McMonagle B, Al-Sanosi A, Croxson G, Fagan P
Department of Otolaryngology Head and Neck Surgery, St Vincent's Hospital, Sydney, New South Wales, Australia.
J Laryngol Otol. 2008 Nov;122(11):1139-50. doi: 10.1017/S0022215107000667. Epub 2008 Jan 7.
To report a series of 53 cases of facial schwannoma, to review the current literature, addressing contentious issues, and to present a management algorithm.
Retrospective case review combined with review of current literature.
A review of the case notes of 53 patients with intracranial and intratemporal facial schwannoma, from two tertiary referral centres, was undertaken. This represents the largest series of facial schwannomas with clinical correlations in the literature. Data relating to epidemiological, clinical and management details were tabulated and compared with other large series. A review of the current literature was performed, and a management algorithm presented.
There were 23 (43 per cent) female and 30 (57 per cent) male patients. Patients' ages at presentation ranged from five to 84 years, with a mean of 49 years. Twenty-five (47 per cent) of the tumours were present on the left side and 28 (53 per cent) on the right side. Hearing loss was the most common presenting symptom, being present in 31/53 (58 per cent) patients, followed by facial weakness in 27/53 (51 per cent). Two patients (4 per cent) were completely asymptomatic, and their facial neuromas were diagnosed incidentally. The schwannoma extended along more than one segment of the facial nerve in 39 patients (74 per cent), with the mean number of segments involved being 2.5. A conservative approach of clinical observation was undertaken in 20 patients (38 per cent). Thirty-three patients (62 per cent) underwent surgery, with a total of 36 procedures. The translabyrinthine approach was most common, being utilised in 17 of the 36 procedures. Two patients underwent revision surgery for residual or recurrent disease on three occasions. There was total removal of tumour in 21 cases; the remainder had subtotal or no removal with drainage or decompression of the tumours. Twenty-one nerve reconstructions were performed, and 18 facial rehabilitation procedures were performed on 14 patients.
The results of this case series are similar to those of other reported series. The diagnosis of facial schwannoma is now generally made pre-operatively, due to improved imaging techniques and heightened awareness. Clinical assessment of facial function and imaging form the mainstays of surveillance for these tumours. These tumours are managed via clinical observation or surgical intervention; the latter can range from simple procedures (such as drainage of cystic components) to aggressive tumour removal and facial nerve reconstruction. Facial rehabilitation procedures may also be applied. The timing of intervention is contentious; surgical intervention is indicated when facial function deteriorates to a House-Brackmann grade IV level.
Facial schwannomas are rare lesions, and reported series are generally small. Due to the complex management issues involved, these tumours are best managed in a tertiary referral setting. Observation is preferred until facial function deteriorates to a House-Brackmann grade III level, at which time surgery is considered. When facial function deteriorates to House-Brackmann grade IV, surgical intervention is indicated. We advocate surgical management based on the treatment algorithm described.
报告53例面部神经鞘瘤病例系列,回顾当前文献,探讨有争议的问题,并提出一种治疗方案。
回顾性病例分析并结合当前文献综述。
对来自两个三级转诊中心的53例颅内及颞内面部神经鞘瘤患者的病历进行回顾。这是文献中最大的一系列具有临床相关性的面部神经鞘瘤病例。将流行病学、临床及治疗细节数据制成表格,并与其他大型病例系列进行比较。对当前文献进行综述,并提出一种治疗方案。
女性患者23例(43%),男性患者30例(57%)。患者就诊时年龄从5岁至84岁不等,平均年龄49岁。25例(47%)肿瘤位于左侧,28例(53%)位于右侧。听力损失是最常见的首发症状,53例患者中有31例(58%)出现,其次是面部无力,53例患者中有27例(51%)出现。2例患者(4%)完全无症状,其面部神经鞘瘤为偶然发现。39例患者(74%)的神经鞘瘤沿面神经的一个以上节段延伸,平均受累节段数为2.5个。20例患者(38%)采取了临床观察的保守方法。33例患者(62%)接受了手术,共进行了36次手术。经迷路入路最为常见,36次手术中有17次采用该入路。2例患者因残留或复发性疾病接受了3次翻修手术。21例肿瘤完全切除;其余患者肿瘤部分切除或未切除,并行肿瘤引流或减压。进行了21次神经重建,14例患者进行了18次面部康复手术。
本病例系列的结果与其他报道的系列相似。由于成像技术的改进和认识的提高,目前面部神经鞘瘤一般在术前作出诊断。面部功能的临床评估和成像检查是这些肿瘤监测的主要手段。这些肿瘤通过临床观察或手术干预进行治疗;后者范围从简单手术(如囊性成分引流)到积极的肿瘤切除及面神经重建。也可应用面部康复手术。干预时机存在争议;当面部功能恶化至House-Brackmann IV级时,应进行手术干预。
面部神经鞘瘤是罕见病变,报道的病例系列一般规模较小。由于涉及复杂的治疗问题,这些肿瘤最好在三级转诊机构进行治疗。在面部功能恶化至House-Brackmann III级之前,首选观察,此时考虑手术。当面部功能恶化至House-Brackmann IV级时,应进行手术干预。我们提倡根据所描述的治疗方案进行手术治疗。