Faculty Bibliography

BACKGROUND: Meningiomas exhibit a wide range of histomorphologic features, including variable mesenchymal and epithelioid phenotypes. Meningiomas also represent the most common host tumors for systemic metastases, particularly carcinomas. Recently, however, 3 unique dural-based neoplasms were encountered, wherein malignant-appearing gland-like structures were intermixed with meningothelial elements, yet genetic data suggested epithelial metaplasia rather than metastatic carcinoma. OBJECTIVE: To describe and characterize a rare meningioma pattern with potential diagnostic pitfalls. METHODS: In addition to routine clinical, radiologic, and histopathological analyses, cases were studied with immunohistochemistry and fluorescence in situ hybridization (FISH) to elucidate the origins of 2 seemingly disparate tumoral components. RESULTS: Immunohistochemistry confirmed an epithelial ontogeny of gland-like structures, with extensive CK7 positivity suggesting possible lung or breast primaries. However, identical losses of chromosomes 1p, 14q, and 22q in meningothelial and epithelial components were identified by FISH, an observation consistent with a monoclonal derivation and supporting the diagnosis of malignant meningioma with adenocarcinoma-like metaplasia. Although this phenomenon was reminiscent of gland-like metaplasia in secretory meningioma, it differed in that the gland-forming cells were cytologically malignant, formed extracellular rather than intracellular lumina, and were unassociated with pseudopsammoma bodies. Nevertheless, intermingled secretory and adenocarcinoma-like features were seen in one case, suggesting some relationship between these 2 forms of epithelial metaplasia. CONCLUSION: Recognition of adenocarcinoma-like metaplasia in meningiomas can prevent a misdiagnosis of metastatic carcinoma, with all its associated implications for patient management.

Hydrocephalus caused by stenosis of the foramen of Monro is rare. The authors describe a 28-year-old female patient with bilateral foraminal stenosis treated using endoscopic septostomy and unilateral foraminal balloon plasty (foraminoplasty). The patient's hydrocephalus and symptoms resolved postoperatively. Endoscopic strategies may be employed as first-line therapy in this condition

OBJECT: Selective dorsal rhizotomy (SDR) is a highly effective and well-established surgical tool for correction of lower-extremity spasticity in children with spastic diplegia caused by cerebral palsy (CP). Although the literature demonstrates considerable immediate and long-term functional benefits in children treated with SDR, the effects of SDR on adults with spastic diplegia have not been thoroughly investigated. The purpose of this retrospective study was to examine the objective and subjective clinical outcomes of SDR on an adult population. METHODS: We reviewed the charts of 21 consecutive adult patients who underwent SDR for treatment of CP-related spastic diplegia between the years of 1989 and 2007. All patients were treated by a single surgeon (T.S.P.) and underwent formal pre- and postoperative physical therapy assessments to examine joint range of motion (ROM), gross motor function measure (GMFM), and muscle tone. The majority of patients (15/21) exhibited preoperative ambulatory independence without an assistive device. Postoperative assessments were performed at 4 months, but most patients (11/21) had longer follow-up periods (mean, 17.6 +/- 30.2 months). All patients were assessed with a telephone survey to estimate pre- and postoperative function with the Katz and Lawton Activities of Daily Living (ADL) Scale. RESULTS: After SDR surgery, patients experienced significant improvements in lower-extremity passive joint ROM (namely, decreases in hamstring and gastrocnemius tightness) as well as in GMFM crawling and kneeling scores. In addition, spasticity in all measured lower-extremity muscle groups was decreased as compared with preoperative levels. On the basis of our patient self-assessments conducted via telephone, each patient demonstrated subjective improvements in ambulatory ability, spasticity, coordination, joint ROM, pain, overall quality of life, and independence. Also, the Lawton total instrumental ADL scale scores were subjectively improved from preoperative levels. We documented no complications, including postoperative sensory deficits, in any of our patients. CONCLUSIONS: Our experience suggests that SDR can be an effective treatment for CP-related spastic diplegia in ambulatory adults who are unresponsive to medical therapy and should be considered as a therapeutic option in carefully selected patients. Although our study represents the largest series of adult patients with spastic diplegia to date treated with SDR, the data collected will need to be validated in a larger, prospective clinical trial.

