Intramedullary Cavernous Angiomas of the Spinal Cord in the Pediatric Age Group: A Pediatric Series

Abstract

The authors have reviewed available data from 7 pediatric patients with intramedullary spinal cord cavernous angioma (ISCCA) reported in the literature, and added from their own series 2 pediatric patients, for a total of 9 patients. This group of pediatric patients’ clinical presentation, course, management and outcome were compared to their adult counterparts as reported in the literature. In contrast to adults, children with symptomatic ISCCA do not show a gender imbalance and the thoracic spinal cord is not predominantly involved. Pediatric patients commonly present with an acute episode and rapid deterioration. A more favorable outcome has been reported in children as compared to adults in the face of relatively similar presenting deficits. As in adults, magnetic resonance imaging (MRI) remains the diagnostic and postoperative test of choice. Complete resection affords the best chance for cure. Symptomatic children with ISCCA characteristically present with an acute deficit and rapid deterioration. MRI of the entire neuraxis is recommended for lesion multiplicity. An attempt at total resection and long-term MRI follow-up are recommended.

Intramedullary spinal cord cavernous angioma in children: case report and literature review

“…Intramedullary cavernous malformations of the spinal cord are very uncommon, especially in children where only ten cases have yet been reported, mostly as isolated cases [1,3,6,14,17]. In 2002, Nagib and O'Fallon [14] summarized nine cases including their two personal cases, and in 2006, Bakir et al [3] reported another pediatric patient with ImCA: Although the number of reported pediatric cases with ImCA is small, we can outline some differences from that of the While ImCA in the adult occur more frequently in women than in men [12,17], with a female/male ratio of approximately 2:1, seven of ten cases of symptomatic ImCA reported in children were male, thus accounting for a male/ female ratio of 2:1.…”

“…In 2002, Nagib and O'Fallon [14] summarized nine cases including their two personal cases, and in 2006, Bakir et al [3] reported another pediatric patient with ImCA: Although the number of reported pediatric cases with ImCA is small, we can outline some differences from that of the While ImCA in the adult occur more frequently in women than in men [12,17], with a female/male ratio of approximately 2:1, seven of ten cases of symptomatic ImCA reported in children were male, thus accounting for a male/ female ratio of 2:1. The hypothesis of a hormonal effect leading to a greater risk of bleeding in female adults does not seem to be verified in children, but the mean age at bleeding is 13.2 years (range [8][9][10][11][12][13][14][15][16][17] and can also suggest the hormonal influence of puberty.…”

“…Intramedullary spinal cord cavernous malformations are much less common: To our knowledge, only 122 cases have been published until now [1,3,4,8,11,12,15,17,19], with only ten of those in pediatric patients [1,3,6,14,17]. This scarcity makes it very difficult to draw some guidelines for the management of these lesions in children.…”

“…Microsurgical resection is the only available therapeutic option for intramedullary cavernous malformations [3,14,19]. However, indications for operating on asymptomatic cavernous malformations remain controversial; although some authors [1,12] do not recommend surgery in these cases, one has to weigh the risks of surgery against those of sudden hemorrhage with serious neurological consequences in case of surgical abstention.…”

“…However, indications for operating on asymptomatic cavernous malformations remain controversial; although some authors [1,12] do not recommend surgery in these cases, one has to weigh the risks of surgery against those of sudden hemorrhage with serious neurological consequences in case of surgical abstention. The facts that all cavernous hemangiomas of the central nervous system show histological evidence of previous hemorrhage [1,18] and that catastrophic neurological deterioration caused by hematomyelia has been documented [1,3,4,14] underscore the risks of surgical abstention.…”