Hirschsprung's disease: problems of diagnosis and treatment

Due to its varied mode of presentation, Hirschsprung's disease continues to be a diagnostic problem. To identify features that might facilitate diagnosis a retrospective review of 37 patients seen was carried out over a nine-year period. The usual presentation in neonates was failure to pass first meconium, or its delayed passage associated with abdominal distention: in a few of these the distension was accompanied by persistent non-specific diarrhoea. In many of the older children, the distension was accompanied by progressive constipation with or without episodic diarrhoea, usually following delayed passage of first meconium. The classical textbook findings on rectal examination and following barium enema were absent in most of the patients and especially in the neonates. Infection and malnutrition were identified as two of the main causes of a high post-operative mortality of 35%.