Tag: Vestibular schwannoma

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Combined use of diffusion tensor tractography and multifused contrast-enhanced FIESTA for predicting facial and cochlear nerve positions in relation to vestibular schwannoma

Combined use of diffusion tensor tractography and multifused contrast-enhanced FIESTA for predicting facial and cochlear nerve positions in relation to vestibular schwannoma

J Neurosurg 123:1480–1488, 2015

The authors assessed whether the combined use of diffusion tensor tractography (DTT) and contrastenhanced (CE) fast imaging employing steady-state acquisition (FIESTA) could improve the accuracy of predicting the courses of the facial and cochlear nerves before surgery.

Methods The population was composed of 22 patients with vestibular schwannoma in whom both the facial and cochlear nerves could be identified during surgery. According to DTT, depicted fibers running from the internal auditory canal to the brainstem were judged to represent the facial or vestibulocochlear nerve. With regard to imaging, the authors investigated multifused CE-FIESTA scans, in which all 3D vessel models were shown simultaneously, from various angles. The low-intensity areas running along the tumor from brainstem to the internal auditory canal were judged to represent the facial or vestibulocochlear nerve.

Results For all 22 patients, the rate of fibers depicted by DTT coinciding with the facial nerve was 13.6% (3/22), and that of fibers depicted by DTT coinciding with the cochlear nerve was 63.6% (14/22). The rate of candidates for nerves predicted by multifused CE-FIESTA coinciding with the facial nerve was 59.1% (13/22), and that of candidates for nerves predicted by multifused CE-FIESTA coinciding with the cochlear nerve was 4.5% (1/22). The rate of candidates for nerves predicted by combined DTT and multifused CE-FIESTA coinciding with the facial nerve was 63.6% (14/22), and that of candidates for nerves predicted by combined DTT and multifused CE-FIESTA coinciding with the cochlear nerve was 63.6% (14/22). The rate of candidates predicted by DTT coinciding with both facial and cochlear nerves was 0.0% (0/22), that of candidates predicted by multifused CE-FIESTA coinciding with both facial and cochlear nerves was 4.5% (1/22), and that of candidates predicted by combined DTT and multifused CE-FIESTA coinciding with both the facial and cochlear nerves was 45.5% (10/22).

Conclusions By using a combination of DTT and multifused CE-FIESTA, the authors were able to increase the number of vestibular schwannoma patients for whom predicted results corresponded with the courses of both the facial and cochlear nerves, a result that has been considered difficult to achieve by use of a single modality only. Although the 3D image including these prediction results helped with comprehension of the 3D operative anatomy, the reliability of prediction remains to be established.

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Prognostic Significance of Peritumoral Edema in Patients With Vestibular Schwannomas

Prognostic Significance of Peritumoral Edema in Patients With Vestibular Schwannomas

Neurosurgery 77:81–86, 2015

Peritumoral edema (PTE) in skull base meningiomas correlates to the absence of an arachnoid plane and difference in outcome. In vestibular schwannomas (VS), PTE and its significance for microsurgery and outcome have never been systematically evaluated.

OBJECTIVE: To evaluate whether PTE correlates with tumor characteristics, the presence of an arachnoid plane, and outcome.

METHODS: A retrospective study of the institutional database. PTE was evaluated on fluid-attenuated inversion recovery magnetic resonance images. Preoperative patient data and intraoperative tumor features (presence of tumor pseudocapsule, vascularity, degree of adhesion/invasion of the arachnoid) were noted. Outcome measures were completeness of removal, neurological outcome, and complication rate. These parameters in patients with PTE (group A) were correlated to those in matched series without edema (group B).

RESULTS: Thirty patients presented with PTE (5%). The mean VS size was 3.4 cm. No major differences in the degree of adhesion or presence of an arachnoid plane were found. VS with PTE were more frequently hypervascular (26.7% in group A vs 6.7% in group B). The presence of PTE in VS was not related to surgical radicality. VS with PTE had worse early postoperative facial nerve function, but at 12 months, there was no major difference. VS with PTE were prone to cause postoperative hemorrhages in the tumor bed.

CONCLUSION: PTE in VS does not correlate with the degree of tumor adhesion and the presence of an arachnoid dissection plane. The radicality of tumor removal and longterm functional outcome in patients with and without PTE was similar. VS with PTE are more vascular and prone to cause postoperative hemorrhages. Therefore, meticulous hemostasis is advisable.

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Hearing preservation surgery for vestibular schwannomas via the retrosigmoid transmeatal approach

Hearing preservation surgery in VS-1

Neurosurg Rev (2014) 37:431–444

Maximum tumor extirpation with preservation of the facial and cochlear nerve function is the goal of surgery for vestibular schwannoma. To preserve cochlear nerve function, the surgeon must employ a detailed knowledge of microanatomy, precise microsurgical techniques, and persistence. This paper describes the “pearls” of surgical techniques based on the anatomical study inside the mastoid from the view of the retrosigmoid transmeatal approach.

