Ruano, Rodrigo

AbstractObjectiveTo evaluate variability in antenatal sonographic prognostication of congenital diaphragmatic hernia (CDH) within the North American Fetal Therapy Network (NAFTNet).MethodsNAFTNet centre were invited to complete a questionnaire and participate in videoconference calls, during which participants were observed while measuring lung area by ultrasound using the anteroposterior (AP) method, longest method, and trace method. Each center identified 1–2 experienced fetal medicine specialist(s) or medical imaging specialists locally to participate in the study. Practices were compared among NAFTNet centre within and without the fetal endoscopic tracheal occlusion (FETO) consortium.ResultsNineteen participants from 9 FETO center and 30 participants from 17 non‐FETO center completed the survey and 31 participants were interviewed and observed while measuring sonographic lung area. All Centres measured observed‐to‐expected lung‐to‐head ratio (o/e LHR) or LHR for CDH prognostication. Image selection criteria for lung area measurement were consistent, including an axial section of the chest with clear lung borders and a 4‐chamber cardiac view. Lung area measurement methods varied across NAFTNet, with most centre using longest (4/9 FETO vs. 13/29 non‐FETO) or trace (3/9 FETO vs. 11/29 non‐FETO) method. Centres differed in expected reference ranges for o/e LHR determination and whether the lowest, highest or average o/e LHR was utilized.ConclusionVariability in antenatal sonographic prognostication of CDH was identified across NAFTNet, indicating a need for consensus‐based standardization.

OBJECTIVE Open prenatal myelomeningocele (MMC) repair is typically associated with reversal of in utero hindbrain herniation (HBH) and has been posited to be associated with a reduction in both postoperative prenatal and immediate postnatal hydrocephalus (HCP) risks. However, the long-term postnatal risk of HCP following HBH reversal in these cases has not been well defined. The authors describe the results of a long-term HCP surveillance in a cohort of patients who underwent prenatal MMC repair at their institution. METHODS A retrospective review of all prenatal MMC repair operations performed at the Mayo Clinic between 2012 and 2017 was conducted. Pertinent data regarding the clinical courses of these patients before and after MMC repair were summarized. Outcomes of interest were occurrences of HBH and HCP and the need for intervention. RESULTS A total of 9 prenatal MMC repair cases were identified. There were 7 cases in which MRI clearly demonstrated prenatal HBH, and of these 86% (6/7) had evidence of HBH reversal after repair and prior to delivery. After a mean postnatal follow-up of 20 months, there were 3 cases of postnatal HCP requiring intervention. One case that failed to show complete HBH reversal after MMC repair required early ventriculoperitoneal shunting. The other 2 cases were of progressive, gradual-onset HCP despite complete prenatal HBH reversal, requiring endoscopic third ventriculostomy with choroid plexus cauterization at ages 5 and 7 months. CONCLUSIONS Although prenatal MMC repair can achieve HBH reversal in a majority of well-selected cases, the prevention of postnatal HCP requiring intervention appears not to be predicated on this outcome alone. In fact, it appears that in a subset of cases in which HBH reversal is achieved, patients can experience a progressive, gradual-onset HCP within the 1st year of life. These findings support continued rigorous postnatal surveillance of all prenatal MMC repair patients, irrespective of postoperative HBH outcome.

AbstractObjectivesThe purpose of this study was to evaluate the reproducibility of stomach position grading in congenital diaphragmatic hernia (CDH) as proposed by Cordier et al and Basta et al after standardization of the methods at our center.MethodsWe collected sonographic images from 23 fetuses with left‐sided CDH at our center from 2010 to 2018. Nine operators (one maternal fetal medicine expert and eight sonographers) reviewed the selected images and graded the stomach position according to the methods of Cordier et al and Basta et al. We assessed the interoperator agreement with Fleiss's kappa statistics.ResultsOverall agreement amongst all operators was moderate for both methods proposed by Cordier et al (k = 0.60, SE 0.07, 95% CI 0.47‐0.73, P < .0001) and Basta et al (k = 0.60, SE 0.06, 95% CI 0.47‐0.73, P < .0001). Interoperator agreement was moderate for grade 3 with the method by Cordier et al (k = 0.45, SE 0.09, 95% CI 0.27‐0.64, P < .0001) and fair for grade 4 with the method by Basta et al (k = 0.33, SE 0.08, 95% CI 0.18‐0.49 P < .0001).ConclusionsOur study demonstrates a fair to moderate interoperator agreement of the stomach position grading methods proposed in the literature after standardization of the methods at our center. Further multicenter studies are needed to confirm our results.

Importance Bilateral renal agenesis is a rare congenital anomaly associated with poor prognosis. Objective The aims of this article are to review and summarize evidence on prenatal diagnosis and outcomes of bilateral renal agenesis. Evidence Acquisition A search was undertaken using PubMed and ClinicalTrials.gov databases from January 1, 1998, to September 1, 2018. Search terms include “prenatal diagnosis” OR “outcomes” AND “bilateral renal agenesis.” Search was limited to English language. Results Fetal ultrasonography is the primary imaging modality for prenatal diagnosis of fetal urogenital tract abnormalities. However, ultrasonography is limited by several factors; it is operator dependent and associated with small field of view, has limited soft-tissue acoustic contrast, and is also influenced by patient habitus and fetal position. Color Doppler ultrasonography can be used as an adjunct to exclude bilateral renal agenesis by visualizing renal arteries. In the literature, prenatal magnetic resonance imaging has been reported to be equal to or superior to prenatal ultrasonography. Bilateral renal agenesis with oligohydramnios/anhydramnios is associated with a poor prognosis; perinatal death occurs secondary to pulmonary hypoplasia in the majority of cases. Conclusions Ultrasonography in combination with color Doppler ultrasonography permits the fetal urinary tract to be assessed in the first and early second trimester of gestation. The magnetic resonance imaging can be used as a complementary adjunctive modality in equivocal or inconclusive ultrasonographic findings. Target Audience Obstetricians and gynecologists, family physicians. Learning Objectives After completing this activity, the learner should be able to describe the natural history of fetuses with bilateral renal agenesis; explain to patients the accuracy and limitations of the prenatal diagnosis of the anomaly; and counsel patients regarding the perinatal outcome and prognosis of fetuses with this condition.