Non-Cemented Hip Replacements in Patients with Gauchers Disease


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The successful outcome of non-cemented hip replacements in patients with Gaucher disease was presented by Dr Menachem Itzchaki, Consultant Orthopaedic Surgeon to the Gaucher Clinic at the Shaare Zedek Medical Center in Israel, at the EWGGD meeting on 1 May 2002. These findings are comparable to Dr Itzchaki's experience in cemented hip replacements for Gaucher disease and to the experience of hip replacements in the general population.


Twenty-two years ago Dr Jack Goldblatt and his colleagues in South Africa reviewed the results of eight patients with Gauchers disease who had had 15 total hip replacements after 14 years of follow-up. The results gauged by limb mobility and freedom from pain, were encouraging.

The recommendation given was that total hip replacement was the treatment of choice in patients with Gauchers disease needing hip replacements despite the 'resistance among colleagues to perform this operation among Gauchers disease'.

Among the problems encountered at that time were risk of infection and bleeding, the use of a cemented prosthesis (artificial hip) in a matrix (bone) which was damaged and likely to continue to disintegrate, and the inability to predict the outcome, particularly in young patients, because of the assumption that there was conti-nuous infiltration of Gaucher cells.

Currently 150,000 total hip replacements for various conditions are carried out in the USA every year.

Recent Experience is Good

Recent experiences have shown that meticulous attention to antibiotic cover and haematological stability ensures a good outcome in patients with Gaucher disease. Evidence has shown that hip replacements have lasted from 13 to 20 years in patients with Gauchers disease as well as for other conditions.

With acceptance of the feasibility of total hip replacement for avascular necrosis in patients with Gaucher disease, the question arises as to which procedure and which prosthetic devices are preferred.

Dr Itzchaki presented his experience using cementless total hip replacements in a cohort of relatively young patients.

Twelve cementless total hip replacements were performed in 11 patents with Type 1 Gaucher disease (from 1986 to 2001). All had replacements without the use of cement for fixing the prosthesis (artificial hip). All patients had sustained degenerative hip joint disease due to osteonecrosis of the femoral head . The age of the patients at surgery ranged from 17 to 64 and they were followed up from one to 15 years. Circumstances and occurrences during the operation and immediately afterwards were comparable to those of patients without Gaucher disease. Partial weight bearing with the use of crutches was advised at discharge.

Functional evaluation was good to excellent in all but one patient who eventually required revision (a further replacement). There were no cases of loosening during the follow-up period other than the above patient.

Safe and Functional

Our conclusion is that non-cemented total hip replacement is a safe and functionally predictable procedure for patients requiring hip replacement.

These findings are comparable to our experience in cemented total hip replacement for Gaucher disease and to the worldwide experience of total hip replacements in an otherwise healthy population.

Thus we believe that patients with Gaucher disease who require total hip replacement should be allowed to avail themselves of the state-of-the-art surgical option comparable to any other patient with an equal degree of disability and/or pain. This is especially true for patients in whom haematological abnormalities and/or the tendency to infections have been ameliorated by enzyme replacement therapy. Our current hypothesis is that the Gaucher cell-infiltrated bone is no less capable of accepting a prosthetic device than that of normal bone.


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Source: Gauchers News October 2002.
© Copyright Gauchers Association 2002