Jaffe Lichtenstein Type Polyostotic Fibrous Dysplasia with Unilateral Absent Testis

Abstract

Fibrous dysplasias are developmental bone disorders in which the medullary cavity is replaced by fibrous tissue. It can be monostotic involving a single bone or polyostotic involving multiple bones. Unlike the monostotic type, the polyostotic type of fibrous dysplasia is associated with non skeletal manifestations like café au lait macule and/or endocrine hyperfunction. The presence of endocrine involvement differentiates McCune Albright syndrome from Jaffe Lichtenstein syndrome. We hereby report a case of an 11-yearold male child presenting with polyostotic type of fibrous dysplasia involving bilateral femurs with café au lait macule having no endocrine disease consistent with the diagnosis of Jaffe Lichtenstein disease. He also had unilateral absent testis which is rarely reported in this syndrome.

Keywords

• anorchia
• bisphosphonate
• café au lait
• cortical bone grafting