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Prenatal diagnosis and postnatal outcome of closed spinal dysraphism

Ivonne Bedei, Eyal Krispin, Magdalena Sanz Cortés, Hennie Lombaard, Roni Zemet, William E. Whitehead, Michael A. Belfort, Thierry A.G.M. Huisman

2023Prenatal Diagnosis11 citationsDOIOpen Access PDF

Abstract

OBJECTIVE: To evaluate the prenatal diagnosis of closed dysraphism (CD) and its correlation with postnatal findings and neonatal adverse outcomes. METHODS: A retrospective cohort study including pregnancies diagsnosed with fetal CD by prenatal ultrasound (US) and magnetic resonance imaging (MRI) at a single tertiary center between September 2011 and July 2021. RESULTS: CD was diagnosed prenatally and confirmed postnatally in 12 fetuses. The mean gestational age of prenatal imaging was 24.2 weeks, in 17% the head circumference was ≤fifth percentile and in 25% the cerebellar diameter was ≤fifth percentile. US findings included banana sign in 17%, and lemon sign in 33%. On MRI, posterior fossa anomalies were seen in 33% of cases, with hindbrain herniation below the foramen magnum in two cases. Mean clivus-supraocciput angle (CSA) was 74°. Additional anomalies outside the CNS were observed in 50%. Abnormal foot position was demonstrated prenatally in 17%. Neurogenic bladder was present in 90% of patients after birth. CONCLUSION: Arnold Chiari II malformation and impaired motor function can be present on prenatal imaging of fetuses with CD and may be associated with a specific type of CD. Prenatal distinction of CD can be challenging. Associated extra CNS anomalies are frequent and the rate of neurogenic urinary tract dysfunction is high.

Topics & Concepts

MedicinePrenatal diagnosisForamen magnumMagnetic resonance imagingGestational ageFetusRetrospective cohort studyPregnancyPediatricsSpina bifidaObstetricsSurgeryRadiologyBiologyGeneticsSpinal Dysraphism and MalformationsFetal and Pediatric Neurological DisordersAssisted Reproductive Technology and Twin Pregnancy