Introduction: Achalasia cardia (AC) is a primary motility disorder of esophagus, characterized by aperistalsis and defective relaxation of lower esophageal sphincter. It is predominantly a disease of adults and the incidence in children is extremely rare, 0.11 in 100,000. The presenting symptoms among children predominantly are dysphagia, regurgitation, vomiting, and failure to thrive. The diagnosis is made by barium studies and esophageal manometry. Per oral endoscopic myotomy (POEM) is a novel technique in adult population but its efficacy and safety in pediatric population is not known. Cardiomyotomy is the treatment of choice for childhood achalasia.
Case description: A 4-year-old boy presented to us with complaints of recurrent vomiting since 6 months of age and failure to thrive. He used to vomit immediately after ingestion of both solids and liquids. He had history of bronchopneumonia at around 1 year of age. He was malnourished and less than the third percentile for his age. His barium esophagogram (Fig. 1) showed persistent narrowing at the lower end of esophagus with proximal dilatation suggestive of achalasia. He was nutritionally rehabilitated and taken up for laparoscopic Heller's cardiomyotomy. Post-surgery, he improved well and was able to tolerate both solids and liquids. On follow-up, he had gained weight and was feeding normally.
This case highlights the importance of recognizing the fact that achalasia though rare can present in pediatric age-group as well. Diagnosis is usually delayed or misdiagnosed as gastroesophageal reflux disease (GERD), esophageal webs, etc. Patients usually become extremely malnourished and developmental milestones are delayed. Hence, the early diagnosis and treatment with cardiomyotomy is the key.
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