面部渐进性隆起伴视力下降的骨纤维异常增殖症1例

A case of fibrous dysplasia of bone with progressive facial deformity and decreased vision

20岁女性,因“左侧眶周渐进性隆起伴眼球突出20年,左眼视力下降1年”就诊。查体提示患者眼球突出度12 mm>90 mm<16 mm,左眼最佳矫正视力0.8,伴周边视野轻度受损和左眼眼球运动受限,色觉正常。结合眼眶CT表现,考虑为“左侧眶周多骨性骨纤维异常增殖症”。遂于内镜导航引导下,眼科与口腔科联合手术,行眶缘修整术、眼眶减压术和颌骨修整术、颧弓缩窄术,术后病理和分子学检测进一步确诊为骨纤维异常增殖症。患者术后左眼眼球突出和外观畸形均显著改善,最佳矫正视力恢复至1.0,眼球运动受限明显好转;术后4年,患者病灶骨稍有缓慢增长,无症状复发。讨论体会:骨纤维异常增殖症的治疗难点在于手术时机和手术方案的选择。手术时机的选择需综合考虑患者年龄、病灶骨增长速率、功能受损情况以及患者心理因素;手术方案的确定需要综合考虑病灶数目、部位和引发功能障碍的原因。

A 20-year-old woman presented with progressive facial deformity and exophthalmos for 20 years and with decreased vision for about 1 year in her left eye.Exophthalmometry measurements was 12 mm>90 mm<16 mm and her best corrected visual acuity was 0.8 in the left eye,with mild impairment of peripheral visual field and limited eye movement in the left eye as well.According to clinical manifestations and orbital CT images,the patient was diagnosed with polyosseous fibrous dysplasia of bone and subsequently underwent endoscope-navigation-assisted orbital margin trimming,orbital decompression,jaw trimming and zygomatic arch coarctation for her left periorbital lesion.Postoperative pathology and molecular tests further confirmed the diagnosis of fibrous dysplasia of bone.The patient’s lesions have remained stable without any symptom recurrence for four years after surgery.The difficulty in the treatment of fibrous dysplasia of bone lies in the selection of surgical time and surgical procedure.The patients’age,the number and location of lesions,the growth rate of lesion bones,the severity and cause of functional impairment,and patients’psychological status should be considered comprehensively before making treatment decisions.