小肠淋巴管扩张症15例的诊断和治疗

摘要

Objective To evaluate the diagnosis and therapy of intestinal lymphangiectasia.Methods In this study 15 patients were admitted in our hospital during recent 7 years.Clinical manifestations included hypoalbuminemia,symmetrical edema,emaciation,diarrhea and lymphopenia.Lymphangiography,lympanscintigraphy and biopsy were performed for diagnosis.Therapy conducted included conservative therapy,low-fat and medium-chain triglycerides(MCT)diet,albumin infusions,diuretics,total parenteral nutrition and octreotide.Surgical therapy ineluded thoracic duct-vein anastomasis and segmental resection.Results In this group 8 patients receiving conservative therapy were followed-up from 1.5 to 7 years(average 2.5 years).Symptoms were alleviated in 6 patients.Seven patients underwent operative therapy,among them,4 patients received thoracic duct-exterior jugular vein anastomasis and followed-up from 1 to 5 years,with symptoms mitigated in 2 patients.3 patients underwent local intestinal resection,follow-up from 1 to 3 years found one patient was cured,one was improved,and 1 patient died 3 months afterthe operation. Conclusion Intestinal lymphangiectasia is rather rare and there was no definite and effective therapy.%目的 总结小肠淋巴管扩张症的诊断和治疗经验.方法 1999-2006年收治小肠淋巴管扩张症15例.该症的临床表现为水肿、腹泻、低蛋白血症以及外周淋巴细胞降低,结合淋巴管造影、核素标记白蛋白腹部显像和病理学检查确诊.治疗方法:(1)内科治疗:低脂、中链甘油脂肪酸(MCT)饮食;支持治疗:补充白蛋白、利尿剂;重症者给予,TPN及生长抑素;(2)手术治疗:采用胸导管-颈外静脉吻合、肠切除和肠淋巴瘘切除等手术治疗.结果 内科治疗8例,随访1年6个月至7年,平均2年6个月.明显好转4例,好转2例,无效2例;手术治疗7例,胸导管-颈外静脉吻合4例,随访1～5年,明显好转2例,无效2例.对2例无效者,行肠切除1例,另1例保守治疗4年无效,行十二指肠淋巴瘘切除加腹膜后淋巴管静脉吻合,随访1年7个月治愈;肠切除术3例,随访1～3年,治愈1例,好转1例,无效1例.肝前型门静脉高压症继发小肠淋巴管扩张症行肠-腔静脉分流1例,随访2年无效.结论 小肠淋巴管扩张症是一种少见病,目前尚无确切的治疗方法.