Sporadic unilateral vestibular schwannoma in the pediatric population

2009 ◽  
Vol 4 (2) ◽  
pp. 125-129 ◽  
Author(s):  
Brian P. Walcott ◽  
Ganesh Sivarajan ◽  
Bronislava Bashinskaya ◽  
Douglas E. Anderson ◽  
John P. Leonetti ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Tumor Size ◽  
Pediatric Population ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Duration Of Symptoms

Object Vestibular schwannomas (VSs) are rare in the pediatric population. Most often, these lesions manifest as a bilateral disease process in the setting of neurofibromatosis Type 2. Even in the absence of additional clinical diagnostic criteria, the presentation of a unilateral VS in a young patient may be a harbinger of future penetrance for this hereditary tumor syndrome. Methods The authors retrospectively reviewed the charts of a cohort of 7 patients who presented with apparently sporadic, unilateral VSs. These patients had previously undergone surgery via translabyrinthine, retrosigmoid, or combined approaches. Clinical outcomes were reviewed with emphasis on facial nerve function and follow-up for signs and symptoms of a heritable disorder. Results All patients underwent microsurgical resection in a multidisciplinary effort by the senior authors. The average tumor size was 4.57 cm, with an average duration of symptoms prior to definitive diagnosis of 31.2 months. The tumor size at the time of presentation followed a trend different from reports in adults, while the duration of symptoms did not. At a follow-up average of 6.3 years (range 1–12 years), 100% of patients demonstrated good facial function (House-Brackmann Grade I or II). No patient in this cohort demonstrated symptoms, objective signs, or genetic analysis indicating the presence of neurofibromatosis Type 2. Conclusions Diagnosis and management of sporadic, unilateral VSs in children is complicated by clinical presentations and surgical challenges unique from their adult counterparts. Careful consideration should be given to a heritable genetic basis for sporadic unilateral VS in the pediatric population. Results of genetic testing do not preclude the necessity for long-term follow-up and systemic investigation. In patients who present with large tumors, preliminary experience leads the authors to suggest that a combined retrosigmoid-translabyrinthine approach offers the greatest opportunity for preservation of facial nerve function.

Early Proactive Management of Vestibular Schwannomas in Neurofibromatosis Type 2

Neurosurgery ◽  
2001 ◽  
Vol 49 (2) ◽  
pp. 274-283 ◽  
Author(s):  
Derald E. Brackmann ◽  
Jose N. Fayad ◽  
William H. Slattery ◽  
Rick A. Friedman ◽  
John Diaz Day ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Neck Surgery ◽  
Neurofibromatosis Type 2 ◽  
Nerve Function ◽  
Speech Discrimination ◽  
Facial Nerve Function ◽  
Proactive Management

Abstract OBJECTIVE The treatment of patients with neurofibromatosis Type 2 has always been challenging for neurosurgeons and neurotologists. Guidelines for appropriate management of this devastating disease are controversial. METHODS A retrospective study of 28 patients with neurofibromatosis Type 2 who underwent 40 middle fossa craniotomies for excision of their acoustic tumors is reported. Eleven patients underwent bilateral procedures. The study focused on hearing preservation and facial nerve results for this group of patients. The 16 male patients and 12 female patients ranged in age (at the time of surgery) from 10 to 70 years, with a mean age of 22.6 years. The mean tumor size was 1.1 cm (range, 0.5–3.2 cm), and the majority of tumors were less than 1.5 cm. RESULTS Measurable hearing was preserved in 28 ears (70%), with 42.5% being within 15 dB pure-tone average and 15% speech discrimination score of preoperative levels. In 55% of cases there was no change in the hearing class, as defined by the American Academy of Otolaryngology-Head and Neck Surgery. Of the 11 patients who underwent bilateral operations, 9 (82%) retained some hearing bilaterally. After 1-year follow-up periods (mean, 12.8 mo), 87.5% of patients exhibited normal facial nerve function (House-Brackmann Grade I). CONCLUSION Early surgical intervention to treat acoustic tumors among patients with neurofibromatosis Type 2 is a feasible treatment strategy, with high rates of hearing and facial nerve function preservation.

