Oral manifestations of Fanconi's anaemia: a case report.

BACKGROUND

Fanconi's anaemia (FA) is a rare, inherited autosomal recessive genetic disease characterized by progressive bone marrow failure, congenital abnormalities, and high predisposition to malignancies. The incidence of the disease is unusually high in the Afrikaner population of South Africa with calculated heterozygote prevalence being approximately 1 in 77. This report describes the oral clinical and radiological findings in a 16 year old male with FA.

METHODS

Medical and dental history was noted, the oral mucosa was examined and the teeth were screened for fillings and caries. Periodontal examination consisted of: measurement of probing depths, recession, mobility of teeth, furcation involvement, plaque index and gingival index. An orthopantomograph was taken.

RESULTS

There was a generalized black hyper-pigmentation on the buccal mucosa, tongue and palate. The patient had generalized gingival recession and most of the periodontal pockets were deeper than 5mm. Several teeth were mobile and the orthopantomograph showed loss of alveolar bone in the furcation areas of most posterior teeth. A diagnosis of severe generalized periodontitis associated with systemic disease was made.

CONCLUSIONS

Patients with Fanconi's anaemia are susceptible to severe dental and periodontal diseases.