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Awake craniotomy for auditory brainstem implant in patients with neurofibromatosis type 2:Four case reports

World Journal of Clinical Cases 2021年第25期9卷 7512-7519页

作者： De-Xiang Wang Shuo Wang Min-Yu Jian Ru-Quan Han Department of Anesthesiology Beijing Tiantan HospitalCapital Medical UniversityBeijing 100070China

BACKGROUND The auditory brainstem implant(ABI)is a significant treatment to restore hearing sensations for neurofibromatosis type 2(NF2)***,there is no ideal method in assisting the placement of *** this case series,intraoperative cochlear nucleus mapping was performed in awake craniotomy to help guide the placement of the electrode *** SUMMARY We applied the asleep-awake-asleep technique for awake craniotomy and hearing test via the retrosigmoid approach for acoustic neuroma resections and ABIs,using mechanical ventilation with a laryngeal mask during the asleep phases,utilizing a ropivacaine-based regional anesthesia,and sevoflurane combined with propofol/remifentanil as the sedative/analgesic agents in four NF2 *** electrode arrays were placed in the awake phase with successful intraoperative hearing tests in three *** was one uncooperative patient whose awake hearing test needed to be *** all cases,tumor resection and ABI were performed *** electrode effectiveness was achieved in awake ABI *** This case series suggests that awake craniotomy with an intraoperative hearing test for ABI placement is safe and well *** craniotomy is beneficial for improving the accuracy of ABI electrode placement and meanwhile reduces non-auditory side effects.

关键词： Awake craniotomy neurofibromatosis type 2 Auditory brainstem implant Hearing test Case report

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Two Cases of Spinal Tanycytic Ependymoma Associated with neurofibromatosis type 2

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Chinese Medical Journal 2017年第7期130卷 872-873页

作者： Xiao-Gang Tao Zong-Gang Hou Shu-Yu Hao Jun-Ting Zhang Bai-Yun Liu Department of Neurosurgery Beijing Tiantan Hospital Capital Medical University Beijing 100050 China

INTRODUCTION Tanycytic ependymoma is a rare subtype of the World Health Organization Grade il ependymoma most commonly found in the cervical and thoracic segments of the spinal cord. Spinal tumors in neurofibromatosis type 2 （NF-2） can be intramedullary, extramedullary, or associated with the nerve roots, and there may be multiple tumors at multiple locations in the spine.

关键词： neurofibromatosis type 2 Spinal Tumor Tanycytic Ependymoma

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MISME Syndrome:A Case Report Of neurofibromatosis type-2

MISME Syndrome:A Case Report Of Neurofibromatosis Type-2

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中国中西医结合学会神经外科专业委员会第一届学术大会暨神经外科专业委员会成立大会

作者： Jin Xin He Junhua Vang Dingkun Zhang Xinwen Wu Ye Liu Hanjiang Wu Zhangyi Jin Jun Luo Neurosurgery Department Zhejiang Provincial Tongde Hospital Department of Neurology Temple University School of Medicine

Mnemonic MISME syndrome is a rare neurofibromatosis type-2(NF2) for multiple inherited schwannomas,meningiomas,and *** is an autosomal dominantly inherited disorder due to mutations of a tumor-suppressor gene on the chromosome *** it is characterized by multiple benign tumors arising in both the central and the peripheral nervous system,particularly from the bilateral vestibulocochlear nerve in more than 90%of the patients with NF2 and more than two thirds of them develop spinal tumors,which are often devastating and difficult to *** occurrence of triple tumors in an NF2 patient is ***,simultaneous occurrence of the triple tumors in a shared common location is even *** reported here a young woman with MISME syndrome with triple tumors affecting the cervical spinal cord in whom staging microsurgical resection intervention produced a clinically favorable *** reports with immunochemical staining showed the presence of schwannomas,meningiomas,and *** claimed that surgical debulking on those symptomatic craniospinal tumors is the option of treatment of the *** microsurgical techniques may reduce adverse outcomes.

