Painful chest wall swelling: First rib osteochondroma in a paediatric patient with Hereditary Multiple Exostoses.

Abstract

Hereditary multiple exostoses (HME) is an autosomal dominant condition with variable penetrance. As per Dr Waller's audit of the Leeds regional bone tumour registry, 90 primary bone tumours of the thoracic skeleton were recorded, accounting for 4.5% of all cases in the registry. Osteochondromas represent 8% of rib tumours and approximately 50% of benign rib tumours. The patient presented with a chronic swelling in the right supraclavicular region with restricted shoulder motion and was diagnosed with osteochondroma of the first rib after evaluation. Surgical management by excision of the first rib tumour was done. Excision of the osteochondroma in the concerned case showed drastic symptomatic relief with complete recovery in range of motion. The hypothesis regarding common association of rib osteochondroma with a syndromic condition, namely HME, was also found to be fitting in the given case.