颅骨骨纤维结构不良的诊断和治疗

Diagnosis and Treatment of Osteofibrous Dysplasia of Skull (a Report of 33 Cases)

目的探讨颅骨骨纤维结构不良的诊断和治疗。方法回顾性分析33例经病理证实为颅骨骨纤维结构不良病例的临床资料,探讨其手术原则及其疗效。结果33例颅骨骨纤维结构不良患者中,男13例,女20例;年龄6～58岁,其中小于18岁者15例。该病常见累及额骨、筛骨、蝶骨,其主要临床表现为头痛、局部肿物、眼球突出、视力障碍、面部畸形等。X线平片及CT检查主要表现为局部骨密度增高,出现占位效应。病灶位于颅骨一侧皮质者,边界清楚,可有硬化;而病灶在骨髓腔内者,则多边界不清。CT增强扫描可见病灶明显强化,密度不均匀。MRI检查骨纤维结构不良信号呈多样性,无特异性,因此MRI对其的诊断意义不大。术后随访,33例患者经手术治疗后临床症状均有改善,2例复发。结论该病多见于儿童和青少年,女性较男性多见。外科手术为颅骨骨纤维结构不良的主要治疗手段,早期积极手术可使骨质愈合良好,缓解症状,改善预后。

Objective To investigate the diagnosis and treatment of osteofibrous dysplasia of the skull. Methods The clinical data of 33 patients with osteofibrous dysplasia of the skull, who underwent surgery for the osteofibrous dysplasia in our department from January, 1988 to July, 2008, were analyzed retrospectively. Results Of 33 patients with osteofibrous dysplasia of skulls, 13 were male and 20 female. Fifteen patients were under 18 years of age. The clinical manifestations mainly included headache, local lump, exophthalmos, visual disorder, facial malformation and so on in the patients with the osteofibrous dysplasia of skulls. X-ray and CT examination showed that there were high density of sclerotin and ground glass appearance in the lesions. The demarcation between the destroyed and normal bones was clear or unclear. The osteofibrous dysplasia was low signal intensity on T1-weighted MRI and variable signal intensity on T2-weighted MRI. The role of MRI examination in diagnosing osteofibrous dysplasia was＇probably limited and not specific. The data of following-up showed that all the patients were improved in the clinical symptoms and signs after the surgery. The osteofibrous dysplasia recurred in 2 patients. Conclusions The osteofibrous dysplasia of skull is more common in the persons under 18 years of age and females than that in the persons over 18 years of age and males. Surgery is an effective method to treat the osteofibrous dysplasia of skull. The symptoms may be relieved and the prognosis may be improved after the surgery in the patients with the osteofibrous dysplasia of skull.