Thoracic spinal cord compression by intramedullary hamartomatous tissue in a young boy: case report.

Abstract

Spinal cord hamartomas are a rare occurrence characterized by well- differentiated mature elements located in an irregular position in the spinal cord. The authors present the unique case of a 12-year-old boy who originally presented to our center for treatment of a right thalamic astrocytoma. One year after his initial presentation, the child developed progressive bilateral leg weakness with decreased sensation. Spinal magnetic resonance imaging performed at this time revealed an intramedullary spinal cord lesion from T4 to T8, which was later found to be composed of hamartomatous tissue. The patient underwent bilateral T4 to T8 laminectomies with subtotal resection of the hamartomatous lesion, and his symptoms improved postoperatively. Although typically described in the literature as spinal cord hamartomas because of their histological composition, these rare hamartomatous lesions may be the result of a neurulation defect rather than true hamartomas. In patients presenting with multiple neurological conditions, as in our case, diagnosis may be more complex. For those presenting with neurological signs and symptoms, surgical treatment is essential.