Type 2 neurofibromatosis: the need for supraregional care?

1993 ◽  
Vol 107 (5) ◽  
pp. 401-406 ◽  
Author(s):  
D. Gareth R. Evans ◽  
Richard Ramsden ◽  
Susan M. Huson ◽  
Rodney Harris ◽  
Richard Lye ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Vestibular Schwannoma ◽  
Acoustic Neuroma ◽  
Hearing Preservation ◽  
Definitive Treatment ◽  
Facial Nerve Function ◽  
Operative Results ◽  
Nerve Preservation ◽  
Quality Life

The results of a U.K. study of 145 cases of type 2 neurofibromatosis has shown generally very poor operative results in terms of hearing and facial nerve preservation. Only 9 out of 118 vestibular schwannoma (acoustic neuroma) operations resulted in any clinically detectable hearing preservation and only 32 left the patient with good or normal (House grade I or II) ipsilateral facial nerve function. Although operation is still the definitive treatment of vestibular schwannoma (acoustic neuroma) and may be a lifesaving procedure, it appears that the evidence in favour of early operation is only valid when carried out in highly specialized centres. The special problems of NF2 cases who may go on to develop multiple spinal and cranial tumours making them wheelchair bound and blind as well as deaf warrants a careful experienced approach. Timing of operations may be critical for the enhancement of useful years of quality life. We therefore propose the setting up of a national NF2 register, with the management of cases at a few supraregional centres.

scholarly journals Retrosigmoid Transmeatal Approach for Resection of Acoustic Neuroma: Operative Video and Technical Nuances of Subperineural Dissection for Facial Nerve Preservation

2019 ◽  
Vol 80 (S 03) ◽  
pp. S269-S270
Author(s):  
James K. Liu ◽  
Vincent N. Dodson ◽  
Robert W. Jyung
Keyword(s):  
Facial Nerve ◽  
Acoustic Neuroma ◽  
Protective Layer ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Tumor Removal ◽  
Nerve Preservation ◽  
Tumor Capsule ◽  
Acoustic Neuromas ◽  
Dissection Technique

The retrosigmoid (suboccipital) approach is the workhorse for most acoustic neuromas in the cerebellopontine angle. In this operative video atlas manuscript, the authors demonstrate the nuances of the subperineural dissection technique for microsurgical resection of an acoustic neuroma via the retrosigmoid transmeatal approach. The plane is developed by separating the perineurium of the vestibular nerve away from the tumor capsule. This perineurium provides a protective layer between the tumor capsule and the facial nerve which serves as a buffer to avoid direct dissection and potential trauma to the facial nerve. Using this technique during extracapsular tumor dissection helps to maximize the extent of tumor removal while preserving facial nerve function. A gross total resection of the tumor was achieved, and the patient exhibited normal facial nerve function (Fig. 1). In summary, the retrosigmoid transmeatal approach with the use of subperineural dissection are important strategies in the armamentarium for surgical management of acoustic neuromas with the goal of maximizing tumor removal and preserving facial nerve function (Fig. 2).The link to the video can be found at: https://youtu.be/L3lPtSvJt60.

scholarly journals Translabyrinthine Approach for Resection of Large Cystic Acoustic Neuroma: Operative Video and Technical Nuances of Subperineural Dissection for Facial Nerve Preservation

2019 ◽  
Vol 80 (S 03) ◽  
pp. S267-S268
Author(s):  
James K. Liu ◽  
Vincent N. Dodson ◽  
Robert W. Jyung
Keyword(s):  
Hearing Loss ◽  
Facial Nerve ◽  
Acoustic Neuroma ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Nerve Preservation ◽  
Translabyrinthine Approach ◽  
Acoustic Neuromas ◽  
Extracapsular Dissection ◽  
Cerebellar Retraction

The translabyrinthine approach is advantageous for the resection of large acoustic neuromas compressing the brainstem when hearing loss is nonserviceable. This approach provides wide access through the presigmoid corridor without prolonged cerebellar retraction. Early identification of the facial nerve at the fundus is also achieved. In this operative video atlas manuscript, the authors demonstrate a step-by-step technique for microsurgical resection of a large cystic acoustic neuroma via a translabyrinthine approach. The nuances of microsurgical and skull base technique are illustrated including performing extracapsular dissection of the tumor while maintaining a subperineural plane of dissection to preserve the facial nerve. This strategy maximizes the extent of removal while preserving facial nerve function. A microscopic remnant of tumor was left adherent to the perineurium. A near-total resection of the tumor was achieved and the facial nerve stimulated briskly at low thresholds. Other than preexisting hearing loss, the patient was neurologically intact with normal facial nerve function postoperatively. In summary, the translabyrinthine approach and the use of subperineural dissection are important strategies in the armamentarium for surgical management of large acoustic neuromas while preserving facial nerve function.The link to the video can be found at: https://youtu.be/zld2cSP8fb8.

