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OBJECTIVE: A) To describe an improved methodology for continuously monitoring the functional integrity of facial nerve by eliciting facial corticobulbar motor-evoked potentials (FCoMEP) and B) To establish the prognosis of facial nerve function based on changes in FCoMEP during skull base surgery.METHODS: Intraoperative monitoring of FCoMEP performed in 100 patients. Previously published methodology has been improved upon by a) doing preoperative mapping of the facial nerve, b) facilitating the corticobulbar tract (CBT) by continuous transcranial electrical stimulation (TES) at 2Hz repetition rate, c) recording from multiple facial nerve innervated muscles, and d) eliciting blink reflex (BR). We analyzed changes in FCoMEP, comparing them with the clinical facial nerve outcome scored with the House-Brackman (HB) scale.RESULTS: The monitorability rate was 100%. Out of 100 patients, nine presented a new facial deficit after surgery. Eight of these showed significant changes in FCoMEP. In four patients FCoMEPs were lost ; they presented a complete facial paralysis from which they did not recover. To discriminate the prognosis of patients, ROC analysis identified a cut-off at 65% for FCoMEPs amplitude decrease with a sensitivity of 89% and specificity of 99%. In four patients FCoMEP showed a decrease in amplitude greater than 65%, and they presented mild/moderate facial paresis that was transient. One patient did not present changes in FCoMEP but had a mild facial paresis from which the patient recovered.CONCLUSIONS: The improved methodology allows the maximum rate of monitorability and minimizes false positive and false negative results. This study shows that prognosis of facial nerve may be reliably established based on FCoMEP parameters.SIGNIFICANCE: We improved the previously described methodology for continuously monitoring the functional integrity of the facial nerve by increasing the monitorability rate, and we describe the impact of FCoMEP intraoperative management of facial nerve. This method may permit establishing the short-term and long-term prognosis of facial nerve function in skull base surgery.

Inflammatory orbital lesions include a broad list of diagnoses, many of them with overlapping clinical and radiographic features. They often present a diagnostic conundrum, even to the most experienced orbital specialist, thus placing considerable weight on surgical biopsy and histopathological analysis. However, histopathological diagnosis is also inherently challenging due to the rarity of these lesions and the overlaps in histologic appearance among distinct disease entities. We herein present the case of an adolescent male with a subacutely progressive orbital mass that generated a significant diagnostic dilemma. Early orbital biopsy was consistent with a benign fibro-inflammatory lesion, but corticosteroid therapy was ineffective in halting disease progression. After an initial substantial surgical debulking, histopathological analysis revealed several key features consistent with IgG4-related disease (IgG4-RD), a systemic fibro-inflammatory process typically accompanied by multifocal tumor-like lesions. Surprisingly, within months, there was clear evidence of clinical and radiographic disease progression despite second-line rituximab treatment, prompting a second surgical debulking. This final specimen displayed distinctive features of Rosai-Dorfman disease (RDD), a systemic inflammatory disease characterized by uncontrolled histiocytic proliferation. Interestingly, certain features of this re-excision specimen were still reminiscent of IgG4-RD, which not only reflects the difficulty in differentiating RDD from IgG4-RD in select cases, but also illustrates that these diagnoses may exist along a spectrum that likely reflects a common underlying pathogenetic mechanism. This case emphasizes the importance of surgical biopsy or resection and histopathological analysis in diagnosing—and, ultimately, treating—rare, systemic inflammatory diseases involving the orbit, and, furthermore, highlights the shared histopathological features between RDD and IgG4-RD.

Background Intraosseous petrous apex schwannomas are an exceedingly rare entity; little is known about their epidemiology, natural history, and postoperative outcomes. Case Description Here, we present the fourth known case of a primary intraosseous schwannoma of the petrous apex: a 68-year-old woman presenting with diplopia, facial numbness, progressive intermittent vertigo, tinnitus, diminished hearing, and ataxia. She underwent a transtemporal approach for subtotal resection of the tumor with subsequent stereotactic radiosurgery. Conclusions Our 2-year follow-up demonstrates slow growth and success of multimodal management in the treatment of these tumors. We review the 3 prior reports of petrous apex schwannomas and identify unifying radiographic and clinical characteristics to aid future diagnostic considerations of lesions of the petrous apex.

The prognostic value of mutations in G-protein genes GNAQ and GNA11 in patients with intracranial and orbital melanocytomas is unknown. The authors present a case of GNA11 mutation (GNA11Q209L) in a 32-year-old male suffering from a meningeal melanocytoma with orbital involvement and ipsilateral Nevus of Ota. The patient underwent gamma knife stereotactic radiosurgery without biopsy and later partial transcranial resection of the melanocytic tumor that was subject to immunohistochemical and molecular analysis. A 50-gene next-generation sequencing panel revealed a 626A>T mutation in the GNA11 gene. One year later, intracranial extension of the melanocytoma necessitated a ventriculoperitoneal shunt and immunotherapy. Future work is needed to determine how GNA11 mutations in melanocytomas influence prognosis and monitoring strategies.

