An epidemiological and clinical analysis of craniomaxillofacial fibrous dysplasia in a Chinese population
1 Department of Oral and Maxillofacial Surgery, Affiliated Stomatological Hospital, Nanjing Medical University, Jiangsu, China
2 Department of Oral and Maxillofacial Surgery, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
3 , Hanzhong Road 136, Nanjing, 210029, PR China
Orphanet Journal of Rare Diseases 2012, 7:80 doi:10.1186/1750-1172-7-80Published: 17 October 2012
Craniomaxillofacial fibrous dysplasia (FD) is a benign bone lesion characterized by facial disfigurement and functional impairment. The aim of this study was to characterize the epidemiological and clinical features of craniomaxillofacial FD by presenting data from a representative Chinese population during a 15-year period (1994–2009).
The craniomaxillofacial disease registries of two Chinese tertiary referral hospitals (Shanghai Ninth People’s Hospital and Stomatological hospital of Jiangsu Province) were searched and reviewed to collect relevant information for patients with craniomaxillofacial FD between Jan.1994 and Dec.2009. All included cases were further analyzed with regard to associated epidemiological and clinicopathological variables.
A total number of 266 cases with definitive diagnosis were identified with 219 primary cases and 47 recurrent cases. There were 111 males and 155 females with a male to female ratio of 0.716:1. They were clinically categorized into three groups: monostotic (71.1%), polysotic (27.4%) and Albright syndrome (1.5%). Maxilla alone or with adjacent bones was the most common affected site. The serum alkaline phosphatase (ALP) in patients was much higher than that in healthy control, whereas comparable between primary patients and recurrent ones. Three patients (3/266, 1.1%) with polysotic lesions underwent spontaneous malignant transformation into osteosarcoma. The majority of patients underwent conservative surgery, while the others received radical resection with or without reconstruction.
Craniomaxillofacial FD is a rare bony disorder with defined epidemiological and clinicopathological features in Chinese population. Further investigations are warranted to establish the optimized timing, treatment strategy and prognostic prediction for this clinical entity.