HEREDITARY MULTIPLE EXOSTOSES: CASE REPORT

Objective: Hereditary Multiple Exostoses (HME) is a genetically transmitted bone dysplasia that is inherited in an autosomal dominant manner. It usually presents after the age of two years as multiple bony growths on the appendicular skeleton. As a rare condition (incidence of 0.9-2/100,000), it is not commonly seen in our environment. This paper presents the clinical and radiological features of two patients seen in our hospital within a period of six months. Method: The case records of two patients with hereditary multiple exostoses are presented to highlight the clinical presentation and management options of the condition. Results: A boy and a girl who respectively manifested the features of hereditary multiple exostoses at the age of two and six years are presented. The main presenting features were painless progressively increasing bony swellings in both upper and lower limbs, with forearm deformity and ulnar deviation of the wrist. One of them had pressure symptoms which necessitated surgical excision of the symptomatic exostosis. Fine needle aspiration cytology confirmed the diagnosis of osteochondroma. Conclusion: Hereditary multiple exostoses though rare, do occur in our environment and the management is essentially by masterly inactivity except when the bony swellings exhibit any complications or there is concomitant deformity.