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产前诊断的颈面部淋巴管畸形的子宫外产时治疗。

Ex Utero Intrapartum Treatment for Prenatally Diagnosed Cervicofacial Lymphatic Malformations.

机构信息

Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Division of Pediatric Surgery, Department of Surgery, Texas Children's Hospital, Houston, Texas.

Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Division of Pediatric Surgery, Department of Surgery, Texas Children's Hospital, Houston, Texas.

出版信息

J Surg Res. 2024 Nov;303:628-635. doi: 10.1016/j.jss.2024.08.026. Epub 2024 Oct 21.

DOI:10.1016/j.jss.2024.08.026
PMID:39437601
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.

摘要

简介

颈部和面部的淋巴畸形(cf-LM)可能在产前超声检查中被发现,这促使人们考虑进行体外宫内分娩(EXIT)以确保胎儿的气道通畅。此外,cf-LM 的产后管理从单纯切除转变为包括西罗莫司和硬化治疗的多模式方法,这可能会影响 cf-LM 的新生儿结局。本研究旨在描述接受产前诊断为 cf-LM 并接受 EXIT 到气道的患者的新生儿结局。

方法

对 2011 年至 2020 年期间所有因 cf-LM 接受 EXIT 到气道的患者进行回顾性单中心回顾。使用描述性统计(中位数[四分位间距])分析人口统计学、产前影像学、术中细节和结局。

结果

六名产前诊断为 cf-LM 的患者在中位妊娠 36(33.8-36.9)周时接受了 EXIT 到气道。胎儿磁共振成像的中位数体积为 187.5 mL(142.3-237.8),中位数气管食管移位指数为 11 mL(9.25-15)。所有患者在胎盘支持下均成功插管,中位时间为 25(15.25-91)天。1 名患者在出生后第 10 天因坏死性小肠结肠炎总发生死亡。在幸存者中,5 名患者中有 2 名接受了气管造口术,5 名患者中有 4 名接受了胃造口术,所有患者均在出生后第 9(8-10)天行西罗莫司治疗。4 名患者在初次住院期间接受了 cf-LM 的减瘤或切除术。患者的中位住院时间为 68(45-129)天。1 名患者在 EXIT 到气道后发生气胸,有气压伤证据,需要放置胸腔引流管(持续 8 天)。

结论

EXIT 到气道手术仍然是缓解产前诊断的颈部和面部淋巴畸形引起的新生儿缺氧的可行策略。然而,产后结局是多变的,可能存在长期的呼吸道后遗症。

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