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.

Improved preservation of hearing and facial nerve function in vestibular schwannoma surgery via the retrosigmoid approach in a series of 200 patients

2006 ◽  
Vol 105 (4) ◽  
pp. 527-535 ◽  
Author(s):  
Madjid Samii ◽  
Venelin Gerganov ◽  
Amir Samii
Keyword(s):  
Facial Nerve ◽  
Hearing Preservation ◽  
Neurological Status ◽  
Retrosigmoid Approach ◽  
Cerebrospinal Fluid Leakage ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Nerve Preservation ◽  
Permanent Morbidity

Object The aim of this study was to evaluate and present the results of current surgical treatment of vestibular schwannomas (VSs) and to report the refinements in the operative technique. Methods The authors performed a retrospective study of 200 consecutive patients who had undergone VS surgery over a 3-year period. Patient records, operative reports, follow-up data, and neuroradiological findings were analyzed. The main outcome measures were magnetic resonance imaging, neurological status, patient complaints, and surgical complications. Complete tumor removal was achieved in 98% of patients. Anatomical preservation of the facial nerve was possible in 98.5% of patients. In patients treated for tumors with extension Classes T1, T2, and T3, the rate of facial nerve preservation was 100%. By the last follow-up examination, excellent or good facial nerve function had been achieved in 81% of the cases. By at least 1 year postsurgery, no patients had total facial palsy. In the patients with preserved hearing, the rate of anatomical preservation of the cochlear nerve was 84%. The overall rate of functional hearing preservation was 51%. There was no surgery-related permanent morbidity in this series of patients. Cerebrospinal fluid leakage was diagnosed in 2% of the patients. The mortality rate was 0%. Conclusions The goal of VS treatment should be total removal in one stage and preservation of neurological function, as they determine a patient’s quality of life. This goal can be safely and successfully achieved using the retrosigmoid approach.

scholarly journals Retrosigmoid approach for giant cystic vestibular schwannoma: subperineural dissection technique for facial nerve preservation

2021 ◽  
Vol 5 (2) ◽  
pp. V7
Author(s):  
Ali Tayebi Meybodi ◽  
Robert W. Jyung ◽  
James K. Liu
Keyword(s):  
Facial Nerve ◽  
Thin Layer ◽  
Vestibular Schwannoma ◽  
Cranial Nerve ◽  
Retrosigmoid Approach ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Total Resection ◽  
Nerve Preservation ◽  
Dissection Technique

In this illustrative video, the authors demonstrate retrosigmoid resection of a giant cystic vestibular schwannoma using the subperineural dissection technique to preserve facial nerve function. This thin layer of perineurium arising from the vestibular nerves is used as a protective buffer to shield the facial and cochlear nerves from direct microdissection trauma. A near-total resection was achieved, and the patient had an immediate postoperative House-Brackmann grade I facial nerve function. The operative nuances and pearls of technique for safe cranial nerve and brainstem dissection, as well as the intraoperative decision and technique to leave the least amount of residual adherent tumor, are demonstrated. The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID21128

Preservation of Function in Vestibular Schwannoma Surgery

2007 ◽  
Vol 60 (suppl_2) ◽  
pp. ONS-124-ONS-128 ◽  
Author(s):  
Alireza Gharabaghi ◽  
Amir Samii ◽  
Andrei Koerbel ◽  
Steffen K. Rosahl ◽  
Marcos Tatagiba ◽  
...  
Keyword(s):  
Surgical Technique ◽  
Facial Nerve ◽  
Vestibular Schwannoma ◽  
Acoustic Neuroma ◽  
Patient Positioning ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Tumor Removal ◽  
Presurgical Evaluation ◽  
Microsurgical Techniques

Abstract THE MANAGEMENT AND surgical technique for microsurgical tumor removal of vestibular schwannomas (acoustic neuroma) with the suboccipital retrosigmoid approach and semi-sitting patient positioning is described. An emphasis is placed on the preservation of auditory and facial nerve function with a stepwise description of the technical and operative nuances, including presurgical evaluation, positioning, anesthesiological and neurophysiological aspects, approach, microsurgical techniques, and postsurgical care.

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