A total of 592 consecutive patients underwent surgical removal of unilateral vestibular schwannoma (VS) between January 1994 and December 2009. The hearing preservation rate was 53.7 % for large vestibular schwannomas (>20 mm in diameter) and 74.1 % for tumors of all sizes.

The key procedures for hearing preservation surgery are as follows: bloodless microdissection, sufficient coring-debulking, capsular elevation to locate the facial and cochlear nerves both electrophysiologically and by visual observation, sharp dissection of the facial and cochlear nerves, and avoidance of heat and mechanical injury to the nerves, the internal auditory artery, and the brain stem. Besides these techniques, appropriate instruments are essential to preserve hearing.

The function of the facial and cochlear nerves should be the foremost concern. Meticulous techniques and the knowledge of microsurgical anatomy lead to hearing preservation with maximum tumor removal.

 

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The behavior of residual tumors and facial nerve outcomes after incomplete excision of vestibular schwannomas

 incomplete excision of vestibular schwannomas

J Neurosurg 120:1278–1287, 2014

The authors evaluated the behavior of residual tumors and facial nerve outcomes after incomplete excision of vestibular schwannomas (VSs).

Methods. The case records of all patients who underwent surgical treatment of VSs were analyzed. All patients in whom an incomplete excision had been performed were analyzed. Incomplete excision was defined as near-total resection (NTR), subtotal resection (STR), and partial resection (PR). Tumors in the NTR and STR categories were followed up with a wait-and-rescan approach, whereas the tumors in the PR category were subjected to a second- stage surgery and were excluded from this series. All patients included in the study underwent baseline MRI at the 3rd and 12th postoperative months, and repeat imaging was subsequently performed every year for 7–10 years postoperatively or as indicated clinically. Preoperative and postoperative facial function was noted.

Results. Of the 2368 patients who underwent surgery for VS, 111 patients who had incomplete excisions of VSs were included in the study. Of these patients, 73 (65.77%) had undergone NTR and 38 (34.23%) had undergone STR. Of the VSs, 62 (55.86%) were cystic and 44 (70.97%) of these cystic VSs underwent NTR. The residual tumor was left behind on the facial nerve alone in 62 patients (55.86%), on the facial nerve and vessels in 2 patients (1.80%), on the facial nerve and brainstem in 15 patients (13.51%), and on the brainstem alone in 25 patients (22.52%). In the 105 patients with normal preoperative facial nerve function, postoperative facial nerve function was House-Brackmann (HB) Grades I and II in 51 patients (48.57%), HB Grade III in 34 patients (32.38%), and HB Grades IV–VI in 20 pa- tients (19.05%). Seven patients (6.3%) showed evidence of tumor regrowth on follow-up MRI. All 7 patients (100%) who showed evidence of tumor regrowth had undergone STR. No patient in the NTR group exhibited regrowth. The Kaplan-Meier plot demonstrated a 5-year tumor regrowth-free survival of 92%, with a mean disease-free interval of 140 months (95% CI 127–151 months). The follow-up period ranged from 12 to 156 months (mean 45.4 months).

Conclusions. The authors’ report and review of the literature show that there is undoubtedly merit for NTR and STR for preservation of the facial nerve. On the basis of this they propose an algorithm for the management of incomplete VS excisions. Patients who undergo incomplete excisions must be subjected to follow-up MRI for a period of at least 7–10 years. When compared with STR, NTR via an enlarged translabyrinthine approach has shown to have a lower rate of regrowth of residual tumor, while having almost the same result in terms of facial nerve function.

 

 

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Medial acoustic neuromas: clinical and surgical implications

Medial VIII schwannoma

J Neurosurg 120:1095–1104, 2014

Medial acoustic neuroma is a rare entity that confers a distinct clinical syndrome. It is scarcely discussed in the literature and is associated with adverse features. This study evaluates the clinical and imaging features, pertinent surgical challenges, and treatment outcome in a large series of this variant. The authors postulate that the particular pathological anatomy with its arachnoidal rearrangement has a profound implication on the surgical technique and outcome.

Methods. The authors conducted a retrospective analysis of 52 cases involving 33 women and 19 men who underwent resection of medial acoustic neuromas performed by the senior author (O.A.) over a 20-year period (1993– 2013). Clinical, radiological, and operative records were reviewed, with a specific focus on the neurological outcomes and facial nerve function and hearing preservation. Intraoperative findings were analyzed with respect to the effect of arachnoidal arrangement on the surgeon’s ability to resect the lesion and the impact on postoperative function.