Inhibition of mTORC1 signaling has been shown to diminish growth of meningiomas and schwannomas in preclinical studies, and clinical data suggest that everolimus, an orally administered mTORC1 inhibitor, may slow tumor progression in a subset of patients with neurofibromatosis type 2 (NF2) with vestibular schwannoma. To assess the pharmacokinetics, pharmacodynamics, and potential mechanisms of treatment resistance, we performed a presurgical (phase 0) clinical trial of everolimus in patients undergoing elective surgery for vestibular schwannoma or meningiomas. Eligible patients with meningioma or vestibular schwannoma requiring tumor resection enrolled on study received everolimus 10 mg daily for 10 days immediately prior to surgery. Everolimus blood levels were determined immediately before and after surgery. Tumor samples were collected intraoperatively. Ten patients completed protocol therapy. Median pre- and postoperative blood levels of everolimus were found to be in a high therapeutic range (17.4 ng/mL and 9.4 ng/mL, respectively). Median tumor tissue drug concentration determined by mass spectrometry was 24.3 pg/mg (range, 9.2–169.2). We observed only partial inhibition of phospho-S6 in the treated tumors, indicating incomplete target inhibition compared with control tissues from untreated patients (P = 0.025). Everolimus led to incomplete inhibition of mTORC1 and downstream signaling. These data may explain the limited antitumor effect of everolimus observed in clinical studies for patients with NF2 and will inform the design of future preclinical and clinical studies targeting mTORC1 in meningiomas and schwannomas.

OBJECTIVE Meningiomas that arise primarily within the cavernous sinus are often believed to be more indolent in their growth pattern. Despite this perceived growth pattern, disabling symptoms can arise even with small tumors. While research has been done on cavernous sinus meningiomas (CSMs) and their treatment, very little is known about their natural growth rates. With a better understanding of the growth rate of CSM, patient treatment and guidance can be can optimized and individualized. The goal of this study was to determine volumetric growth rates of untreated CSMs. METHODS Thirty-seven patients with 166 MR images obtained between May 2004 and September 2019 were reviewed, with a range of 2–13 MR images per patient (average of 4.5 MR images per patient). These scans were obtained over an average follow-up period of 45.9 months (median 33.8, range 2.8–136.9 months). All imaging prior to any intervention was included in this analysis. Volumetric measurements were performed and assessed over time. RESULTS The estimated volumetric growth rate was 23.3% per year (95% CI 10.2%–38.0%, p < 0.001), which is equivalent to an estimated volume doubling time (VDT) of 3.3 years (95% CI 2.1–7.1 years). There was no significant relationship between growth rate and patient age (p = 0.09) or between growth rate and patient sex (p = 0.78). The median absolute growth rate was 41% with a range of −1% to 1793%. With a definition of “growth” as an increase of greater than 20% during the observed period, 65% of tumors demonstrated growth within their observation interval. Growth rates for each tumor were calculated and tumors were segmented based on growth rate. Of 37 patients, 22% (8) demonstrated no growth (< 5% annual growth, equivalent to a VDT > 13.9 years), 32% (12) were designated as slow growth (annual growth rate 5%–20%, VDT 3.5–13.9 years), 38% (14) were found to have medium growth (annual growth rate 20%–100%, VDT 0.7–3.5 years), and 8% were considered fast growing (annual growth rate > 100%, VDT < 0.7 years). CONCLUSIONS This study evaluated CSM volumetric growth rates. A deeper understanding of the natural history of untreated CSMs allows for better counseling and management of patients.

Soon after the World Health Organization declared the severe acute respiratory syndrome coronavirus 2 a global health emergency on January 30, 2020, New York City was plagued by the virus and its health system and economy pushed to their limits. The majority of the limited neurosurgical data in relation to COVID-19 is anecdotal and the higher theoretical risk of transmission of the virus among skull base aerosol generating (SBAG) cases has not been investigated or discussed in a neurosurgical population. We discuss a series of 13 patients who underwent 15 SBAG surgical procedures during the peak of COVID-19 in our hospital system and the protocols use perioperatively for their procedures. Our data support that with proper preoperative testing, a well-delineated surgical algorithm, and appropriate personal protective equipment, emergent/urgent cases can be done safely in hospitals that are currently experiencing high volumes of COVID-19 cases as we did in March to May of 2020.

Background and purpose Preoperative localization of the pituitary gland with imaging in patients with macroadenomas has been inadequately explored. The pituitary gland enhancing more avidly than a macroadenoma has been described in the literature. Taking advantage of this differential enhancement pattern, our aim was to evaluate the role of high-resolution dynamic MR imaging with golden-angle radial sparse parallel reconstruction in localizing the pituitary gland in patients undergoing trans-sphenoidal resection of a macroadenoma. Materials and methods A retrospective study was performed in 17 patients who underwent trans-sphenoidal surgery for pituitary macroadenoma. Radial volumetric interpolated brain examination sequences with golden-angle radial sparse parallel technique were obtained. Using an ROI-based method to obtain signal-time curves and permeability measures, 3 separate readers identified the normal pituitary gland distinct from the macroadenoma. The readers' localizations were then compared with the intraoperative location of the gland. Statistical analyses were performed to assess the interobserver agreement and correlation with operative findings. Results The normal pituitary gland was found to have steeper enhancement-time curves as well as higher peak enhancement values compared with the macroadenoma (P Conclusions This study confirms our ability to consistently and accurately identify the normal pituitary gland in patients with macroadenomas with the golden-angle radial sparse parallel technique with quantitative permeability measurements and enhancement-time curves.

