Ileocolic intussusception: an unusual complication in a newborn with intestinal neuronal dysplasia

Abstract

Intestinal neuronal dysplasia type B (IND B) represents a congenital malformation of the enteric nervous system causing disorders of intestinal motility, e. g., chronic constipation. We report a newborn who primarily suffered from intussusception and peritonitis. He required a subtotal colectomy for gangrene, but since IND B had not been expected at this time, no specific immunhistochemical workup for IND was initiated. Following recurrent episodes of ileus and subileus within the next years, colonic biopsies were taken and histotopochemical staining revealed IND B. The remaining colon required resection; an ileorectostomy was performed and the patient is now asymptomatic. This case report discusses the causality of IND B for intussusception and stresses that in newborn patients the clinical presentation may be misleading, and adequate histochemical evaluation is essential for early detection.