Nondysraphic pediatric intramedullary spinal cord lipomas Report of 5 cases

Object. 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. Methods. With institutional review board approval, a retrospective chart review was undertaken at Childrens Hospital Los Angeles. Results. Four patients with intramedullary spinal cord lipomas and I 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. Conclusions. 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. (DOI: 10.3171/2009.9.PEDS09238)