Department of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, Netherlands; Department of Radiology and Nuclear Medicine, division of Pediatric Radiology, Radboudumc-Amalia Children's Hospital, Nijmegen, Netherlands.
Department of Radiology and Nuclear Medicine, division of Pediatric Radiology, Radboudumc-Amalia Children's Hospital, Nijmegen, Netherlands.
J Pediatr Surg. 2021 Oct;56(10):1764-1770. doi: 10.1016/j.jpedsurg.2021.03.011. Epub 2021 Mar 18.
To evaluate imaging, treatment, and outcomes in neonates with a lymphatic malformation (LM) adjacent to the airway and to evaluate risk factors that can predict outcome.
A retrospective case series was conducted of ten patients treated between January 2011 and July 2019. The main outcome measures included airway compromise and clinical response to sclerotherapy ± surgery. Categorical data were compared using the Fisher's exact test.
Ex-utero intrapartum therapy was performed in four cases, among whom one died due to sepsis. All patients underwent schlerotherapy, with surgical debulking in two. Four patients showed a good clinical response, and five started experimental systemic treatment. Patients with bilateral disease and patients with an LM with >180° tracheal surrounding were significantly at risk for airway compromise (bilateral: n = 6/6 versus n = 0/4, p = 0.005; >180°: n = 5/5 versus n = 1/5, p = 0.048). The need for LM treatment in the neonatal period was indicative of a poor clinical response ('non-responders' 5/6, 'responders' 0/4; p = 0.048).
This study indicates that bilateral disease and >180° tracheal surrounding are risk factors for airway compromise in neonates with an LM adjacent to the airway. In addition, the need for early treatment seems to be indicative of a poor clinical response.
评估毗邻气道的淋巴管畸形（LM）新生儿的影像学表现、治疗方法和结局，并评估可预测结局的风险因素。
对 2011 年 1 月至 2019 年 7 月期间治疗的 10 例患者进行了回顾性病例系列研究。主要结局指标包括气道阻塞和对硬化治疗+手术的临床反应。使用 Fisher 确切检验比较分类数据。
4 例患者进行了子宫外产时治疗，其中 1 例因败血症死亡。所有患者均接受硬化治疗，其中 2 例接受手术去瘤治疗。4 例患者的临床反应良好，5 例开始接受实验性系统治疗。双侧疾病和 LM 周围>180°气管的患者发生气道阻塞的风险显著增加（双侧：n=6/6 与 n=0/4，p=0.005；>180°：n=5/5 与 n=1/5，p=0.048）。新生儿期 LM 治疗的需要提示临床反应不良（“无反应者”5/6，“有反应者”0/4，p=0.048）。
本研究表明，双侧疾病和>180°气管周围是毗邻气道的 LM 新生儿气道阻塞的风险因素。此外，早期治疗的需要似乎预示着临床反应不良。
