Pediatr Surg Int (1994) 9:501-502 © Springer-Verlag 1994 Neonatal intestinal perforation in Hirschsprung's disease R. Surana, F. M. J. Quinn, and P. Puri Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, Dublin 12, Ireland Accepted 15 February 1994 Abstract. Over a period of 18 years, 77 of 135 patients treated for Hirschsprung's disease (HD) presented in the neonatal period. Of these 77 patients, 8 had gastrointestinal (GI) perforations. Seven patients were born at full term and 1 at 32 weeks of gestation. Three patients had associated trisomy 21. Tl~e site of perforation included rectum in 1 patient, sigmoid in 1, descending colon in 1, transverse colon in 2, caecum in 2, and jejunum in 1. Perforations occurred in ganglionic bowel in 7 patients and in the aganglionic segment in 1. One patient died in the newborn period of overwhelming sepsis secondary to enterocolitis, and histology of the bowel confirmed HD. In 6 patients HD was confirmed on barium enema and suction rectal biopsy, and they subsequently underwent a definitive pull-through operation. The 1 patient in whom the initial barium enema was normal continued to suffer from constipation until the age of 7 years, when the diagnosis of HD was established. He then underwent a pull-through procedure with no fur- ther problems. An association between neonatal intestinal perforation and HD must therefore be recognised to avoid delay in the management. Key words: Hirschsprung's disease - Intestinal perforation - Neonate Introduction The characteristic presentation of Hirschsprung's disease (HD) in the neonatal period includes delayed passage of meconium and/or intestinal obstruction. However, occa- sionally gastrointestinal (GI) perforation may be a pre- senting feature. If the perforation occurs, it invariably oc- curs in the neonatal period [6, 11]. Failure to recognise this Correspondence to: R Purl association may lead to delay in the diagnosis and specific management [3-5, 7, 10]. This study was undertaken to identify patients with HD and GI perforations, to follow their clinical course, and to derive guidelines for the management of these patients. Materials and methods A retrospectivereview of all the patients with HD who had developed GI perforations was undertaken. All patients with HD treated over the 18 years 1975- 1992 in three children's hospitals in Dublin (Our Lady's Hospital for Sick Children, Crumlin, The Children's Hospital, Temple Street, National Children's Hospital, Harcourt Street) were included in the study. Data were collected as regards gestational age, sex, site of perforation, investigations, age at diagnosis of HD, operation, length of aganglionosis, complications, and mortality. Results Of 135 patients with HD treated during the period of study, 77 presented in the neonatal period. Eight neonates (5 boys, 3 girls) had GI perforations; 6 presented with GI perfora- tion, 1 developed it after a gastrograffin enema, and 1 de- veloped a jejunal perforation 7 days after a duodenoduo- denostomy for duodenal atresia. Seven of these 8 patients were born at term and 1 at 32 weeks of gestation; 3 also had associated trisomy 21. The age at presentation (GI per- foration) was 2 days in 2 patients, 3 days in 3, 4 days in 1, 5 days in 1, and 8 days in 1. All the patients underwent laparotomy. The site of perforation was as follows: rectum 1, sigmoid colon 1, descending colon 1, transverse colon 2, caecum 2, and jejunum 1. Seven perforations occurred in ganglionic bowel and 1 in aganglionic bowel. At laparotomy, perforations were oversewn in 6 patients. The area of perforation was exteriorised in 1 patient and 7 cm of bowel proximal and distal to the perforation was resected in 1 patient. All the patients had an enterostomy performed. Peroperative biopsies were obtained in all cases, with frozen sections available in 1. The diagnosis of