46例颌面部骨纤维异常增殖症临床分析

目的：分析颌面部骨纤维异常增殖症（fibrous dysplasia，FD）的临床特点、影像学表现以及血清碱性磷酸酶（alkaline phosphatase，ALP）含量与临床相关因素的关系。方法：复习46例FD患者的临床资料并进行随访．测量分析FD患者单骨型、多骨型组和非FD患者对照组ALP的含量。采用SAS6．04软件包对数据进行方差分析。结果：46例患者，单骨型32例（69．6％），多骨型14例（30.4％）。FD在影像学上多无清晰边界，多见毛玻璃样型。复发病例11例．多发生于青春期之前。多骨型组术前血清ALP含量为（257．9±218.0）U／L，显著高于对照组的（58．5±31．3）U／L和单骨型组的（100．0±49．8）U／L。1例行上颌骨全切术＋赝复体修复，3例行下颌骨病变切除＋血管化腓骨肌瓣修复．1例行下颌骨病变切除＋血管化髂骨肌瓣修复，其余病例均行病变骨的修整术。术后随访4个月。4．9a，平均2．4a．所有伤口愈合良好，11例复发病例再次治疗后未见复发。结论：FD是一种纤维骨性病变，颌面部为其好发部位之一。此病无明显性别差异，血清ALP升高可能与FD病变的范围相关。可根据患者的具体情况选择不同术式，手术宜在青春期后进行。

Clinical analysis of 46 patients with fibrous dysplasia of maxillofacial bone

PURPOSE： To analyze the clinical manifestation, radiographic features and serum alkaline phosphatase（ALP） content of patients with fibrous dysplasia （FD） of maxillofacial bone. METHODS： Clinical data of 46 patients with FD of maxillofacial bone were retrospectively reviewed. The serum ALP contents of patients with FD （monostotic form and polyostotic form）, and patients in control group were measured and were analyzed with SAS6.04 software package for ANOVA SNK. RESULTS： Thirty-two （69.6%） patients were of monostotic form and fourteen（30.4%） were of polyostotic form. The radiographic features of FD were ＂ground-glass＂ pattern with opaque rim. Eleven patients were with recurrent lesions at their first visit to our hospital, and the recurrence risk of fibrous dysplasia following curettage was higher in patients prior to puberty than that in adults. The serum ALP content in polyostotic form group was （257.9±218.0）U/L, significantly higher than that in control group （58.5±31.3）U/L（P〈0.01） and of monostotic form group （100.0±49.8）U/L（P〈 0.01）. One patient underwent total maxillectomy and reconstruction with prosthesis. Three patients underwent partial mandibulectomy and reconstruction with vascularized fibular graft. One patient underwent partial mandibulectomy and reconstruction with vascularized iliac bone graft. The remaining patients underwent curettage of lesions. The duration of follow-up period averaged 2.4 years （range, 4 months to 4.9 years） and no recurrence was observed in 11 patients with recurrent lesions. CONCLUSIONS： FD is a fibre-osseous lesion affecting maxillofacial region which has no gender predilection. The serum ALP content may be correlated to the extent of the FD lesion. Delay in surgery until after puberty is recommended. Supported by Shanghai Leading Academic Discipline Project （Grant No. Y0203）.