OBJECT: Controversy persists concerning the optimal treatment of craniopharyngiomas in children, and no standard outcome metric exists for comparison across treatment modalities, nor is there one that adequately reflects the multisystem dysfunction that may arise. METHODS: The authors retrospectively analyzed the records of 86 consecutive children who underwent a uniform treatment paradigm of attempted radical resection performed by a single surgeon. Excluding 3 perioperative deaths and 3 patients with inadequate follow-up, 80 children (34 girls and 46 boys; mean age 9.56 years; mean follow-up 9.6 years) composed the study group (53 primary and 27 previously treated/recurrent tumors). Building on existing classification schemes proposed by De Vile for hypothalamic dysfunction and Wen for overall functional outcome, the authors devised a more nuanced classification system (Craniopharyngioma Clinical Status Scale [CCSS]) that assesses outcome across 5 axes, including neurological examination, visual status, pituitary function, hypothalamic dysfunction, and educational/occupational status at last follow-up (there is a 4-tiered grading scale in each domain, with increasing values reflecting greater dysfunction). RESULTS: There was a significant increase in pituitary dysfunction following treatment-consistent with the high rates of diabetes insipidus and hypopituitarism common to the surgical management of craniopharyngiomas-and less dramatic deterioration in hypothalamic function or cognitive domains. Significant improvement in vision was also demonstrated, with no significant overall change in neurological status. Preoperative CCSS scores predicted postoperative outcome better than clinical characteristics like patient age, sex, tumor size, and the location or presence of hydrocephalus. CONCLUSIONS: Preoperative CCSS scores predicted outcome with higher accuracy than clinical or imaging characteristics. In lieu of randomized trials, the CCSS may provide a useful outcome assessment tool for comparison across treatment paradigms and surgical approaches. Long-term follow-up is critical to the analysis of outcomes of craniopharyngioma treatment, given the often-delayed sequelae of all therapies and the high recurrence rates of these tumors

OBJECT: The aim of this study was to determine the efficacy of venous ultrasonography in screening for deep venous thrombosis (DVT) after subarachnoid hemorrhage (SAH). A large cohort of patients who had suffered SAH was evaluated with the primary end points of ascertaining the incidence of asymptomatic DVT with venous Doppler ultrasonography screening and of identifying risk factors for the development of DVT and subsequent pulmonary embolism. METHODS: Data from patients with aneurysmal SAH who had been admitted to the neurosurgical intensive care unit (ICU) between December 2002 and October 2006 were retrospectively evaluated. Patients who had undergone surgical or endovascular treatment of an aneurysm following SAH and survived > or = 15 days were included in the study. RESULTS: The overall incidence of DVT among the entire study cohort was 18%. A subgroup analysis identified all patients, with or without symptoms for DVT, who had undergone venous Doppler ultrasonography screening. The incidence of asymptomatic DVT was 24%. Univariate analysis of all patients revealed a significant correlation between the risk of DVT and Hunt and Hess grade (r = 0.38, p < 0.0001), Fisher grade (r = 0.31, p < 0.0001), total hospital stay (r = 0.49, p < 0.0001), and number of days in the ICU (r = 0.48, p < 0.0001). Multivariate analysis demonstrated that the total hospital stay and number of days in the ICU were significant predictors of DVT in all patients (p < 0.0001 and p < 0.0002, respectively). In the subgroup of screened patients, Hunt and Hess grade, total hospital stay, and number of days in the ICU were significant predictors of DVT. Although screened patients were more likely to have DVT (chi(2) = 6.0976, p < 0.02), there was no significant difference in the incidence of DVT or pulmonary embolism between patients who did and those who did not undergo routine lower-extremity Doppler ultrasonography screening. CONCLUSIONS: Routine compressive venous Doppler ultrasonography is an efficient, noninvasive means of identifying DVT as a screening modality in both symptomatic and asymptomatic patients following aneurysmal SAH. The ability to confirm or deny the presence of DVT in this patient population allows one to better identify the indications for chemoprophylaxis. Prophylaxis for venous thromboembolism in neurosurgical patients is common. Emerging literature and anecdotal experience have exposed risks of complications with prophylactic anticoagulation protocols. The identification of patients at high risk-for example, those with asymptomatic DVT-will allow physicians to better assess the role of prophylactic anticoagulation.

