Evidence-Based Treatment of Deformity in Multiple Osteochondromatosis

Multiple Osteochondromatosis is a relatively common genetic orthopaedic condition. Although the molecular basis of inheritance is well established, clinical features are variable. It is known that deformity can occur, however its natural history in relation to the presence of local exostoses is poorly understood. The literature review identifies some features of local deformity that suggest a causal effect however there are no level III studies. The result of exostosis excision has only been studied in the forearm in four case-series and the results are contradictory. In a proportion of patients an improvement may be expected to occur. Optimal timing of surgery in relation to patient age and degree of deformity has not been established. Downsides to excision surgery are chiefly exostosis recurrence and failure to achieve the desired improvement in deformity.