关键词： MISME syndrome neurofibromatosis type 2 craniospinal tumors staging operation microsurgery schwannomas meningiomas ependymomas

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Isolated colonic neurofibroma, a rare tumor: A case report and review of literature

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World Journal of Clinical Cases 2020年第10期8卷 1932-1938页

作者： Sara Ghoneim Sonia Sandhu Dalbir Sandhu Department of Internal Medicine Case Western Reserve University at MetroHealth Medical CenterClevelandOH 44109United States Department of Hematology and Oncology Cleveland Clinic Akron GeneralClevelandOH 44307United States Division of Gastroenterology Case Western Reserve University at MetroHealth Medical CenterClevelandOH 44109United States

BACKGROUND Neurofibromas are tumors comprised of peripheral nerve sheath and connective tissue *** can occur sporadically or as part of familial syndromes such as neurofibromatosis type *** colonic neurofibroma without systemic manifestations is a rarely reported clinical *** we present a case of a 51 years old male with an isolated colonic neurofibroma seen on a screening *** SUMMARY Fifty-one years old male who was otherwise healthy without a significant family history of cancer underwent a screening colonoscopy and was found have a 2.3 cm×1.4 cm lesion in the *** biopsy revealed a spindle cell *** resonance imaging of the pelvis was negative for *** underwent an endoscopic ultrasound that showed an ill-defined avascular lesion of mixed echogenicity measuring 2.8 cm×15.2 cm in the submucosa with no communication with muscularis mucosa or *** staining of the tumor was strongly positive for S100,with rare penetrating axons deep within the *** cells were negative for c-kit and desmin and had low Ki-67 *** findings were consistent with a solitary colonic submucosal neurofibroma.A detailed history and physical examination did not reveal any evidence of extraintestinal *** underwent transanal surgical resection of the *** patient tolerated the procedure well without any *** While neurofibromas have been well described in literature,an isolated colonic neurofibroma is a rare pathological *** transformation of neurofibromas has been reported in patients with neurofibromatosis *** report a case of isolated colonic neurofibroma and highlight the importance of resection due to the increased risk of tumorigenesis.

关键词： Neurofibroma Colonoscopy neurofibromatosis type 1 neurofibromatosis type 2 Spindle cell tumor Endoscopic ultrasound Case report

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Clinical and scientific innovations in auditory brainstem implants

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World Journal of Otorhinolaryngology-Head and Neck Surgery 2021年第2期7卷 109-115页

作者： Kunal R.Shetty Sarah E.Ridge Vivek Kanumuri Angela Zhu M.Christian Brown Daniel J.Lee Department of Otorhinolaryngology Head and Neck Surgery McGovern Medical School at the University of Texas Health Science Center at HoustonHoustonTXUSA Eaton-Peabody Laboratories Massachusetts Eye and Ear InfirmaryHarvard Medical SchoolBostonMAf USA

The auditory brainstem implant(ABI)was originally developed to provide rehabilitation of retrocochlear deafness caused by neurofibromatosis type 2(NF2).Recent studies of the ABI have investigated outcomes in non-NF2 cohorts,such as patients with cochlear nerve aplasia or cochlear ossification and more recently,intractable *** technologies that improve the ABI-neural tissue interface are being explored as means to improve performance and decrease side *** discoveries in optogenetics and bioengineering present opportunities to continually evolve this technology into the future,enhancing spatial selectivity of neuronal activation in the cochlear nucleus and preventing side effects through reduction in activation of non-target neuronal *** advances will improve surgical planning and ultimately improve patients1 audiological *** research has rapidly increased in the 21st century and applications of this technology are likely to continually ***,we aim to characterize ongoing clinical,basic science,and bioengineering advances in ABIs and discuss future directions of this technology.

关键词： Auditory brainstem implant Cochlear nucleus neurofibromatosis type 2 Optogenetics ABI Conformable electrode array Otology Neurotology

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