Aims. The diversity of femoral morphology renders femoral component sizing in total hip arthroplasty (THA) challenging. We aimed to determine whether femoral morphology and femoral component filling influence early clinical and radiological outcomes following THA using fully hydroxyapatite (HA)-coated femoral components. Methods. We retrospectively reviewed records of 183 primary uncemented THAs. Femoral morphology, including Dorr classification, canal bone ratio (CBR), canal flare index (CFI), and canal-calcar ratio (CCR), were calculated on preoperative radiographs. The canal fill ratio (CFR) was calculated at different levels relative to the lesser trochanter (LT) using immediate postoperative radiographs: P1, 2 cm above LT; P2, at LT; P3, 2 cm below LT; and D1, 7 cm below LT. At two years, radiological femoral component osseointegration was evaluated using the Engh score, and hip function using the Postel Merle d’Aubigné (PMA) and Oxford Hip Score (OHS). Results. CFR was moderately correlated with CCR at P1 (r = 0.44; p < 0.001), P2 (r = 0.53; p < 0.001), and CFI at P1 (r = − 0.56; p < 0.001). Absence of spot welds (n = 3, 2%) was associated with lower CCR (p = 0.049), greater CFI (p = 0.017), and lower CFR at P3 (p = 0.015). Migration (n = 9, 7%) was associated with lower CFR at P2 (p = 0.028) and P3 (p = 0.007). Varus malalignment (n = 7, 5%), predominantly in Dorr A femurs (p = 0.028), was associated with lower CFR at all levels (p < 0.05). Absence of spot welds was associated with lower PMA gait (p = 0.012) and migration with worse OHS (p = 0.032). Conclusion. This study revealed that femurs with insufficient proximal filling tend to have less favourable radiological outcomes following uncemented THA using a fully HA-coated double-tapered femoral component. Cite this article: Bone Joint Res. 2020;9(4):182–191

Objectives. Our primary aim was to describe migration of the Exeter stem with a 32 mm head on highly crosslinked polyethylene and whether this is influenced by age. Our secondary aims were to assess functional outcome, satisfaction, activity, and bone mineral density (BMD) according to age. Patients and Methods. A prospective cohort study was conducted. Patients were recruited into three age groups: less than 65 years (n = 65), 65 to 74 years (n = 68), and 75 years and older (n = 67). There were 200 patients enrolled in the study, of whom 115 were female and 85 were male, with a mean age of 69.9 years (sd 9.5, 42 to 92). They were assessed preoperatively, and at three, 12 and, 24 months postoperatively. Stem migration was assessed using Einzel-Bild-Röntgen-Analyse (EBRA). Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), Harris Hip Score (HHS), Hip Disability and Osteoarthritis Outcome Score (HOOS), EuroQol-5 domains questionnaire (EQ-5D), short form-36 questionnaire (SF-36,) and patient satisfaction were used to assess outcome. The Lower Extremity Activity Scale (LEAS), Timed Up and Go (TUG) test, and activPAL monitor (energy expelled, time lying/standing/walking and step count) were used to assess activity. The BMD was assessed in Gruen and Charnley zones. Results. Mean varus/valgus tilt was -0.77⁰ and axial subsidence was -1.20 mm. No significant difference was observed between age groups (p ⩾ 0.07). There was no difference according to age group for postoperative WOMAC (p ⩾ 0.11), HHS (p ⩾ 0.06), HOOS (p ⩾ 0.46), EQ-5D (p ⩾ 0.38), patient satisfaction (p ⩾ 0.05), or activPAL (p ⩾ 0.06). Patients 75 years and older had a worse SF-36 physical function (p = 0.01) and physical role (p = 0.03), LEAS score (p < 0.001), a shorter TUG (p = 0.01), and a lower BMD in Charnley zone 1 (p = 0.02). Conclusion. Exeter stem migration is within normal limits and is not influenced by age group. Functional outcome, patient satisfaction, activity level, and periprosthetic BMD are similar across all age groups. Cite this article: N. D. Clement, M. Bardgett, K. Merrie, S. Furtado, R. Bowman, D. J. Langton, D. J. Deehan, J. Holland. Cemented Exeter total hip arthroplasty with a 32 mm head on highly crosslinked polyethylene: Does age influence functional outcome, satisfaction, activity, stem migration, and periprosthetic bone mineral density? Bone Joint Res 2019;8:275–287. DOI: 10.1302/2046-3758.86.BJR-2018-0300.R1

