Nondysraphic pediatric intramedullary spinal cord lipomas

Abstract

Intramedullary spinal cord lipomas not associated with dysraphism are infrequently reported. When present, they typically occur in children and have a predilection for the cervical and thoracic spinal cord. The authors review the presentation, treatment, and disease course in 5 pediatric patients, and compare the outcomes with previously reported cases. With institutional review board approval, a retrospective chart review was undertaken at Children's Hospital Los Angeles. Four patients with intramedullary spinal cord lipomas and 1 patient with a lipoblastoma, none associated with dysraphism, were retrospectively reviewed. There were 2 boys and 3 girls ranging in age from 2 months to 4 years. Four patients underwent a laminectomy or laminoplasty with one or more subtotal resections. One patient initially underwent a decompressive laminoplasty without debulking. The median follow-up was 8 years (range 12 months-11 years). Two patients had regrowth of their lipoma, necessitating a second surgery in one patient and 3 debulking surgeries in the other. Postoperatively, 3 patients developed mild kyphosis, none significant enough to require orthopedic intervention. One patient underwent a stabilization procedure at the time of the initial laminectomy and tumor debulking. No patient received chemotherapy or radiation. At the most recent follow-up visit, patients demonstrated improved neurological function when compared with preoperative status. In addition to a decompressive laminectomy, debulking of the lipoma provides the best long-term neurological outcome. Gross-total excision is not warranted and usually is not possible. Long-term follow-up is needed, and repeat debulking of the lipoma is indicated if there is an increase in tumor size due to hyperplasia of residual adipocytes, when tumor growth is associated with neurological deterioration.