Middle Fossa Approach for Resection of Vestibular Schwannomas: A Decade of Experience

2018 ◽  
Vol 16 (2) ◽  
pp. 147-158 ◽  
Author(s):  
Jennifer A Kosty ◽  
Shawn M Stevens ◽  
Yair M Gozal ◽  
Vincent A DiNapoli ◽  
Smruti K Patel ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Tumor Size ◽  
Pure Tone ◽  
Vestibular Schwannomas ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Pure Tone Average ◽  
Class A ◽  
The Mean

AbstractBACKGROUNDThe middle cranial fossa (MCF) approach is a challenging surgical technique for the resection of small and intermediate sized, primarily intracanalicular, vestibular schwannomas (VS), with the goal of hearing preservation (HP).OBJECTIVETo describe a decade-long, single institutional experience with the MCF approach for resection of VS.METHODSThis is a retrospective cohort study of 63 patients who underwent the MCF approach for resection of VS from 2006 to 2016. Audiometric data included pure-tone average (PTA), low-tone pure-tone average (LtPTA), word recognition score, and American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) hearing classification at presentation and follow-up. Patients with postoperative serviceable (AAO-HNS class A-B) and/or useful (AAO-HNS class A-C) hearing were compared to those without HP. Facial nerve function was assessed using the House–Brackmann scale.RESULTSThe mean age and duration of follow-up were 50 ± 13 yr and 21 ± 21 mo, respectively. The mean tumor size was 10 ± 4 mm. The serviceable and usable HP rates were 54% and 50%, respectively. Some residual hearing was preserved in 71% of patients. Large tumor size (P = .05), volume (P = .03), and extrameatal tumor extension (P = .03) were associated with poor audiometric outcomes. The presence of a fundal fluid cap (P = .01) was a favorable finding. At definitive testing, LtPTA was significantly better preserved than traditional PTA (P = .01). Facial nerve outcomes, tumor control rates, and durability of audiometric outcomes were excellent. 47% of patients pursued aural rehabilitation.CONCLUSIONIn our series, the MCF approach for VS provided excellent rates of tumor and facial nerve function, with durable serviceable HP.

Facial Nerve Outcomes After Vestibular Schwannoma Microsurgical Resection in Neurofibromatosis Type 2

2020 ◽  
pp. 019459982095414
Author(s):  
Catherine Sobieski ◽  
Daniel E. Killeen ◽  
Samuel L. Barnett ◽  
Bruce E. Mickey ◽  
Jacob B. Hunter ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Vestibular Schwannoma ◽  
Tumor Volume ◽  
Tumor Resection ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tumor Diameter ◽  
Microsurgical Resection

Objective The aim of this study is to investigate facial nerve outcomes after microsurgical resection in neurofibromatosis type 2 (NF2) compared to sporadic tumors. Study Design Single institutional retrospective chart review. Setting Tertiary referral center. Methods All adult patients with NF2 vestibular schwannoma (VS) or sporadic VS who underwent microsurgical resection from 2008 to 2019 with preoperative magnetic resonance imaging (MRI) and 1 year of postsurgical follow-up were included. The primary outcome measure was postoperative House-Brackmann (HB) facial nerve score measured at first postoperative visit and after at least 10 months. Results In total, 161 sporadic VSs and 14 NF2 VSs met inclusion criteria. Both median tumor diameter (NF2, 33.5 mm vs sporadic, 24 mm, P = .0011) and median tumor volume (NF2, 12.4 cm3 vs sporadic, 2.9 cm3, P = .0005) were significantly greater in patients with NF2. The median follow-up was 24.9 months (range, 12-130.1). Median facial nerve function after 1 year for patients with NF2 was HB 3 (range, 1-6) compared to HB 1 (range, 1-6) for sporadic VS ( P = .001). With multivariate logistic regression, NF2 tumors (odds ratio [OR] = 13.9, P = .001) and tumor volume ≥3 cm3 (OR = 3.6, P = .025) were significantly associated with HB ≥3 when controlling for age, sex, extent of tumor resection, translabyrinthine approach, and prior radiation. Conclusion Tumor volume >3 cm3 and NF2 tumors are associated with poorer facial nerve outcomes 1 year following microsurgical resection.

