Gaucher Disease: Outcome following Total Hip Replacements and Effect of Enzyme Replacement Therapy in a Cohort of Uk Patients

Abstract

Only a limited number of reports of total hip replacements (THRs) in patients with Gaucher disease (GD) have been published, with the majority showing high rates of early aseptic loosening as well as an increased number of peri-operative complications. We present a series of twelve THRs in nine affected patients, with a mean age of 39 years at the time of surgery (median 37 years; range 27-60 years). We examine the medium- to long-term results and present the outcome scores, survival, and assess the effect of enzyme replacement therapy (ERT). Four hips (33.3%) required revision for aseptic loosening at a mean time of 11.5 years. There was no significant difference in survival between the group on ERT at the time of surgery and group not on ERT. Those on ERT did, however, have fewer peri-operative complications and there was a trend to better outcome scores. Five of the 12 THRs were performed when the diagnosis of GD was unknown and all of these had early complications.?Our results show better survival of THRs in patients with GD than previously thought. The use of ERT pre-operatively did not have an effect on survival, nor outcome scores, but did reduce the peri-operative complication rate. Those not on ERT at the time of surgery were commenced on it at some point in their treatment (at an average of 8.6 years following THR) and we believe the use of ERT, at any time in the treatment, could have contributed to the improved survival seen in our series compared to historical results in the literature.

Longevity of total hip arthroplasty implants in patients with Gaucher disease

“…12 The total number of THR needing a revision is about 3.1% for 10 years, while the younger population is at a higher risk for revision surgery: 4.8% (for 10 years) in patients younger than 49 years. 12,13 These results, which are similar to the current study, propose a resemblance between the general population and this Gaucher disease sub-group. The 3rd parameter related to revision surgery is age at time of surgery.…”

Simultaneous Bilateral Femoral Osteonecrosis in Gaucher Disease

“…In 1994, the systemic treatment of GD made a significant leap with the introduction of ERT [ 23 ]. This treatment decelerated the gradual worsening condition of these patients, allowing for improved general and surgical outcomes [ 24 ]. In the post-ERT era, the discussion regarding THA for patients with GD became entirely different.…”

Skeletal Manifestations of Gaucher's Disease: A Case Report and Literature Review

“…4 After several consultations, it was decided to start enzymatic treatment, particularly because she was able to move unaided, in the hope of improving bone quality, and then to reconsider total hip arthroplasty within a year. 5 Currently, she is still under medical treatment and scheduled for intervals of follow-up.…”