Presentations of Fibrous Dysplasia in South Eastern Nigeria

Abstract

Fibrous dysplasia is a rare disorder, the course of which is unknown and there is no known cure. It is however not a form of cancer. There is abnormal bone growth that destroys and replaces normal bones with fibrous tissues. Most lesions are monostotic, asymptomatic and identified incidentally and can be treated with clinical masterly inactivity and patient education. Clinical presentation is often as a painless tumor or incidental finding, but complications are relatively high due to late recognition. Early diagnosis which usually comes from high index of suspicion and adequate treatment are needed to reduce morbidity and complications which impact on quality of life of the patients.

Aim: To determine the pattern of presentation of fibrous dysplasia amongst patients in South Eastern Nigeria.

Methodology: The histopathology clinical records of all the patients diagnosed of fibrous dysplasia were reviewed for age, gender, provisional clinical diagnosis before tissue biopsy, affected bones, common presenting symptoms, and the side of the body involved. Data were analyzed using the statistical package for social sciences (SPSS) version 17.0 soft ware; and presented in percentages and tables. Statistical significance was inferred at P<0.05; using the students t-test for continuous variables and chi-square test for categorical variables.

Results: 155 clinical records were analyzed, five of which were recurrent cases leaving the actual number of patients at 150. The males were 72 while the females were 78, giving a male to female ratio of 1:1.1. The patients' ages ranged from 4 years to 65 years with a mean age of 34.5 years. The incidence of fibrous dysplasia was highest in the second decade of life, followed by the third decade. Eighty percent (80%) of the cases presented within the first three decades of life; with the incidence dwindling off by the sixth decade. The upper jaws were the most frequently affected bone, accounting for 46.0%. The right upper jaw was affected in 33 (22.0%) while the left was affected in 36 (24.0%) cases. The lower jaws were affected in 62 (41.3%) cases; the right lower jaw in 36 (24.0%) and the left in 26 (17.3%). The upper and lower jaws were affected in 87.3% of the cases. The craniofacial distribution in our series was 93.3%. The bones of the right side of the body were affected in 76 (50.7%) cases while the left side was affected in 69 (46.0%). Central lesion occurred in 3 (2.0%) cases. Two patients (1.3%) only presented with polyostotic lesions. Seventy five patients (50%) presented with painless mass, 16 (10.6%) with painful lesions and 22 (14.7%) with teeth associated symptoms. No case of malignant transformation was recorded in our series.

Conclusion: There is paucity of literature on the pattern of presentation and distribution of Fibrous dysplasia in Nigeria. Fibrous dysplasia in South East Nigeria presents almost exclusively as monostotic form without any gender predilection. It also presents overwhelmingly in the craniofacial region with the appendicular skeleton being sparingly involved.