Qian Y L, Sun Y G, Su W C, Xin J F, Chang K, Xia S, Shen W B
Department of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing100038, China.
Zhonghua Wai Ke Za Zhi. 2024 Dec 1;62(12):1150-1156. doi: 10.3760/cma.j.cn112139-20240620-00307.
To investigate the clinical features of primary intestinal lymphangiectasia (PIL). This study was a retrospective case series study. Fifty consecutive patients diagnosed with PIL in Department of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University from March 2019 to March 2021 were included and their clinical data was retrospectively reviewed. There were 20 males and 30 females included, with an age of ((IQR)) 14 (40) years (range:0 to 67 years). No patient had the family history. There were 26 children, including 9 males and 17 females, aged 0 (7) years (range:0 to 14 years). There were 24 adults, including 11 males and 13 females, aged 40 (26) years (range:20 to 67 years). The clinical manifestations and the results of laboratory examinations, gastrointestinal endoscopy, Tc-labeled human albumin (Tc-HSA) scintigraphy, Tc-DX scintigraphy, direct lymphangiography (DLG), histopathology, diet treatment, surgical intervention, and clinical symptom remission at discharge were collected. Comparisons between groups were performed using independent samples -test, Mann-Whitney test, or test. Among the 50 cases of PIL, the main manifestations were edema (86.0%), diarrhea (76.0%), and abdominal effusion (48.0%). Lymphedema (36.0%) and chylous ascites (18.0%) were not rare in PIL patients. In Tc-HAS scintigraphy, 95.9% (47/49) cases showed signs of intestinal protein loss, and 91.7% (44/48) ceses showed positive findings in Tc-DX scintigraphy. In DLG, 97.8% (45/46) cases showed signs of thoracic duct obstruction, 82.6% (38/46) cases showed retroperitoneal lymphatic hyperplasia, and 23.9% (11/46) cases showed backflow of contrast agent into intestine. No significant difference was seen in gender, course of disease, clinical manifestation, serum level of albumin or globulin, lymphocyte count, positive rate of fecal occult blood and prevalence of lymphedema between adults and children (all >0.05). The clinical presentations of PIL between children and adults had no significant difference. The diagnosis of PIL should be made according to clinical manifestation, Tc-HAS scintigraphy, Tc-DX scintigraphy, DLG, gastrointestinal endoscopy and pathological findings.
探讨原发性肠淋巴管扩张症（PIL）的临床特征。本研究为回顾性病例系列研究。纳入2019年3月至2021年3月在首都医科大学附属北京世纪坛医院淋巴外科确诊为PIL的50例连续患者，并对其临床资料进行回顾性分析。其中男性20例，女性30例，年龄（四分位间距）为14（40）岁（范围：0至67岁）。所有患者均无家族史。儿童26例，其中男性9例，女性17例，年龄0（7）岁（范围：0至14岁）。成人24例，其中男性11例，女性13例，年龄40（26）岁（范围：20至67岁）。收集患者的临床表现、实验室检查结果、胃肠内镜检查、锝标记人血白蛋白（Tc-HSA）闪烁扫描、Tc-DX闪烁扫描、直接淋巴管造影（DLG）、组织病理学、饮食治疗、手术干预及出院时临床症状缓解情况。组间比较采用独立样本t检验、Mann-Whitney检验或χ²检验。50例PIL患者中主要表现为水肿（86.0%）、腹泻（76.0%）和腹腔积液（48.0%）。淋巴水肿（36.0%）和乳糜腹水（18.0%）在PIL患者中并不少见。在Tc-HSA闪烁扫描中，95.9%（47/49）的病例显示有肠道蛋白丢失迹象，Tc-DX闪烁扫描中91.7%（44/48）的病例呈阳性结果。在DLG中，97.8%（45/46）的病例显示胸导管阻塞迹象，82.6%（38/46）的病例显示腹膜后淋巴管增生，23.9%（11/46）的病例显示造影剂反流至肠道。成人与儿童在性别、病程、临床表现、血清白蛋白或球蛋白水平、淋巴细胞计数、粪便潜血阳性率及淋巴水肿患病率方面差异均无统计学意义（均>0.05）。儿童与成人PIL的临床表现无显著差异。PIL的诊断应根据临床表现、Tc-HSA闪烁扫描、Tc-DX闪烁扫描、DLG、胃肠内镜检查及病理结果综合判断。
