目的 分析婴儿原发性小肠淋巴管扩张症(PIL)的临床特征以及治疗方法，提高临床诊治水平。方法 回顾性分析2012年10月至2013年12月北京世纪坛医院淋巴外科收治的4例婴儿PIL的临床表现、实验室检查、核素显像检查以及治疗方法，通过长期随访结果对婴儿期PIL诊治进行总结。结果 患儿年龄4~9个月，均为女性；均以腹泻为首发症状，伴下肢对称性水肿，3例合并呼吸道感染；实验室检查血淋巴细胞绝对值、白蛋白、球蛋白降低，有轻度贫血，低钙血症、铁缺乏；放射性核素显像提示有肠道蛋白丢失。4例患儿采取保肝、补蛋白、利尿等治疗，并予以全胃肠外营养治疗3~4周，出院后个体化的中链三酰甘油（MCT）饮食治疗3~30个月。随访10年，4例患儿恢复正常饮食，复查血白蛋白正常，未再复发。结论 婴儿PIL少见，临床表现以腹泻、水肿为主，常合并呼吸道感染，电解质紊乱以低钙血症、铁缺乏较为常见。对于疑诊为PIL的婴儿，放射性核素检查可作为确诊的首选方法。足疗程的全胃肠外营养治疗配合个体化的MCT饮食是婴儿PIL的有效治疗手段。

Objective To analyze the clinical characteristics and treatment methods of primary intestinal lymphangiectasia (PIL) in infants and to improve clinical diagnosis and treatment. Methods A retrospective analysis was conducted on the clinical manifestations, laboratory tests, nuclear imaging examinations and treatment methods of four cases of PIL in infants admitted to the Department of Lymphatic Surgery at Beijing Shijitan Hospital from October 2012 to December 2013. The diagnosis and treatment of infantile PIL were summarized based on long term follow-up results. Results The patients aged 4-9 months and are all female. All 4 patients presented with diarrhea as the initial symptom, accompanied by symmetrical edema of the lower limbs, and 3 cases were complicated with respiratory infections. Laboratory tests showed a decrease in absolute values of blood lymphocytes, albumin, and globulin. Four patients presented with mild anemia, hypocalcemia, and iron deficiency. Radionuclide imaging indicated loss of intestinal proteins. Four patients were treated with liver protection, protein supplementation, dieresis and total parenteral nutrition for 3-4 weeks. After discharge, personalized medium chain triglycerides (MCT) dietary treatment was administered for 3-30 months. Following up for 10 years, 4 patients resumed normal diet and had normal blood albumin levels rechecked, with no recurrence. Conclusions Infant PIL is relatively rare, with clinical manifestations mainly characterized by diarrhea and edema, it is often accompanied by respiratory infections. Electrolyte disorders, such as hypocalcemia and iron deficiency, are more common. In infant patients suspected of PIL, radioactive nuclide testing may serve as the first choice for definitive diagnosis A sufficient course of total parenteral nutrition in conjunction with personalized MCT diet is an effective treatment for PIL in infants.