Segmental Fibulectomy to Excise the Adherent Distal Tibia Osteochondroma in a Case of Hereditary Multiple Exostosis - A Rare Case Report.

Abstract

Introduction:Hereditary multiple exostosis (HME) is an autosomal dominant disorder affecting the skeletal system, which is characterized by multiple osteochondromas in bones arising from osteochondral ossification and leading to skeletal deformities, short stature, soft tissue, and neurovascular compressive symptoms.Case Report:A 10-year-old female a case of HME presented with painless multiple swelling around knees, wrist, and painful varus deformity in the lower third of the right leg. The large exostosis of the right distal tibia was symptomatic and indenting the fibula which required excision along with the segment of the fibula of about 2.5 cm above the syndesmosis adjacent to the exostosis as the mass was adherent to the fibula.Conclusion:Although distal tibia osteochondromas are rare, they can lead to deformity of the ankle and loss of function if not managed early and properly. Hence, early detection, proper planning, and management of periarticular distal tibia osteochondromas are essential to prevent the development or progression of the deformity. Segmental fibulectomy is required to remove the adherent osteochondromas completely and to prevent the recurrence and secondary surgical procedures. It is very essential to follow up the patient till the skeletal maturity to identify the delayed presentation and late progression of the ankle deformities.