Ex Utero Intrapartum Treatment for Prenatally Diagnosed Cervicofacial Lymphatic Malformations

IF 1.8 3区医学 Q2 SURGERY

Journal of Surgical Research Pub Date : 2024-10-21 DOI:10.1016/j.jss.2024.08.026

Srinithya R. Gillipelli BA , Sarah E. Peiffer MD, MPH , Shannon M. Larabee MD , Pamela Ketwaroo MD , Kristy L. Rialon MD , Joshua Bedwell MD , Deepak Mehta MD , Timothy C. Lee MD , Sundeep G. Keswani MD , Alice King MD

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引用次数: 0

Abstract

Introduction

Cervicofacial lymphatic malformations (cf-LM) may be identified on prenatal ultrasound, prompting consideration of ex utero intrapartum treatment (EXIT) to secure the fetal airway. Furthermore, the recent shift in postnatal management of cf-LM from resection alone toward a multimodal approach including sirolimus and sclerotherapy may impact the neonatal outcomes of cf-LM. This study aims to characterize the neonatal outcomes of patients with prenatally diagnosed cf-LM who underwent EXIT-to-airway.

Methods

Retrospective, single-center review of all patients who underwent EXIT-to-airway for cf-LM (2011-2020) was performed. Demographics, prenatal imaging, intraoperative details, and outcomes were analyzed using descriptive statistics (median [interquartile range]).

Results

Six patients with prenatally diagnosed cf-LM underwent EXIT-to-airway at a median gestational age of 36 (33.8-36.9) wk. The median volume on fetal magnetic resonance imaging was 187.5 mL (142.3-237.8) and median tracheoesophageal displacement index was 11 mL (9.25-15). All were successfully intubated on placental support with a median duration of 25 (15.25-91) d. There was one fatality at day of life 10 due to necrotizing enterocolitis totalis. Among survivors, 2 of 5 underwent tracheostomy placement, 4 of 5 underwent gastrostomy tubes placement, and all 5 received sirolimus at day of life of 9 [8-10] d. Four patients underwent debulking or excision of their cf-LM during the initial hospitalization. Patients had a median length of stay of 68 (45-129) d. One patient experi enced a pneumothorax with evidence of barotrauma following EXIT-to-airway requiring chest tube placement (duration 8 d).

Conclusions

EXIT-to-airway procedure remains a feasible strategy for mitigating neonatal hypoxia in cases of prenatally diagnosed cervicofacial lymphatic malformations. However, postnatal outcomes are variable with potential long-term aerodigestive sequelae.

查看原文本刊更多论文

产前诊断为颈面淋巴畸形的宫内治疗。

导言：颈面部淋巴畸形（cervicofacial lymphatic malformations，cf-LM）可能会在产前超声波检查中被发现，这就需要考虑进行宫外产前治疗（EXIT），以确保胎儿的呼吸道安全。此外，近来cf-LM的产后管理已从单纯切除转向包括西罗莫司和硬化剂治疗在内的多模式方法，这可能会影响cf-LM的新生儿预后。本研究旨在描述产前诊断为锎-胎膜早破并接受EXIT-to-气道治疗的患者的新生儿预后：方法：对所有接受EXIT-to-气道治疗的cf-LM患者（2011-2020年）进行单中心回顾性研究。采用描述性统计（中位数[四分位数间距]）对人口统计学、产前影像学、术中细节和结果进行分析：结果：六名产前诊断为 cf-LM 的患者在中位胎龄 36（33.8-36.9）周时接受了 EXIT 转气道手术。胎儿磁共振成像的中位体积为 187.5 mL（142.3-237.8），中位气管食管移位指数为 11 mL（9.25-15）。所有患儿均在胎盘支持下成功插管，中位持续时间为25（15.25-91）天。在存活者中，5 人中有 2 人接受了气管造口术，5 人中有 4 人接受了胃造瘘管置入术，所有 5 人在生命第 9 天[8-10] d 时均接受了西罗莫司治疗。一名患者在气道切开术后出现气胸并伴有气压创伤，需要放置胸管（持续时间为 8 d）：结论：对于产前诊断为颈面淋巴畸形的病例，EXIT-通气道手术仍是缓解新生儿缺氧的可行策略。结论：对于产前诊断为颈面淋巴畸形的病例，EXIT通气道手术仍是缓解新生儿缺氧的可行策略，但产后效果不一，可能会有长期的气道后遗症。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Surgical Research 医学-外科

CiteScore

3.90

自引率

4.50%

发文量

627

审稿时长

138 days

期刊介绍： The Journal of Surgical Research: Clinical and Laboratory Investigation publishes original articles concerned with clinical and laboratory investigations relevant to surgical practice and teaching. The journal emphasizes reports of clinical investigations or fundamental research bearing directly on surgical management that will be of general interest to a broad range of surgeons and surgical researchers. The articles presented need not have been the products of surgeons or of surgical laboratories. The Journal of Surgical Research also features review articles and special articles relating to educational, research, or social issues of interest to the academic surgical community.