MANOMETRIC PROGRESSION OF ACHALASIA: A PEDIATRIC CASE REPORT

Abstract

119 Pediatric achalasia is a neurologic disorder of unknown etiology resulting in dysphagia. Limited information is available regarding the manometric changes during the development of achalasia. We describe a 10-year old boy with clinical and radiographic manifestations of achalasia, who had a non-specific esophageal motility disorder on initial evaluation and within 6 months developed manometric evidence of achalasia. Case report: A 10-year-old male presented with 1 year history of intermittent vomiting, retrosternal pain and daily episodes of regurgitation. He had no history of caustic ingestion or family history of achalasia. Physical examination was unremarkable. Barium swallow demonstrated a dilated esophagus with distal narrowing. Abdominal and chest CT scan were normal. EGD with biopsies was entirely normal. Esophageal motility demonstrated a mean lower esophageal sphincter (LES) pressure of 26 mmHg with complete LES relaxation. 11% of the swallows induced peristalsis. Mean basal esophageal pressure was 8 mmHg above end-expiratory gastric pressure. Because the manometric study did not demonstrate the presence of classical achalasia, the LES was dilated with a mercury bougie to 46 Fr., with improvement of symptoms. On six months follow-up, he had recurrence of emesis and dysphagia. Repeat manometry demonstrated increased LES pressure with incomplete or absent relaxation in 71% of the swallows. No peristalsis was observed. Basal esophageal pressure was 18 mmHg, with decreased mean esophageal wave pressure throughout the esophagus. Diagnosis of achalasia was made, and the patient underwent pneumatic dilatation with dramatic improvement. Conclusion: Achalasia is a progressive disease, which may first present as a non-specific motility disorder. In case of esophageal dysmotility, if symptoms persist or worsen over time, repeat manometry should be done.