BACKGROUND: Inhibition of mTORC1 signaling has been shown to diminish growth of NF2 deficient tumors in preclinical studies, and clinical data suggest that everolimus, an orally administered mTORC1 inhibitor, may slow tumor progression in a subset of adult and pediatric NF2 patients with VS. To assess the pharmacokinetics, pharmacodynamics and potential mechanisms of treatment resistance, we performed a pre-surgical (“phase 0”) clinical trial of everolimus in patients undergoing surgery for VS or meningiomas. METHODS: Eligible patients with meningioma or VS requiring tumor resection received everolimus 10 mg daily for 10 days immediately prior to surgery. Everolimus blood levels were determined immediately prior to and after surgery. Tumor samples were collected intraoperatively. RESULTS: Ten patients completed protocol therapy, including 5 patients with NF2-related meningioma, 3 patients with sporadic meningioma, and 2 patients with NF2-related VS. Median pre- and post-operative plasma levels of everolimus were found to be in a high therapeutic range (17.4 ng/ml and 9.4 ng/ml, respectively). Median tumor tissue drug concentration determined by mass spectrometry was 24.3 ng/g (range 9.2–169.2), and median tumor tissue to post-operative plasma drug concentration ratio was 0.39. We observed only partial inhibition of phospho-S6 in the treated tumors, indicating incomplete target inhibition compared to matched control tissues from untreated patients (p = 0.005). Consistent with prior observations that inhibition of mTORC1 may lead to MAPK pathway activation through a PI3K-dependent feedback loop, we observed a statistically significant increase of phospho-ERK (p < 0.03) versus untreated controls. CONCLUSIONS: In patients with meningioma or VS, treatment with everolimus leads to incomplete inhibition of mTORC1 signaling and upregulation phospho-ERK. These data may explain the limited anti-tumor effect of everolimus observed in clinical studies for NF2 patients and identify upregulation of phospho-ERK as a likely resistance mechanism that could be addressed with combination therapies.

Background While effective for the repair of large skull base defects, the Hadad-Bassagasteguy nasoseptal flap increases operative time and can result in a several-week period of postoperative crusting during re-mucosalization of the denuded nasal septum. Endoscopic transsphenoidal surgery for pituitary adenoma resection is generally not associated with large dural defects and high-flow cerebrospinal fluid (CSF) leaks requiring extensive reconstruction. Here, we present the posterior nasoseptal flap as a novel technique for closure of skull defects following endoscopic resection of pituitary adenomas. This flap is raised in all surgeries during the transnasal exposure using septal mucoperiosteum that would otherwise be discarded during the posterior septectomy performed in binostril approaches. Methods We present a retrospective, consecutive case series of 43 patients undergoing endoscopic transsphenoidal resection of a pituitary adenoma followed by posterior nasoseptal flap placement and closure. Main outcome measures were extent of resection and postoperative CSF leak. Results The mean extent of resection was 97.16 ± 1.03%. Radiographic measurement showed flap length to be adequate. While a defect in the diaphragma sellae and CSF leak were identified in 21 patients during surgery, postoperative CSF leak occurred in only one patient. Conclusions The posterior nasoseptal flap provides adequate coverage of the surgical defect and is nearly always successful in preventing postoperative CSF leak following endoscopic transsphenoidal resection of pituitary adenomas. The flap is raised from mucoperiosteum lining the posterior nasal septum, which is otherwise resected during posterior septectomy. Because the anterior septal cartilage is not denuded, raising such flaps avoids the postoperative morbidity associated with the larger Hadad-Bassagasteguy nasoseptal flap.

The craniovertebral junction is a complex region that includes the lower clivus, foramen magnum, C1, and C2. Chordomas and chondrosarcomas are slowly growing tumors that arise from notochord rests in the clivus and cartilaginous synchondrosis, respectively. Given their slow growth, they are often large and involve multiple anatomic compartments at clinical presentation. The extreme lateral approach is ideal for accessing tumors that involve the lower clivus, C1/C2, and the retropharyngeal space. Limitations of this approach are access to the contralateral condyle and the clivus above the jugular foramen. Tumors that extend beyond the limits of the approach should be treated with a combined approach. If the occipital condyle or C1/C2 joint are resected, occipital cervical fusion is necessary. Careful planning of the order and timing of multiple approaches and stabilization is necessary for these complex tumors.

BACKGROUND Inhibition of mTORC1 signaling has been shown to diminish growth of NF2 deficient tumors in preclinical studies, and clinical data suggest that everolimus, an orally administered mTORC1 inhibitor, may slow tumor progression in a subset of adult and pediatric NF2 patients with VS. To assess the pharmacokinetics, pharmacodynamics and potential mechanisms of treatment resistance, we performed a pre-surgical (“phase 0”) clinical trial of everolimus in patients undergoing surgery for VS or meningiomas. METHODS Eligible patients with meningioma or VS requiring tumor resection received everolimus 10 mg daily for 10 days immediately prior to surgery. Everolimus blood levels were determined immediately prior to and after surgery. Tumor samples were collected intraoperatively. RESULTS Ten patients completed protocol therapy, including 5 patients with NF2-related meningioma, 3 patients with sporadic meningioma, and 2 patients with NF2-related VS. Median pre- and post-operative plasma levels of everolimus were found to be in a high therapeutic range (17.4 ng/ml and 9.4 ng/ml, respectively). Median tumor tissue drug concentration determined by mass spectrometry was 24.3 ng/g (range 9.2–169.2), and median tumor tissue to post-operative plasma drug concentration ratio was 0.39. We observed only partial inhibition of phospho-S6 in the treated tumors, indicating incomplete target inhibition compared to matched control tissues from untreated patients (p = 0.005). Consistent with prior observations that inhibition of mTORC1 may lead to MAPK pathway activation through a PI3K-dependent feedback loop, we observed a statistically significant increase of phospho-ERK (p < 0.03) versus untreated controls. CONCLUSIONS In patients with meningioma or VS, treatment with everolimus leads to incomplete inhibition of mTORC1 signaling and upregulation phospho-ERK. These data may explain the limited anti-tumor effect of everolimus observed in clinical studies for NF2 patients and identify upregulation of phospho-ERK as a likely resistance mechanism that could be addressed with combination therapies.

