Concurrence of Hirschsprung Disease and congenital duodenal obstruction

Abstract

Hirschsprung disease (HD), duodenal atresia, and annular pancreas are rare congenital gastrointestinal malformations. Although 18% of HD cases are associated with other malformations, little evidence exists of concurrent HD and duodenal atresia or HD and annular pancreas. We present three cases of infants who had evidence of duodenal obstruction on prenatal imaging. Postnatally, all three had obstructive symptoms or constipation, even after duodeno-duodenostomy was performed. They were eventually found to have HD, which was successfully treated through surgical intervention. Although the diagnosis of these conditions can be challenging given similarities in their clinical presentations, proper diagnosis and surgical management is feasible and can significantly reduce morbidity and mortality for the infant.

Keywords

• Hirschsprung disease
• Duodenal obstruction
• Gastrointestinal malformations
• Trisomy 21