Objective: Children with congenital diaphragmatic hernia (CDH) are at risk for neurodevelopmental delay. Herein we report on prenatal changes in biometry and brain perfusion in fetuses with isolated CDH.Study Design: This retrospective study evaluated fetuses with isolated, left-sided CDH in three European referral centers. Abdominal circumference (AC), femur length (FL), head circumference (HC), transcerebellar diameter (TCD), middle cerebral artery (MCA) Doppler, and ventricular width (VW) were assessed during four gestational periods (<24 weeks, 25-28 weeks, 29-32 weeks, >33 weeks). Z-scores were calculated, and growth curves were created based on longitudinal data. Results:In 367 fetuses, HC, AC and FL were within normal ranges throughout gestation. The TCD diminished with advancing gestational age to fall below the fifth percentile after 32 weeks. A less pronounced but similar trend was seen in VW. The peak systolic velocity of the MCA was consistently approximately 10% lower than normal. Disease severity was correlated to TCD (p = 0.002) and MCA doppler values (p = 0.002). There were no differences between fetuses treated with FETO and those managed expectantly.
BACKGROUND AND PURPOSE: Congenital diaphragmatic hernia is associated with high mortality and morbidity, including evidence suggesting neurodevelopmental comorbidities after birth. The aim of this study was to document longitudinal changes in brain biometry and the cortical folding pattern in fetuses with congenital diaphragmatic hernia compared with healthy fetuses. MATERIALS AND METHODS:This is a retrospective cohort study including fetuses with isolated congenital diaphragmatic hernia between January 2007 and May 2019, with at least 2 MR imaging examinations. For controls, we used images from fetuses who underwent MR imaging for an unrelated condition that did not compromise fetal brain development and fetuses from healthy pregnant women. Biometric measurements and 3D segmentations of brain structures were used as well as qualitative and quantitative grading of the supratentorial brain. Brain development was correlated with disease-severity markers.RESULTS: Forty-two fetuses were included, with a mean gestational age at first MR imaging of 28.0 (SD, 2.1) weeks and 33.2 (SD, 1.3) weeks at the second imaging. The mean gestational age in controls was 30.7 (SD, 4.2) weeks. At 28 weeks, fetuses with congenital diaphragmatic hernia had abnormal qualitative and quantitative maturation, more extra-axial fluid, and larger total skull volume. By 33 weeks, qualitative grading scores were still abnormal, but quantitative scoring was in the normal range. In contrast, the extraaxial fluid volume remained abnormal with increased ventricular volume. Normal brain parenchymal volumes were found.CONCLUSIONS: Brain development in fetuses with congenital diaphragmatic hernia around 28 weeks appears to be delayed. This feature is less prominent at 33 weeks. At this stage, there was also an increase in ventricular and extra-axial space volume.ABBREVIATIONS: CDH ¼ congenital diaphragmatic hernia; FETO ¼ fetal endotracheal occlusion; GA ¼ gestational age; SRR ¼ super-resolution reconstruction; TFLV ¼ total fetal lung volume C ongenital diaphragmatic hernia (CDH) is a severe birth defect, occurring in approximately 1 in 3000 live-born neonates. 1 Despite neonatal treatment, the disease is associated with high mortality, and survivors often have short-and long-term morbidities. 1 These include respiratory, gastrointestinal, and neurologic impairments. 2 Neurodevelopmental delays as well as behavioral difficulties have been linked to CDH in the past, and certain risk factors have been suggested, including gestational age at birth, disease severity, associated anomalies, the requirement for extracorporeal membrane oxygenation, and long stays in the neonatal intensive care unit. [2][3][4][5][6][7] In infants with CDH, imaging studies have demonstrated several abnormalities, including increased extra-axial space, delayed sulcation, and white matter injury, but the exact mechanisms
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