There is evidence that primary hemifacial spasm (HFS) in the majority of patients is related to a vascular compression of the facial nerve at its root exit zone (REZ). As a consequence, the hyperexcitability of facial nerve generates spasms of the facial muscles. Microvascular decompression (MVD) of the facial nerve near its REZ has been established as an effective treatment of HFS. Intra-operative disappearance of abnormal muscle responses (lateral spread) elicited by stimulating one of the facial nerve branches has been used as a method to predict MVD effectiveness. Other neurophysiologic techniques, such as facial F-wave, blink reflex and facial corticobulbar motor evoked potentials (FCoMEP), are feasible to intra-operatively study changes in excitability of the facial nerve and its nucleus during MVDs. Intra-operative neuromonitoring with the mentioned techniques allows a better understanding of HFS pathophysiology and helps to optimise the MVD.

Background An intracranial posterior circulation revascularization procedure in the form of a side-to-side in situ posterior inferior cerebellar artery (PICA)-PICA bypass operation was introduced in 1991. This elegant and apparently low-risk operation is performed infrequently. Thus, the operative nuances used in this procedure have not been well reported, limiting the scope of treatment modalities of vertebral artery-PICA aneurysms and vertebral dissections. Objective To repair an incidental right-sided PICA aneurysm noted in a 51-year-old woman in magnetic resonance imaging and subsequent angiography. Methods The patient underwent side-to-side in situ PICA-PICA bypass surgery. Results Immediate indocyanine green angiography suggested that the PICA distal to the aneurysms was filling in a retrograde fashion through the bypass. On the following day, the patient was taken for coil embolization of the aneurysm. However, angiography images revealed that the aneurysm was spontaneously thrombosed, the proximal PICA was patent, and the PICA distal to the aneurysms was filling in a retrograde fashion, as suspected in intraoperative indocyanine green angiography. No further treatments were done. The patient recovered fully. Conclusion We describe in detail the preoperative evaluation, decision process, and operative techniques for a side-to-side in situ PICA-PICA bypass operation, which is a relatively safe and elegant posterior circulation bypass procedure.

Object Chordomas are rare malignant neoplasms arising predominantly at the sacrum and skull base. They are uniformly lethal unless treated with aggressive resection and proton beam irradiation. The authors present results of the surgical management of a large number of patients with clivus chordomas. Factors that influence the surgeon's ability to achieve radical tumor resection are also evaluated. Methods Between 1991 and 2005, 71 patients with clivus chordomas underwent surgery. The average follow-up was 66 months (median 60 months, range 3–189 months). Sixty-five patients had complete records that were analyzed in the present report. Thirty-five percent of them had undergone surgery before being treated by the authors. They were evaluated with MR imaging and CT scanning and underwent surgery utilizing a variety of skull base techniques aimed at achieving radical excision. Many also underwent postoperative radiation, usually in the form of proton beam therapy. The patients were followed up with serial imaging at regular intervals as well as with neurological evaluation. Results Radical tumor resection was achieved in 58% of the group. The overall 5-year survival rate was 75%. Radical resection had a positive impact on survival. The ability to achieve radical resection was dependent on the preoperative tumor volume and the number of anatomical areas involved by the tumor. Cranial nerve impairment and CSF leakage were the most frequent postoperative complications. Conclusions Radical excision is the ideal surgical goal in the treatment of clival chordomas and can be achieved with reasonable risks. Several different surgical approaches may be necessary to accomplish this.

The management of adult patients with Chiari malformation associated with syrinx remains controversial. Although an abundance of literature exists for the pediatric population, there is an absence of guidelines for the adult population. It is unclear which of the different surgical approaches is appropriate in patients with Chiari I malformations and syringomyelia. A 36-year-old female patient had a posterior fossa decompression 3 years prior to recurrence. The patient developed recurrent symptoms with sensory loss and hyperesthesia in the right upper extremity. MRI revealed decreased cerebrospinal fluid flow at the craniocervical junction. The patient was taken to the operating room for revision of the posterior fossa decompression, lysis of adhesions and duraplasty. Re-exploration of a Chiari decompression, lysis of adhesions and revision duraplasty is an effective treatment option for recurrent syringomyelia.