Results. The average tumor size was 34.5 mm (maximum diameter), with over 90% of tumors being 25 mm or larger and 71% being cystic. Cerebellar, trigeminal nerve, and facial nerve dysfunction were common preoperative findings. Hydrocephalus was present in 11 patients. Distinguishing intraoperative findings included marked tumor adherence to the brainstem and frequent hypervascularity, which prompted intracapsular dissection resulting in enhancement on postoperative MRI in 18 cases, with only 3 demonstrating growth on follow-up. There was no mortality or major postoperative neurological deficit. Cerebrospinal fluid leak was encountered in 7 patients, with 4 requiring surgical repair. Among 45 patients who had intact preoperative facial function, only 1 had permanent facial nerve paralysis on extended follow-up. Of the patients with preoperative Grade I–II facial function, 87% continued to have Grade I–II function on follow-up. Of 10 patients who had Class A hearing preoperatively, 5 continued to have Class A or B hearing after surgery.

Conclusions. Medial acoustic neuromas represent a rare subgroup whose site of origin and growth patterns produce a distinct clinical presentation and present specific operative challenges. They reach giant size and are frequently cystic and hypervascular. Their origin and growth pattern lead to arachnoidal rearrangement with marked adherence against the brainstem, which is critical in the surgical management. Excellent surgical outcome is achievable with a high rate of facial nerve function and attainable hearing preservation. These results suggest that similar or better results may be achieved in less complex tumors.

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Contemporary Surgical Management of Vestibular Schwannomas

Vestibular-schwannoma

Neurosurgery 72[ONS Suppl 2]:ons103–ons115, 2013 

Despite advanced microsurgical techniques, more refined instrumentation, and expert team management, there is still a significant incidence of complications in vestibular schwannoma surgery.

OBJECTIVE: To analyze complications from the microsurgical treatment of vestibular schwannoma by an expert surgical team and to propose strategies for minimizing such complications.

METHODS: Surgical outcomes and complications were evaluated in a consecutive series of 410 unilateral vestibular schwannomas treated from 2000 to 2009. Clinical status and complications were assessed postoperatively (within 7 days) and at the time of follow-up (range, 1-116 months; mean, 32.7 months).

RESULTS: Follow-up data were available for 357 of the 410 patients (87.1%). Microsurgical tumor resection was performed through a retrosigmoid approach in 70.7% of cases. Thirty-three patients (8%) had intrameatal tumors and 204 (49.8%) had tumors that were ,20 mm. Gross total resection was performed in 306 patients (74.6%). Hearing preservation surgery was attempted in 170 patients with tumors ,20 mm, and good hearing was preserved in 74.1%. The main neurological complication was facial palsy (House-Brackmann grade III-VI), observed in 14% of patients (56 cases) postoperatively; however, 59% of them improved during the follow-up period. Other neurological complications were disequilibrium in 6.3%, facial numbness in 2.2%, and lower cranial nerve deficit in 0.5%. Nonneurological complications included cerebrospinal fluid leaks in 7.6%, wound infection in 2.2%, and meningitis in 1.7%.

CONCLUSION: Many of these complications are avoidable through further refinement of operative technique, and strategies for avoiding complications are proposed.

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Dorsal location of the cochlear nerve on vestibular schwannoma: preoperative evaluation, frequency, and functional outcome

Dorsal location of the cochlear nerve on vestibular schwannoma

Neurosurg Rev (2013) 36:39–44

The cochlear nerve is most commonly located on the caudoventral portion of the capsule of vestibular schwannomas and rarely on the dorsal portion. In such a condition, total removal of the tumor without cochlear nerve dysfunction is extremely difficult.

The purpose of our study was to identify the frequency of this anatomical condition and the status of postoperative cochlear nerve function; we also discuss the preoperative radiological findings.

The study involved 114 patients with unilateral vestibular schwannomas operated on via a retrosigmoid (lateral suboccipital) approach. Locations of the cochlear nerve on the tumor capsule were ventral, dorsal, caudal, and rostral. Ventral and dorsal locations were further subdivided into rostral, middle, and caudal third of the tumor capsule. The postoperative cochlear nerve function and preoperative magnetic resonance (MR) findings were reviewed retrospectively.

In 56 patients that had useful preoperative hearing, useful hearing was retained in 50.0 % (28 of 56) of patients after surgery. The cochlear nerve was located on the dorsal portion of the tumor capsule in four patients (3.5 %), and useful hearing was preserved in only one of these patients (25 %) in whom the tumor had been partially resected.

This tumor-nerve anatomical relationship was identified in all tumors of <2 cm at preoperative MR cisternography. MR cisternography has the potential to identify the tumor–nerve anatomical relationship, especially in small-sized tumors that usually require therapeutic intervention that ensures hearing preservation. Hence, careful evaluation of the preoperative MR cisternography is important in deciding the therapeutic indications.

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Surgery for vestibular schwannomas: a systematic review of complications by approach

Neurosurg Focus 33 (3):E14, 2012

Various studies report outcomes of vestibular schwannoma (VS) surgery, but few studies have compared outcomes across the various approaches. The authors conducted a systematic review of the available data on VS surgery, comparing the different approaches and their associated complications.

