小肠淋巴管扩张症1例报告并文献复习

Intestinal lymphangiectasia in children: one case report and literature review

目的探讨小肠淋巴管扩张症的诊断方法及治疗。方法分析1例小肠淋巴管扩张症患儿的病史、临床和病理检查结果,以及随访观察半年后复查的各项相关指标,同时复习相关文献。结果小肠淋巴管扩张症确诊依据病理检查,内镜下典型表现也可以诊断,胶囊胃镜、双气囊小肠镜、胃镜及结肠镜、腹部CT、小肠造影、粪便α1抗胰蛋白酶清除率测定等均有诊断意义。治疗上主张控制饮食,给予低脂、高蛋白、补充中链脂肪酸的饮食。该患儿经控制饮食治疗6个月、1年后临床症状、体征好转,部分实验室检查指标亦好转,但随访6个月时镜下小肠改变不明显。结论双气囊小肠镜并病理检查是诊断小肠淋巴管扩张症的最佳方法,胃镜结合结肠镜或小肠造影在一定程度上对诊断有帮助。可用低脂、高蛋白、补充中链脂肪酸的饮食疗法,其治疗是一个长期连续的过程,可能与小肠黏膜修复慢有关。

Objective To explore diagnostic methods and treatments for intestinal lymphangiectasia. Methods The medi- cal history, clinical and pathological findings and the related findings from reexamination at 6-month follow-up in the children with intestinal lymphangiectasia were analyzed, and the related literatures were reviewed. Results The diagnosis of intestinal lymphangiectasia was based on the pathological examination and typical endoscopic manifestation. It was helpful in the diagno- sis for patient to undergo capsule endoscope, double balloon endoscope, gastrointestinal endoscope, abdominal CT scan, small bowel follow through, and stool alpha-l-antitrypsin clearence rate, etc. Diet control was proposed as a treatment for the children. Low fat, high protein diets as well as supplements of medium-chain triglycerides were beneficial. Clinical signs and symptoms and part of the laboratory indexes improved after diet control for 6 months and one year, except the endoscopic manifestation at 6-month follow-up. Conclusions Double ballon endoscope is the best choice in diagnosis of intestinal lymphagiectasia. Gastro- intestinal endoscope or small bowel follow through are also useful for the diagnosis. Diet therapy with low fat, high protein and mediun-chain triglycerides is a long-term continuous process due to slow repair of intestinal mucosal.