Esophageal atresia and achalasialike esophageal dysmotility

Area of Science:

• Pediatric Gastroenterology
• Esophageal Motility Disorders
• Surgical Gastroenterology

Background:

• Esophageal atresia and tracheoesophageal fistula are congenital anomalies requiring surgical repair.
• Post-repair esophageal dysfunction is common, often attributed to gastroesophageal reflux and peristaltic incoordination.
• Achalasia, a primary esophageal motility disorder, is characterized by impaired lower esophageal sphincter relaxation and absent peristalsis.

Observation:

• A 14-year-old male with a history of repaired esophageal atresia presented with regurgitation, malnutrition, and chronic lung insufficiency.
• Diagnostic evaluations, including barium swallow and manometry, revealed findings consistent with achalasia.
• Histopathological examination of the myotomy specimen demonstrated intact esophageal innervation.

Findings:

• Surgical management with a Heller myotomy and antireflux procedure led to complete symptomatic resolution.
• The case highlights a potential etiological link between esophageal atresia and the subsequent development of achalasia.
• Intact neural structures in the myotomy specimen suggest achalasia may arise independently or in conjunction with prior esophageal anomaly repair.

Implications:

• Clinicians should consider achalasia in the differential diagnosis of esophageal dysmotility in patients with a history of esophageal atresia repair.
• This case suggests a possible causal relationship or association between esophageal atresia and achalasia.
• Further research is warranted to elucidate the potential link between these conditions and optimize management strategies.