Methods. MEDLINE searches were conducted to collect studies that reported information on patients undergoing VS surgery. The authors set inclusion criteria for such studies, including the availability of follow-up data for at least 3 months, inclusion of preoperative and postoperative audiometric data, intraoperative monitoring, and reporting of results using established and standardized metrics. Data were collected on hearing loss, facial nerve dysfunction, persistent postoperative headache, CSF leak, operative mortality, residual tumor, tumor recurrence, cranial nerve (CN) dysfunction involving nerves other than CN VII or VIII, and other neurological complications. The authors reviewed data from 35 studies pertaining to 5064 patients who had undergone VS surgery.

Results. The analyses for hearing loss and facial nerve dysfunction were stratified into the following tumor categories: intracanalicular (IC), size (extrameatal diameter) < 1.5 cm, size 1.5–3.0 cm, and size > 3.0 cm. The middle cranial fossa approach was found to be superior to the retrosigmoid approach for hearing preservation in patients with tumors < 1.5 cm (hearing loss in 43.6% vs 64.3%, p < 0.001). All other size categories showed no significant difference between middle cranial fossa and retrosigmoid approaches with respect to hearing loss. The retrosigmoid approach was associated with significantly less facial nerve dysfunction in patients with IC tumors than the middle cranial fossa method was; however, neither differed significantly from the translabyrinthine corridor (4%, 16.7%, 0%, respectively, p < 0.001). The middle cranial fossa approach differed significantly from the translabyrinthine approach for patients with tumors < 1.5 cm, whereas neither differed from the retrosigmoid approach (3.3%, 11.5%, and 7.2%, respectively, p = 0.001). The retrosigmoid approach involved less facial nerve dysfunction than the middle cranial fossa or translabyrinthine approaches for tumors 1.5–3.0 cm (6.1%, 17.3%, and 15.8%, respectively; p < 0.001). The retrosigmoid approach was also superior to the translabyrinthine approach for tumors > 3.0 cm (30.2% vs 42.5%, respectively, p < 0.001). Postoperative headache was significantly more likely after the retrosigmoid approach than after the translabyrinthine approach, but neither differed significantly from the middle cranial fossa approach (17.3%, 0%, and 8%, respectively; p < 0.001). The incidence of CSF leak was significantly greater after the retrosigmoid approach than after either the middle cranial fossa or translabyrinthine approaches (10.3%, 5.3%, 7.1%; p = 0.001). The incidences of residual tumor, mortality, major non-CN complications, residual tumor, tumor recurrence, and dysfunction of other cranial nerves were not significantly different across the approaches.

Conclusions. The middle cranial fossa approach seems safest for hearing preservation in patients with smaller tumors. Based on the data, the retrosigmoid approach seems to be the most versatile corridor for facial nerve preservation for most tumor sizes, but it is associated with a higher risk of postoperative pain and CSF fistula. The translabyrinthine approach is associated with complete hearing loss but may be useful for patients with large tumors and poor preoperative hearing.

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Technical nuances of resection of giant (> 5 cm) vestibular schwannomas: pearls for success

Neurosurg Focus 33 (3):E15, 2012

Removal of vestibular schwannomas (VSs, or acoustic neuromas) remains one of the most challenging operations in neurosurgery. Giant or huge tumors (> 5 cm) heighten these challenges, and technical nuances play a special role in maximizing tumor resection while minimizing complications.

In this article, the senior author describes his technical experience with microsurgical excision of giant VSs. The accompanying video further illustrates these details.

 

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Surgery for vestibular schwannomas: a systematic review of complications by approach

Neurosurg Focus 33 (3):E14, 2012

Various studies report outcomes of vestibular schwannoma (VS) surgery, but few studies have compared outcomes across the various approaches. The authors conducted a systematic review of the available data on VS surgery, comparing the different approaches and their associated complications.

Methods. MEDLINE searches were conducted to collect studies that reported information on patients undergoing VS surgery. The authors set inclusion criteria for such studies, including the availability of follow-up data for at least 3 months, inclusion of preoperative and postoperative audiometric data, intraoperative monitoring, and reporting of results using established and standardized metrics. Data were collected on hearing loss, facial nerve dysfunction, persistent postoperative headache, CSF leak, operative mortality, residual tumor, tumor recurrence, cranial nerve (CN) dysfunction involving nerves other than CN VII or VIII, and other neurological complications. The authors reviewed data from 35 studies pertaining to 5064 patients who had undergone VS surgery.

