Tachibana E, Saito K, Takahashi M, Fukuta K, Yoshida J
Department of Neurosurgery, Nagoya University, Postgraduate School of Medicine, Nagoya, Japan.
Surg Neurol. 2000 Aug;54(2):165-9; discussion 169-70. doi: 10.1016/s0090-3019(00)00252-4.
A successfully treated massive chondrosarcoma in the skull base associated with Maffucci's syndrome is presented. The purpose of this report is to discuss the surgical approach to the tumor and reconstruction of the skull base.
A 36-year-old woman who had a history of multiple enchondromas and subcutaneous hemangiomas presented with decreased right visual acuity and left papilledema. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated a mass in the skull base. The tumor occupied the nasal and paranasal cavities, and extended to the anterior, middle, and posterior intracranial spaces. The midline skull base structures and the left middle cranial base were destroyed. Using a combined anterior craniofacial and left orbitozygomatic approach, the tumor was totally resected. The large skull base defect was reconstructed with a vascularized outer table parietal bone graft attached to a bipedicled temporoparietal galeal flap. The postoperative course was uneventful except for decreased left visual acuity, and temporary diplopia and facial hypesthesia. In 40 months of follow-up there was no recurrence. CONCLUSIONSA skull base approach should be selected to perform total resection of an extensive skull base tumor. The bipedicled temporoparietal galeal flap and vascularized calvarial bone was useful for simultaneous reconstruction.
本文报告一例成功治疗的与马富西综合征相关的颅底巨大软骨肉瘤病例。本报告的目的是讨论该肿瘤的手术入路及颅底重建方法。
一名36岁女性，有多发内生软骨瘤和皮下血管瘤病史，出现右眼视力下降和左眼视乳头水肿。计算机断层扫描（CT）和磁共振成像（MRI）显示颅底有一肿块。肿瘤占据鼻腔和鼻窦，并延伸至颅内前、中、后间隙。颅底中线结构及左侧中颅底被破坏。采用前颅面联合左侧眶颧入路，将肿瘤完全切除。用带血管蒂的顶骨外板骨瓣附着于双蒂颞顶部帽状腱膜瓣修复巨大的颅底缺损。术后除左眼视力下降、短暂复视和面部感觉减退外，病程顺利。随访40个月无复发。
对于广泛的颅底肿瘤，应选择颅底入路进行全切除。双蒂颞顶部帽状腱膜瓣和带血管蒂的颅骨对同期重建很有用。
