Abstract：
BACKGROUND: 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: 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).
METHODS: 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.
RESULTS: 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).
CONCLUSIONS: 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.
OBJECTIVE: 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).
METHODS: 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.
RESULTS: 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).
CONCLUSIONS: 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.
摘要：
背景：脐膨出是一种先天性腹壁中线缺损，导致内脏疝入膜覆盖囊。肺部并发症,包括肺发育不全,肺动脉高压,和长时间的呼吸支持是新生儿发病和死亡的主要原因。
目的：本研究旨在评估胎儿MRI来源的肺容量和脐膨出缺损大小作为预测产前诊断为脐膨出(PDO)患者出生后肺部发病率和新生儿死亡率的临床工具的作用。
方法：这是2007-2023年在我们的胎儿中心进行的所有PDO妊娠的回顾性队列研究。排除具有非整倍性或并发生命限制性胎儿异常的妊娠。用胎儿核磁共振,观察到的与预期的胎儿肺总容积(O/ETLV)比是通过先前公布的方法确定的.还测量了腹部缺损的横向直径。将O/ETLV比率和腹部缺损测量值与产后结局进行比较。主要结果是随时死亡。次要结局包括出生后前30天或出生住院出院前的死亡，需要插管和机械通气的呼吸支持，或肺动脉高压的发展。
结果：在101例PDO妊娠中，54例（53.5％）的产前诊断为脐膨出的妊娠符合纳入标准。与三种O/ETLV分类相比，死亡率显着增加：O/E≥50％组中的1/36（2.8％），O/E25-49.9%组的3/14（21.4%），0/E<25%组(p<0.001)为4/4(100%)。插管率随O/ETLV分级的严重程度增加,O/E组27.8%≥50%，在O/E25-49.9%组中占64.3%，和100%在O/E<25%组(p=0.003)。与O/E≥50％组相比，O/E25-49.9％（50.0％）和O/E<25％（50.0％）组的肺动脉高压发生率也较高（8.3％，p=0.002)。腹壁缺损的横径与死亡的主要结局之间没有相关性（OR=1.0895％CI=[0.65-1.78]，p=0.77）。
结论：在我们的PDO患者队列中，O/ETLV<50%与死亡有关,需要插管，长时间插管,和肺动脉高压。相比之下,脐膨出大小对这些结果没有预后价值。MRI上的低胎儿肺容量与新生儿结局不良之间的强关联突出了胎儿MRI对估计出生后预后的实用性。临床医生可以利用胎儿肺容量指导围产期咨询并优化护理计划。
目的：本研究旨在评估胎儿MRI来源的肺容量和脐膨出缺损大小作为预测产前诊断为脐膨出(PDO)患者出生后肺部发病率和新生儿死亡率的临床工具的作用。
方法：这是2007-2023年在我们的胎儿中心进行的所有PDO妊娠的回顾性队列研究。排除具有非整倍性或并发生命限制性胎儿异常的妊娠。用胎儿核磁共振,观察到的与预期的胎儿肺总容积(O/ETLV)比是通过先前公布的方法确定的.还测量了腹部缺损的横向直径。将O/ETLV比率和腹部缺损测量值与产后结局进行比较。主要结果是随时死亡。次要结局包括出生后前30天或出生住院出院前的死亡，需要插管和机械通气的呼吸支持，或肺动脉高压的发展。
结果：在101例PDO妊娠中，54例（53.5％）的产前诊断为脐膨出的妊娠符合纳入标准。与三种O/ETLV分类相比，死亡率显着增加：O/E≥50％组中的1/36（2.8％），O/E25-49.9%组的3/14（21.4%），0/E<25%组(p<0.001)为4/4(100%)。插管率随O/ETLV分级的严重程度增加,O/E组27.8%≥50%，在O/E25-49.9%组中占64.3%，和100%在O/E<25%组(p=0.003)。与O/E≥50％组相比，O/E25-49.9％（50.0％）和O/E<25％（50.0％）组的肺动脉高压发生率也较高（8.3％，p=0.002)。腹壁缺损的横径与死亡的主要结局之间没有相关性（OR=1.0895％CI=[0.65-1.78]，p=0.77）。
结论：在我们的PDO患者队列中，O/ETLV<50%与死亡有关,需要插管，长时间插管,和肺动脉高压。相比之下,脐膨出大小对这些结果没有预后价值。MRI上的低胎儿肺容量与新生儿结局不良之间的强关联突出了胎儿MRI对估计出生后预后的实用性。临床医生可以利用胎儿肺容量指导围产期咨询并优化护理计划。