Object Intramedullary spinal cord tumors can displace the surrounding neural tissue, causing enlargement and distortion of the normal cord anatomy. Resection requires a midline myelotomy to avoid injury to the posterior columns. Locating the midline for myelotomy is often difficult because of the distorted anatomy. Standard anatomical landmarks may be misleading in patients with intramedullary spinal cord tumors due to cord rotation, edema, neovascularization, or local scar formation. Misplacement of the myelotomy places the posterior columns at risk of significant postoperative disability. The authors describe a technique for mapping the dorsal column to accurately locate the midline. Methods A group of 10 patients with cervical and thoracic intramedullary spinal cord lesions underwent dorsal column mapping in which a strip electrode was used to define the midline. After the laminectomy and durotomy, a custom-designed multielectrode grid was placed on the exposed dorsal surface of the spinal cord. The electrode is made up of 8 parallel Teflon-coated stainless-steel wires (76-μm diameter, spaced 1 mm apart) embedded in silastic with each of the wires stripped of its insulating coating along a length of 2 mm. This strip electrode maps the amplitude gradient of conducted spinal somatosensory evoked potentials elicited by bilateral tibial nerve stimulation. Using these recordings, the dorsal columns are topographically mapped as lying between two adjacent numbers. Results The authors conducted a retrospective analysis of the preoperative, immediate, and short-term postoperative neurological status, focusing especially on posterior column function. There were 8 women and 2 men whose mean age was 52 years. There were 4 ependymomas, 1 subependymoma, 1 gangliocytoma, 1 anaplastic astrocytoma, 1 cavernous malformation, and 2 symptomatic syringes requiring shunting. In all patients the authors attempted to identify the midline by using anatomical landmarks, and then proceeded with dorsal column mapping to identify the midline electrophysiologically. In the 2 patients with syringomyelia and in 5 of the patients with tumors, the authors were unable to identify the midline anatomically with any certainty. In 2 patients with intramedullary tumors, they were able to identify the midline anatomically with certainty. Dorsal column mapping allowed identification of the midline and to confirm the authors' anatomical localization. In 2 patients with intramedullary tumors, posterior column function was preserved only on 1 side. All other patients had intact posterior column function preoperatively. Conclusions Dorsal column mapping is a useful technique for guiding the surgeon in locating the midline for myelotomy in intramedullary spinal cord surgery. In conjunction with somatosensory evoked potential, motor evoked potential, and D-wave recordings, we have been able to reduce the surgical morbidity related to dorsal column dysfunction in this small group of patients.

We report a young man who presented with atrial fibrillation and was subsequently found to have a markedly elevated serum thyroid stimulating hormone level and a solid, noncalcified intrasellar mass on imaging. The patient was treated with thyroid radioablation and hormone replacement and followed for 7 years, during which time the tumor grew to 4.6 cm. He had no visual disturbances or other hormonal symptoms. At transsphenoidal surgery, a tumor consisting of a pituitary adenoma and adamantinomatous craniopharyngiomalike components was resected. Both components were closely intermingled, but there was no evidence of an intermediate morphologic phenotype. Immunohistochemically, the adenoma was not only positive for beta-thyroid stimulating hormone, alpha subunit, and pituitary transcription factor 1, but also stained for beta-follicle stimulating hormone, steroidogenic factor-1, adrenocorticotropic hormone, and pituitary-restricted transcription factor (Tpit), exhibiting an unusual plurihormonal profile. The craniopharyngiomalike component showed immunoreactivity for steroidogenic factor-1, one of the pituitary transcription factors. This lesion may represent an unusual composite tumor attributable to divergent differentiation of a common precursor. Alternatively, it may be viewed as a pituitary adenoma showing metaplastic change analogous to the development of squamous cell nests of the pars tuberalis from adenohypophyseal endocrine cells.

Object Chordomas of the cervical spine are rare tumors. Although en bloc resection has proven to be the ideal procedure in other areas, there is controversy regarding this approach in the cervical spine. The goal in this study was to determine whether piecemeal tumor resection was efficient in the management of chordomas that arise in this location. Methods The authors retrospectively reviewed all 74 cases of chordoma treated by their group. Seven patients with isolated cervical chordomas who were treated between October 1992 and January 2006 were identified. There were four male and three female patients, whose ages ranged from 6 to 61 years (mean 34.4 years). Follow-up duration ranged from 7 to 169 months (median 23 months). All cases were managed using a retrocarotid approach with mobilization of the vertebral artery. When the tumor could not be completely resected via the initial anterior approach, a subsequent posterior resection was performed. Tumor resection was intralesional in all cases, and gross-total tumor resection was achieved in six cases. One patient required a second resection 4 months later. In all cases, a posterior stabilization procedure was performed. Five patients underwent anterior fusion (three with fibular allograft and two with iliac crest), whereas two underwent occipitocervical fusion. In two patients with dedifferentiated chordoma metastasis developed, and one of them died 7 months later. The other patient with metastasis died suddenly at home 26 months postsurgery, presumably from aspiration. At the time of this submission, there were no signs of recurrence in five patients. Conclusions The authors believe that, in most cases, en bloc resection of cervical chordoma is not feasible. This is due to the tendency of chordomas to involve multiple compartments at the time of diagnosis. In the authors' experience, intralesional radical resection remains an effective surgical approach to this disease entity.

