Complications of hemophilia in the elbow: current management

ABSTRACT

Introduction

After the ankle and knee, the elbow is the most commonly impaired joint in patients with hemophilia.

Areas covered

A Cochrane Library and PubMed (MEDLINE) search related to elbow problems in hemophilia was conducted. Early and continuous primary hematological prophylaxis (ideally starting in infancy) is essential, given that the juvenile elbow is highly prone to the musculoskeletal complications of hemophilia. If primary hematological prophylaxis is not administered (due either to lack of affordability or lack of venous access), persistent hemarthrosis can occur in the elbow. Hemarthroses will then require aggressive treatment (through arthrocentesis under factor factor coverage) to prevent progression to synovitis (requiring prompt radiosynovectomy or arthroscopic synovectomy) and ultimately hemophilic arthropathy (requiring rehabilitation and orthopedic surgery). Between the second and fourth decade of life, many patients with hemophilia present elbow joint destruction.

Expert opinion

In the initial stage of joint destruction, rehabilitation and intraarticular injections of hyaluronic acid can relieve the symptoms. In the advanced stage of hemophilic arthropathy, however, surgery is necessary. The main surgical procedures currently available are arthroscopic debridement, radial head resection, and total elbow arthroplasty. Transposition of the ulnar nerve and removal of heterotopic ossifications might also be necessary.

KEYWORDS:

• Hemophilia
• elbow
• arthrocentesis
• radiosynovectomy
• arthroscopic synovectomy
• arthroscopic debridement
• total elbow arthroplasty

Article highlights

• Early and continuous primary hematological prophylaxis (ideally from cradle to grave) is paramount.

• If primary hematological prophylaxis is not administered (e.g., due to high cost or lack of venous access), persistent hemarthrosis will occur in the elbow.

• Hemarthrosis should be treated aggressively (by arthrocentesis under factor replacement coverage and aseptic conditions) to prevent progression to synovitis and ultimately hemophilic arthropathy.

• In the initial stage of joint destruction, rehabilitation (including elbow braces) and intraarticular injections of hyaluronic acid can relieve the symptoms.

• Synovitis requires early radiosynovectomy or arthroscopic synovectomy.

• In the advanced stage of hemophilic arthropathy, total elbow arthroplasty might be necessary.

• The best method for managing advanced hemophilic elbow arthropathy is patient education, hematological prophylaxis, and a combination of medical treatment, physical maintenance, rehabilitation after a bleeding, and the use of orthoses, when required.

• None of these procedures is sufficient in isolation to prevent or treat hemophilic elbow arthropathy.

• Hemophilia is a disease that requires a multidisciplinary approach, and hematologists need to be the main coordinators and managers of the care for patients with hemophilia.

Declaration of interest

EC Rodriguez-Merchan has received reimbursement for collaborating as a speaker and researcher from Pfizer and Roche and has received honoraria for consulting or collaborating as a speaker from Pfizer, Roche, Sobi, Takeda, Novo Nordisk and Bayer. H De la Corte-Rodriguez has received reimbursement for collaborating as a speaker and researcher from Pfizer and Roche and has received honoraria for consulting or collaborating as a speaker from Sobi, Takeda, Novo Nordisk and Bayer.

The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.