Hirschsprung's disease and neonatal intestinal obstruction: Where does it lie in the spectrum?

Abstract

Introduction: Neonatal intestinal obstruction may be caused by varied etiologies. These different etiologies differ in presentation, management, and outcome. Hirschsprung's disease (HD) is an important differential diagnosis in such patients and differs in presentation, management, and outcome. Objective: The objective of the study was to compare the clinical presentation and outcome of HD with other causes of neonatal intestinal obstruction. Methods: The medical record of all the patients, presented at our institute with intestinal obstruction in the neonatal period during 2014–2015, was reviewed retrospectively for etiology, clinical features, investigations, management, and outcome. The cases diagnosed with HD (Cohort A) were compared with the cohort containing patients with all other causes of neonatal intestinal obstruction (Cohort B). Results: A total of 53 cases with congenital neonatal intestinal obstruction were identified. Among them, 17 had HD and 36 had other causes of intestinal obstruction (27 intestinal atresia, 7 malrotation, and 2 Meckel's diverticulum). The male:female ratio in Cohort A and B was 4.66:1 and 2.6:1, respectively. The median age of presentation in Cohort A and B was 7 days and 4 days, respectively. All patients in Cohort A presented with abdominal distension and delayed passage of meconium. In Cohort B, bilious vomiting was the chief complaint. There were no associated congenital anomalies in Cohort A, whereas 22% of the patients in Cohort B had associated anomalies. There was no mortality in Cohort A, while there was 27% mortality in Cohort B. Conclusion: HD is an important cause of neonatal intestinal obstruction. HD is not usually associated with other congenital anomalies. It has excellent survival in comparison to other causes of neonatal intestinal obstruction.