성인에서 발생한 대장의 분절형 저신경절증 3예: 증례 보고 및 국내 문헌 고찰

Abstract

Hypoganglionosis is a rare form of intestinal neuronal disorder which is characterized by reduced number and size of ganglion cells in the intestinal wall occurring usually in childhood. Pathophysiology is not yet known but intestinal inflammation, ischemia, neurotoxin, or autoimmune mechanism may play a role in degeneration or reduction of ganglion cells in submucosal or myenteric plexus in the intestinal wall. Hypganglionosis in adults is even less clarified and defined. Clinically patients present with constipation, severe abdominal distension, or intestinal pseudo-obstruction. However, there are no demonstrable obstructive lesions in abdomen CT and other bowel studies. We report three cases of colonic segmental hypoganglionosis in adult patients who were treated successfully by surgical resection with a review of previously reported hypoganglionosis cases in Korean literatures.