Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, CA; Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA.
Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, CA; Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA.
J Pediatr Surg. 2021 Aug;56(8):1425-1429. doi: 10.1016/j.jpedsurg.2020.10.007. Epub 2020 Oct 19.
BACKGROUND/PURPOSE: Abdominal lymphatic malformations (LM) are a rare subset of vascular anomaly caused by abnormal development of the lymphatic system. They are classified as macrocystic, microcystic or combination macrocystic and microcystic. Surgical resection, percutaneous sclerotherapy, and medical therapy are all employed to treat these complex and often symptomatic lesions. No standardized treatment algorithm exists currently. The purpose of this study was to establish a multidisciplinary treatment approach to abdominal LMs.
A retrospective observational study was conducted from 2013 to 2019 on patients with abdominal LMs at a single tertiary children's hospital vascular anomalies center. Demographics, imaging, and treatment modality were recorded. Clinical and/or radiographic response to the primary treatment modality as well as complications was the outcomes measured.
Nineteen patients (12 macrocystic, 5 microcystic and 2 combined) were identified, with a median age at diagnosis of 2.2 years (range 0.1-20.8 years). Sclerotherapy was the most common primary treatment, followed by surgical resection and sirolimus. No difference in clinical response (p = 0.58) or complications (p = 0.31) was observed based on primary treatment or subtypes.
Based on our institutional experience, we propose an LM subtype-based treatment algorithm for abdominal LMs. It employs a multidisciplinary approach, and results in satisfactory patient outcomes with minimal complications.
Level III, retrospective comparative study.
背景/目的：腹部淋巴管畸形（LM）是一种罕见的血管畸形，由淋巴系统异常发育引起。它们分为巨囊型、微囊型或巨囊型和微囊型混合。手术切除、经皮硬化治疗和药物治疗都可用于治疗这些复杂且常伴有症状的病变。目前尚无标准化的治疗方案。本研究旨在建立一种治疗腹部 LM 的多学科治疗方法。
对 2013 年至 2019 年期间在一家三级儿童医院血管畸形中心就诊的腹部 LM 患者进行回顾性观察性研究。记录患者的人口统计学、影像学和治疗方式。主要治疗方式的临床和/或影像学反应以及并发症是测量的结果。
共确定了 19 例患者（12 例巨囊型、5 例微囊型和 2 例混合型），诊断时的中位年龄为 2.2 岁（范围 0.1-20.8 岁）。硬化治疗是最常见的主要治疗方法，其次是手术切除和西罗莫司。基于主要治疗或亚型，临床反应（p=0.58）或并发症（p=0.31）无差异。
根据我们的机构经验，我们提出了一种基于 LM 亚型的腹部 LM 治疗方案。它采用多学科方法，结果令人满意，并发症最小。
III 级，回顾性比较研究。
