儿童小肠淋巴管扩张症消化内镜特点及临床15例分析

Gastrointestinal endoscopic characteristics and clinical analysis of 15 children with intestinal lymphangiectasia

目的提高对儿童小肠淋巴管扩张症(IL)临床、内镜及病理特点的认识,减少误诊。方法回顾性分析2006年3月～2009年3月该院收治年龄3～16岁,15例小肠淋巴管扩张症临床表现、实验室检查、消化内镜特点及其病理结果。结果消化内镜特点:8例消化内镜发现十二指肠黏膜白色点状或小斑块(胃镜6例,小肠镜2例),其中2例发现病变附近有肿块或不规则溃疡,同时结肠镜发现回肠末端局部黏膜息肉样隆起;7例小肠镜发现空回肠黏膜白色粟米样改变,6例病变附近见肿块或不规则溃疡。病理特点:8例十二指肠黏膜慢性炎症伴淋巴管扩张(其中2例回肠末端黏膜慢性炎症,伴淋巴滤泡增生,淋巴管扩张),十二指肠非霍奇金淋巴瘤、间质瘤各1例;7例空回肠黏膜慢性炎症,伴淋巴管扩张,回肠非霍奇金淋巴瘤、小肠淋巴管瘤、小肠间质瘤、回肠绒毛状腺癌、炎性病变并肌纤维母细胞瘤样增生及Cohn's病各1例。结论有血浆蛋白与球蛋白同时下降的浮肿患儿,伴或不伴淋巴细胞减少,要重点怀疑IL,行消化内镜及病理检查明确诊断,并尽量寻找其原发病因。

[ Objective ] To analyse the clinical and gastrointestinal endoscopic features, and pathological results of intestinal lymphangieetasia in 15 children cases. [Methods] The clinical manifestations, laboratory examinations, gastrointestinal endoscopic features and pathological results were retrospectively analysed in 15 cases of intestinal lymphang/ectasia who were admitted into our hospital from March 2006 to March 2009 and aged 3 - 16 years. [ Results ] Digestive endoscopy revealed small white spots or patches of duodenal mucosa in eight cases [gastroseopy for six cases, double balloon enteroscopy （DBE） for two cases], among which two cases had mass or irregular ulcer near pathological lesions, at the same time colonoscopy disclosed polypoid bulge in local ileal mucosa. White miliary lesions were found by DBE in the jejunum of seven cases, and mass lesions or irregular ulcers were seen near the pathological changes in six cases. Pathological diagnosis confirmed 8 eases of chronic inflammation of duodenal mucosa with lymphangieetasia, 1 case of duodenal non-Hodgkin＇s lymphoma, and 1 case of mesenchymal; 7 cases of chronic inflammatory of jejunal mueosa with lymphangiectasia, ileal non-Hodgkin＇s lymphoma, 1 lymphangioma of the small intestine, 1 small intestine stromal tumor, 1 ileum villous adenocarcinoma, 1 inflammatory lesion and myofibroblastoma hyperplasia, and 1 Cohn＇s disease. [Conclusions] For edematous children with simultaneous decrease in plasma protein and globulin, with or without reduction in lymphocytes, IL should be suspected. Gastrointestinal endoscopy, biopsy and pathological examination should be applied as early as possible to find the primary diseases.