Pediatric Intramedullary Cavernoma: Surgical Treatment

Abstract

The natural history of pediatric intramedullary cavernomas (ImCs) is essentially unknown, however, they tend to present at a mean age between 9.1 and 13.2 years with an acute neurological deficit, often followed by rapid deterioration due to hematomyelia. Imaging of the entire neuraxis is warranted, as those affected by multiple lesions and the familial form of the disease (12–40%) tend to suffer a more aggressive behaviour and earlier age of clinical onset. The cumulative literature is inconclusive with regard to indications for surgical treatment, thus supporting the concept of highly individualized decision making. However, considering the lifetime risks in children, surgical treatment to prevent future hemorrhage should strongly be considered, at least for those lesions that are surgically accessible. Except for the very young child, most authors would favor the use of intraoperative neuromonitoring during resection using either a posterior midline, dorsal root entry zone, or lateral myelotomy approach. With adequate surgical treatment either in the acute phase or after clinical recuperation, prognosis may be surprisingly good. Incompletely resected lesions and/or syndromal cases tend to recur, requiring further treatment. Definite answers await more cases with longer follow-up especially after subtotal resection, however, as long as the child remains in a stable condition, careful clinical and radiological follow-up may be an alternative to reoperation.