Zerin J M, Kuhn-Fulton J, White S J, Chong S K, Stevens J C, West K W, Teitelbaum D H, Nasr S Z
Department of Radiology, Indiana University Medical Center, Riley Hospital for Children, Indianapolis 46202-2920.
Radiology. 1995 Jan;194(1):223-6. doi: 10.1148/radiology.194.1.7997557.
To determine the radiographic, clinical, surgical, and histologic findings in children with cystic fibrosis who develop strictures of the colon.
Ten children (five boys, five girls; age range, 2.5-9.0 years; mean age, 5.5 years), who were treated at the practices of the authors, were retrospectively identified and their medical records reviewed.
Radiographic manifestations of the colonic disease included mucosal irregularity and spiculation with nodular thickening of the colonic wall and loss of normal colonic haustration. Luminal narrowing involved long segments of the colon. Longitudinal shortening of the colon was also a prominent feature. The decrease in caliber of the bowel ranged from mild narrowing to complete occlusion of the lumen. Histologic examination revealed severe submucosal fibrosis and fatty infiltration with transmural extension of the fibrosis to involve the serosa in some cases. Unlike in Crohn disease, however, acute inflammatory changes were minimal or absent.
Colonic stricture in children with cystic fibrosis is due to irreversible and frequently progressive narrowing of the colonic lumen.
确定患有囊性纤维化且出现结肠狭窄的儿童的影像学、临床、手术及组织学表现。
回顾性分析在作者所在医疗机构接受治疗的10名儿童（5名男孩，5名女孩；年龄范围2.5 - 9.0岁，平均年龄5.5岁）的病历资料。
结肠疾病的影像学表现包括黏膜不规则、毛刺样改变、结肠壁结节状增厚以及正常结肠袋消失。管腔狭窄累及结肠长段。结肠纵向缩短也是一个显著特征。肠管管径缩小范围从轻度狭窄到管腔完全闭塞。组织学检查显示严重的黏膜下纤维化和脂肪浸润，部分病例纤维化可透壁延伸至浆膜层。然而，与克罗恩病不同的是，急性炎症改变轻微或不存在。
囊性纤维化患儿的结肠狭窄是由于结肠管腔不可逆且常呈进行性狭窄所致。
