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先天性高气道梗阻综合征胎儿喉手术再通的胎儿镜激光消融技术探讨

A Technical Look at Fetoscopic Laser Ablation for Fetal Laryngeal Surgical Recanalization in Congenital High Airway Obstruction Syndrome.

作者信息

Peiro Jose L, Nolan Heather R, Alhajjat Amir, Diaz Ramiro, Gil-Guevara Enrique, Tabbah Sammy M, Lim Foong Y

机构信息

Cincinnati Fetal Center, Cincinnati Children's Hospital Medical Center (CCHMC), Cincinnati, Ohio, USA.

出版信息

J Laparoendosc Adv Surg Tech A. 2020 Jun;30(6):695-700. doi: 10.1089/lap.2019.0808. Epub 2020 Apr 29.

Abstract

Congenital high airway obstruction syndrome (CHAOS) is a rare condition characterized by complete obstruction of the upper fetal airways. Left untreated, it is uniformly fatal. intrapartum treatment (EXIT) has been used to establish a surgical airway in affected fetuses during delivery. While this procedure benefits those fetuses that survive to delivery, high mortality in the prenatal period necessitates earlier innovative strategies. Herein, we report a novel technique for intervention. A fetoscopic intervention was performed at 28 weeks on a 35-year-old G1P0 woman with fetal CHAOS from a laryngeal obstruction measuring 11 mm in length on prenatal imaging. Under ultrasound guidance, a 3.3-mm curved fetoscope was used to access the uterine cavity through a single subcentimeter maternal skin incision. The scope was driven through the fetal oral cavity and manipulated to attain a view of the vocal cords. A subglottic obstruction was observed. A 600-micron laser fiber was passed through the working channel of the scope and used to ablate the obstructed airway. Using the laser fiber and a guidewire, the ablated opening was traversed with the fetoscope to the level of the carina. Postoperatively, the lungs became less hyperinflated. There was improvement in ascites and diaphragmatic eversion. At 31 1/7 weeks' gestation, the mother experienced preterm premature rupture of membranes with active labor and the fetus was delivered through EXIT to tracheostomy. The infant was managed on mechanical ventilation and is currently thriving at home with a tracheostomy at 2 years of age. Fetoscopy with laser ablation of the airway obstruction is an effective prenatal management strategy that offers the potential to alter the devastating natural course of CHAOS.

摘要

先天性高气道梗阻综合征(CHAOS)是一种罕见疾病,其特征为胎儿上呼吸道完全梗阻。若不治疗,通常会致命。产时治疗(EXIT)已被用于在分娩期间为受影响的胎儿建立手术气道。虽然该手术使那些存活至分娩的胎儿受益,但产前高死亡率需要更早的创新策略。在此,我们报告一种新的干预技术。对一名35岁、孕1产0、胎儿患有CHAOS的女性在孕28周时进行了胎儿镜干预,产前影像显示其喉部梗阻长度为11毫米。在超声引导下,使用一根3.3毫米的弯曲胎儿镜通过一个小于1厘米的母体皮肤切口进入子宫腔。将内镜经胎儿口腔推进并操作以观察声带。观察到声门下梗阻。一根600微米的激光光纤通过内镜的工作通道,用于消融梗阻气道。使用激光光纤和导丝,将消融后的开口用胎儿镜穿过至隆突水平。术后,肺部过度膨胀减轻。腹水和膈肌外翻情况有所改善。在妊娠31又1/7周时,母亲出现胎膜早破且伴有规律宫缩,胎儿通过EXIT至气管造口术分娩。婴儿接受机械通气治疗,目前2岁,在家中带气管造口管健康成长。胎儿镜下激光消融气道梗阻是一种有效的产前管理策略,有可能改变CHAOS的灾难性自然病程。

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