德國INI國際神經(jīng)科學(xué)研究所在神經(jīng)外科界享譽國際，擅長對各種神外疾病的手術(shù)治療，是聽神經(jīng)瘤、顱底及腦干疾病方面！德國INI在聽神經(jīng)瘤方面的許多理念、手術(shù)術(shù)式、術(shù)后并發(fā)癥控制和管理等方面的貢獻可謂比比皆是。此篇我們精選匯編了近十余年來其在聽神經(jīng)瘤方面手術(shù)的研究。

　　關(guān)鍵詞：家族性纖維瘤病，術(shù)后復(fù)發(fā)性，放療后復(fù)發(fā)性，較大型，囊性。

　　RISIA，神經(jīng)移植術(shù)，面神經(jīng)重建修復(fù)。

　　1. 乙狀竇后內(nèi)聽道上-內(nèi)擴展硬膜內(nèi)入路（RISIA）切除累及巖骨的復(fù)發(fā)性較大聽神經(jīng)瘤：手術(shù)技術(shù)報告。

　　Retrosigmoid Intradural Suprameatal-Inframeatal Approach for Complete Surgical Removal of a Giant Recurrent Vestibular Schwannoma with Severe Petrous Bone Involvement: Technical Case Report.

　　手術(shù)切除嚴(yán)重累及巖骨的復(fù)發(fā)性較大聽神經(jīng)瘤具有嚴(yán)重的并發(fā)癥風(fēng)險，手術(shù)挑戰(zhàn)較大。病例描述：27歲男性患者,較大的聽神經(jīng)瘤復(fù)發(fā)（4.5cm），腫瘤累及Meckel腔和左巖段頸內(nèi)動脈。由于腫瘤壓迫和先前手術(shù)史，癥狀包括左面部完全麻痹和聽力喪失，左側(cè)三叉神經(jīng)、外展神經(jīng)麻痹及后組顱神經(jīng)功能障礙。該患者還表現(xiàn)出嚴(yán)重的不協(xié)調(diào)和共濟失調(diào)。經(jīng)磨除巖骨上-內(nèi)方部分骨質(zhì)的RISIA入路，腫瘤得到完全切除，術(shù)后未發(fā)現(xiàn)相關(guān)并發(fā)癥。術(shù)后患者平衡和顱神經(jīng)功能完全恢復(fù)（除面神經(jīng)和耳蝸神經(jīng)）。結(jié)論：RISIA入路可以順利、合適地實現(xiàn)完全切除腫瘤，即使是在嚴(yán)重累及Meckel腔和巖段頸內(nèi)動脈的病例中。這是一個通過RISIA入路成功地完全切除復(fù)發(fā)性較大聽神經(jīng)瘤的報告。

　　Surgical removal of giant vestibular schwannomas with severe petrous bone involvement remains challenging due to the high risk of complications. The retrosigmoid intradural suprameatal-inframeatal approach (RISIA) allows for safe exposure extending from Meckel's cave to the petrous internal carotid artery (ICA). CASE DESCRIPTION: A 27-year-old man presented with recurrence of a giant vestibular schwannoma (4.5 cm) invading Meckel's cave and the left petrous ICA. Symptoms included complete left facial palsy and hearing loss due to tumor invasion and previous operations, as well as left-sided trigeminal hypesthesia, abducens nerve palsy, and lower cranial nerve dysfunction due to tumor compression. The patient also exhibited severe discoordination and ataxia. The tumor was completely resected via the RISIA, which involved drilling of the suprameatal and inframeatal portions of the petrous bone. No approach-related complications were observed. Full recovery of cranial nerve functions (with the exception of those related to the facial and cochlear nerves) and balance were observed postoperatively. CONCLUSIONS: The RISIA allows for safe and effective surgical access during complete tumor resection, even when severe involvement of Meckel's cave and the petrous ICA are observed. The present report is the first to demonstrate the usefulness of this approach in patients with recurrent giant vestibular schwannoma.

復(fù)發(fā)性較大聽神經(jīng)瘤經(jīng)RISIA全切，術(shù)前術(shù)后MR對比

　　2. 2型神經(jīng)纖維瘤病聽神經(jīng)瘤的顯微外科治療：適應(yīng)癥和結(jié)果。

　　Microsurgery management of vestibular schwannomas in neurofibromatosis type 2: indications and results.