Results. The analyses for hearing loss and facial nerve dysfunction were stratified into the following tumor categories: intracanalicular (IC), size (extrameatal diameter) < 1.5 cm, size 1.5–3.0 cm, and size > 3.0 cm. The middle cranial fossa approach was found to be superior to the retrosigmoid approach for hearing preservation in patients with tumors < 1.5 cm (hearing loss in 43.6% vs 64.3%, p < 0.001). All other size categories showed no significant difference between middle cranial fossa and retrosigmoid approaches with respect to hearing loss. The retrosigmoid approach was associated with significantly less facial nerve dysfunction in patients with IC tumors than the middle cranial fossa method was; however, neither differed significantly from the translabyrinthine corridor (4%, 16.7%, 0%, respectively, p < 0.001). The middle cranial fossa approach differed significantly from the translabyrinthine approach for patients with tumors < 1.5 cm, whereas neither differed from the retrosigmoid approach (3.3%, 11.5%, and 7.2%, respectively, p = 0.001). The retrosigmoid approach involved less facial nerve dysfunction than the middle cranial fossa or translabyrinthine approaches for tumors 1.5–3.0 cm (6.1%, 17.3%, and 15.8%, respectively; p < 0.001). The retrosigmoid approach was also superior to the translabyrinthine approach for tumors > 3.0 cm (30.2% vs 42.5%, respectively, p < 0.001). Postoperative headache was significantly more likely after the retrosigmoid approach than after the translabyrinthine approach, but neither differed significantly from the middle cranial fossa approach (17.3%, 0%, and 8%, respectively; p < 0.001). The incidence of CSF leak was significantly greater after the retrosigmoid approach than after either the middle cranial fossa or translabyrinthine approaches (10.3%, 5.3%, 7.1%; p = 0.001). The incidences of residual tumor, mortality, major non-CN complications, residual tumor, tumor recurrence, and dysfunction of other cranial nerves were not significantly different across the approaches.

Conclusions. The middle cranial fossa approach seems safest for hearing preservation in patients with smaller tumors. Based on the data, the retrosigmoid approach seems to be the most versatile corridor for facial nerve preservation for most tumor sizes, but it is associated with a higher risk of postoperative pain and CSF fistula. The translabyrinthine approach is associated with complete hearing loss but may be useful for patients with large tumors and poor preoperative hearing.

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Staged resection of large vestibular schwannomas

J Neurosurg 116:1126–1133, 2012.(http://thejns.org/doi/abs/10.3171/2012.1.JNS111402)

Staged resection of large vestibular schwannomas (VSs) has been proposed as a strategy to improve facial nerve outcomes and morbidity. The authors report their experience with 2-stage resections of large VSs and analyze the indications, facial nerve outcomes, surgical results, and complications. The authors compare these results with those of a similar cohort of patients who underwent a single-stage resection.

Methods. A retrospective review of all patients (age > 18 years) who underwent surgery from 2002 to 2010 for large (≥ 3 cm) VSs at the authors’ institution with a minimum of 6 months follow-up was undertaken. A first-stage retrosigmoid approach (without meatal drilling) was performed to remove the cerebellopontine angle portion of the tumor and to decompress the brainstem. A decision to stage the operation was made intraoperatively if there was cerebellar or brainstem edema, excessive tumor adherence to the facial nerve or brainstem, a poorly stimulating facial nerve, or a thinned or splayed facial nerve. A second-stage translabyrinthine approach was performed at a later date to remove the remaining tumor. The single-stage resection consisted of a retrosigmoid approach with meatal drilling. Patient charts were evaluated for tumor size, extent of resection, tumor recurrence, House-Brackmann facial nerve function grade, and complications.

Results. Twenty-eight and 19 patients underwent 2- or single-stage resection of a large VS, respectively. The average tumor size was 3.9 cm (range 3.2–7 cm) in the 2-stage group and 3.9 cm (range 3.1–5 cm) in the single-stage group. The mean follow-up was 36 ± 19 months in the 2-stage group versus 24 ± 14 months in the single-stage group. Gross-total or near-total resection was achieved in 27 (96.4%) of 28 patients in the 2-stage group and 15 (79%) of 19 patients in the single-stage group (p < 0.01). Anatomical facial nerve preservation was achieved in all but 1 patient (94.7%), and there were no recurrences on follow-up imaging in the 2-stage group. Good facial nerve functional outcome (House-Brackmann Grades I and II) at last follow-up was achieved in 23 (82%) of 28 patients in the 2-stage group and 10 (53%) of 19 patients in the single-stage group (p < 0.01). Cerebrospinal fluid leak–related complications (intracranial hypotension, blood patch, and lumboperitoneal shunt for pseudomeningocele) were more common in the 2-stage group. There were no postoperative strokes, hemorrhages, or deaths in either group.

Conclusions. The authors’ results suggest that staged resection of large VSs may potentially achieve better facial nerve outcomes. There does not appear to be added neurological morbidity with staged resections

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Preoperative identification of the facial nerve in patients with large cerebellopontine angle tumors using high-density diffusion tensor imaging


 

J Neurosurg 116:697–702, 2012. http://thejns.org/doi/abs/10.3171/2011.12.JNS111404

Facial nerve paresis can be a devastating complication following resection of large (> 2.5 cm) cerebellopontine angle (CPA) tumors. The authors have developed and used a new high-density diffusion tensor imaging (HD-DT imaging) method, aimed at preoperatively identifying the location and course of the facial nerve in relation to large CPA tumors. Their study objective was to preoperatively identify the facial nerve in patients with large CPA tumors and compare their HD-DT imaging method with a traditional standard DT imaging method and correlate with intraoperative findings.

