Achalasia Cardia

Achalasia is a rare disease in children with an incidence of  less than 5 % of patients have symptoms when they are below 15 years of age.Achalasia is a primary esophageal motility disorder with unknown etiology, characterized by absence of esophageal peristalsis and increased or normal resting pressure of the lower esophageal sphincter (LES) which fails to relax completely in response to swallowing.

The most common symptoms in children and adolescents are vomiting, progressive dysphagia and weight loss, whereas in adults symptoms are progressive dysphagia to solids and liquids, regurgitation, and chest pain. Recurrent pneumonia, nocturnal cough and feeding problem can also occur in children.

The diagnosis is made by careful symptomatic evaluation, barium swallow study and confirmed by esophageal manometry. Endoscopy with biopsy is necessary to rule out other diseases such as eosinophilic esophagitis (EoE)and esophageal stricture, cancer, or gastroduodenal pathology.

Treatment is palliative and the goal is symptomatic relief of the functional obstruction at the level of the LES. Both pneumatic dilations of the LES and Esophageal myotomy (Heller myotomy) are the most used treatment in children and they seem to be safe and effective .

The optimal management of achalasia in children and adolescents remains unclear and the literature lacks standardized technical procedures and follow-up protocols. However, it has been shown that surgical therapy is the best treatment for young male adults as they are not good responders to balloon dilation.

With the recent advances in minimal invasive techniques, laparoscopic Heller’s myotomy is our treatment of choice. Following surgery, there was a definite improvement in their symptoms as well as an increase in their weight. We like others advocate  Laparoscopic Heller’s myotomy as the procedure of choice in the management of achalasia in children.

 

Suggested Reading:

 

Cristiane Hallal • Carlos O. Kieling • Daltro L. Nunes • Cristina T. Ferreira • Guilherme Peterson • Se´rgio G. S. Barros • Cristina A. Arruda • Jose´ C. Fraga • Helena A. S. Goldani. Diagnosis, misdiagnosis, and associated diseases of achalasia in children and adolescents: a twelve-year single center experience. Pediatr Surg Int (2012) 28:1211–1217

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