Mid Shaft Femoral Fracture in a Young Haemophilia A Patient - A Case Report

Abstract

Introduction/Aim: Haemophilia is a rare X chromosome linked inherited bleeding disorder, characterized by deficiency of coagulation factor VIII in Haemophilia A or factor IX in Haemophilia B. Patients deficient of either of these coagulation factors are prone to fracture as a result of reduced bone mineral density. Our aim is to present a case of Haemophilia A with mid shaft femoral fracture and difficulties in his management. Presentation of Case: We present a case of mid shaft femoral fracture in a eight year old boy with Haemophilia A who slipped on a wet floor in his home. He initially presented at a private facility where he experienced mismanagement by the use of intramuscular injection before coming to our tertiary health care center, University College Hospital (UCH), Ibadan. Management of the fracture was also hampered by non availability of recombinant factor VIII concentrate at presentation; he was transfused with both whole blood and fresh frozen plasma. He had skin tractions done on two occasions with no alignment of the fractured bones before he subsequently had Plaster of Paris cast under recombinant factor VIII concentrate cover. He was discharged after 14 weeks on admission with x-ray at discharge showing over riding of the involved bones. Conclusion: Management of fracture in this haemophilia patient was a difficult task especially with his active lifestyle. Delayed presentation at a tertiary health care facility and unavailability of recombinant factor VIII concentrate negatively affected the outcome.