Department of Obstetrics & Gynecology, Texas Children's Hospital and Baylor College of Medicine, Houston, TX (Dadoun).
Division of Maternal-Fetal Medicine, Dept of Obstetrics, Gynecology, & Reproductive Sciences, Magee-Womens Hospital University of Pittsburgh School of Medicine, Pittsburgh, PA (Shanahan).
Am J Obstet Gynecol MFM. 2024 Oct;6(10):101457. doi: 10.1016/j.ajogmf.2024.101457. Epub 2024 Aug 3.
Omphalocele is a congenital midline abdominal wall defect resulting in herniation of viscera into a membrane-covered sac. Pulmonary complications, including pulmonary hypoplasia, pulmonary hypertension, and prolonged respiratory support are a leading cause of neonatal morbidity and mortality.
OBJECTIVE(S): This study aimed to assess the role of fetal MRI-derived lung volumes and omphalocele defect size as clinical tools to prognosticate postnatal pulmonary morbidity and neonatal mortality in those with a prenatally diagnosed omphalocele (PDO).
This was a retrospective cohort study of all pregnancies with PDO at our fetal center from 2007-2023. Pregnancies with aneuploidy or concurrent life-limiting fetal anomalies were excluded. Using fetal MRI, observed-to-expected total fetal lung volume (O/E TLV) ratios were determined by a previously published method. The transverse diameter of the abdominal defect was also measured. The O/E TLV ratios and abdominal defect measurements were compared with postnatal outcomes. The primary outcome was death at any time. Secondary outcomes included death in the first 30 days of life or before discharge from birth hospitalization, the requirement of respiratory support with intubation and mechanical ventilation, or development of pulmonary hypertension.
Of 101 pregnancies with a PDO, 54 pregnancies (53.5%) with prenatally diagnosed omphalocele met inclusion criteria. There was a significant increase in the rate of death when compared between the three O/E TLV classifications: 1/36 (2.8%) in the O/E≥50% group, 3/14 (21.4%) in the O/E 25%-49.9% group, and 4/4 (100%) in the O/E<25% group (P<.001). The rate of intubation increased with the severity of O/E TLV classification, with 27.8% in the O/E≥50% group, 64.3% in the O/E 25%-49.9% group, and 100% in the O/E<25% group (P=.003). The rate of pulmonary hypertension was also higher in the O/E 25%-49.9% (50.0%) and the O/E<25% (50.0%) groups compared to the O/E≥50% group (8.3%, P=.002). There was no association between the transverse diameter of the abdominal wall defect and the primary outcome of death (OR=1.08 95% CI=[0.65-1.78], P=.77).
In our cohort of patients with PDO, O/E TLV<50% is associated with death, need for intubation, prolonged intubation, and pulmonary hypertension. In contrast, omphalocele size demonstrated no prognostic value for these outcomes. The strong association between low fetal lung volume on MRI and poor neonatal outcomes highlights the utility of fetal MRI for estimating postnatal prognosis. Clinicians can utilize fetal lung volumes to direct perinatal counseling and optimize the plan of care.
脐膨出是一种先天性中线腹壁缺陷，导致内脏疝入膜覆盖的囊袋中。肺部并发症，包括肺发育不全、肺动脉高压和延长的呼吸支持，是新生儿发病率和死亡率的主要原因。
本研究旨在评估胎儿 MRI 衍生的肺容积和脐膨出缺陷大小作为临床工具，预测产前诊断的脐膨出（PDO）患者的产后肺部发病率和新生儿死亡率。
这是对我们胎儿中心 2007 年至 2023 年期间所有 PDO 妊娠的回顾性队列研究。排除了染色体异常或同时存在危及生命的胎儿异常的妊娠。使用胎儿 MRI，通过之前发表的方法确定观察到的与预期的总胎儿肺容积（O/E TLV）比值。还测量了腹部缺陷的横径。将 O/E TLV 比值和腹部缺陷测量值与产后结果进行比较。主要结局是任何时间的死亡。次要结局包括 30 天内死亡或在出生住院期间出院前死亡、需要插管和机械通气的呼吸支持、或发展为肺动脉高压。
在 101 例 PDO 妊娠中，54 例（53.5%）具有产前诊断的脐膨出符合纳入标准。在 O/E TLV 三个分类组之间比较时，死亡率显著增加：O/E≥50%组为 1/36（2.8%），O/E 25%-49.9%组为 3/14（21.4%），O/E<25%组为 4/4（100%）（P<.001）。O/E TLV 分类严重程度与插管率相关，O/E≥50%组为 27.8%，O/E 25%-49.9%组为 64.3%，O/E<25%组为 100%（P=.003）。O/E 25%-49.9%（50.0%）和 O/E<25%（50.0%）组的肺动脉高压发生率也高于 O/E≥50%组（8.3%，P=.002）。腹壁缺陷的横径与主要结局死亡之间无关联（OR=1.08 95% CI=[0.65-1.78]，P=.77）。
在我们的 PDO 患者队列中，O/E TLV<50%与死亡、需要插管、延长插管和肺动脉高压有关。相比之下，脐膨出大小对这些结局没有预测价值。MRI 上胎儿肺容积低与新生儿不良结局之间的强烈关联突出了胎儿 MRI 用于估计产后预后的效用。临床医生可以利用胎儿肺容积来指导围产期咨询并优化护理计划。
