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Isolated intraspinal juvenile xanthogranuloma in an infant presenting as acute paraplegia

David-Alexander Wille1, Oliver Bozinov, Ianina Scheer

  • 1Department of Neuropediatrics, University Children's Hospital Zurich, Zurich, Switzerland. david.wille@kispi.uzh.ch

Neuropediatrics
|December 21, 2012
PubMed

Insights

A rare case of juvenile xanthogranuloma (JXG) in an infant caused acute paraplegia. Surgical removal of the intraspinal JXG led to complete neurologic recovery, highlighting a unique presentation.

Area of Science:

  • Pediatric Neurology
  • Dermatopathology
  • Neurosurgery

Background:

  • Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis primarily affecting the skin.
  • Extracutaneous manifestations of JXO are uncommon, with spinal involvement being exceedingly rare, especially in infants.

Observation:

  • A 6-month-old infant presented with sudden onset of paraplegia.
  • MRI revealed a thoracic intraspinal extradural mass causing significant spinal cord compression.

Findings:

  • Histopathological examination confirmed the mass as juvenile xanthogranuloma.
  • This represents, to our knowledge, the first reported case of an isolated intraspinal JXG in an infant under 12 months of age.

Implications:

  • Early diagnosis and surgical intervention are crucial for favorable outcomes in spinal JXG.
  • This case expands the spectrum of extracutaneous presentations of JXO in pediatric patients.
  • Highlights the importance of considering JXO in the differential diagnosis of spinal masses in infants presenting with neurological deficits.

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