Managing Surgical Risks in People with Inhibitors

These procedures pose additional challenges
Author: James V. Luck Jr., MD

Reconstructive surgery on people with hemophilia is more challenging because of the added need of assuring coagulation, the increased incidence of infection and reduced range of motion.

I was co-author of a study, published in The Journal of Bone & Joint Surgery in 2005, that reported a 16% incidence of late infection occurring between one and 25 years after surgery in men with hemophilia without inhibitors. Other studies have shown similar results, which is significant compared to the general population incidence of about 1%. Loss of motion is the result of arthrofibrosis, or scar tissue formation, within and around the joint. It is more severe in hemophilia than in other conditions.

In patients without inhibitors, these problems have been relatively manageable. But in those with inhibitors, these risks are increased. At the World Federation of Hemophilia World Congress in Paris, France, July 8 to 12, 2012, Nicholas Goddard, MB, FRCS, of the United Kingdom, indicated that approximately 50% of people with inhibitors who have knee replacement surgery have continued oozing from the wound after surgery, which can be very difficult to control. Despite the availability of bypassing agents, such as FEIBA and factor VIIa, many people with inhibitors experience wound healing that does not progress normally, which can increase the risk of infection of the implant. Improper wound healing also prevents early aggressive range of motion, which in turn results in more arthrofibrosis and further limited range of motion.

The cost of reconstructive surgery on a patient with hemophilia is several times more expensive than in a nonhemophilic patient, but the cost in inhibitor patients dwarfs those expenses. The cost of care can exceed $1 million, and in some cases $2 million, for the primary surgery alone. If further reconstructive surgery is needed, expenses can mount.

For all of these reasons, surgery in patients with hemophilia and an inhibitor is anything but straightforward. (See “No Routine Matter,” page 26.) I sincerely look forward to the time when we can provide joint replacement surgeries for our patients with inhibitors and anticipate similar results to those of our noninhibitor patients. Hopefully, that day is not far away.

James V. Luck Jr., MD, is director of orthopaedics and rehabilitation at the Hemophilia Treatment Center at Los Angeles Orthopaedic Hospital.