The role of orthopaedic surgery in haemophilia: current rationale, indications and results

Abstract

The musculoskeletal problems of haemophilic patients begin in infancy when minor injuries lead to haemarthroses and haematomas.Early continuous haematological primary prophylaxis by means of the intravenous infusion of the deficient coagulation factor (ideally from cradle to grave) is of paramount importance because the immature skeleton is very sensitive to the complications of haemophilia: severe structural deficiencies may develop quickly.If primary haematological prophylaxis is not feasible due to expense or lack of venous access, joint bleeding will occur. Then, the orthopaedic surgeon must aggressively treat haemarthrosis (joint aspiration under factor coverage) to prevent progression to synovitis (that will require early radiosynovectomy or arthroscopic synovectomy), recurrent joint bleeds, and ultimately end-stage osteoarthritis (haemophilic arthropathy).Between the second and fourth decades, many haemophilic patients develop articular destruction. At this stage the main possible treatments include arthroscopic joint debridement (knee, ankle), articular fusion (ankle) and total joint arthroplasty (knee, hip, ankle, elbow). Cite this article: EFORT Open Rev 2019;4:165-173. DOI: 10.1302/2058-5241.4.180090.

Keywords: haemophilia; haemophilic arthropathy; orthopaedic surgery.

Fig. 1

Elbow haemophilic arthropathy: (a) anteroposterior radiograph; (b) lateral view.

Fig. 2

Haemophilic arthropathy of the knee joint: (a) anteroposterior view; (b) lateral radiograph.

Fig. 3

Bilateral ankle haemophilic arthropathy: (a) anteroposterior radiograph of both ankles; (b) lateral view of the left ankle; (c) lateral radiograph of the right ankle.

Fig. 4

Joint aspiration in acute haemophilic haemarthrosis.

Fig. 5

Ultrasonography (US) of acute haemarthrosis.

Fig. 6

Total knee replacement (TKR) in a patient with inhibitor: (a) anteroposterior preoperative radiograph; (b) lateral preoperative view; (c) anteroposterior postoperative radiograph; (d) lateral postoperative view. The result was excellent.

Fig. 7

Aseptic loosening of a primary total knee replacement 18 years after implantation that required revision arthroplasty with a CCK (constrained condylar knee) implant: (a) anteroposterior preoperative view; (b) anteroposterior postoperative radiograph. The result was excellent.

Fig. 8

Two-stage revision total knee replacement (TKR) in a periprosthetic infection: (a) anteroposterior view of the knee before TKR; (b) lateral radiograph of the knee prior to TKR; (c) anteroposterior view of the knee after TKR; (d) lateral view of the knee after TKR; (e) articulated spacer implanted in the first stage of the two-stage revision arthroplasty performed (anteroposterior view); (f) lateral radiograph of the articulated spacer; (g) anteroposterior view of the revised implant (constrained condylar knee [CCK] design) after the second stage of the two-stage revision arthroplasty; (h) lateral radiograph of the revised implant (CCK design) after the second stage of the two-stage revision arthroplasty. The result was excellent.

Fig. 9

Pseudoaneurysm after primary total knee replacement (TKR) with a constrained condylar knee (CCK) design due to severe varus deformity and muscular atrophy: (a) anteroposterior view of the knee before TKR; (b) lateral radiograph prior to TKR; (c) severe postoperative haemarthroses five days after surgery was treated with joint aspiration; (d) angiogram demonstrated the existence of an arterial pseudoaneurysm (arrow); (e) arterial embolization (arrow) solved the problem.

Fig. 10

Ultrasonography (US) of a muscle haematoma.

Fig. 11

Computerized tomography (CT) scan showing an iliopsoas haematoma (arrow).