CASE REPORT Midgut malrotation and associated Hirschsprung’s disease: a diagnostic dilemma Hany O. S. Gabra Æ Richard J. Stewart Æ Shawqui Nour Accepted: 11 December 2006 / Published online: 9 January 2007 Ó Springer-Verlag 2007 Abstract Malrotation and Hirschsprung’s disease (HSD) are rarely reported together. We report our experience with three patients who presented during the neonatal period in whom the association resulted in diagnostic difficulty. In this report, we focus on the clinical presentation, diagnosis and appropriate man- agement. Keywords Midgut malrotation Á Hirschsprung’s disease Á Neonatal bowel obstruction Introduction We report our experience with three patients who presented during the neonatal period with a clinical picture of neonatal bowel obstruction. A provisional diagnosis of malrotation was made in the first two patients but at the time of surgery, an abnormally dilated left colon was detected in both cases. Intra- operative colonic biopsies confirmed the diagnosis of Hirschsprung’s disease. The third baby was confirmed to have Hirschsprung’s disease on rectal biopsies but underwent a laparotomy for persistent obstructive picture and was found to have midgut malrotation. In this report, we highlight the lessons gained from these cases. Case reports Case 1 A 5-day-old male baby was born at term with no antenatal problems. He passed meconium within 24 h of delivery and was fed normally and was passing stools normally in the first 4 days of life. He presented with 1 day history of bilious vomiting and abdominal dis- tension. After obtaining IV access, fluid resuscitation was commenced. A plain abdominal X-ray showed a picture of ‘‘non specific’’ dilated bowel loops with no gas in the rectum. Further imaging, in the form of upper gastrointestinal (GI) contrast, showed abnor- mally situated low duodeno-jejunal (DJ) junction, which was suggestive of small bowel malrotation. At laparotomy, midgut malrotation was confirmed with the DJ flexure was situated at the right side of the midline. Ladd’s procedure was performed and division of the classical bands was conducted. Inspection of the large bowel at the time of surgery, revealed a markedly dilated left colon. Biopsies were taken from the rec- tum, sigmoid and transverse colon and sent for histo- pathology. Post-operatively the results of the biopsies confirmed the diagnosis of Hirschsprung’s disease. The patient had an uneventful post-operative recovery and he was discharged on regular rectal washouts. Six weeks later, a one stage Duhamel pull through proce- dure was performed. H. O. S. Gabra Á R. J. Stewart Paediatric Surgical Unit, Nottingham University Hospitals, Queen’s Medical Centre Campus, Nottingham, UK S. Nour Paediatric Surgical Unit, Leicester Royal Infirmary, University Hospitals of Leicester, Leicester, UK H. O. S. Gabra (&) Paediatric Surgical Unit, Queen’s Medical Centre Campus, Nottingham NG7 2UH, UK e-mail: hosgabra@hotmail.com 123 Pediatr Surg Int (2007) 23:703–705 DOI 10.1007/s00383-006-1869-3