Hawes M J
University of Colorado Medical Center, Denver.
Ophthalmic Plast Reconstr Surg. 1989;5(2):133-40. doi: 10.1097/00002341-198906000-00010.
Cherubism is a rare, fibroosseous lesion of the jaws that may have orbital manifestations of proptosis, lower eyelid retraction, superior globe displacement, and visual loss. Management of the orbital tumors in two cases from the same family is described. The original description of the disorder is reviewed. The inheritance pattern is autosomal dominant. Pathology findings show multinucleated giant cells in a vascular fibrous stroma. Teeth are often missing or displaced. Radiographic studies show symmetric cystic lesions in the jaws and sometimes involvement of the orbital floor and lateral orbital wall. Fibrous dysplasia is in the differential diagnosis, but can be distinguished by clinical characteristics. Treatment may involve curettement or recontouring of the bony lesions.
cherubism是一种罕见的颌骨纤维骨性病变，可能有眼球突出、下睑退缩、眼球上移和视力丧失等眼眶表现。本文描述了来自同一家族的两例眼眶肿瘤的治疗情况。回顾了该疾病的最初描述。其遗传模式为常染色体显性遗传。病理检查结果显示在血管性纤维基质中有多核巨细胞。牙齿常缺失或移位。影像学研究显示颌骨有对称性囊性病变，有时累及眶底和眶外侧壁。骨纤维异常增殖症需鉴别诊断，但可通过临床特征加以区分。治疗可能包括刮除或重塑骨病变。
