Intramedullary cavernous angioma of the spinal cord in a pediatric patient, with multiple cavernomas, familial occurrence and partial spontaneous regression: case report and review of the literature

Abstract

The rarity of the pediatric intramedullary cavernoma, the familial occurrence, and the spontaneous regression of the pontine cavernoma make this case very peculiar.

Infantile Cervical Intramedullary Cavernous Angioma Manifesting as Hematomyelia -Case Report-

“…The prevalence of females with adult intramedullary spinal cord cavernous angiomas is shown by a female/male ratio of 2:1 32,36,42,45) and 1.1:1. 35,36) In contrast, 15 of 22 cases were in male children. The female/male ratio of child intramedullary spinal cord cavernous angiomas is 0.47:1, which is different from adult cases.…”

“…However, they are evenly distributed in the thoracic and cervical spinal cord in children. 8,24,25,35) Neurological deterioration is usually slowly progressive in adults. 1,6,15) Other forms of clinical presentation of intramedullary spinal cord cavernous angiomas include acute deficit followed by slow and stepwise deterioration with periods of clinical improvement or acute episodes of mild symptoms with gradual decline over months.…”

“…8,10,24,25,35) In a review of the pediatric cases, 50-100% of the cases presented with acute hemorrhage and neurological deterioration. 8,10,24,25,35) Similarly in our case, the clinical presentation began Cervical Intramedullary Cavernous Angioma in an Infant with acute neurological deficit due to hematomyelia which was clearly demonstrated by MR imaging. The hemorrhage rates of cerebral cavernous angiomas are 0.5-1.0% per year, whereas the ratio of intramedullary spinal cord cavernous angiomas is 1.6%.…”

“…years) have been reported. 1,3,8,9,14,17,19,21,[24][25][26][35][36][37]39,40) Here we describe an infantile case of cervical intramedullary spinal cord cavernous angioma and discuss the usefulness of osteoplastic laminotomy for an infant.…”

“…8,19,24) Since the introduction of magnetic resonance (MR) imaging, the number of intramedullary spinal cord cavernous angiomas reported in the literature has increased greatly. 3,[8][9][10]17,19,24,25,35,36,40,47) However, only 21 cases of intramedullary spinal cord cavernous angioma among children (not older than 18 , and gadolinium-enhanced T 1 -weighted (C) magnetic resonance images disclosing a mass lesion at the level between C4-C7 with no enhancement by gadolinium injection. years) have been reported.…”