小肠淋巴管扩张症15例的诊断和治疗

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.

The diagnosis and therapy of intestinal lymphangiectasia,report of 15 cases

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.