Gonçalves M B, Siqueira S B P, Christoph D H, Klescoski J, Melo M H A, Landeiro J A
Neurosurgery Department, Hospital de Força Aérea do Galeão, HFAG, Rio de Janeiro, Brazil.
Minim Invasive Neurosurg. 2010 Feb;53(1):37-9. doi: 10.1055/s-0030-1247553. Epub 2010 Apr 7.
Fibrous dysplasia is a non-heritable, congenital disease involving the bones. Its diagnosis relies on image examination and pathology. Treatment is usually conservative, but in cases of cranial nerve impingement or extension to the condyles, surgery should be considered. In this paper, we describe a singular case of monostotic fibrous dysplasia of the clivus in a child.
A 14-year-old boy presented with chronic headache and diplopia. A neurological examination revealed palsy of the right VIth cranial nerve. The radiological findings were consistent with fibrous dysplasia, with a hypointense and isointense clival lesion on T (1)- and T (2)-weighted images, respectively. Surgical treatment was indicated by cranial nerve impairment. An endoscopic endonasal approach with an image-guided system was used. The patient was discharged four days after surgery and his diplopia improved postoperatively.
A variety of surgical approaches have been used in the treatment of midline extra-axial cranial base tumors. Fibrous dysplasia of the clivus without expansion to the condylus can be removed with a transsphenoidal endonasal endoscopic approach. Preoperative imaging reveals the individual patient's anatomy and improves the intraoperative orientation.
Image-guided transsphenoidal endoscopic surgery is recommended for its safety and minimal invasiveness.
骨纤维异常增殖症是一种累及骨骼的非遗传性先天性疾病。其诊断依赖于影像学检查和病理学检查。治疗通常较为保守，但在出现颅神经受压或病变延伸至髁突的情况下，应考虑手术治疗。在本文中，我们描述了一名儿童斜坡单骨型骨纤维异常增殖症的罕见病例。
一名14岁男孩出现慢性头痛和复视。神经系统检查发现右侧第六颅神经麻痹。影像学检查结果与骨纤维异常增殖症相符，在T1加权像和T2加权像上，斜坡病变分别呈低信号和等信号。由于颅神经受损，需进行手术治疗。采用了带有图像引导系统的鼻内镜经鼻入路。患者术后四天出院，复视术后有所改善。
多种手术入路已被用于治疗中线轴外颅底肿瘤。未向髁突扩展的斜坡骨纤维异常增殖症可通过经蝶窦鼻内镜入路切除。术前影像学检查可显示个体患者的解剖结构，有助于改善术中定位。
鉴于其安全性和微创性，推荐采用图像引导下经蝶窦内镜手术。
