Orthopaedic and surgical features in the management of patients with haemophilia

Eur J Orthop Surg Traumatol. 2014 Jul;24(5):685-92. doi: 10.1007/s00590-013-1361-4. Epub 2013 Dec 3.

Abstract

Haemophilia is a hereditary disease due to a defect of the X chromosome, which determines a faulty production of coagulation factor VIII in haemophilia A (85% of cases) and factor IX in haemophilia B. Three degrees of severity can be distinguished: low, with a deficient factor concentration greater than 5% of normal values, medium, with a concentration between 1 and 5%, and severe, comprising more than half of haemophilia cases, with a concentration of factor VIII or factor IX under 1% of normal. The evolution of haemophilic arthropathy is almost always from haemarthrosis to chronic synovitis and extended erosions of the articular surface, culminating in the final stage of articular destruction-chronic haemophilic arthropathy. This paper analyses the results of the treatment applied to a lot of one hundred and ten patients operated inside the our country's sole Compartment of Osteo-Articular Surgery of haemophiliac patients within the Clinic No. 2 of Orthopaedics and Traumatology between 2001 and 2013. This compartment was founded in 2001, being included in the Romanian Health Ministry's programme for financing the treatment for haemophilia. Within the aforementioned time period, a total of 158 patients from the entire country were consulted. From these, 110 patients underwent a series of surgical procedures, 112 in total. The age interval of the studied lot was between 9 and 45 years, with a maximum between 11 and 20 years. With replacement therapy and correct surgical indication, osteoarticular surgery in haemophiliacs, performed in a specialized centre by a multidisciplinary team, can give good results with acceptable risks.

MeSH terms

  • Adolescent
  • Adult
  • Bone Diseases, Metabolic / complications
  • Child
  • Cohort Studies
  • Hemophilia A / complications*
  • Hemophilia B / complications*
  • Humans
  • Middle Aged
  • Orthopedic Procedures / statistics & numerical data*
  • Osteoporosis / complications
  • Postoperative Complications / etiology
  • Young Adult