Aims. Periprosthetic joint infection (PJI) remains a challenging complication following total hip arthroplasty (THA). It is associated with high levels of morbidity, mortality and expense. Guidelines and protocols exist for the management of culture-positive patients. Managing culture-negative patients with a PJI poses a greater challenge to surgeons and the wider multidisciplinary team as clear guidance is lacking. Patients and Methods. We aimed to compare the outcomes of treatment for 50 consecutive culture-negative and 50 consecutive culture-positive patients who underwent two-stage revision THA for chronic infection with a minimum follow-up of five years. Results. There was no significant difference in the outcomes between the two groups of patients, with a similar rate of re-infection of 6%, five years post-operatively. Culture-negative PJIs were associated with older age, smoking, referral from elsewhere and pre-operative antibiotic treatment. The samples in the culture-negative patients were negative before the first stage (aspiration), during the first-stage (implant removal) and second-stage procedures (re-implantation). Conclusion. Adherence to strict protocols for selecting and treating culture-negative patients with a PJI using the same two-stage revision approach that we employ for complex culture-positive PJIs is important in order to achieve control of the infection in this difficult group of patients. Cite this article: Bone Joint J 2018;(1 Supple A)100-B:3–8

Aims. To evaluate the outcomes of cemented total hip arthroplasty (THA) following a fracture of the acetabulum, with evaluation of risk factors and comparison with a patient group with no history of fracture. . Patients and Methods. Between 1992 and 2016, 49 patients (33 male) with mean age of 57 years (25 to 87) underwent cemented THA at a mean of 6.5 years (0.1 to 25) following acetabular fracture. A total of 38 had undergone surgical fixation and 11 had been treated non-operatively; 13 patients died at a mean of 10.2 years after THA (0.6 to 19). Patients were assessed pre-operatively, at one year and at final follow-up (mean 9.1 years, 0.5 to 23) using the Oxford Hip Score (OHS). Implant survivorship was assessed. An age and gender-matched cohort of THAs performed for non-traumatic osteoarthritis (OA) or avascular necrosis (AVN) (n = 98) were used to compare complications and patient-reported outcome measures (PROMs). Results. The mean time from fracture to THA was significantly shorter for patients with AVN (2.2 years) or protrusio (2.2 years) than those with post-traumatic OA (9.4 years) or infection (8.0 years) (p = 0.03). Nine contained and four uncontained defects were managed with autograft (n = 11), bulk allograft (n = 1), or trabecular metal augment (n = 1). Initial fracture management (open reduction and internal fixation or non-operative), timing of THA (> /< one year), and age (> /< 55 years) had no significant effect on OHS or ten-year survival. Six THAs were revised at mean of 12 years (5 to 23) with ten-year all-cause survival of 92% (95% confidence interval 80.8 to 100). THA complication rates (all complications, heterotopic ossification, leg length discrepancy > 10 mm) were significantly higher following acetabular fracture compared with atraumatic OA/AVN and OHSs were inferior: one-year OHS (35.7 versus 40.2, p = 0.026); and final follow-up OHS (33.6 versus 40.9, p = 0.008). . Conclusion . Cemented THA is a reasonable option for the sequelae of acetabular fracture. Higher complication rates and poorer PROMs, compared with patients undergoing THA for atraumatic causes, reflects the complex nature of these cases. Cite this article: Bone Joint J 2017;99-B:1399–1408

Aims. Fungal prosthetic joint infections (PJIs) are rare and account for about 1% of total PJIs. Our aim was to present clinical and microbiological results in treating these patients with a two-stage approach and antifungal spacers. Patients and Methods. We retrospectively reviewed our institutional database and identified 26 patients with positive fungal cultures and positive Musculoskeletal Infection Society (MSIS) criteria for PJI who were treated between 2009 and 2017. We identified 18 patients with total hip arthroplasty (THA) and eight patients with total knee arthroplasty (TKA). The surgical and antifungal treatment, clinical and demographic patient data, complications, relapses, and survival were recorded and analyzed. Results. The median follow-up was 33 months. The success rate was 38.5% (10/26). Fluconazole resistance was found in 15%. Bacterial co-infection was common in 44% of patients for THA and 66% of patients with TKA. Mortality, reoperations, and treatment failure were common complications. Conclusion. Treatment with a two-stage exchange is a possible option for treatment, although fungal infections have a high failure rate. Therapeutic factors for treatment success remain unclear. Cite this article: Bone Joint J 2019;101-B:589–595

