Current Thoracic Surgery

Achalasia cardia and Hirschsprung’s disease are distinct pediatric gastrointestinal motility disorders that rarely manifest in a single patient. While both involve a failure of muscular relaxation within the enteric nervous system, they typically present with disparate clinical features. We report an exceedingly rare case of a 3-year-old girl who initially presented with classic signs of achalasia cardia, including progressive dysphagia and regurgitation. Following a successful Heller’s cardiomyotomy, the patient’s esophageal symptoms improved; however, her recovery was hampered by emerging constipation and significant abdominal distension. Initially attributed to protein-energy malnutrition, these lower gastrointestinal symptoms prompted further investigation, which ultimately revealed a concurrent diagnosis of Hirschsprung’s disease. The patient subsequently underwent a posterior anal myectomy, which led to the complete resolution of her symptoms and a full recovery. This case underscores the diagnostic complexity of dual motility pathologies. It emphasizes that persistent or evolving symptoms following a primary surgical intervention should prompt a high index of suspicion for additional underlying conditions, requiring a comprehensive and multidisciplinary diagnostic approach to ensure favorable pediatric outcomes.