🧒 Pediatric Spinal Cavernous Malformation

• Abdominal Pain Mimicking a Neurological Disorder: A Case Report of Spinal Cavernous Malformation in a Pediatric Patient
• Pediatric Central Nervous System Vascular Malformation : Pathological Review with Diagram
• Clinical and genetic characteristics of 9 rare cases with coexistence of dual genetic diagnoses
• Giant Cavernous Malformation Mimicking an Infiltrative Intracranial Neoplasm in Children-Case Report and Systematic Review of the Literature
• Multiple spinal intramedullary cavernous angiomas with bleeding episode mimicking an intramedullary tumor
• Extradural hemorrhagic spinal cavernous angioma in a paucisymptomatic child: A rare case with review of the current literature
• Spinal involvement in pediatric familial cavernous malformation syndrome
• Natural History of Spinal Cord Cavernous Malformations: A Multicenter Cohort Study

A Pediatric Spinal Cavernous Malformation (SCM) is a rare vascular lesion of the spinal cord in children, composed of dilated, thin-walled vascular channels without intervening normal neural tissue.

SCMs consist of:

• Compact clusters of sinusoidal capillaries
• Thin walls lacking smooth muscle and elastin
• No arterial supply; low-flow lesion
• Often associated with microhemorrhages and hemosiderin deposits

• Extremely rare in children
• More common in adults (peak: 30–50 years)
• Pediatric cases may present earlier due to larger lesions or genetic predisposition

• Sporadic: Most pediatric cases
• Familial: Linked to *CCM1*, *CCM2*, or *CCM3* gene mutations (often multiple lesions)
• May coexist with cerebral cavernous malformations (CCM)

• Progressive myelopathy
• Sudden neurological decline due to hemorrhage
• Pain (back or radicular)
• Weakness, sensory loss
• Bowel/bladder dysfunction
• Sometimes incidental finding

• MRI with and without contrast:
  • “Popcorn” or “mulberry” appearance
  • Mixed signal intensity (T1/T2) with hemosiderin ring (T2 hypointensity)
  • Gradient echo or SWI: sensitive to hemorrhage
• No enhancement or mild enhancement post-Gd

• Ependymoma
• Astrocytoma
• Hemangioblastoma
• Spinal arteriovenous malformations (AVMs)
• Lipomas or dermoids

• Observation if asymptomatic or mild stable symptoms
• Surgical resection:
  • Indicated for progressive deficits or recurrent hemorrhage
  • Best done with neurophysiological monitoring
  • Gross total resection is curative
• Radiosurgery: not typically used due to spinal cord risk

• Favorable with early diagnosis and complete resection
• Risk of rebleed if untreated (especially in symptomatic cases)
• Long-term monitoring recommended (especially in familial forms)

• Gross BA, Du R. Spinal Cavernous Malformations: Clinical features and surgical outcomes. *Neurosurg Focus*. 2010.
• Labauge P et al. Genetics of cavernous angiomas. *Lancet Neurol*. 2007.
• Wang X, et al. Pediatric spinal cavernous malformations: A review. *Childs Nerv Syst*. 2020.