Faculty Bibliography

The authors present a series of six patients with large symptomatic benign pineal cysts and review the 27 patients previously reported in the literature. Patients with symptomatic pineal cysts most often present with one of three syndromes: 1) paroxysmal headache with gaze paresis; 2) chronic headache, gaze paresis, papilledema, and hydrocephalus; or 3) pineal apoplexy with acute hydrocephalus. Surgical intervention with radical cyst removal is the treatment of choice for all symptomatic pineal cysts. Complete cyst removal is desirable; however, radical subtotal resection is appropriate if the cyst cannot be easily separated from the quadrigeminal plate. Ventricular shunting should be reserved for patients with persistent hydrocephalus after cyst resection

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

Choroid plexus carcinoma (CPC) arising in the infant poses several treatment dilemmas. The tumor is often not totally resectable at presentation given its large size and tendency to invade adjacent brain. Because of its predisposition to regrow and metastasize, some form of postoperative cytotoxic therapy is required. Chemotherapy (CHT), as opposed to radiotherapy (RT), has a more desirable risk/benefit role in infants, since it is relatively sparing of late neurologic sequelae. Three young male children presented with large intraventricular CPC at 9, 18, and 27 months of age. One child had subarachnoid metastases at diagnosis and the other two had localized disease. Subtotal resections were accomplished and all three required VP shunts. Initial CHT consisted of four monthly courses of cisplatin (20 mg/m2) and etoposide (100 mg/m2), both administered intravenously, daily, for five days. After four courses, two children had complete responses and one had stable disease. Additional CHT was given but one child developed a local recurrence and another diffuse CNS metastases. Both died with intratumoral hemorrhages at 5 and 57 months following diagnosis. The third child remains in continuous remission 46 months after diagnosis. None of the children received RT. Chemotherapy may permit long term deferral of RT. More aggressive CHT regimens should be explored in infants with CPC

The article reviews 180 cases of osteoplastic laminotomies, 1 year after operation. The patients were predominantly children undergoing selective dorsal rhizotomies. Also included were a few pediatric patients who received laminotomies for spinal cord tumors. Spinal X-rays were reviewed at 1 and 3 years of follow-up. There was complete to partial bridging of the laminar roof in all cases. This experience confirms that the procedure is well tolerated and is associated with reossification of the replaced bony segment