (benign or malignant mass or stricture.) Esophagogastroduodenoscopy
Retained saliva, food particles, dilated upper esophageal segment and narrowed distal part of the esophagus are suggestive of achalasia. This also helps in excluding the possibility of a mechanical obstruction of the esophageal lumen. (benign or malignant mass or stricture.)
Endoscopic ultrasound Helps in excluding malignancy. Endoscopic ultrasound
Helps in excluding malignancy.
Bronchoscopy Pressure effects of dilated esophagus compresses the trachea. Bronchoscopy can detect this. However this is not routinely used in the diagnosis of achalasia. Bronchoscopy
Pressure effects of dilated esophagus compresses the trachea. Bronchoscopy can detect this. However this is not routinely used in the diagnosis of achalasia.

Investigations - Management

Fact Explanation
Esophagogastroduodenoscopy Evaluates the success of treatment. This also helps in detection of squamous cell carcinoma and adenocarcinoma of the esophagus which are known complications of achalasia. (Squamous cell carcinoma is more common than adenocarcinoma) Achalasia is a chronic disease and needs lifelong follow up, preferably once in every three years. Esophagogastroduodenoscopy
Evaluates the success of treatment. This also helps in detection of squamous cell carcinoma and adenocarcinoma of the esophagus which are known complications of achalasia. (Squamous cell carcinoma is more common than adenocarcinoma) Achalasia is a chronic disease and needs lifelong follow up, preferably once in every three years.
Esophageal manometry Measures post treatment lower esophageal sphincter pressure. Lower the lower esophageal sphincter pressure after the pressure lesser the risk of recurrence. Esophageal manometry
Measures post treatment lower esophageal sphincter pressure. Lower the lower esophageal sphincter pressure after the pressure lesser the risk of recurrence.
Barium swallow study Detects progressive dilatation of the esophagus and aids in deciding the treatment. Barium swallow study
Detects progressive dilatation of the esophagus and aids in deciding the treatment.
Hemoglobin levels Detects anemia secondary to poor nutrition. Hemoglobin levels
Detects anemia secondary to poor nutrition.
Chest X- ray Detects pulmonary infection. Chest X- ray
Detects pulmonary infection.
CT scan of the chest Detects pulmonary infection. CT scan is superior than the chest X-ray. CT scan of the chest
Detects pulmonary infection. CT scan is superior than the chest X-ray.
Barium swallow study Detects the diameter of the dilated esophagus and helps grading the severity of the achalasia. Diameter more than 6cm is indicative of severe achalasia. Barium swallow study
Detects the diameter of the dilated esophagus and helps grading the severity of the achalasia. Diameter more than 6cm is indicative of severe achalasia.

Management - Supportive

Fact Explanation
Nutritional support If the child is severely wasted optimization of the nutrition plays an important role in the management. Nutritional support
If the child is severely wasted optimization of the nutrition plays an important role in the management.
Treatment of respiratory tract infections Due to the recurrent aspiration, patients are more susceptible to develop aspiration pneumonia. This should be treated prior to the surgery. Treatment of respiratory tract infections
Due to the recurrent aspiration, patients are more susceptible to develop aspiration pneumonia. This should be treated prior to the surgery.

Management - Specific

Fact Explanation
Calcium channel blockers (CCB) Reduces the esophageal smooth muscle spasm and relaxes the lower esophageal sphincter. Nifedipine is the commonly used CCB. Calcium channel blockers (CCB)
Reduces the esophageal smooth muscle spasm and relaxes the lower esophageal sphincter. Nifedipine is the commonly used CCB.
Long acting nitrates The action is similar to CCBs. CCB and Long acting nitrates are the most commonly used pharmacological treatment options. Long acting nitrates
The action is similar to CCBs. CCB and Long acting nitrates are the most commonly used pharmacological treatment options.
Phosphodiesterase-5-inhibitors The mechanism of action is similar to the above drugs. Sildenafil is the commonly used drug. Phosphodiesterase-5-inhibitors
The mechanism of action is similar to the above drugs. Sildenafil is the commonly used drug.
Anticholinergics Atropine, dicyclomine and cimetropium bromide are commonly used drugs. These drugs reduces the lower esophageal sphincter tone. Anticholinergics
Atropine, dicyclomine and cimetropium bromide are commonly used drugs. These drugs reduces the lower esophageal sphincter tone.
β -adrenergic agonists Terbutaline is the commonly used drug of this group. The final action is the reduction of the lower esophageal sphincter tone. Pharmacological management is less successful than the other modalities of treatment. β -adrenergic agonists
Terbutaline is the commonly used drug of this group. The final action is the reduction of the lower esophageal sphincter tone. Pharmacological management is less successful than the other modalities of treatment.
Botulinum toxin (Botox) Botox is injected to the site of the lesion via endoscopy. Botox inhibits the release of acetylcholine neurotransmitter at the presynaptic ending and prevents the propagation of the nerve impulse. Botulinum toxin (Botox)
Botox is injected to the site of the lesion via endoscopy. Botox inhibits the release of acetylcholine neurotransmitter at the presynaptic ending and prevents the propagation of the nerve impulse.
Pneumatic dilation (balloon dilation) This is better than the pharmacological management options. Air dilates the lower esophagus and damages the circular muscle fibers of the lower esophageal sphincter. Pneumatic dilation (balloon dilation)
This is better than the pharmacological management options. Air dilates the lower esophagus and damages the circular muscle fibers of the lower esophageal sphincter.
Surgical myotomy Circular muscle fibers of the lower esophageal sphincter is surgically divided. Laparoscopic treatment is preferred over the open surgical access. Heller myotomy is the commonly used option. Surgical myotomy
Circular muscle fibers of the lower esophageal sphincter is surgically divided. Laparoscopic treatment is preferred over the open surgical access. Heller myotomy is the commonly used option.
Esophagectomy In the presence of severe esophageal dilation and tortuosity (megaesophagus or sigmoid esophagus) are eligible for this treatment option. Esophagectomy
In the presence of severe esophageal dilation and tortuosity (megaesophagus or sigmoid esophagus) are eligible for this treatment option.

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