Cerebrospinal fluid (CSF) leaks result from a communication between the subarachnoid space and the upper aerodigestive tract. Because of the risk of complications such as meningitis, brain abscess, and pneumocephalus, all persistent CSF leaks should be repaired. Surgical repair may be achieved transcranially or extracranially using a wide variety of autogenous, allogenic, and synthetic patching materials. We report our results with a transnasal transsphenoidal endoscopic approach for the repair of CSF leaks coupled with a multilayer closure using acellular dermis (Alloderm™). We conducted a retrospective review of all patients presenting to our institution over the past 5 years with isolated sphenoid sinus CSF fistulas. Results: Twenty-one patients were included in the study. Nineteen patients (90.5%) had their sphenoid sinus CSF fistula repaired during the first attempt; 2 patients (9.5%) needed a second attempt. The multilayer repair of the CSF leak using acellular dermis via a transsphenoidal endoscopic approach is an effective and successful method of surgical repair of the fistula site. Neither the number, size, nor cause of the CSF fistula affected surgical outcomes. However, the presence of hydrocephalus was a significant negative variable, altering the surgical outcomes of our patients. The acellular dermis offers the advantage of not requiring autogenous tissue for the effective repair of CSF leaks in the sphenoid sinus.

The local invasiveness and occasional rapid growth of chordomas, despite optimal treatment, highlight the need to develop ways to predict their biologic behavior. Alterations in adhesion proteins have been shown to participate in proliferation, invasiveness, and metastasis in epithelial tumors. We therefore analyzed the expression of E-cadherin, N-cadherin, as well as their cytosolic binding proteins alpha-catenin, beta-catenin, and gamma-catenin, in 51 paraffin archived and 17 cryopreserved chordoma specimens. In the majority of chordomas, E-cadherin and N-cadherin expression was inversely correlated, whereas beta-catenin and gamma-catenin expression was directly correlated. By multivariate analysis, N-cadherin up-regulation correlated with a diminished recurrence-free survival, and E-cadherin down-regulation strongly correlated with increased probabilities of death as determined by the Kaplan-Meier log-rank test. There was a 3.28-fold increased probability of having a tumor recurrence and a 10.98-fold increased probability of dying when, respectively, N-cadherin was up-regulated and E-cadherin down-regulated. These results suggest that changes in the relative expression of the cadherin-catenin complex reflect chordoma aggressiveness; and that decreased expression of E-cadherin and increased expression of N-cadherin may underlie the transition from a less to a more aggressive tumor phenotype.

Sphenoorbital meningiomas (SOMs) are complex tumors involving the sphenoid wing, orbit, and cavernous sinus, which makes their complete resection difficult or impossible. Sphenoidal hyperostosis that results in incomplete resection makes these tumors prone to high rates of recurrence with postoperative morbidity resulting in a nonfunctional globe. High-dose radiation therapy has often been described as the only treatment capable of achieving tumor control, although often at the expense of the patient's progressive visual deterioration.This series consisted of 25 patients who were retrospectively analyzed over a 12-year period. Visual function was evaluated pre- and postoperatively in all patients. A standardized surgical approach to a frontotemporal craniotomy and orbitozygomatic osteotomy with intra- and extradural drilling of the optic canal and all the hyperostotic bone was performed. Orbital and cranial reconstruction was performed in all patients. The follow-up period was 6 months to 12 years (average 5 years). The patients presented with the classic triad of SOM: proptosis (86%), visual impairment (78%), and ocular paresis (20%). A gross-total resection was achieved in 70% of patients with surgery limited by the superior orbital fissure and the cavernous sinus. Proptosis improved in 96% of patients with 87% improvement in visual function. Ocular paresis improved in 68%, although 20% of patients experienced a temporary ocular paresis postoperatively. There were no perioperative deaths or morbidity related to the surgical approach or reconstruction. Ninety-five percent of patients reported an improved functional orbit. There was tumor recurrence in 8% of patients; in one case recurrence was delayed for longer than 11 years.Sphenoorbital meningiomas are a distinct category of tumors complicated by potentially extensive hyperostosis of the skull base. Successful resection requires extensive intra- and extradural surgery, necessitating drilling of the optic canal and an orbital osteotomy within anatomical limitations. The bone resection requires reconstruction with autograft, allografts, or alloplast for improved orbital function. All aspects of the clinical triad improved. A radical resection can be achieved with low morbidity, providing a significantly improved clinical outcome in the long-term period.

The subtemporal and preauricular infratemporal fossa approach provides direct access to the middle cranial fossa, petrous bone, clivus, and cavernous sinus. This approach is ideally suited for use in the resection of chordomas and chondrosarcomas because most are extradural tumors. This approach requires a thorough knowledge of the surgical anatomy of the middle cranial fossa, cavernous sinus, temporal bone, and clivus. A frontotemporal craniotomy is made, and an orbitozygomatic osteotomy is performed to provide a low approach to this area. Dissection is performed extradurally under the temporal lobe to identify the petrous segment of the internal carotid artery. The artery is exposed completely in its entire course through the temporal bone and displaced laterally. The temporal bone and clivus are drilled medial to the petrous internal carotid artery. The surgeon approaches the area in front of the brainstem from an anterolateral direction.

