Yang Congxia, Qiu Tong, Yang Min, Zhou Jiangyuan, Gong Xue, Yang Kaiying, Zhang Zixin, Lan Yuru, Zhang Xuepeng, Zhou Zilong, Zhang Yujia, Xiang Shanshan, Chen Siyuan, Ji Yi
Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China.
Department of Pediatric Surgery, Guangzhou Women and Children's Medical Center, National Children's Medical Center for South Central Region, Guangzhou Medical University, Guangzhou, China.
J Vasc Surg Venous Lymphat Disord. 2025 Jan;13(1):101969. doi: 10.1016/j.jvsv.2024.101969. Epub 2024 Sep 19.
The diagnosis of abdominal lymphatic malformations (ALMs) is often overlooked in clinical practice. However, reports in the literature about ALMs are limited to case reports and series with small sample sizes. This study aimed to review our currently available data to describe the clinical characteristics of ALMs and evaluate the risk factors for acute abdomen caused by ALMs.
We reviewed the records of patients with ALMs who were diagnosed between December 2008 and January 2023 in our institution. The associations between acute abdomen and ALMs were analyzed based on single-factor and multivariate logistic regression analyses.
This study included 345 patients with pathologically confirmed ALMs, with a slight female predominance of 1:1.4. Approximately 39.1% (135/345) of patients were asymptomatic, and 24.6% (85/345) presented with acute abdomen. Among the ALMs in the cohort, 42.6% (147/345) were retroperitoneal lymphatic malformations (LMs). The maximal lesion dimensions in patients with acute abdomen and nonacute abdomen were 10.0 cm and 7.8 cm, respectively, with no significant difference based on multivariate analyses. Children were more likely to develop acute abdomen than adults were (P = .002; odds ratio, 5.128; 95% confidence interval, 1.835-14.326). ALMs accompanying acute abdomen were more common for lesions involving the small intestinal mesentery (P = .023; odds ratio, 2.926; 95% confidence interval, 1.157-7.400).
ALMs are rare with an insidious onset, and retroperitoneal LMs are the most common ALMs, followed by jejunal mesenteric LMs. Our retrospective analysis suggested that young age and small intestinal mesenteric lymphatic malformation are independent risk factors for acute abdomen with ALMs.
腹部淋巴管畸形（ALMs）的诊断在临床实践中常被忽视。然而，文献中关于ALMs的报道仅限于病例报告和小样本系列研究。本研究旨在回顾我们现有的数据，以描述ALMs的临床特征，并评估由ALMs引起急腹症的危险因素。
我们回顾了2008年12月至2023年1月期间在我院诊断为ALMs的患者记录。基于单因素和多因素逻辑回归分析，分析急腹症与ALMs之间的关联。
本研究纳入了345例经病理证实的ALMs患者，女性略占优势，男女比例为1:1.4。约39.1%（135/345）的患者无症状，24.6%（85/345）表现为急腹症。在该队列的ALMs中，42.6%（147/345）为腹膜后淋巴管畸形（LMs）。急腹症患者和非急腹症患者的最大病变尺寸分别为10.0 cm和7.8 cm，多因素分析显示无显著差异。儿童比成人更易发生急腹症（P = 0.002；比值比，5.128；95%置信区间，1.835 - 14.326）。累及小肠系膜的病变伴随急腹症的ALMs更为常见（P = 0.023；比值比，2.926；95%置信区间，1.157 - 7.400）。
ALMs罕见，起病隐匿，腹膜后LMs是最常见的ALMs，其次是空肠系膜LMs。我们的回顾性分析表明，年龄小和小肠系膜淋巴管畸形是ALMs并发急腹症的独立危险因素。