scholarly journals Long-term facial nerve clinical evaluation following vestibular schwannoma surgery

2008 ◽  
Vol 66 (2a) ◽  
pp. 194-198 ◽  
Author(s):  
Rafaela Julia Batista Veronezi ◽  
Yvens Barbosa Fernandes ◽  
Guilherme Borges ◽  
Ricardo Ramina
Keyword(s):  
Facial Nerve ◽  
Vestibular Schwannoma ◽  
Tumor Size ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Significant Difference ◽  
Long Term Follow Up ◽  
Facial Function

BACKGROUND: Facial function is important in accompaniment of patients operated on vestibular schwannoma (VS). OBJETIVE: To evaluate long term facial nerve function in patients undergoing VS resection and to correlate tumor size and facial function in a long-term follow-up. METHOD: Transversal study of 20 patients with VS operated by the retrosigmoid approach. House-Brackmann Scale was used preoperatively, immediately after surgery and in a long-term follow-up. Student t test was applied for statistic analysis. RESULTS: In the immediate postoperative evaluation, 65% of patients presented FP of different grades. Improvement of facial nerve function (at least of one grade) occurred in 53% in the long-term follow-up. There was statistically significant difference in facial nerve outcome in long-term follow-up when tumor size was considered (p<0.05). Conclusion: The majority of patients had improvement of FP in a long-term follow-up and tumor size was detected to be a factor associated with the postoperative prognostic.

Translabyrinthine resection of neurofibromatosis type 2 associated vestibular and facial schwannomas, repair of facial nerve, and placement of auditory brainstem implant

2014 ◽  
Vol 36 (v1supplement) ◽  
pp. 1 ◽  
Author(s):  
Marc S. Schwartz ◽  
Gregory P. Lekovic ◽  
Derald E. Brackmann ◽  
Courtney C. J. Voelker
Keyword(s):  
Facial Nerve ◽  
Vocal Cord Paralysis ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Facial Nerve Function ◽  
Ipsilateral Side ◽  
Total Resection ◽  
Auditory Brainstem Implant

We present video of gross-total resection of a large cerebellopontine angle tumor consisting of both vestibular and facial schwannoma components via the translabyrinthine route in a patient with neurofibromatosis type 2. The facial nerve is reconstructed using a greater auricular nerve graft, and an auditory brainstem implant is placed. Prior to surgery the patient had no facial nerve function on the operative side and had lost useful hearing. He also had usable vision only on the ipsilateral side and had contralateral vocal cord paralysis.The video can be found here: http://youtu.be/IOkEND-0vhI.

scholarly journals Stereotactic radiosurgery in the management of acoustic neuromas associated with neurofibromatosis Type 2

1999 ◽  
Vol 6 (3) ◽  
pp. E1
Author(s):  
Brian R. Subach ◽  
Douglas Kondziolka ◽  
L. Dade Lunsford ◽  
David J. Bissonette ◽  
John C. Flickinger ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tumor Control ◽  
Nerve Function ◽  
Tumor Control Rate ◽  
Acoustic Neuromas ◽  
The Mean

