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Spontaneous Third Ventriculostomy in Krabbe Disease.

Giulio Zuccoli1, Aram Kim2, Michele Poe3

  • 1Department of Radiology, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, Pennsylvania.

Pediatric Neurology
|March 22, 2020
PubMed
Summary

Spontaneous third ventriculostomies were found in patients with infantile Krabbe disease, indicating increased intracranial pressure. Monitoring for this pressure is crucial for managing Krabbe disease and preventing complications.

Keywords:
Globoid cell leukodystrophyIntracranial pressureKrabbe diseaseSpontaneous third ventriculostomy

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Area of Science:

  • Neurology
  • Pediatric Neurology
  • Neuroimaging

Background:

  • Spontaneous third ventriculostomies are associated with obstructive hydrocephalus and elevated intracranial pressure.
  • Krabbe disease can present with hydrocephalus, but its symptoms overlap with increased intracranial pressure, complicating diagnosis.
  • Monitoring hydrocephalus in Krabbe disease is challenging due to overlapping clinical signs.

Purpose of the Study:

  • To investigate the occurrence of spontaneous third ventriculostomies in infantile Krabbe disease.
  • To correlate spontaneous third ventriculostomies with increased intracranial pressure in this population.
  • To assess the impact of spontaneous third ventriculostomies on developmental outcomes in infantile Krabbe disease.

Main Methods:

  • Retrospective analysis of brain MRI scans from patients with infantile Krabbe disease.
  • Assessment of ventricular size using a standardized brain atlas.
  • Identification of spontaneous third ventriculostomies on mid-sagittal, T2-weighted images.
  • Evaluation of developmental outcomes using standardized tests.

Main Results:

  • Spontaneous third ventriculostomies were identified in 12 out of 75 patients with infantile Krabbe disease.
  • Patients with spontaneous third ventriculostomies exhibited significantly larger head circumference and ventricular volumes.
  • Delayed development in adaptive, gross motor, and fine motor functions was observed in patients with spontaneous third ventriculostomies.

Conclusions:

  • Spontaneous third ventriculostomies are present in infantile Krabbe disease, likely due to increased intracranial pressure.
  • The findings underscore the importance of monitoring for increased intracranial pressure in infantile Krabbe disease.
  • Early detection and management of increased intracranial pressure may be vital for improving outcomes in Krabbe disease.