Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, Haidian District, Beijing, China.
Department of Gastroenterology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Orphanet J Rare Dis. 2023 Oct 2;18(1):308. doi: 10.1186/s13023-023-02914-z.
Intestinal lymphangiectasia (IL) is a rare protein-losing enteropathy caused by disorders of the intestinal lymphatics. There are only a few case reports and case series concerning the VCE (video capsule endoscopy) findings of IL. This work aimed to evaluate the VCE characteristics of small intestinal mucosal abnormalities in patients with IL, and to investigate the relationship between clinical and VCE characteristics.
Consecutive patients with IL who underwent VCE were enrolled in this retrospective study. The cases were classified into the white villi group and non-white villi group according to mucosal abnormalities detected by VCE. Clinical and endoscopic characteristics were investigated and analyzed.
A total of 98 patients with IL with a median onset age of 26.3 ± 19.2 years were included. VCE revealed the following small intestinal lesions: (i) white villi type (57/98, 58.2%), i.e.: white-tipped or granular villi, white nodular villi or plaques; (ii) non-white villi type (41/98, 41.8%), i.e.: diffused low and round villi; (iii) complications (46/98, 46.9%), i.e.: bleeding, ulcers, protruding or vesicular-shaped lesions, stenosis and lymphatic leakage. A total of 58.2% (57) and 41.8% (41) of the cases were classified into the white villi and non-white villi groups respectively. The percentage of chylothorax in the white villi group was significantly lower than that in the non-white villi group (12/57 vs. 19/41, p = 0.008). In VCE, there were no significant differences in the involved segments and total detected rate of complications between the white villi and non-white villi groups (p > 0.05), while the detected rate of lymphatic leakage in the white villi group was significantly higher than that in the non-white villi group (31.6% vs. 12.2%, p = 0.026).
Our study evaluated the entire small intestinal mucosal abnormalities of IL by VCE, especially endoscopic complications. IL has specific VCE abnormalities in addition to classical endoscopic findings.
肠淋巴管扩张症（IL）是一种罕见的蛋白丢失性肠病，由肠淋巴管紊乱引起。仅有少数关于 IL 视频胶囊内镜（VCE）检查结果的病例报告和病例系列。本研究旨在评估 IL 患者小肠黏膜异常的 VCE 特征，并探讨临床与 VCE 特征之间的关系。
连续纳入接受 VCE 检查的 IL 患者，回顾性分析其临床及内镜资料。根据 VCE 检查发现的黏膜病变，将病例分为白苔组和非白苔组。
共纳入 98 例 IL 患者，中位发病年龄为 26.3±19.2 岁。VCE 显示小肠病变如下：（i）白苔型（57/98，58.2%），即：白尖或颗粒状绒毛、白结节状绒毛或斑块；（ii）非白苔型（41/98，41.8%），即：弥漫性低而圆的绒毛；（iii）并发症（46/98，46.9%），即：出血、溃疡、突出或疱疹样病变、狭窄和淋巴漏。58.2%（57）和 41.8%（41）的病例分别归入白苔组和非白苔组。白苔组乳糜胸的发生率明显低于非白苔组（12/57 比 19/41，p=0.008）。VCE 中，两组受累肠段及并发症总检出率无显著差异（p>0.05），但白苔组淋巴漏的检出率明显高于非白苔组（31.6%比 12.2%，p=0.026）。
本研究通过 VCE 评估了 IL 整个小肠黏膜异常，特别是内镜并发症。IL 除了具有经典的内镜表现外，还有其特定的 VCE 异常。
