A Case of Hirschsprung Disease With Segmental Dilatation of Intestine Diagnosed in Newborn

Abstract

Segmental dilatation of intestine (SDI) is a relatively rare disease that was first reported by Swenson in 1959 and has since been reported in various disease forms and concepts. We report a case of SDI with Hirschsprung disease in a 0-day-old girl. Her gestational age was 38 weeks and 6 days, and her birth weight was 2,396 g. After birth, the baby was transferred to our hospital because of respiratory distress. She had a defecation disorder; roentgenography showed an abnormal distribution of intestinal gas indicative of a pelvic mass, and enema examination showed marked dilatation of the sigmoid colon and narrowing of the rectum. We suspected SDI and short-segment Hirschsprung disease. We performed rectal biopsy and colostomy at 8 days of age and laparoscopic-assisted transanal pull-through and resection of the dilated intestine at 10 months of age. After that, she was able to self-defecate. Although the treatment of SDI is excision of the lesion, the possibility of the coexistence of other diseases cannot be ruled out. Careful attention should be paid to diagnosis and surgical treatment.