OBJECTIVEThe objective of this study was to determine the utility and safety of rigid endoscopy as an adjunct during posterior fossa surgery to treat cranial neuropathies.METHODSA suboccipital craniotomy was performed for 19 patients with non-neoplastic processes involving the Vth, VIIth, and/or VIIIth cranial nerves. Ten patients with trigeminal neuralgia (n = 8), hemifacial spasm (n = 1), or intractable tinnitus (n = 1) underwent primarily microvascular decompression procedures. One patient with geniculate neuralgia underwent nervus intermedius sectioning combined with microvascular decompression. Eight patients underwent unilateral vestibular nerve neurectomies for treatment of Ménière's disease. A 0- or 30-degree rigid endoscope was used in conjunction with the standard microscopic approach for all procedures.RESULTSAll patients experienced resolution or significant improvement of their preoperative symptoms after posterior fossa surgery. The endoscope allowed improved definition of anatomic neurovascular relationships without the need for significant cerebellar or brainstem retraction. Cleavage planes between the cochlear and vestibular nerves entering the internal auditory canal and sites of vascular compression could not be microscopically observed for several patients; however, endoscopic identification was possible for all patients. There were no complications related to the use of the endoscope.CONCLUSIONThe rigid endoscope can be used safely during posterior fossa surgery to treat cranial neuropathies, and it allows improved observation of the cranial nerves, nerve cleavage planes, and vascular anatomic features without significant cerebellar or brainstem retraction.

OBJECTIVEOur goals were to study the normal histological features of the jugular foramen, compare them with the histopathological features of glomus tumors involving the temporal bone, and thus provide insight into the surgical management of these tumors with respect to cranial nerve function.METHODSTen jugular foramen blocks were obtained from five human cadavers after removal of the brain. Microscopic studies of these blocks were performed, with particular attention to fibrous or bony compartmentalization of the jugular foramen, the relationships of the caudal cranial nerves to the jugular bulb/jugular vein and internal carotid artery, and the fascicular structures of the nerves. In addition, we studied the histopathological features of 11 glomus tumors involving the temporal bone (10 patients), with respect to nerve invasion, associated fibrosis, and carotid artery adventitial invasion.RESULTSA dural septum separating the IXth cranial nerve from the fascicles of Cranial Nerves X and XI, at the intracranial opening, was noted. Only two specimens, however, had a septum (one bony and one fibrous) producing internal compartmentalization of the jugular foramen. The cranial nerves remained fasciculated within the foramen, with the vagus nerve containing multiple fascicles and the glossopharyngeal and accessory nerves containing one and two fascicles, respectively. All of these nerve fascicles lay medial to the superior jugular bulb, with the IXth cranial nerve located anteriorly and the XIth cranial nerve posteriorly. All nerve fascicles had separate connective tissue sheaths. A dense connective tissue sheath was always present between the IXth cranial nerve and the internal carotid artery, at the level of the carotid canal. The inferior petrosal sinus was present between the IXth and Xth cranial nerves, as single or multiple venous channels. The glomus tumors infiltrated between the cranial nerve fascicles and inside the perineurium. They also produced reactive fibrosis. In one patient, in whom the internal carotid artery was also excised, the tumor invaded the adventitia.CONCLUSIONWithin the jugular foramen, the cranial nerves lie anteromedial to the jugular bulb and maintain a multifascicular histoarchitecture (particularly the Xth cranial nerve). Glomus tumors of the temporal bone can invade the cranial nerve fascicles, and infiltration of these nerves can occur despite normal function. In these situations, total resection may not be possible without sacrifice of these nerves.

Object The authors analyze their experience with the treatment of 29 patients who underwent radical excision of skull base chordomas. Methods Modern skull base surgical techniques were used in all patients who were treated between August 1991 and July 2000. The degree of tumor resection was gauged according to intraoperative inspection and postoperative high-resolution imaging findings. There were 21 patients with primary disease and eight with recurrent disease. Total resection was accomplished in 18 patients. Five patients had undergone radiotherapy prior to the present surgery, and an additional eight patients underwent postoperative radiotherapy. There were no surgery-related deaths. In five patients who died of the disease, surgery and radiotherapy had failed to effect a cure. Two of the remaining patients are alive with recurrent disease, and there is questionable evidence of recurrence in another patient. All 24 patients are functioning independently. Cranial nerve impairment was the most common postoperative deficit, followed by cerebrospinal fluid (CSF) leakage and infection. Conclusions The use of skull base techiniques in radical surgery provides an opportunity to excise the tumor and the involved bone. In most cases the procedure-related cranial nerve deficits improve over time. The complications of CSF leakage and infection can be minimized and are preventable. Proton beam irradiation is an excellent adjuvant treatment but is reserved for patients with definite tumor recurrence or residual tumor that can be identified on the imaging studies.