Aims. The optimal treatment for independent patients with a displaced intracapsular fracture of the hip remains controversial. The recognised alternatives are hemiarthroplasty and total hip arthroplasty. At present there is no established standard of care, with both types of arthroplasty being used in many centres. Patients and Methods. We conducted a feasibility study comparing the clinical effectiveness of a dual mobility acetabular component compared with standard polyethylene component in total hip arthroplasty for independent patients with a displaced intracapsular fracture of the hip, for a 12-month period beginning in June 2013. The primary outcome was the risk of dislocation one year post-operatively. Secondary outcome measures were EuroQol 5 Dimensions, ICEpop CAPability measure for Older people, Oxford hip score, mortality and re-operation. Results. Only 20 patients were recruited during this time. The baseline demographics were similar in the two groups and no patient suffered a dislocation. Differences in secondary outcomes were not analysed due to the small sample. Conclusion. This feasibility study suggests that any trial investigating the effectiveness of total hip arthroplasty for fracture of the hip might not be deliverable within the constraints of current systems of care in the United Kingdom. Cite this article: Bone Joint J 2016;98-B:1431–5

We evaluated 31 patients with bilateral dysplastic hips who had undergone periacetabular osteotomy for early (Tönnis grade 0 or 1) or moderate (Tönnis grade 2) osteoarthritis in one hip and total hip replacement for advanced (Tönnis grade 3) osteoarthritis in the other. At a mean follow-up of 5.5 years (2 to 9) after periacetabular osteotomy and 6.7 years (3 to 10) after total hip replacement, there was no difference in the functional outcome in hips undergoing osteotomy for early or moderate osteoarthritis and those with a total hip replacement, as determined by the Merle d’Aubigné and Postel score and the Western Ontario and McMaster Universities osteoarthritis index. More patients preferred the spherical periacetabular osteotomy to total hip replacement (53% vs 23%; p = 0.029). Osteoarthritis secondary to hip dysplasia is often progressive. Given the results, timely correction of dysplasia by periacetabular osteotomy should be considered whenever possible in young patients since this could produce a favourable outcome which is comparable with that of total hip replacement

Aims. There is evidence that prior lumbar fusion increases the risk of dislocation and revision after total hip arthroplasty (THA). The relationship between prior lumbar fusion and the effect of femoral head diameter on THA dislocation has not been investigated. We examined the relationship between prior lumbar fusion or discectomy and the risk of dislocation or revision after THA. We also examined the effect of femoral head component diameter on the risk of dislocation or revision. Methods. Data used in this study were compiled from several Finnish national health registers, including the Finnish Arthroplasty Register (FAR) which was the primary source for prosthesis-related data. Other registers used in this study included the Finnish Health Care Register (HILMO), the Social Insurance Institutions (SII) registers, and Statistics Finland. The study was conducted as a prospective retrospective cohort study. Cox proportional hazards regression and Kaplan-Meier survival analysis were used for analysis. Results. Prior lumbar fusion surgery was associated with increased risk of prosthetic dislocation (hazard ratio (HR) = 2.393, p < 0.001) and revision (HR = 1.528, p < 0.001). Head components larger than 28 mm were associated with lower dislocation rates compared to the 28 mm head (32 mm: HR = 0.712, p < 0.001; 36 mm: HR = 0.700, p < 0.001; 38 mm: HR = 0.808, p < 0.140; and 40 mm: HR = 0.421, p < 0.001). Heads of 38 mm (HR = 1.288, p < 0.001) and 40 mm (HR = 1.367, p < 0.001) had increased risk of revision compared to the 28 mm head. Conclusion. Lumbar fusion surgery was associated with higher rate of hip prosthesis dislocation and higher risk of revision surgery. Femoral head component of 32 mm (or larger) associates with lower risk of dislocation in patients with previous lumbar fusion. Cite this article: Bone Joint J 2020;102-B(8):1003–1009