Methods. The authors prospectively studied 5 patients with large (> 2.5 cm) CPA tumors. All patients underwent preoperative traditional standard- and HD-DT imaging. Imaging results were correlated with intraoperative findings.

Results. Utilizing their HD-DT imaging method, the authors positively identified the location and course of the facial nerve in all patients. In contrast, using a standard DT imaging method, the authors were unable to identify the facial nerve in 4 of the 5 patients.

Conclusions. The HD-DT imaging method that the authors describe and use has proven to be a powerful, accurate, and rapid method for preoperatively identifying the facial nerve in relation to large CPA tumors. Routine integration of HD-DT imaging in preoperative planning for CPA tumor resection could lead to improved facial nerve preservation.

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Diffusion tensor imaging–based fiber tracking for prediction of the position of the facial nerve in relation to large vestibular schwannomas

J Neurosurg 115:1087–1093, 2011. DOI: 10.3171/2011.7.JNS11495

The reliable preoperative visualization of facial nerve location in relation to vestibular schwannoma (VS) would allow surgeons to plan tumor removal accordingly and may increase the safety of surgery. In this prospective study, the authors attempted to validate the reliability of facial nerve diffusion tensor (DT) imaging–based fiber tracking in a series of patients with large VSs. Furthermore, the authors evaluated the potential of this visualization technique to predict the morphological shape of the facial nerve (tumor compression–related flattening of the nerve).

Methods. Diffusion tensor imaging and anatomical images (constructive interference in steady state) were acquired in a series of 22 consecutive patients with large VSs and postprocessed with navigational software to obtain facial nerve fiber tracking. The location of the cerebellopontine angle (CPA) part of the nerve in relation to the tumor was recorded during surgery by the surgeon, who was blinded to the results of the fiber tracking. A correlative analysis was performed of the imaging-based location of the nerve compared with its in situ position in relation to the VS.

Results. Fibers corresponding to the anatomical location and course of the facial nerve from the brainstem to the internal auditory meatus were identified with the DT imaging–based fiber tracking technique in all 22 cases. The location of the CPA segment of the facial nerve in relation to the VS determined during surgery corresponded to the location of the fibers, predicted by the DT imaging–based fiber tracking, in 20 (90.9%) of the 22 patients. No DT imaging–based fiber tracking correlates were found with the 2 morphological types of the nerve (compact or flat).

Conclusions. The current study of patients with large VSs has shown that the position of the facial nerve in relation to the tumor can be predicted reliably (in 91%) using DT imaging–based fiber tracking. These are preliminary results that need further verification in a larger series.

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The molecular biology and novel treatments of vestibular schwannomas

J Neurosurg 115:906–914, 2011. DOI: 10.3171/2011.6.JNS11131

Vestibular schwannomas are histopathologically benign tumors arising from the Schwann cell sheath surrounding the vestibular branch of cranial nerve VIII and are related to the NF2 gene and its product merlin.

Merlin acts as a tumor suppressor and as a mediator of contact inhibition. Thus, deficiencies in both NF2 genes lead to vestibular schwannoma development.

Recently, there have been major advances in our knowledge of the molecular biology of vestibular schwannomas as well as the development of novel therapies for its treatment.

In this article the authors comprehensively review the recent advances in the molecular biology and characterization of vestibular schwannomas as well as the development of modern treatments for vestibular schwannoma. For instance, merlin is involved with a number of receptors including the CD44 receptor, EGFR, and signaling pathways, such as the Ras/raf pathway and the canonical Wnt pathway. Recently, merlin was also shown to interact in the nucleus with E3 ubiquitin ligase CRL4DCAF1.

A greater understanding of the molecular mechanisms behind vestibular schwannoma tumorigenesis has begun to yield novel therapies. Some authors have shown that Avastin induces regression of progressive schwannomas by over 40% and improves hearing. An inhibitor of VEGF synthesis, PTC299, is currently in Phase II trials as a potential agent to treat vestibular schwannoma.

Furthermore, in vitro studies have shown that trastuzumab (an ERBB2 inhibitor) reduces vestibular schwannoma cell proliferation.

With further research it may be possible to significantly reduce morbidity and mortality rates by decreasing tumor burden, tumor volume, hearing loss, and cranial nerve deficits seen in vestibular schwannomas.

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Tumor origin and hearing preservation in vestibular schwannoma surgery

J Neurosurg 115:900–905, 2011. DOI: 10.3171/2011.7.JNS102092

Preservation of cochlear nerve function in vestibular schwannoma (VS) removal is usually dependent on tumor size and preoperative hearing status. Tumor origin as an independent factor has not been systematically investigated.

