Nanami T, Hidaka T, Kidoguchi J, Ogawa A
Department of Neurosurgery, Iwate Medical University, Japan.
No Shinkei Geka. 1994 Oct;22(10):967-71.
A surgical case of monostotic fibrous dysplasia of the left frontal and sphenoidal bone in a 14-year-old girl is described. This girl was admitted to our hospital in March, 1992, with a chief complaint of facial deformity and asymmetry due to a painless and progressive bony bulging over the left fronto-orbital region. But she denied any symptoms such as proptosis, diplopia, optic atrophy and visual loss. Other data found on neurological examination and laboratory tests were normal. In addition, she had no history of skin lesions, precocious puberty or other endocrine abnormalities. Plain craniogram showed remarkable thickening of the left frontal bone and of the anterior cranial fossa of the sphenoidal bone with irregular stenosis of the left optic canal. CT scan showed the diffuse enlargement of the affected bone and involvement of the paranasal sinuses. Angiography revealed no positive findings. On December 10, 1992, orbito-cranial reconstruction and unilateral optic canal release were performed using an extradural approach through a left fronto-temporal craniectomy. Histological findings confirmed the lesion to be typical fibrous dysplasia. She recovered completely one month after the operation, but she suffered transient blurred vision, diplopia and left ptosis. Most of the decreased vision caused by fibrous dysplasia cannot be reversed after surgical treatment. So, if optic canal stenosis is evident, even when visual loss is not clear, release of the optic canal stenosis should be done as early as possible in association with experienced neurosurgeons and with meticulous dissection.
本文描述了一名14岁女孩左额骨和蝶骨单骨型骨纤维异常增殖症的手术病例。该女孩于1992年3月因左额眶区无痛性进行性骨质膨隆导致面部畸形和不对称而入住我院。但她否认有任何诸如眼球突出、复视、视神经萎缩和视力丧失等症状。神经系统检查和实验室检查的其他结果均正常。此外，她无皮肤病变、性早熟或其他内分泌异常病史。头颅X线平片显示左额骨及蝶骨前颅窝明显增厚，左侧视神经管不规则狭窄。CT扫描显示患骨弥漫性增大并累及鼻窦。血管造影未发现阳性结果。1992年12月10日，通过左额颞开颅硬膜外入路进行了眶颅重建和单侧视神经管减压术。组织学检查结果证实病变为典型的骨纤维异常增殖症。术后1个月她完全康复，但出现了短暂的视力模糊、复视和左侧上睑下垂。骨纤维异常增殖症导致的大多数视力下降在手术治疗后无法恢复。因此，如果视神经管狭窄明显，即使视力丧失不明显，也应尽早与经验丰富的神经外科医生联合进行视神经管减压术，并进行细致的解剖操作。
