小肠淋巴管扩张症的诊断与治疗

目的提高对小肠淋巴管扩张症(IL)的诊治水平。方法回顾性分析我院近20年收治12例IL患者的临床、实验室、影像和病理资料。结果男7例,女5例,平均年龄40.2岁。患者以腹泻、浮肿、浆膜腔积液为主要表现。其实验室检查以淋巴细胞减少,白、球蛋白减少为主要表现。内镜主要表现为小肠黏膜弥漫性分布小白点、小息肉样改变,其它如消化道造影、淋巴管造影均有一定阳性表现。所有患者的内镜下小肠黏膜活检病理均提示不同部位的小肠淋巴管扩张。治疗上以对症支持、低脂、中链脂肪酸饮食为主。部分继发性IL通过根治原发病,或针对病变部位进行局部手术切除病变肠段,或淋巴管、静脉吻合术等使病情得到缓解。结论对于浮肿、腹泻、腹腔积液的患者,同时有明显的低蛋白血症、低淋巴细胞血症,应高度怀疑IL,若活检证实淋巴管扩张,则可诊断淋巴管扩张。治疗可给予对症支持、低脂、MCT饮食,有部分患者可尝试进行手术治疗。

The Diagnosis and Treatment of Intestinal Lymphangiectas

ObjectiveTo improve the identification of intestinal lymphangiectasia(IL).Methods12 cases of IL treated in our hospital since 1986 were studied and the clinical,laboratory data,and pathological findings were analyzed.ResultsThe sex ratio of male to female was 7 to 5,the mean age in this series of 12 patients was 40.2 years.All patients presented with diarrhea,edema and chylous effusions.The principal laboratory findings in these patients were hypoproteinemia and lymphocytopenia.Endoscope revealed dilated lacteals that appears as white opaques spots or white-tipped villi,and nodular lesions in almost patients.Lymphangiography and small bowel X-ray examinations were abnormal in the majority.The diagnosis was established by biopsy of the intestinal mucosa,which demonstrates dilated mucosal and submucosal lymphatic channels.The mainstay of treatment for IL was correcting the underlying disease responsible for the IL,reintroduction of a low-fat,MCT-supplemented diet.ConclusionIL should be considered if any patient with edema and diarrhea also has hypoproteinemia and lymphocytopenia.A diagnosis of IL rests on jejunal biopsy demonstrating dilated lymphatic lacteals.The therapy of IL should be support treatment,reintroduction of a low-fat,MCT-supplemented diet.