Fetal Therapy Unit "U. Nicolini," Buzzi Children's Hospital, University of Milan, Milan, Italy.
Department of Women, Mother and Neonate, Buzzi Children's Hospital, University of Milan, Milan, Italy.
Fetal Diagn Ther. 2023;50(2):115-120. doi: 10.1159/000529334. Epub 2023 Jan 27.
We aimed to identify maternal and fetal complications and investigate postnatal and long-term outcomes of fetal hydrothorax (FHT) treated with pleuro-amniotic shunting (shunt).
Single-center retrospective observational cohort of shunt cases performed from 2000 to 2021. Risk factors for maternal complications, fetal demise, neonatal death (NND), and postnatal outcomes were identified.
Out of 88 cases, 70 (79.5%) were complicated by hydrops, with an average gestational age (GA) at diagnosis of 27 weeks (range 16-34). In 16 cases, definitive etiology of FHT was identified; five cases of Noonan syndrome and three cases of monogenic disorders diagnosed by whole-exome sequencing (EPHB4, VEGFR3, RASA1). Shunt was performed at an average GA of 28 weeks (20-34), with a dislodgement in 10 cases (11.4%). Maternal: Complications occurred in three cases; survival rate was 76.1% (67/88). Follow-up data were available for 57/67 (85.1%) children. Incidence of severe neurodevelopmental impairment and pneumopathy (broncho dysplasia, persistent pulmonary hypertension of newborn, and asthma) was 5.3% and 8.8%, respectively. Post-treatment persistence of hydrops, FHT associated with genetic syndromes, and GA at birth were risk factors for fetal demise, NND, and postnatal complications.
In truly isolated FHT, whenever indicated, pleuro-amniotic shunting is a safe procedure associated with good survival rate and long-term outcome.
我们旨在确定胎儿胸腔积液（FHT）治疗中胸腔-羊膜分流（分流）的母体和胎儿并发症，并调查产后和长期结局。
对 2000 年至 2021 年进行的分流病例进行单中心回顾性观察队列研究。确定母体并发症、胎儿死亡、新生儿死亡（NND）和产后结局的危险因素。
88 例中，70 例（79.5%）合并胎儿水肿，诊断时平均胎龄（GA）为 27 周（范围 16-34）。在 16 例中，明确了 FHT 的明确病因；5 例 Noonan 综合征和 3 例通过全外显子组测序诊断的单基因疾病（EPHB4、VEGFR3、RASA1）。分流在平均 GA 为 28 周（20-34）时进行，10 例（11.4%）发生移位。母体：有 3 例发生并发症；存活率为 76.1%（67/88）。57/67（85.1%）名儿童有随访数据。严重神经发育障碍和肺病（支气管发育不良、新生儿持续性肺动脉高压和哮喘）的发生率分别为 5.3%和 8.8%。治疗后持续存在的水肿、与遗传综合征相关的 FHT 和出生时 GA 是胎儿死亡、NND 和产后并发症的危险因素。
在真正孤立的 FHT 中，只要有指征，胸腔-羊膜分流是一种安全的手术，存活率和长期结局良好。