Object Stereotactically guided radiosurgery is one of the primary treatment modalities for patients with acoustic neuromas (vestibular schwannomas). The goal of radiosurgery is to arrest tumor growth while preserving neurological function. Patients with acoustic neuromas associated with neurofibromatosis Type 2 (NF2) represent a special challenge because of the risk of complete deafness. To better define the tumor control rate and long-term functional outcome, the authors reviewed their 10-year experience in treating these lesions. Methods Forty patients underwent stereotactic radiosurgery at the University of Pittsburgh, 35 of them for solitary tumors. The other five underwent staged procedures for bilateral lesions (10 tumors, 45 total). Thirteen patients (with 29% of tumors) had undergone a median of two prior resections. The mean tumor volume at radiosurgery was 4.8 ml and the mean tumor margin dose was 15 Gy (range 12–20 Gy). The overall tumor control rate was 98%. During the median follow-up period of 36 months, 16 (36%) tumors regressed, 28 (62%) remained unchanged, and one (2%) grew. In the 10 patients for whom more than 5 years of clinical and neuroimaging follow-up results were available (median 92 months), five tumors were smaller and five remained unchanged. Surgical resection was performed in three patients (7%) after radiosurgery; only one showed radiographic evidence of progression. Useful hearing (Gardner-Robertson Class I or II) was preserved in six (43%) of 14 patients and this rate improved to 67% after modifications made in 1992. Normal facial nerve function (House-Brackmann Grade 1) was preserved in 25 (81%) of 31 patients. Normal trigeminal nerve function was preserved in 34 (94%) of 36 patients. Conclusions Stereotactically guided radiosurgery is a safe and effective treatment for patients with acoustic tumors in the setting of NF2. The rate of hearing preservation may be better with radiosurgery than with other available techniques.

scholarly journals Disease course of Neurofibromatosis Type 2; a 30-year follow-up study of 353 patients seen at a single institution

2020 ◽  
Author(s):  
Claire Forde ◽  
Andrew T King ◽  
Scott A Rutherford ◽  
Charlotte Hammerbeck-Ward ◽  
Simon K Lloyd ◽  
...  
Keyword(s):  
De Novo ◽  
Radiation Treatment ◽  
Univariate Analysis ◽  
Age At Onset ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Disease Course ◽  
Number Of Patients

Abstract Background Limited data exists on the disease course of Neurofibromatosis Type 2 (NF2) to guide clinical trial design. Methods A prospective database of patients meeting NF2 diagnostic criteria, reviewed between 1990–2020, was evaluated. Follow-up to first vestibular schwannoma (VS) intervention and death was assessed by univariate analysis and stratified by age at onset, era referred and inheritance type. Interventions for NF2-related tumours were assessed. Cox regression was performed to determine the relationship between individual factors from time of diagnosis to NF2-related death. Results Three-hundred-and-fifty-three patients were evaluated. During 4643.1 follow-up years from diagnosis to censoring 60 patients (17.0%) died. The annual mean number of patients undergoing VS surgery or radiotherapy declined, from 4.66 and 1.65 respectively per 100 NF2 patients in 1990-1999 to 2.11 and 1.01 in 2010-2020, as the number receiving bevacizumab increased (2.51 per 100 NF2 patients in 2010-2020). Five patients stopped bevacizumab to remove growing meningioma or spinal schwannoma. 153/353 (43.3%) had at least one neurosurgical intervention/radiation treatment within 5 years of diagnosis. Patients asymptomatic at diagnosis had longer time to intervention and better survival compared to those presenting with symptoms. Those symptomatically presenting &lt;16 and &gt;40 years had poorer overall survival than those presenting at 26-39 years (P=0.03 and P=0.02 respectively) but those presenting between 16-39 had shorter time to VS intervention. Individuals with de novo constitutional variants had worse survival than those with de novo mosaic or inherited disease (P=0.004). Conclusion Understanding disease course improves prognostication, allowing for better informed decisions about care.

