成人発症した結腸segmental oligoganglionosisの1例

Segmental Colonic Oligoganglionosis in Adult, Report of a Case

要旨　患者は40歳，男性．発熱と腸閉塞様の症状で来院し，X線・内視鏡検査にてS状～横行結腸にわたる原因不明の拡張不良とその口側結腸の二次的拡張が認められた．症状が完治しないため外科的に拡張不良部の切除を施行し，術後の病理組織学的検査で結腸segmental oligoganglionosisと診断した．本例は原因が不明であったが，成人発症のHirschsprung病とは異なる病態と考えられ，成人大腸疾患における本症鑑別の重要性が示唆された症例である．

　A 40 year-old man with no known history of difficulty in defecation in his childhood, visited our hospital complaining of periodic severe abdominal pain, abdominal distension and fever. Plain abdominal x-ray film showed marked dilatation of the ascending and transverse colon with fluid level formation. Barium enema examination revealed sigmoid and transverse colon to be poorly distended with marked dilatation proximal to it. The mucosal pattern was almost normal in the poorly distended segment while shallow and irregular ulcers were seen in the dilated transverse colon. These findings were also confirmed by colonoscopic examination.

　The patient was clinically diagnosed as having idiopathic long segmental narrowing of the colon with chronic obstruction. As ileus symptom was not relieved by conservative therapy, surgery was perfomed, which revealed neither mechanical obstruction nor abnormal findings in other abdominal viscera. Narrow part of the colon was resected. Histological examination revealed marked decrease in number of normal ganglion cells in both Auerbach's and Meissner's plexuses in the affected part of the resected specimen. In contrast, normal ganglion cells were found in the areas proximal and distal to the involved segment. Based on these histological findings, the final diagnosis of segmental colonic oligoganglionosis was made.

　The features of this case were that symptoms appeared for the first time in his forties, that the colon was involved segmentally and that oligoganglionosis was seen in both Auerbach's and Meissner's plexuses. This case seems to differ from classical Hirschsprung's disease in adult.