Faculty Bibliography

FROM THE RECORDS of approximately 1500 shunt operations performed between 1987 and 1992, we identified 37 adults between ages 38 and 86 years (mean, 70 yr) with the normal-pressure hydrocephalus (NPH) syndrome who underwent surgery by a single surgeon. Since 1990, we have routinely used a flow-regulated shunt system (Orbis-Sigma valve [OSV]; Cordis Corporation, Miami, FL) in these patients. In this study, we compared the OSV system with conventional differential-pressure (DP) shunt systems uniformly used before 1990. This series (n = 37) consisted of 62% men (n = 23) and 38% women (n = 14). We excluded all patients with hydrocephalus associated with central nervous system neoplasms, intracerebral hemorrhage, or trauma as well those with radiographically documented late-onset aqueductal stenosis. All patients presented with the NPH clinical syndrome, chiefly with magnetic gait. In addition, 75% of patients experienced cognitive loss and 59% experienced urinary incontinence. The mean duration of preoperative symptoms was 35 months (range, 7-120 mo). Eight patients (22%) had undergone previous shunting procedures before referral to our service. A total of 89 shunt operations were performed in the 37 patients. Using actuarial methods and controlling for a history of prior shunt surgery, we found no significant difference in the time to initial malfunction (shunt survival) between the OSV and the DP shunts. There were three subdural hematomas and one infection in the OSV group compared with no complications in the DP valve group (P = 0.11). Thirty-six patients were available for follow-up, at a mean of 14 months after surgery. Nearly 90% of all patients experienced improvement in gait after shunting, regardless of the valve system that was used. There was one unrelated death. Realizing the limitations of a retrospective analysis and on the basis of the limited number of patients in this study, we conclude that using actuarial methods, we found no significant difference in shunt survival when comparing the OSV with the standard DP valve shunt systems with antisiphon devices in patients with NPH. Contrary to previous reports, the OSV is not free of overdrainage complications. Most patients (89%) with the NPH syndrome who primarily presented with gait disorder experienced significant improvement in gait after either OSV or DP shunting procedures when selected for surgery on the basis of the clinical syndrome and confirmatory radiographic data

Future advances in neuro-oncology will increasingly rely on an understanding of the molecular biology of brain tumors. Recent laboratory work, including the identification of oncogenes and tumor suppressor genes, has elucidated many of the molecular events contributing to oncogenesis. In particular, the signaling pathways for the growth factors have been implicated in the genesis and the maintenance of several human tumors, including neoplasms of the central nervous system (CNS). Growth factor autocrine and paracrine stimulatory loops promote tumor proliferation and angiogenesis. A family of structurally related growth factor receptors, the receptor tyrosine kinases, are particularly relevant to tumors of the CNS. This large family includes the receptors for the epidermal growth factor, the platelet-derived growth factor, the fibroblast growth factor, the insulin-like growth factor, the neurotrophins related to the nerve growth factor, and the vascular endothelial growth factor, as well as several receptors for which no growth factor ligand has been identified. Several of these receptor molecules and their growth factor ligands are preferentially expressed in the embryonic brain and are thought to play a central role in regulating the determination of the cell fate during the development of the CNS. Moreover, the overexpression or the mutation of genes encoding these receptors can be oncogenic. Researchers think that some receptors in this family (i.e., those that have been shown to be overexpressed or mutated in human brain tumors) contribute to brain tumor oncogenesis. This article will focus on recent experimental work and will discuss the classification and the biology of the receptor tyrosine kinases, as well as their roles in the development of the CNS and in tumorigenesis

Fourth-ventricular shunting is commonly used to treat symptomatic posterior fossa cysts of the Dandy-Walker malformation and trapped fourth ventricle. Although the benefits of this procedure have been widely reported, there is a paucity of data on the pitfalls of posterior fossa shunting in the neurosurgical literature. During the 4-year period from July 1989 to June 1993, we placed fourth-ventricular shunts in 12 patients. Remarkably, 5 patients suffered complications related to posterior fossa catheter placement (42% rate). Three of these patients developed new cranial nerve dysfunction caused by direct injury to the floor of the fourth ventricle, 1 patient suffered an intracystic hemorrhage and acute shunt malfunction, and 1 patient had the catheter tip in the brainstem on postoperative studies without new neurological deficit. We conclude that placement of fourth-ventricular shunts can be fraught with complications which we believe is related to technique. We propose that altering the trajectory of the ventricular catheter from our usual midline technique to a more lateral position will lessen the chances for injury to the floor of the fourth ventricle. In this manner we hope to decrease our incidence of complications for this procedure