Aims. To our knowledge, no study has compared the long-term results of cemented and hybrid total hip arthroplasty (THA) in patients with osteoarthritis (OA) secondary to congenital hip disease (CHD). This is a demanding procedure that may require special techniques and implants. Our aim was to compare the long-term outcome of cemented low-friction arthroplasty (LFA) and hybrid THA performed by one surgeon. Patients and Methods. Between January 1989 and December 1997, 58 hips (44 patients; one man, 43 woman; mean age 56.6 years (25 to 77)) with OA secondary to CHD were treated with a cemented Charnley LFA (group A), and 55 hips (39 patients; two men, 37 women; mean age 49.1 years (27 to 70)) were treated with a hybrid THA (group B), by the senior author (GH). The clinical outcome and survivorship were compared. Results. At all timepoints, group A hips had slightly better survivorship than those in group B without a statistically significant difference, except for the 24-year survival of acetabular components with revision for aseptic loosening as the endpoint, which was slightly worse. The survivorship was only significantly better in group A compared with group B when considering reoperation for any indication as the endpoint, 15 years postoperatively (74% vs 52%, p = 0.018). Conclusion. We concluded that there was not a substantial difference at almost any time in the outcome of cemented Charnley LFAs compared with hybrid THAs when treating patients with OA of the hip secondary to CHD. We believe, however, that after improvements in the design of components used in hybrid THA, this could be the method of choice, as it is technically easier with a shorter operating time. Cite this article: Bone Joint J 2019;101-B:1050–1057

Aims. The aim of this study was to determine if the Oxford Knee and Hip Score (OKHS) can accurately predict when a primary knee or hip referral is deemed nonsurgical versus surgical by the surgeon during their first consultation, and to identify nonsurgical OKHS screening thresholds. Patients and Methods. We retrospectively reviewed pre-consultation OKHS for all consecutive primary total knee arthroplasty (TKA) and total hip arthroplasty (THA) consultations of a single surgeon over three years. The 1436 knees (1016 patients) and 478 hips (388 patients) included were categorized based on the surgeon’s decision into those offered surgery during the first consultation versus those not (nonsurgical). Spearman’s rank correlation coefficients and receiver operating characteristic (ROC) curve analysis were performed. Results. Oxford Scores were better for the nonsurgical cohorts (p < 0.001) and correlated with the surgical decision (p < 0.001). ROC area under the curve values for knees (0.83, 95% confidence intervals (CI) 0.81 to 0.85) and hips (0.87, 95% CI 0.84 to 0.91) were excellent. A conservative and effective threshold for knees is Oxford Knee Score (OKS) > 32 points (sensitivity = 0.997, negative predictive value (NPV) = 0.992) and for hips is Oxford Hip Score (OHS) > 34 points (sensitivity = 0.997, NPV = 0.978). Severable potential lower OKHS thresholds were identified. Conclusion. Pre-consultation OKHS demonstrate good ability to predict when a primary TKA or THA referral will be deemed nonsurgical in a single surgeon’s practice. Multiple OKHS thresholds can effectively screen out nonsurgical referrals. Cite this article: Bone Joint J 2019;101-B(6 Supple B):23–30

We reviewed the seven- to ten-year results of our previously reported prospective randomised controlled trial comparing total hip replacement and hemiarthroplasty for the treatment of displaced intracapsular fracture of the femoral neck. Of our original study group of 81 patients, 47 were still alive. After a mean follow up of nine years (7 to 10) overall mortality was 32.5% and 51.2% after total hip replacement and hemiarthroplasty, respectively (p = 0.09). At 100 months postoperatively a significantly greater proportion of hemiarthroplasty patients had died (p = 0.026). Three hips dislocated following total hip replacement and none after hemiarthroplasty. In both the total hip replacement and hemiarthroplasty groups a deterioration had occurred in walking distance (p = 0.02 and p < 0.001, respectively). One total hip replacement required revision compared with four hemiarthroplasties which were revised to total hip replacements. All surviving patients with a total hip replacement demonstrated wear of the cemented polyethylene component and all hemiarthroplasties had produced acetabular erosion. There was lower mortality (p = 0.013) and a trend towards superior function in patients with a total hip replacement in the medium term

