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Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

Olga A de Wit1, Wilfred Fa den Dunnen, Krystyne M Sollie

  • 1Department of Pediatrics, University Medical Center, University of Groningen, Hanzeplein 1, 9700 RB, Groningen, The Netherlands. d.a.sival@bkk.umcg.nl.

Cerebrospinal Fluid Research
|March 4, 2008
PubMed
Abstract

Insights

Neuroepithelial/ependymal denudation in fetal spina bifida aperta (SBA) occurs before Chiari II malformation and hydrocephalus. This early denudation may lead to permanent cerebral pathology due to irreversible cell damage.

Area of Science:

  • Neuroscience
  • Developmental Biology
  • Pediatric Pathology

Background:

  • Fetal spina bifida aperta (SBA), characterized by spinal meningomyelocele (MMC), is linked to cerebral pathologies like hydrocephalus and Chiari II malformation.
  • Animal models suggest that loss of ventricular lining (neuroepithelial/ependymal denudation) may initiate cerebral pathology in SBA.
  • The precise timing and initiating events of neuroepithelial/ependymal denudation in human fetal MMC remain unclear.

Purpose of the Study:

  • To investigate the occurrence of neuroepithelial/ependymal denudation in human fetuses and neonates with MMC.
  • To determine if neuroepithelial/ependymal denudation is associated with the onset of hydrocephalus in MMC cases.

Main Methods:

  • Study included seven fetuses and one neonate (16-40 weeks gestational age) with MMC and six control fetuses.
  • Ultrasound (US) was used for fetal MMC identification and monitoring of head circumference and ventricular width.
  • Histological confirmation of MMC and characterization of hydrocephalus (ventriculomegaly) were performed postnatally.

Main Results:

  • Neuroepithelial/ependymal denudation was observed as early as 16 weeks gestational age in the aqueduct and telencephalon, preceding hydrocephalus and Chiari II malformation.
  • By 21-34 weeks gestational age, denudation and progenitor cell loss coincided with Chiari II malformation, but hydrocephalus was absent.
  • At 37-40 weeks gestational age, denudation was observed concurrently with Chiari II malformation and hydrocephalus.

Conclusions:

  • Neuroepithelial/ependymal denudation in the telencephalon and aqueduct can precede the development of Chiari II malformation and hydrocephalus in fetal SBA.
  • Given the inability of denuded areas to regain cell function, these early neurodevelopmental changes may result in permanent cerebral pathology.

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