Methods. A series of 90 patients with VSs, who underwent surgery via a suboccipitolateral route, was evaluated with respect to cochlear nerve function, tumor size, radiological findings, and intraoperatively confirmed tumor origin. All patients were reevaluated 12 months after surgery.

Results. Despite comparable preoperative cochlear nerve status and larger tumor sizes, hearing preservation was achieved in 42% of patients with tumor originating from the superior vestibular nerve, compared with 16% of those with tumor originating from the inferior vestibular nerve.

Conclusions. Tumor origin is an important prognostic factor for cochlear nerve preservation in VS surgery.

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Dural Landmark to Locate the Internal Auditory Canal in Large and Giant Vestibular Schwannomas: The Tübingen Line

Neurosurgery 69[ONS Suppl 1]:ons99–ons102, 2011.  DOI: 10.1227/NEU.0b013e31821664c6

In cases of large and giant vestibular schwannomas (VS), the visualization of the internal auditory canal (IAC) opening is difficult or impossible.

OBJECTIVE: To describe the Tübingen line and explore its relationships with the IAC as a landmark to help locate the IAC.

METHODS: Ten cadaveric heads were used in this study. Between 2004 and 2009, the senior author (M.T.) used the Tübingen line as a landmark to recognize the IAC in 300 consecutive patients with VS. To locate the Tübingen  line, the initial step was to identify several vertical foldings of dura located around the area of the vestibular aqueduct. After this, foldings upward consistently reached a linear level where all of the foldings ended and the dura tightly adhered to the bony surface in a smooth, foldless shape.

RESULTS: The Tübingen line was identified in all temporal bones studied and in all 300 patients operated on, with the exception of 2 cases (,1%). Removal of the bone just above the Tübingen line located the IAC in all temporal bone specimens studied. Similarly, the surgical cases showed that the Tübingen line helped locate the IAC in all patients.

CONCLUSION: The Tübingen line is an easy, consistent, and safe method to locate the projection of the IAC along the posterior surface of the temporal bone.

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Hydrocephalus associated with vestibular schwannomas: management options and factors predicting the outcome

J Neurosurg 114:1209–1215, 2011. DOI: 10.3171/2010.10.JNS1029

The current, generally accepted optimal management for hydrocephalus related to vestibular schwannomas (VSs) is primary tumor removal, with further treatment reserved only for patients who remain symptomatic. Previous studies have shown, however, that this management can lead to an increase in surgery-related complications. In this study, the authors evaluated their experience with the treatment of such patients, with the aim of identifying the following: 1) the parameters correlating to the need for specific hydrocephalus treatment following VS surgery; and 2) patients at risk for developing hydrocephalus-related complications.

Methods. This was a retrospective study of a 400-patient series. The complication rates and outcomes following primary hydrocephalus treatment versus primary VS removal were compared. Patients undergoing primary tumor removal were further subdivided on the basis of the need for subsequent hydrocephalus treatment. The 3 categories of parameters tested for correlation with the need for such subsequent treatment as well as with heightened risk for developing complications were patient-, tumor-, and hydrocephalus-related.

Results. Of the entire series, 53 patients presented with hydrocephalus. Forty-eight of 53 patients underwent primary VS surgery, of whom 42 (87.5%) did not require additional hydrocephalus treatment. Of the 6 patients who did require additional hydrocephalus treatment, only 3 ultimately required a VP shunt. Factors correlating to the need of hydrocephalus treatment after VS removal were large tumor size, irregular tumor surface, and severe preoperative hydrocephalus. Patients with a longer symptom duration prior to surgery, those with polycyclic tumors, or with inhomogeneous VS, were at heightened risk for the development of CSF leaks. The general and functional outcome of surgery showed no correlation to the presence of preoperative hydrocephalus.

Conclusions. Primary tumor removal is the optimum management of disease in patients with VS with associated hydrocephalus; it leads to resolution of the hydrocephalus in the majority of cases, and the outcome is similar to that of patients without hydrocephalus. Certain factors may aid in identifying patients at risk for developing persistent hydrocephalus as well as those at risk for CSF leaks

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Gamma Knife radiosurgery for larger-volume vestibular schwannomas

J Neurosurg 114:801–807, 2011.DOI: 10.3171/2010.8.JNS10674

Stereotactic radiosurgery (SRS) is an important management option for patients with small- and medium- sized vestibular schwannomas. To assess the potential role of SRS in larger tumors, the authors reviewed their recent experience.

Methods. Between 1994 and 2008, 65 patients with vestibular schwannomas between 3 and 4 cm in one extracanalicular maximum diameter (median tumor volume 9 ml) underwent Gamma Knife surgery. Seventeen patients (26%) had previously undergone resection.

