End Stage Hip Osteoarthritis in Klippel-Trenaunay Syndrome - A Case Report

Klippel-Trenaunay syndrome (KTS) affects the development of blood vessels, soft tissues (such as skin and muscles), and bone. It is a rare cause of severe degenerative joint disease at an early age. Orthopedic interventions in these patients bring numerous difficulties for various reasons. Up to this point, only six cases of attempted total hip arthroplasty (THA) in patients with KTS have been reported in the literature. The two most recent cases were prematurely aborted due to excessive bleeding and imminent risk of exsanguination. One of the most recent published case reports suggested that hip joint replacement should be avoided in this patient population.

A middle-aged female presented with end stage coxarthrosis secondary to KTS. A thorough workup was performed and magnetic resonance imaging revealed that the direct anterior interval was relatively free of vascular malformations. After intensive work-up, uneventful THA was performed, resulting in dramatic improvement of quality of life.

Effort should be put into identifying those patients with a relatively easy accessible joint so they are not unnecessarily being denied successful joint replacement.