[A neonate with aganglionosis of the entire colon]. / Een neonaat met aganglionose van het gehele colon.

ABSTRACT

BACKGROUND: 1 in every 5,000 children is born with Hirschsprung's disease (HD). Total colonic aganglionosis is an extended form of HD and is present in 2-13% of all patients with the disease. CASE DESCRIPTION Paediatricians from a general hospital referred a 12-day-old male neonate on account of suspected abdominal obstruction and sepsis. An ileostomy was created and biopsies were taken during a laparotomy. Examination of the biopsies indicated total colonic aganglionosis. This diagnosis was unexpected because the symptoms of obstruction had occurred late and the radiological findings were not characteristic of Hirschsprung's disease. CONCLUSION: Persistent passage problems after delayed meconium passage are important alarm symptoms of Hirschsprung's disease. This diagnosis must be considered and ruled out in these patients in order to avoid an acute presentation of this disease.