Results. The median follow-up duration was 36 months (range 1–146 months). At the first planned imaging follow-up at 6 months, 5 tumors (8%) were slightly expanded, 53 (82%) were stable in size, and 7 (11%) were smaller. Two patients (3%) underwent resection within 6 months due to progressive symptoms. Two years later, with 63 tumors overall after the 2 post-SRS resections, 16 tumors (25%) had a volume reduction of more than 50%, 22 (35%) tumors had a volume reduction of 10–50%, 18 (29%) were stable in volume (volume change < 10%), and 7 (11%) had larger volumes (5 of the 7 patients underwent resection and 1 of the 7 underwent repeat SRS). Eighteen (82%) of 22 patients with serviceable hearing before SRS still had serviceable hearing after SRS more than 2 years later. Three patients (5%) developed symptomatic hydrocephalus and underwent placement of a ventriculoperitoneal shunt. In 4 patients (6%) trigeminal sensory dysfunction developed, and in 1 patient (2%) mild facial weakness (House-Brackmann Grade II) developed after SRS. In univariate analysis, patients who had a previous resection (p = 0.010), those with a tumor volume exceeding 10 ml (p = 0.05), and those with Koos Grade 4 tumors (p = 0.02) had less likelihood of tumor control after SRS.

Conclusions. Although microsurgical resection remains the primary management choice in patients with low comorbidities, most vestibular schwannomas with a maximum diameter less than 4 cm and without significant mass effect can be managed satisfactorily with Gamma Knife radiosurgery.

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Decision analysis of treatment options for vestibular schwannoma

J Neurosurg 114:400–413, 2011. (DOI: 10.3171/2010.3.JNS091802)

Widespread use of MR imaging has contributed to the more frequent diagnosis of vestibular schwannomas (VSs). These tumors represent 10% of primary adult intracranial neoplasms, and if they are symptomatic, they usually present with hearing loss and tinnitus. Currently, there are 3 treatment options for quality of life (QOL): wait and scan, microsurgery, and radiosurgery. In this paper, the authors’ purpose is to determine which treatment modality yields the highest QOL at 5- and 10-year follow-up, considering the likelihood of recurrence and various complications.

Methods. The MEDLINE, Embase, and Cochrane online databases were searched for English-language articles published between 1990 and June 2008, containing key words relating to VS. Data were pooled to calculate the prevalence of treatment complications, tumor recurrence, and QOL with various complications. For parameters in which incidence varied with time of follow-up, the authors used meta-regression to determine the mean prevalence rates at a specified length of follow-up. A decision-analytical model was constructed to compare 5- and 10-year outcomes for a patient with a unilateral tumor and partially intact hearing. The 3 treatment options, wait and scan, microsurgery, and radiosurgery, were compared.

Results. After screening more than 2500 abstracts, the authors ultimately included 113 articles in this analysis. Recurrence, complication rates, and onset of complication varied with the treatment chosen. The relative QOL at the 5-year follow-up was 0.898 of normal for wait and scan, 0.953 for microsurgery, and 0.97 for radiosurgery. These differences are significant (p < 0.0052). Data were too scarce at the 10-year follow-up to calculate significant differences between the microsurgery and radiosurgery strategies.

Conclusions. At 5 years, patients treated with radiosurgery have an overall better QOL than those treated with either microsurgery or those investigated further with serial imaging. The authors found that the complications associated with wait-and-scan and microsurgery treatment strategies negatively impacted patient lives more than the complications from radiosurgery. One limitation of this study is that the 10-year follow-up data were too limited to analyze, and more studies are needed to determine if the authors’ results are still consistent at 10 years.

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A Critical Evaluation of Vestibular Schwannoma Surgery for Patients Younger Than 40 Years of Age

Neurosurgery 67:1646–1654, 2010 DOI: 10.1227/NEU.0b013e3181f8d3d3

There are few published prospective data sets specifically focusing on patients younger than 40 years old undergoing microsurgery for vestibular schwannoma.

OBJECTIVE: We describe functional outcomes and long-term tumor control after surgery in patients younger than 40 years old enrolled in a prospectively collected database over a 25-year period.

METHODS: We selected all vestibular schwannoma patients from a prospectively collected database who were younger than 40 years old at the time of surgical resection for a vestibular schwannoma. Rates of tumor control and hearing preservation were analyzed using Kaplan-Meier analysis, and risk factors for facial nerve palsy, hearing loss, and trigeminal neuropathy were analyzed using multivariate logistic regression.

RESULTS: A total of 204 patients younger than 40 years of age met our inclusion criteria and were included in the analysis. Our data indicate that surgical resection leads to durable long-term freedom from tumor recurrence or progression in 89% of young patients at 15 years of follow-up. Consistent with other published series, hearing was preserved in 68% of patients with smaller tumors (,3 cm). Facial nerve function was preserved in 76% of patients with smaller tumors and 52% of patients with larger tumors (P , .001). On multivariate logistic regression, tumor size was a significant predictor of hearing loss, whereas gross total resection was nearly a significant predictor of hearing loss controlling for other variables (P = .06).

CONCLUSION: We present the largest prospectively studied cohort of young patients undergoing microsurgical resection of vestibular schwannoma. These data suggest that surgical resection provides excellent long-term tumor control in these patients

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