Pathological and clinical data from 56 patients operated on for craniopharyngioma since 1981 were analyzed to determine the utility of dividing patients with this tumor into distinct clinical groups based on recognized pathological type and to determine the prognostic import of brain invasion. Of the tumors in the 30 adult patients, 66% were adamantinomatous, 28% were squamous papillary, and the remainder were mixed. However, of the tumors in the 26 children, 96% were adamantinomatous and none were pure squamous papillary (P < 0.01). Forty-six percent of the children compared with 17% of the adults had brain invasion (P < 0.01). Brain invasion was present in 37% of the adamantinomatous but in only 13% of the squamous papillary tumors. Seventy-seven percent of the children underwent gross total resection (GTR) compared with 27% of the adults (P < 0.01). Sixty-three percent of the squamous papillary tumors underwent GTR compared with 54% of the adamantinomatous and mixed tumors. Follow-up ranged from 7 to 187 months (mean, 49 mo). After subtotal resection, with or without radiation therapy, 58% of the tumors recurred compared with 17% recurrence after GTR (P < 0.01), with a mean time to recurrence of 34 months. In both tumor histological types, subtotal resection was associated with a higher rate of tumor recurrence compared with gross total resection. Among the subtotally resected craniopharyngiomas, 2 of the 3 (67%) squamous papillary and 11 of the 21 (52%) adamantinomatous and mixed tumors recurred. In contrast, among the totally resected tumors, none of the 5 squamous papillary and only 5 of the 25 (20%) adamantinomatous and mixed tumors recurred. There were no significant differences in Karnofsky performance status score, mortality rate, or visual and endocrine outcomes when comparing patients based on histological tumor type. When controlling for age and extent of resection, we found that brain invasion had no significant effect on recurrence rate in totally resected tumors. Based on the limited number of patients in this series, we conclude as follows. 1) Contrary to previous reports, squamous papillary craniopharyngiomas, like adamantinomatous tumors, may recur when subtotally resected. 2) For both tumor variants, the most significant factor associated with craniopharyngioma recurrence is the extent of surgical resection rather than histopathological subtype. 3) Contrary to prior hypotheses, brain invasion in totally resected tumors does not predict higher recurrence. 4) GTR is associated with a significantly lower recurrence rate and can be achieved without sacrificing functional outcome

In 15% of patients with spontaneous subarachnoid hemorrhage (SAH), the source of bleeding cannot be determined despite repeated cerebral angiography. However, some patients diagnosed as having 'SAH of unknown cause' actually harbor undetected aneurysms. The authors report six patients with SAH who, despite multiple negative cerebral angiograms, underwent exploratory surgery due to a high clinical and radiographic suspicion for the presence of an aneurysm. Brain computerized tomography (CT) scans revealed blood located mainly in the basal frontal interhemispheric fissure in four patients, in the sylvian fissure in one patient, and in the interpeduncular cistern in one patient. The patients were evaluated as Hunt and Hess Grades I to III, and had undergone at least two high-quality cerebral angiograms that did not reveal an aneurysm. Vasospasm was visualized in two patients. Three patients rebled while in the hospital. Exploratory surgery was performed at an average of 12 days post-SAH. Five aneurysms were discovered at surgery and were successfully clipped. All four patients with interhemispheric blood were found to have an anterior communicating artery (ACoA) aneurysm. The patient with blood in the sylvian fissure was found to have a middle cerebral artery aneurysm. These aneurysms were partially thrombosed. No aneurysm was detected in the patient with interpeduncular SAH, despite extensive basilar artery exploration. Five patients had an excellent outcome and one patient developed diabetes insipidus. These results show that exploratory aneurysm surgery is warranted, despite repeated negative cerebral angiograms, if the patient manifests the classical signs of SAH with CT scans localizing blood to a specific cerebral blood vessel (particularly the ACoA) and if a second SAH is documented at the same site

Perforation of colonic diverticula is a complication of corticosteroid use that has not been described in the neurosurgical literature. Between 1987 and 1992, 719 patients who underwent surgery for primary and metastatic brain and spinal tumors of the central nervous system received 2246 to 4936 mg of methylprednisolone given over at least 7 days. Five patients in this group (all men, ages 50-69 yr) experienced a sigmoid diverticular perforation at a mean dose of 3947 mg of methylprednisolone (range, 2240-6160 mg). Of these five, two had a known history of diverticular disease. In contrast, during this same period, 3749 patients who underwent neurosurgical procedures for non-neoplastic conditions did not receive corticosteroids and experienced no colonic perforations. All five patients with colonic perforations presented with abdominal pain and had free intraperitoneal air that was revealed on radiographs of the abdomen. Perforation of a sigmoid diverticulum was confirmed in all five at exploratory laparotomy. Four patients had good outcomes, and one died. We conclude the following: 1) patients over age 50 who receive high-dose corticosteroids are at risk for sigmoid colonic perforation, and these medications should be used with caution in such patients; 2) if possible, lower total doses of perioperative corticosteroids should be used in patients with known diverticular disease; and 3) because corticosteroids mask many of the inflammatory signs of perforation, this diagnosis should be considered in any patient with abdominal discomfort, fever of unknown origin, or unexplained leukocytosis

Ninety patients were followed after undergoing multilevel cervical laminectomy for cervical myeloradiculopathy secondary to cervical stenosis. Follow-up data was obtained by chart review and phone interview for all patients; 20% were examined at follow-up as well; 77% of patients were improved, 13% without change, and 10% worse at follow-up. No patients deteriorated in the immediate postoperative period. Greater than 50% of severely disabled patients had an excellent result, and 78% improved somewhat. It is concluded that cervical laminectomy is an effective procedure for treating patients with cervical spondylosis causing myeloradiculopathy. Reasons for failure of this procedure, although rarely identified on follow-up examination, are reviewed and briefly discussed