OBJECTIVE: Cranial dural arteriovenous fistulae (dAVF) with cortical venous drainage (CVD) (Borden Types 2 and 3) are reported to carry a 15% annual risk of intracranial hemorrhage (ICH) or nonhemorrhagic neurological deficit (NHND). The purpose of this study was to compare the clinical course of Type 2 and 3 dAVFs that present with ICH or NHND with those that do not. METHODS: Twenty-eight patients with Type 2 or 3 dAVFs were retrospectively evaluated. CVD was classified as asymptomatic (aCVD) if patients presented incidentally or with pulsatile tinnitus or orbital phenomena. CVD was classified as symptomatic (sCVD) if patients presented with ICH or NHND. Occurrence of new ICH or new or worsening NHND between diagnosis and disconnection of CVD or last follow-up (if not disconnected) was noted. Overall frequency of events was compared using Fisher's exact test. Cumulative, event-free survival was compared using Kaplan-Meier analysis with log-rank testing. RESULTS: Of 17 patients with aCVD, 1 (5.9%) developed ICH and none experienced NHND or death during the median 31.4-month follow-up period. Of 11 patients with sCVD, 2 (18.2%) developed ICH and 3 (27.3%) experienced new or worsened NHND over the median 9.7-month follow-up period. One of these patients subsequently died. Overall frequency of ICH or NHND was significantly lower in patients with aCVD versus sCVD (P = 0.022). Respective annual event rates were 1.4 versus 19.0%. aCVD patients had significantly higher cumulative event-free survival (P = 0.0016). CONCLUSION: Cranial dAVFs with aCVD may have a less aggressive clinical course than those with sCVD.

OBJECT: The clinical characteristics and overall outcome in patients with spontaneous isolated convexity subarachnoid hemorrhage (SAH) are not well described in the literature. The purpose of this study was to examine the mode of presentation, common origins, radiographic findings, and clinical course in a large case series of such patients. METHODS: A retrospective single-center chart review of all patients in whom nontraumatic primary convexity SAH was diagnosed between 2002 and 2007 was performed. Twenty patients were identified and analyzed for presenting symptoms, radiological and laboratory findings, hospital course, and outcome. RESULTS: There were 15 women and 5 men in our series, and the mean age was 52 years (range 18-86 years). The most common presenting symptom was headache, with 15 patients experiencing it as a chief complaint. Other frequent manifestations included altered mental status (8 patients), focal neurological deficits (7), and seizure (4 patients). An underlying cause of the hemorrhage was identified in 13 cases, whereas the remainder went unresolved. Of the known causes, 5 were due to posterior reversible encephalopathy syndrome, 3 were caused by thrombocytopenia or anticoagulation, and the remainder were isolated cases of lupus vasculitis, drug-induced vasculopathy, postpartum cerebral angiopathy, hypertensive microangiopathy, and Call-Fleming syndrome. All patients with unknown disease origins had favorable outcomes, whereas 8 of 13 patients with an identifiable underlying disorder experienced favorable outcomes. CONCLUSIONS: Spontaneous isolated convexity SAH is rarely caused by aneurysm rupture, has a distinct mode of presentation, and generally carries a more favorable prognosis than that of aneurysmal SAH.