Orthopaedic and surgical features in the management of patients with haemophilia

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.

Perioperative Period of a Hemophilia‐related Osteoarthropathy Therapeutic Regimen and Analysis of Complications

“…The patients who underwent hip and elbow replacement had no perioperative wound complications, which is in line with reports in other countries . The above data show that with full perioperative preparation, joint replacement surgery is safe and effective in patients with hemophilia.…”

Management of severe perioperative bleeding

“…However, recent data demonstrated that good surgical results are achievable over a range of procedures when there is appropriate careful preoperative planning, appropriate replacement/ substitution therapy, and multidisciplinary team management. [501][502][503][504][505][506] Although surgery is a highly demanding intervention in patients with severe IBDs, especially in low-resource countries, 507 it often represents a life or limb-saving and quality of life-improving measure, which has to be taken.…”

Perioperative management of haemophilia B: A critical appraisal of the evidence and current practices

“…Length of stay in hospital for perioperative management is not only affected by clinical characteristics (eg surgery complexity, existence of treatment complications) but can also be affected by local protocols arising from funding restrictions; ultimately, this encouragespreoperative factor dosing in outpatients and early discharge for outpatient dosing of additional doses. The appropriate use of coagulation factor products combined with regular assessments at a haemophilia (or bleeding‐disorder) comprehensive care centre, supplemented with symptomatic support (eg physiotherapy around orthopaedic surgery), was shown to assist the majority of patients in recovering from surgery …”

“…Low incidences of postoperative bleeding and other complications were reported in studies from Romania, France and the United Kingdom. 4,24,25 These studies highlighted the importance of timely monitoring of factor levels and close collaboration within the MDT during the perioperative period.…”