Rare Association of Congenital Megaoesophagus and Aortomesenteric Clamp Syndrome (AMCS): An Exceptional Case in an Infant at Yaoundé Central Hospital (YCH)

Oesophageal achalasia is a rare oesophageal motility disorder in children and remains exceptional in infants. Its association with Superior Mesenteric Artery Syndrome (SMAS), characterized by compression of the third portion of the duodenum between the abdominal aorta and the superior mesenteric artery, is extremely uncommon and has mainly been reported in adults. We report the first case of this association, which occurred in a 10-month-old female infant presenting with chronic regurgitation since birth and severe acute malnutrition. Imaging investigations, including abdominal computed tomography and upper gastrointestinal contrast study, confirmed the diagnosis of oesophageal achalasia associated with SMAS. The patient underwent Heller cardiomyotomy combined with Dor fundoplication. In addition, during follow-up, further oesophageal dilatation using Savary-Gilliard dilators was done, resulting in progressive clinical improvement and weight gain. This case highlights the importance of considering SMAS in infants presenting with severe malnutrition secondary to achalasia.