Faculty Bibliography

Radical surgery is the most important treatment modality for ependymoma. The benefit of adjuvant radiotherapy and/or chemotherapy following a gross total resection of a low grade intracranial ependymoma is uncertain. Since 1990 we elected to defer adjuvant therapy in 7 pediatric patients with a median age of 7 years (range 3-16 years) who had a radical resection of an intracranial ependymoma and no evidence of central nervous system metastases. The primary tumor site was the cerebral hemisphere (6) and the cerebellum (1). A gross total resection was radiologically confirmed in 5 of the 7 patients. Two of the patients had a blood clot in the resection site on the postoperative magnetic resonance imaging scan. All patients are alive after a median follow-up of 44 months and the median progression-free survival is 38+ months. Five of the patients remain in continuous remission. The 2 patients with postoperative blood clots developed subclinical local recurrences, 10 and 11 months, respectively, after diagnosis. They remain in remission for 13+ and 27+ months after subsequent radical surgical procedures. Involved field radiotherapy was administered to 1 patient. After a limited period of follow-up, radical surgery alone appears to be sufficient for the majority of children with low grade ependymomas diagnosed at > 3 years of age when postoperative imaging confirms a gross total resection. This is more likely to occur in supratentorial ependymomas arising in older children

Neurological deterioration in the tethered cord syndrome has been postulated to result from a compromise of blood flow in the distal spinal cord. In order to evaluate vascular perfusion in human subjects, a new technique of laser-Doppler flowmetry was used to monitor continuously the microcirculation of the distal spinal cord during surgery for tethered cord release in 10 children. For further comparison, five children undergoing selective dorsal rhizotomy were also monitored. In the tethered cord syndrome group, spinal cord blood flow before untethering was a mean of 12.6 ml/min per 100 g of tissue and increased in all cases after release to a mean of 29.4 ml/min per 100 g of tissue. All patients improved neurologically. The selective dorsal rhizotomy group had a preoperative mean spinal cord blood flow of 30.8 ml/min per 100 g of tissue, which was not altered by the operative procedure. Significant improvement occurs in distal spinal cord blood flow after tethered cord release, which may be representative of an important mechanism in the pathophysiology of the tethered cord syndrome

Twenty children with vein of Galen malformations and hydrocephalus required a ventriculoperitoneal shunt. Shunt placement was associated with a 70% incidence of complications that included status epilepticus in 3 patients, intraventricular hemorrhage in 7 patients, and subdural hematoma or hygroma in 5 patients. Based upon this experience, the authors recommend the use of preoperative prophylactic anticonvulsants and the placement of medium pressure frontal ventriculoperitoneal shunts. This report will address the overall experience with and the basis on which these therapeutic recommendations were made

The echographic characteristics of 186 suspected intramedullary spinal cord tumors were reviewed. Ultrasonography was found to be specific in distinguishing the tumor type, the extent of the lesion, and the presence and type of associated cysts. Ultrasonography greatly facilitates the selection of respective sites for the placement of a myelotomy, and for initiation of the resection. Additionally, this indispensable adjunct provides ongoing images that allow the preoperative plan to be precisely followed in a surgical field where anatomical landmarks are limited and the margin for error is minimal

We report the case of a 13-year-old boy with disabling chorea due to moyamoya disease. His chorea seemed to improve with steroid therapy. We conclude that steroid therapy may ameliorate moyamoya-associated chorea, and perioperative steroids can confound neurosurgical outcome. We are unable to assess the effect of cerebrovascular bypass procedures on the outcome of chorea in this patient.

A case of transdural herniation of a cervical disk is presented. The diagnosis was most clearly established by magnetic resonance imaging. The literature is reviewed and possible mechanisms of transdural herniation are discussed

Two cases of posterior lumbar vertebral rim fracture and associated disc protrusion in adolescents are presented. Disc compliance and developing vertebral structure were thought to be primarily responsible for this unusual injury. Occurring primarily in adolescent males, this entity most commonly affects the L-4 vertebra. One of the cases involved disc rupture at three levels. This injury is thought to be due in part to union of the anuli by the posterior longitudinal ligament, and in part to rim fracture and limited prolapse rather than frank single-level herniation in an adolescent. A review of the embryology, literature, and theoretical mechanism for occurrence at multiple levels is included

Melanotic neoplasms of the spinal nerve root are rare but well-documented occurrences. The authors report the case of a 68-year-old woman with a malignant melanotic neoplasm containing premelanosomes and melanosomes and lacking a basal lamina. This tumor was found in a lumbar nerve root and is believed to represent an instance of a primary melanoma arising in a spinal nerve root. The differential diagnosis of pigmented lesions of spinal nerve root includes melanoma, pigmented nerve sheath tumor, blue nevus, melanotic clear-cell sarcoma, and meningeal melanocytoma. Surgeons should be aware of the potential for encountering unexpected malignant lesions involving spinal nerve roots