Age at symptom onset and long-term survival in patients with neurofibromatosis Type 2

2003 ◽  
Vol 99 (3) ◽  
pp. 480-483 ◽  
Author(s):  
Goro Otsuka ◽  
Kiyoshi Saito ◽  
Tetsuya Nagatani ◽  
Jun Yoshida
Keyword(s):  
Symptom Onset ◽  
Survival Rates ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Content Type ◽  
Fine Print

Object. Neurofibromatosis Type 2 (NF2) is an intractable disorder predisposing to multiple, recurrent tumors of the central nervous system (CNS). To clarify the survival rate and characteristics that predict poor survival, we retrospectively reviewed clinical data in cases of NF2. Methods. From among 283 patients with neurofibromatosis who had been registered in a nationwide study in Japan between 1986 and 1987, 74 patients with bilateral vestibular schwannomas were analyzed. The mean duration of follow up after diagnosis was 121 months (range 2–287 months). Results of a Kaplan—Meier product-limit analysis indicated that overall 5-, 10-, and 20-year patient survival rates following diagnosis of NF2 were 85, 67, and 38%, respectively. Early onset of the initial symptom significantly compromised survival; 5-, 10-, and 20-year survival rates in patients with symptom onset at an age younger than 25 years were 80, 60, and 28%, respectively, whereas in patients with symptom onset at an age of 25 years or older the rates were 100, 87, and 62%, respectively. Patients with small vestibular schwannomas at diagnosis (< 2 cm in diameter) had better rates of survival. Other variables such as sex, additional tumors in the CNS, or dermal abnormalities did not significantly affect survival. Conclusions. This first report of long-term follow-up results concerning the survival of patients with NF2 indicates an adverse effect of early symptom onset.

Effect of Anatomic Segment Involvement on Stereotactic Radiosurgery for Facial Nerve Schwannomas: An International Multicenter Cohort Study

Neurosurgery ◽  
2020 ◽  
Vol 88 (1) ◽  
pp. E91-E98 ◽  
Author(s):  
Gautam U Mehta ◽  
Gregory P Lekovic ◽  
William H Slattery ◽  
Derald E Brackmann ◽  
Hao Long ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Stereotactic Radiosurgery ◽  
Radiation Effects ◽  
Progression Free Survival ◽  
Functional Improvement ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Multicenter Cohort Study ◽  
Facial Numbness

Abstract BACKGROUND Facial nerve schwannomas are rare, challenging tumors to manage due to their nerve of origin. Functional outcomes after stereotactic radiosurgery (SRS) are incompletely defined. OBJECTIVE To analyze the effect of facial nerve segment involvement on functional outcome for these tumors. METHODS Patients who underwent single-session SRS for facial nerve schwannomas with at least 3 mo follow-up at 11 participating centers were included. Preoperative and treatment variables were recorded. Outcome measures included radiological tumor response and neurological function. RESULTS A total of 63 patients (34 females) were included in the present study. In total, 75% had preoperative facial weakness. Mean tumor volume and margin dose were 2.0 ± 2.4 cm3 and 12.2 ± 0.54 Gy, respectively. Mean radiological follow-up was 45.5 ± 38.9 mo. Progression-free survival at 2, 5, and 10 yr was 98.1%, 87.2%, and 87.2%, respectively. The cumulative proportion of patients with regressing tumors at 2, 5, and 10 yr was 43.1%, 63.6%, and 63.6%, respectively. The number of involved facial nerve segments significantly predicted tumor progression (P = .04). Facial nerve function was stable or improved in 57 patients (90%). Patients with involvement of the labyrinthine segment of the facial nerve were significantly more likely to have an improvement in facial nerve function after SRS (P = .03). Hearing worsened in at least 6% of patients. Otherwise, adverse radiation effects included facial twitching (3 patients), facial numbness (2 patients), and dizziness (2 patients). CONCLUSION SRS for facial nerve schwannomas is effective and spares facial nerve function in most patients. Some patients may have functional improvement after treatment, particularly if the labyrinthine segment is involved.

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