骨性纤维发育异常——好发于儿童的新病种

Osteofibrous Dysplasia: a New Entity in Children

目的：为了提高骨性纤维发育异常的诊断水平，避免治疗失误及降低复发率。方法：通过３５例长骨纤维性骨病变的复查，并加用特殊染色观察组织病变，发现本病１０例，平均年龄６．７岁。全部经手术刮除病灶。结果：６例获１～９年随访，５例术后１～２年内复发，复发率高达８３％，其中２例分别于术后５年和８年自愈。结论：综合临床、Ｘ线以及病理资料已足以将本病和单骨性纤维结构不良等病区别开来成为一个新的病种。本病预后良好，因手术年龄愈小，术后复发率愈高，故手术不宜在１０岁以下进行。

Objective: To improve the diagnosis of osteofibrous dysplasia. Methods: Ten out of 35 patients with fibroosseous lesions of long bone were diagnosed as osteofibrous dysplasia after reassessment with the histology and immunohistochemical stain. All the patients underwent surgical curettage with or without bone grafts. Results: Six patients were followedup for 1 to 9 years after the operation. Five of them recurred (83%). The condition of two out the 5 patients improved spontaneously 5 and 8 years later. Conclusions: Unlike fibrous dysplasia or other fibroosseous lesions, osteofibrous dysplasia is a clinically and radiologically distinctive entity. It has a favorable prognosis. Surgery under the age of 10 should be avoided in view of the high recurrence rate.