Cervical Intramedullary Cavernoma in Children: Case report and literature review

Abstract

Case presentation: A 10-year-old male patient was admitted due to posterior cervicalgia, vomiting and progressive generalized weakness. Physical examination showed difficulty in ambulation and tetraparesis. Magnetic resonance imaging (MRI) of the cervical medulla enhanced by the contrast agent, gadolinium, showed a poorly contrasted mass with a hemorrhagic center. We undertook a posterior cervicotomy (C1-C3). Post-operative evolution was satisfactory with complete regression of the deficits 90 days after surgery. Control MRI at intervals of 1 and 4 years confirmed absence of the tumor. Discussion: Cavernomas are vascular malformations, consisting of coarsely dilated vascular channels and coated by a single layer of endothelial cells, devoid of endothelium and myothelium. They represent only 5-12% of all vascular pathologies of the medulla, with only 10% affecting the pediatric population. There are only thirty cases of pediatric intramedullary spinal cavernomas in the literature, with predominance among males (2.1:1). Thoracic and cervical spinal cavernomas consist in 55% and 45% of the cases, respectively. In six cases (20%) cavernomatous lesions were associated with synchronic intracranial cavernoma. Among the reported cases, only one had poor evolution after surgery, whereas six patients persisted with prior symptoms. Conclusion: Spinal intramedullary cavernomas are rare entities, especially in the pediatric population, and are treated with surgery which improves prior neurologic deficits, besides preventing rebleeding