🧒 Pediatric Spinal Cavernous Malformation [Neurosurgery Wiki]

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====== 🧒 Pediatric Spinal Cavernous Malformation ======

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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.

===== 🧠 Pathophysiology =====
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

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

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

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

===== 🧪 Diagnosis =====
  * **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

===== 🩺 Differential Diagnosis =====
  * [[Ependymoma]]
  * [[Astrocytoma]]
  * [[Hemangioblastoma]]
  * [[Spinal arteriovenous malformation]]s (AVMs)
  * [[Lipoma]]s or [[dermoid]]s

===== ⚖️ Management =====
  * **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

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

===== 📚 References =====
  * 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.