　　目的：分析對2型神經(jīng)纖維瘤?。∟F2）患者的經(jīng)驗和治療策略，側(cè)重于聽神經(jīng)瘤（VS）手術(shù)。方法：在35年的時間里，術(shù)者（M.S.）對165例NF2患者進行了手術(shù)，手術(shù)的VS總數(shù)為210。該回顧性分析包括145位手術(shù)的患者。分析了病歷、手術(shù)報告、神經(jīng)系統(tǒng)檢查、聽力檢查和影像學(xué)檢查。結(jié)果：在85％的病例中實現(xiàn)了腫瘤全切。在15％的患者中，有意次全切是為了聽力保護及腦干減壓。聽神經(jīng)保留率為35％，當(dāng)術(shù)前患者有合適聽力誘發(fā)時，聽神經(jīng)保留率為65％，23％患者實現(xiàn)了雙側(cè)聽神經(jīng)保留。面神經(jīng)的解剖保留率為89％。

　　結(jié)論：NF2患者的VS手術(shù)目標(biāo)應(yīng)該是在不以犧牲神經(jīng)功能為代價下全切腫瘤，精心的個性化治療策略有助于延長生存期并保留神經(jīng)功能。

　　Aim:To analyze the senior author's experience and strategy of treatment of patients with neurofibromatosis type 2 (NF2), with particular emphasis on vestibular schwannoma (VS) surgery.

　　MATERIALS AND METHODS: Over a period of more than 35 years, the senior author (M.S.) has operated on more than 165 patients with NF2. The total number of VS surgeries was 210. This retrospective analysis includes 145 consecutively operated patients. Medical records, operative reports, follow-up neurological, audiometric examinations, and neuroradiological findings were analyzed. RESULTS: Total tumor removal was achieved in 85% of the operated tumors. In 15%, deliberately subtotal removal was performed for brain stem decompression and hearing preservation in the only hearing ear. The overall rate of hearing preservation was 35%. When only patients with preserved useful preoperative hearing were included, the rate was 65%. Bilateral hearing after surgery was preserved in 23% of the patients. The anatomical integrity of the facial nerve was preserved in 89%.

　　CONCLUSIONS: The goal of VS surgery in patients with NF2 should be complete removal but not at the expense of functional impairment. Carefully individualized treatment strategy offers the possibility of prolongation of life and preservation of neurological functions.

　　3. 聽神經(jīng)瘤術(shù)后使用端-側(cè)腓腸神經(jīng)異位移植術(shù)修復(fù)面部神經(jīng)功能：手術(shù)技術(shù)報告

　　Using an end-to-side interposed sural nerve graft for facial nerve reinforcement after vestibular schwannoma resection. Technical note.

　　在過去的30年中，聽神經(jīng)瘤手術(shù)后面部和聽神經(jīng)保存率提高了。然而，部分或完全受損的面神經(jīng)的治療仍然面臨嚴(yán)峻的形勢。在這種情況下，可以通過即時或擇期手術(shù)進行治療?；诮诔晒Φ亩?側(cè)神經(jīng)吻合術(shù)的研究，作者將該技術(shù)應(yīng)用于在聽神經(jīng)瘤手術(shù)中面神經(jīng)解剖保留但功能部分缺損的患者，作者使用端-側(cè)腓腸神經(jīng)異位移植以增強部分面神經(jīng)解剖的連續(xù)性。術(shù)后18個月的隨訪檢查顯示，面部神經(jīng)功能的效果令人滿意。在部分面部神經(jīng)損傷的病例，端-側(cè)神經(jīng)異位移植是一種合理的修復(fù)方式，并且可能是未來其他顱神經(jīng)損傷的治療選擇。

　　Increasing rates of facial and cochlear nerve preservation after vestibular schwannoma surgery have been achieved in the last 30 years. However, the management of a partially or completely damaged facial nerve remains an important issue. In such a case, several immediate or delayed repair techniques have been used. On the basis of recent studies of successful end-to-side neurorrhaphy, the authors applied this technique in a patient with an anatomically preserved but partially injured facial nerve during vestibular schwannoma surgery. The authors interposed a sural nerve graft to reinforce the facial nerve whose partial anatomical continuity had been preserved. On follow-up examinations 18 months after surgery, satisfactory cosmetic results for facial nerve function were observed. The end-to-side interposed nerve graft appears to be a reasonable alternative in cases of partial facial nerve injury, and might be a future therapeutic option for other cranial nerve injuries.

