Hirschsprung’s disease in twin to twin transfusion syndrome: A case report

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.