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
Santoro, A., Piccirilli, M., Brunetto, G. M., Delfini, R., & Cantore, G. (2007). 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. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 23(11), 1319–1326. https://doi.org/10.1007/s00381-007-0395-5
View Original Source →Abstract
OBJECT: The authors present their experience with the 17th pediatric intramedullary cavernoma reported in English literature. METHODS: The patient firstly underwent surgery for a left frontal cavernoma when he was 2 years old. Also the child's mother was operated for a C2-C3 intramedullary cavernoma. He grew up normally and the radiological follow-up was negative for other brainstem cavernous malformations. When he was 11 years old he complained a worsening tetraparesis. A cerebral and spinal magnetic resonance (MR) imaging revealed the presence of a C1 intramedullary cavernoma and a pontine cavernoma. He underwent surgery for the cervical lesion, which was completely removed. The postoperative course was regular with a total recovery from the neurological deficit. The boy underwent a radiological follow-up, monitoring the pontine lesion, which spontaneously regressed when he was 19 years old. CONCLUSION: The rarity of the pediatric intramedullary cavernoma, the familial occurrence, and the spontaneous regression of the pontine cavernoma make this case very peculiar
Case Details
Disease Location
Spinal cord (c1) frontal lobe pons
Personal Characteristics
11 -year-old male, first surgery at 2 -year-old for frontal cavernoma, resulted in complete surgical removal of the left frontal cavernoma. Mother was operated for a c2-c3 intramedullary cavernoma at age 32--cavernoma was 1.5 cm in diameter. In the following year, she had two more cerebral cavernomas removed and had complete postop neurological recovery
Clinical Characteristics
At 11 -year-old, complained about tetraparesis, mr revealed c1 intramedullary cavernoma and a pontine cavernoma, surgery removed the cervical lesion and experienced worsening onset of weakness in lower and upper extremitiesover a months, associated with headache eventually was unable to deambulate independently examination resulted in significant tetraparesis, bilateral lower and upper extremities hyperreflexia with bilateral ankle clonus. Bilateral babinki's signs were elicited sensory and bladder function were preserved at 8 underwent MRI which was negative for any other cavernomas at 11, cerebral and spinal MRI revealed intramedullary cavernoma at the c1 level in the posterior part, associated with a covernoma in the upper and anterior part of the pons MRI performed 24 hours after the surgery to remove the lesion at age 11 confirmed the complete removal of the lesion. Patient was discharged after 5 days the evolution of the cavernoma of the pons was checked in the 1 month follow-up using consecutive brain and spinal MRI
Remission Characteristics
Pontine lesion (pons cavernoma) remissed when 19 yo 1 month after surgery (11 yo), complete recovery of neurological deficits
Treatment & Mechanisms
Clinical Treatment
Complete surgical removal of the lesion at 11 yo (removal of posterior arch of c1 , and c2 laminectomy)
Non-Clinical Treatment
None reported