　　4. 放療術(shù)失敗后的聽神經(jīng)瘤患者的外科治療。

　　Surgical treatment of patients with vestibular schwannomas after failed previous radiosurgery.

　　越來越多的聽神經(jīng)瘤（VSs）患者接受放療，但是，據(jù)報道有2％9％的患者出現(xiàn)放療失敗-或繼發(fā)性再生長。在壓迫腦干的大腫瘤中以及神經(jīng)功能快速惡化的患者中，手術(shù)切除是合理的治療選擇。方法：作者評估了28例VS患者中先前放療與手術(shù)結(jié)果的相關(guān)性。該研究進一步細(xì)分為A組（術(shù)前有過放療的患者）和B組（術(shù)前有過放療且腫瘤部分切除的患者）。將這兩組患者的功能和一般結(jié)果與對照組進行比較（無先前治療的患者）。結(jié)果：A組15例，B組13例，對照組30例。手術(shù)適應(yīng)癥情況：腫瘤的持續(xù)性增大和神經(jīng)功能的惡化（12例），腫瘤持續(xù)的增大（15例），神經(jīng)癥狀的惡化而沒有腫瘤生長（1例）。A、B兩組及對照組的全部患者中，96.7％均實現(xiàn)了腫瘤全切。任何一組都沒有死亡病例。盡管未觀察到術(shù)后神經(jīng)缺損或并發(fā)癥發(fā)生率的顯著差異，但B組患者出現(xiàn)新發(fā)的顱神經(jīng)功能缺損和CSF漏的風(fēng)險較高。接受過放療的患者（A和B組）傾向于在腫瘤床或小腦中發(fā)生術(shù)后血腫的風(fēng)險更高。先前未接受治療的患者的面部神經(jīng)解剖保存率較高（93.3％），而在A組和B組中分別降至86.7％和61.5％。面神經(jīng)功能保留率被發(fā)現(xiàn)與先前的治療有關(guān)，在對照組為87％，A組為78％和B組為68％。結(jié)論：放療失敗后，顯微手術(shù)可以成功全切腫瘤。然而，術(shù)后功能結(jié)果往往比未經(jīng)治療的患者差。先前有過放療及部分切除的患者，手術(shù)較具挑戰(zhàn)性，且預(yù)后差。

　　OBJECT: An increasing number of patients with vestibular schwannomas (VSs) are being treated with radiosurgery. Treatment failure or secondary regrowth after radiosurgery, however, has been observed in 2%-9% of patients. In large tumors that compress the brainstem and in patients who experience rapid neurological deterioration, surgical removal is the only reasonable management option. METHODS: The authors evaluated the relevance of previous radiosurgery for the outcome of surgery in a series of 28 patients with VS. The cohort was further subdivided into Group A (radiosurgery prior to surgery) and Group B (partial tumor removal followed by radiosurgery prior to current surgery). The functional and general outcomes in these 2 groups were compared with those in a control group (no previous treatment, matched characteristics). RESULTS： There were 15 patients in Group A, 13 in Group B, and 30 in the control group. The indications for surgery were sustained tumor enlargement and progression of neurological symptoms in 12 patients, sustained tumor enlargement in 15 patients, and worsening of neurological symptoms without evidence of tumor growth in 1 patient. Total tumor removal was achieved in all patients in Groups A and B and in 96.7% of those in the control group. There were no deaths in any group. Although no significant differences in the neurological morbidity or complication rates after surgery were noted, the risk of new cranial nerve deficits and CSF leakage was highest in patients in Group B. Patients who underwent previous radiosurgical treatment (Groups A and B) tended to be at higher risk of developing postoperative hematomas in the tumor bed or cerebellum. The rate of facial nerve anatomical preservation was highest in those patients who were not treated previously (93.3%) and decreased to 86.7% in the patients in Group A and to 61.5% in those in Group B. Facial nerve function at follow-up was found to correlate to the previous treatment; excellent or good function was seen in 87% of the patients from the control group, 78% of those in Group A, and 68% of those in Group B. CONCLUSIONS: Complete microsurgical removal of VSs after failed radiosurgery is possible with an acceptable morbidity rate. The functional outcome, however, tends to be worse than in nontreated patients. Surgery after previous partial tumor removal and radiosurgery is most challenging and related to worse outcome.

