Fetal syringomyelia

Area of Science:

• Neuroscience
• Developmental Biology
• Pediatric Pathology

Background:

• Syringomyelia is a complex spinal cord condition.
• Fetal spinal dysraphism and hindbrain crowding are associated with neurological abnormalities.
• Understanding the origins of fetal syringomyelia is crucial for diagnosis and management.

Purpose of the Study:

• To determine the prevalence of syringomyelia in fetuses with dysraphism and hindbrain crowding.
• To characterize the anatomical features and potential causes of fetal syringomyelia.
• To differentiate fetal syringomyelia from post-natal forms.

Main Methods:

• Retrospective analysis of 113 fetal cases (17.5–34 weeks gestation).
• Examination of cases with Chiari II malformations, OEIS, Meckel Gruber syndrome, and conjoined twins.
• Histopathological assessment for secondary injury markers like vernicomyelia and hemorrhage.

Main Results:

• Syringomyelia was identified in 13 cases of Chiari II malformations, 5 OEIS cases, 2 Meckel Gruber syndrome cases, and 1 conjoined twin pair.
• Secondary injuries (vernicomyelia, infarct-like histology, hemorrhage) were observed in 8 cases.
• Syringes were typically distinct from the central canal, located dorsally/paramedially, and extended proximally or distally from dysraphic sites.

Conclusions:

• Fetal syringomyelia in Chiari II malformations and dysraphic states often forms before midgestation.
• Both primary malformation and secondary in-utero injury contribute to its development.
• Fetal syringomyelia is anatomically and pathophysiologically distinct from post-natal syringomyelia secondary to hindbrain crowding.