Case Report
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World J Gastroenterol. Dec 14, 2014; 20(46): 17666-17669
Published online Dec 14, 2014. doi: 10.3748/wjg.v20.i46.17666
Hirschsprung’s disease in twin to twin transfusion syndrome: A case report
Hye Won Park, Min Jeng Cho, Wook Youn Kim, Byung Ok Kwak, Min Hee Kim
Hye Won Park, Byung Ok Kwak, Min Hee Kim, Department of Pediatrics, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul 143-729, South Korea
Min Jeng Cho, Department of Surgery, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul 143-729, South Korea
Wook Youn Kim, Department of Pathology, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul 143-729, South Korea
Author contributions: Park HW drafted the manuscript and was primarily responsibility for its final content; Kwak BO and Cho MJ provided advice and reviewed the article; Kim WY provided advice and help with pathological findings; Kim MH approved the final manuscript.
Correspondence to: Min Hee Kim, MD, PhD, Department of Pediatrics, Division of Neonatology, Konkuk University Medical Center, Konkuk University School of Medicine, 4-12 Hwayang-dong, Gwangjin-gu, Seoul 143-729, South Korea. kmh@kuh.ac.kr
Telephone: +82-2-20305120 Fax: +82-2-20305122
Received: April 4, 2014
Revised: May 26, 2014
Accepted: July 11, 2014
Published online: December 14, 2014
Processing time: 258 Days and 5.3 Hours
Abstract

Twin to twin transfusion syndrome (TTTS) is caused by aberrant vascular connections between infant twins and results in high morbidity and mortality in the perinatal period. In donor infants with TTTS and symptoms of intestinal obstruction, small-bowel lesions have been reported in most cases. We report on a 33+6 gestational wk donor infant with TTTS who had intermittent obstructive episodes, including delayed meconium passage and colonic dilatation on abdominal X-ray. The diagnosis of Hirschsprung’s disease was based on a lateral pelvic film with a reversed rectosigmoid ratio. A subsequent barium colon study and rectal suction biopsy indicated a short segment aganglionosis of the colon.

Keywords: Hirschsprung’s disease; Twin to twin transfusion syndrome; Donor; Aganglionosis; Delayed meconium passage

Core tip: Twin to twin transfusion syndrome (TTTS) is caused by an aberrant vascular connection between monochorionic infant twins. Necrotizing enterocolitis, jejunal/ileal atresia, and perforation have previously been reported, but there have been no reports of Hirschsprung’s disease in a donor infant with TTTS. The intrauterine hypoxemia in the case we report here may have inhibited neuroblast cell migration or caused the destruction of ganglion cells in the gut. The patient was a donor baby with symptoms of feeding intolerance and marked colon dilatation who was suffering from Hirschsprung’s disease and TTTS.