Hirschsprung’s Disease in a Twin Neonate With One Suffering From Multiple Skip Segments

Razieh Sangsari; Maliheh Kadivar; Maryam Saeedi; Kayvan Mirnia; Maryam  Ghavami Adel; Moeinedin Safavi

doi:10.18502/crcp.v6i3.7128

Razieh Sangsari Department of Pediatrics, Division of Neonatology, Children’s Medical Center, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
Maliheh Kadivar Department of Pediatrics, Division of Neonatology, Children’s Medical Center, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
Maryam Saeedi Department of Pediatrics, Division of Neonatology, Children’s Medical Center, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
Kayvan Mirnia Department of Pediatrics, Division of Neonatology, Children’s Medical Center, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
Maryam Ghavami Adel Department of Surgery, Division of Pediatrics Surgery, Neonatology, Children’s Medical Center, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Moeinedin Safavi Department of Pathology, Children’s Medical Center, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.

DOI: https://doi.org/10.18502/crcp.v6i3.7128

Keywords: Hirschsprung’s disease; Frozen sections; Meconium

Abstract

This is a new case, affecting a twin that both with Hirschsprung’s disease, one was suffering from long-segment Hirschsprung’s disease with skip segmented. Our surgeon suspected the absence of abnormal vessel tortuosity in the transitional zone; thus, the appendix was sent for permanent pathology. There were ganglion cells in the colostomy site but no ganglion was found in the appendix. Complete biopsies from different parts of the intestine demonstrated positive ganglion cells in the transverse colon, ascending colon, and the distal ileum, jejunum, and duodenum, and negative ganglion cells in the rectum, sigmoid, descending colon, appendix, and 5 cm far from the ileocecal valve were observed. Therefore, the Kimura procedure was done. The most important key in such cases is the surgeons’ observation during operation.