　　5. 聽神經(jīng)瘤伴瘤周水腫的手術(shù)

　　Surgery of Vestibular Schwannomas with Peritumoral Edema

　　腦膜瘤中腫瘤周圍水腫的存在與手術(shù)的復(fù)雜性和預(yù)后有關(guān)，其在聽神經(jīng)瘤（VS）中的意義尚未得到系統(tǒng)評估。方法：對系列患者進行回顧性研究，將VS瘤周水腫與以下因素進行相關(guān)性分析：腫瘤影像學(xué)特征、手術(shù)難度、腫瘤粘連、血管分布、包膜存在、功能預(yù)后和并發(fā)癥發(fā)生率。將實驗組結(jié)果與沒有腫周水腫的對照組進行比較。結(jié)果：共有30例患者（30/605例患者或5％）出現(xiàn)腫瘤周圍水腫。具有瘤周水腫患者更經(jīng)常發(fā)生耳鳴和腦積水，這些VS常富血供。手術(shù)難度與瘤周水腫沒有明顯關(guān)系，但和腫瘤的蛛網(wǎng)膜包膜相關(guān)。 A組的全部患者均實現(xiàn)了完全切除（對照組為97％）。面部神經(jīng)保留率為97％（原為98％）。兩組的功能結(jié)局相似。但是，對照組的術(shù)后血腫發(fā)生率更高（10％vs. 3％）。結(jié)論：伴有局灶性瘤周水腫的VS更富血供，應(yīng)警惕其術(shù)后更高的出血風(fēng)險。但是，以精良的顯微手術(shù)技術(shù)為前提，腫周水腫的存在不會影響腫瘤全切率及術(shù)后功能。

　　Introduction: The presence of peritumoral edema in meningiomas correlates with complexity of surgery and outcome. Its significance in vestibular schwannomas (VS) has not yet been evaluated systematically. Methods: Retrospective study of patients’ series. Correlative analysis of presence of edema to: radiological tumor characteristics, operative difficulty, tumor adhesion, vascularity, presence of capsule, functional outcome, and complication rate. The findings were compared with those in a matched control group without peritumoral edema (group B). Results: A total of 30 patients (30/605 patients or 5%) presented with peritumoral edema. Patients with edema had more frequently tinnitus and hydrocephalus. At surgery these VS were more frequently hypervascular. No major difference in the operative difficulty in patients with/without edema in regards to difficulty of tumor dissection and presence of arachnoid plane was found. Complete resection was achieved in all patients in group A (vs.97% in the control group B); the facial nerve was preserved in 97% (vs.大概率). Functional outcome in both groups was similar. However, the postoperative hemorrhage rate was higher in the current control Group (10 vs. 3%). Conclusion: VS with perifocal edema are more frequently hypervascular. This may lead to a higher rate of postoperative hemorrhages in the tumor bed and should always be considered. With adequate microsurgical technique, however, the presence of peritumoral edema does not influence the rate of complete tumor removal and the functional outcome.

　　參考文獻：

　　1. Sato Y, Mizutani T, Shimizu K, Freund HJ, Samii M. Retrosigmoid Intradural Suprameatal-Inframeatal Approach for Complete Surgical Removal of a Giant Recurrent Vestibular Schwannoma with Severe Petrous Bone Involvement: Technical Case Report. World Neurosurg. 2018;110:93-8.

　　2. Samii M, Gerganov V, Samii A. Microsurgery Management of Vestibular Schwannomas in Neurofibromatosis Type 2: Indications and Results. 2008;21:169-75.

　　3. Samii M, Koerbel A, Safavi-Abbasi S, Di Rocco F, Gharabaghi A. Using an end-to-side interposed sural nerve graft for facial nerve reinforcement after vestibular schwannoma resection. Technical note. Journal of Neurosurgery. 2006;105(6):920-3.

　　4. Gerganov V, Giordano M, Samii M. Surgical treatment of patients with vestibular schwannomas after failed previous radiosurgery. Journal of Neurosurgery. 2012;116(4):713-20.

　　5. Gerganov V, Giordano MG, Metwalli H, Samii A, Samii M. Surgery of Vestibular Schwannomas with Peritumoral Edema. Journal of Neurological Surgery Part B: Skull Base. 2014;75(S 02):a064.

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• 更新時間：2019-12-19 16:51:34