Abstract: Objective　To investigate the clinical manifestations，diagnosis，treatment and prognosis of intestinal lymphangiectasia（IL） in order to improve the levels of diagnosis and treatment of IL in children. Methods　The clinical data，laboratory examinations，gastroenteroscopic findings，histopathological examinations and other results of 18 children with IL were analyzed retrospectively，who were diagnosed in the Department of Gastroenterology of Guangzhou Women and Children’s Medical Center from January 2010 to December 2019. Compare the related indicators between the children with onset age within 1 year old and those over 1 year old. Results　Among 18 children with IL，there were 8 boys and 10 girls；there were 13（72.2%） children with the onset age younger than 1 year old，while 5（27.8%） children with onset over 1 year old；the main clinical manifestations of IL were edema（17，94.4%），abdominal distention（16，88.8%），diarrhea（15，83.8%），ascites（16，88.8%） and being complicated with infection（14，77.7%）. There was no significant difference between the two groups in gender，lymphocyte absolute count，serum albumin，globulin，IgG or positive histopathology（P＞0.0 5）. However，there was significant difference between the two groups in terms of infection rate（χ2＝4.11，P＜0.05）. One case（5.6%） was lost in follow-up，and 15 cases（83.3%） had good prognosis. The diet rich in medium-chain triglyceride（MCT） was effective. Two cases needed supplementation with the serum albumin irregularly. Conclusion　The main clinical manifestations of IL are edema，abdominal distention and diarrhea，which is likely to be combined with ascites and infection；the younger the onset age，the more susceptible to be infected and the higher the risk of critical illness. The diet rich in MCT is effective and should be in long-term maintenance；a small number of children need irregular supplementation with the serum albumin. Long term follow-up is needed for future prognosis.

Key words: lymphangiectasia, intestine, child, clinical analysis

摘要： 目的　探讨儿童小肠淋巴管扩张症（intestinal lymphangiectasia， IL）的临床表现、 诊断、 治疗和预后， 提高对儿童IL的认识和诊治水平。方法　回顾性分析2010年1月至2019年12月在广州市妇女儿童医疗中心消化科诊断IL 18例患儿临床资料、 实验室检查、 胃肠镜、 组织病理学等结果， 比较1岁内起病与1岁以上起病IL患儿的相关指标差异。结果　18例IL患儿中男8例， 女10例。起病年龄＜1岁13例（72.2%）， 起病年龄≥1岁5例（27.8%）；IL的临床表现主要为水肿（17例， 94.4%）、腹胀（16例， 88.8%）、腹泻（15例， 83.3%），腹腔积液（16例， 88.8%）， 合并感染（14例， 77.7%）。起病年龄＜1岁和≥1岁两组比较， 在性别分布、 淋巴细胞绝对计数、 血清白蛋白、 球蛋白、 IgG、 组织病理阳性方面差异无统计学意义（P均＞0.05）， 在合并感染方面差异有统计学意义（χ2＝4.11，P＜0.05）。18例中有1例（5.6%）失访； 15例（83.3%）预后良好， 富含中链甘油三酯（medium-chain triglyceride， MCT）饮食治疗有效； 另2例（11.1%）需不定期静脉补充白蛋白。结论　儿童小肠淋巴管扩张症以水肿、 腹胀、 腹泻为主要临床表现， 易出现腹腔积液并合并感染； 起病年龄越小， 越容易罹患感染， 甚至发展成危重症； 高MCT饮食治疗有效， 需长期维持， 部分患儿需不定期补充静脉白蛋白， 远期预后需长期观察随访。

关键词: 淋巴管扩张, 小肠, 儿童, 临床分析