Open Spinal Dysraphism Without Hindbrain Herniation-Natural History and Postnatal Outcome
I Bedei1, C C Kik2, R Axt-Fliedner1
1Department of Prenatal Medicine and Fetal Therapy, Justus-Liebig University Giessen, Giessen, Germany.
Prenatal Diagnosis
|July 8, 2025
Summary
Fetuses with open spinal dysraphism (OSD) but no hindbrain herniation (HBH) have a low shunt rate. However, motor function is often impaired prenatally and may worsen after birth, impacting counseling for fetal surgery.
Area of Science:
- Prenatal diagnosis and management of congenital anomalies.
- Fetal neurosurgery and developmental neurology.
- Spinal dysraphism and its impact on neurological development.
Background:
- Open spinal dysraphism (OSD) is a complex congenital condition.
- The presence of hindbrain herniation (HBH) influences OSD prognosis.
- Understanding OSD natural history without HBH is crucial for accurate counseling.
Purpose of the Study:
- To describe the natural history of fetuses with OSD but without HBH.
- To evaluate prenatal and postnatal outcomes in this specific OSD subgroup.
- To inform clinical decision-making regarding interventions like fetal surgery.
Main Methods:
- Retrospective cohort study across three prenatal centers.
- Inclusion of fetuses with postnatally confirmed OSD and no prenatal HBH (19-27 weeks).
- Utilized serial ultrasound, MRI, and postnatal assessments (imaging, surgery, neurological function).
Main Results:
- 12 out of 280 OSD fetuses (4%) lacked HBH.
- Moderate ventriculomegaly occurred in 33%; no corpus callosum anomalies detected.
- Postnatal HBH developed in 50%, shunt rate was 20%.
- 80% had stable or improved motor function (MF) relative to anatomical level.
- 33% experienced a decline in MF compared to second-trimester assessment.
Conclusions:
- OSD fetuses without HBH show a low postnatal shunt rate.
- Prenatal motor function is frequently impaired, with potential worsening postnatally.
- Findings challenge the role of fetal surgery in this OSD subgroup and guide counseling.
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