Objective: To determine the efficacy and safety of quick-setting hydroxyapatite cement in eliminating cerebrospinal fluid (CSF) rhinorrhea following neurotologic surgery. Study Design: A prospective study of 40 consecutive patients undergoing neurotologic surgery in whom the dura was opened. Setting: All patients were treated as hospital inpatients at a tertiary referral center. Patients: 25 men and 15 women between the ages of 20 and 72 years (mean age 51 years) underwent neurotologic surgery at the parent institution. Intervention: Various neurotologic procedures were performed for the resection of 25 acoustic tumors, 5 meningiomas, 3 glomus tumors, 2 vestibular nerve sections, 2 chordomas, 1 epidermoid tumor, and 1 meningoencephelocele, and for 2 patients referred to our institution with known CSF leaks following acoustic tumor surgery. A new form of quick-setting hydroxyapatite cement, which that hardens within 3 to 5 minutes was used to seal the air cell tracts of the temporal bone in all cases. Main Outcome Measure: The presence of CSF rhinorrhea postoperatively. Results: CSF rhinorrhea occurred in 2 patients following acoustic tumor surgery, the first through an occult air cell tract at the margin of the drilled internal auditory canal, and the second via an oval window fistula 1 month after a translabyrinthine approach. Conclusions: This form of hydroxyapatite cement appears safe, reliable, effective, and economical for the prevention of CSF rhinorrhea following neurotologic surgery. CSF rhinorrhea cannot be eliminated unless our ability to identify all potential air cell tract communications improves.

Background The search for the ideal dural replacement in the setting where autogenous tissues are unavailable or inadequate still persists. Because of the ability of acellular dermis (AlloDerm, LifeCell Corporation, The Woodlands, TX) to remodel itself into native tissue, this dynamic quality is hypothesized to occur when used as a dural replacement. Methods We report the long-term outcome of a small cohort of patients who, to our knowledge, were the first patients to receive AlloDerm for dural replacement. In addition, to put these current findings in a historical perspective, we present a review of the literature for dural replacement. Results Ten patients all successfully underwent duraplasty with AlloDerm with only one postoperative complication that was not related to the acellular dermal dural repair. Conclusions We contend that AlloDerm is a safe and viable option for dural replacement in cases in which autogenous tissues are either unavailable or insufficient for proper reconstruction. © 2000 John Wiley & Sons, Inc. Head Neck 22: 765–771, 2000.

Surgical access to the central and paracentral skull base, infratemporal fossa, nasopharynx and posterior sinus cavities for the management of benign and malignant tumors in these areas is challenging and often results in less than optimal exposure. (J Laryngol Otol 92:949–967, 1978; Skull Base Surg 1:26–32, 1991; Arch Otolaryngol Head Neck Surg 116:92–104, 1990 and 107:698–702, 1981; J Neurosurg 67:488–499, 1987). 1–5 The advent of the osteoplastic maxillotomy and facial translocation procedures, while greatly improving this exposure, also provide the surgeon control of critical anatomic structures in the surgical field and adjacent anatomic areas.

Different surgical approaches to the brain stem with results of excision of brain stem cavernous malformations are reported. Nine patients with brain stem cavernous malformations were operated with six different approaches. The patients were evaluated neurologically, and by magnetic resonance imaging (MRI) examination. All patients had at least one and often more episodes of haemorrhage. Seven patients underwent the total removal of the malformation, without recurrence. One patient with a large medullary cavernoma had recurrence, but without further growth. One patient with a large pontomesencephalic malformation and partial resection, suffered rebleeding due to residual cavernoma. The mean follow-up period was 49 months (range 3-112). The results of surgery were excellent or good in eight patients, and poor (worsening) in one. It is concluded that surgical resection is recommended for the cavernous malformations of the brain stem which are symptomatic, have bled or are growing, and are approachable through one of the pial surfaces of the brain stem. Different skull base approaches are useful for this procedure. Radiosurgery is not recommended in such cases.

BACKGROUND The retinoblastoma (Rb) gene is a well characterized tumor suppressor gene in which loss of heterozygosity has been implicated in a number of malignancies including osteosarcoma and breast carcinoma. Chordomas and chondrosarcomas are rare skull base neoplasms with a propensity for local recurrences, resistance to conventional radiotherapy, and a 5%-30% incidence of metastases. Except for the so called "chondroid chordoma," histologic features do not correlate with the clinical behavior or growth patterns of these tumors. No study to date has investigated what role tumor suppressor genes or oncogenes play in the development and continued growth of these rare neoplasms. METHODS In order to evaluate the role of the retinoblastoma tumor suppressor gene in chordomas and chondrosarcomas we screened seven chordomas and two chondrosarcomas located at the skull base for loss of heterozygosity (LOH) of the Rb gene. Genomic DNA was extracted from tumor specimens as well as matched control tissue and utilizing a polymerase chain reaction technique, intron 17 and 20 were amplified from each specimen. The intron 17 product was then digested with the restriction endonuclease X ba1 followed by electrophoresis on a 1% agrose gel. The intron 20 amplified products were electrophoresed on a nondenaturing 6% polyacrylamide gel. RESULTS We demonstrated LOH at intron 17 of the retinoblastoma gene in 2/7 chordomas and in 0/2 chondrosarcomas. The two chordomas possessing LOH were particularly aggressive tumors demonstrating extensive involvement of the skull base and rapid recurrences following radical resections. CONCLUSIONS Alterations of the Rb gene may play a role in the growth of skull base chordomas with LOH of the Rb gene serving as a marker for more aggressive tumors. This report represents the first study evaluating the Rb gene in chordomas or chondrosarcomas and is the first report of allelic loss of the Rb gene in skull base chordomas.

In this article, the evaluation and management of benign and malignant lesions of the anterior ethmoid and frontal sinuses are discussed, with emphasis on preoperative evaluation, indications and contraindications to surgery, and reconstruction following ablative procedures. Surgical approaches to access this anatomic area are presented, and the role of adjuvant therapy in the treatment of malignant disease is reviewed.