Ulnar hemimelia in deformed left forearm treated with ilizarov fixator

Abstract

Introduction: Ulnar hemimelia is a congenital ulnar deficiency of the forearm characterized by complete or partial absence of the ulna bone. History and Examination: 18 years female presented to us with deformed left upper limb. Cosmetically, the forearm looked curved with concavity towards the ulnar side. Materials and Method:The correction was carried out in 2 stages. In first stage, the wedge shaped bone was osteotomised and correction was then fixed with dynamic compression plate. The ulna was fixed with ilizarov frame. In second stage, the corticotomy of proximal ulna was done and rush nail was passed intra medullary before distraction to prevent translation. The ulna was lengthened until it reached the distal radio ulnar joint. Result and Conclusion: In our case report, the girl with ulnar hemimelia was not able to do routine activities of daily living before the operation. Hence treatment was necessitated to improve cosmetic and functional outcomes. Management of such cases is highly individualised and mainly involves improvement of function.

Ulnar longitudinal deficiency: a rare case report and review

“…It may be a part of Poland syndrome, Klippel-Feil syndrome, Goltz-Gorlin syndrome, Cornelia De Lange syndrome, or Femur Fibula Ulna syndrome. 5,7 The radius is typically longer than the ulna in patients with achondroplasia, and in patients with mucopolysaccharidosis, the distal ulna and radius can be hypoplastic and may slope toward each other. 6,8 In syndromic cases the clinical course and the prognosis depends on the severity of the syndrome.…”