Aims. The aims of this study were to characterize antibiotic choices for perioperative total knee arthroplasty (TKA) and total hip arthroplasty (THA) prophylaxis, assess antibiotic allergy testing efficacy, and determine rates of prosthetic joint infection (PJI) based on perioperative antibiotic regimen. Patients and Methods. We evaluated all patients undergoing primary TKA or THA at a single academic institution between January 2004 and May 2017, yielding 29 695 arthroplasties (22 705 patients), with 3411 arthroplasties in 2576 patients (11.5%) having undergone preoperative allergy testing. A series of institutional databases were combined to identify allergy consultation outcomes, perioperative antibiotic regimen, and infection-free survivorship until final follow-up. Results. Among 2576 allergy-tested patients, 2493 patients (97%) were cleared to use cephalosporins. For the entire cohort, 28 174 arthroplasties (94.9%) received cefazolin and 1521 (5.1%) received non-cefazolin antibiotics. Infection-free survivorship was significantly higher among arthroplasties receiving cefazolin compared with non-cefazolin antibiotics, with 0.06% higher survival free of infection at one month, 0.56% at two months, 0.61% at one year, and 1.19% at ten years (p < 0.001). Overall, the risk of PJI was 32% lower in patients treated with cefazolin after adjusting for the American Society of Anesthesiologists (ASA) classification, joint arthroplasty (TKA or THA), and body mass index (BMI; p < 0.001). The number needed to treat with cefazolin to prevent one PJI was 164 patients at one year and 84 patients at ten years. Therefore, potentially 6098 PJIs could be prevented by one year and 11 905 by ten years in a cohort of 1 000 000 primary TKA and THA patients. Conclusion. PJI rates are significantly higher when non-cefazolin antibiotics are used for perioperative TKA and THA prophylaxis, highlighting the positive impact of preoperative antibiotic allergy testing to increase cefazolin usage. Given the low rate of true penicillin allergy positivity, and the readily modifiable risk factor that antibiotic choice provides, we recommend perioperative testing and clearance for all patients presenting with penicillin and cephalosporin allergies. Cite this article: Bone Joint J 2019;101-B(6 Supple B):9–15

Aims. Total hip arthroplasty (THA) is gaining popularity as a treatment for displaced femoral neck fractures (FNFs), especially in physiologically younger patients. While THA for osteoarthritis (OA) has demonstrated low complication rates and increased quality of life, results of THA for acute FNF are not as clear. Currently, a THA performed for FNF is included in an institutional arthroplasty bundle without adequate risk adjustment, potentially placing centres participating in fracture care at financial disadvantage. The purpose of this study is to report on perioperative complication rates after THA for FNF compared with elective THA performed for OA of the hip. Patients and Methods. The American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) database between 2008 and 2016 was queried. Patients were identified using the THA Current Procedural Terminology (CPT) code and divided into groups by diagnosis: OA in one and FNF in another. Univariate statistics were performed. Continuous variables were compared between groups using Student’s t-test, and the chi-squared test was used to compare categorical variables. Multivariate and propensity-matched logistic regression analyses were performed to control for risk factors of interest. Results. Analyses included 139 635 patients undergoing THA. OA was the indication in 135 013 cases and FNF in 4622 cases. After propensity matching, mortality within 30 days (1.8% vs 0.3%; p < 0.001) and major morbidity (24.2% vs 19%; p < 0.001) were significantly higher among FNF patients. Re-operation (3.7% vs 2.7%; p = 0.014) and re-admission (7.3% vs 5.5%; p = 0.002) were significantly higher among FNF patients. Hip fracture patients had significantly longer operative time and length of stay (LOS), and were significantly less likely to be discharged to their home. Multivariate analyses gave similar results. Conclusion. This large database study showed a higher risk of postoperative complications including mortality, major morbidity, re-operation, re-admission, prolonged operative time, increased LOS, and decreased likelihood of discharge home in patients undergoing THA for FNF compared with OA. While THA is a good option for FNF patients, there are increased costs and financial risks to centres with a joint arthroplasty bundle programme participating in fracture care. Cite this article: Bone Joint J 2019;101-B(6 Supple B):84–90

Aims

Although the short stem concept in hip arthroplasty procedure shows acceptable clinical performance, we sometimes get unexplainable radiological findings. The aim of this retrospective study was to evaluate changes of radiological findings up to three years postoperatively, and to assess any potential contributing factors on such radiological change in a Japanese population.

Aims

The aim of this study was to compare the mid-term patient-reported outcome, bone remodelling, and migration of a short stem (Collum Femoris Preserving; CFP) with a conventional uncemented stem (Corail).

Aims. Surface replacement arthroplasty (SRA), compared with traditional total hip arthroplasty (THA), is more expensive and carries unique concern related to metal ions production and hypersensitivity. Additionally, SRA is a more demanding procedure with a decreased margin for error compared with THA. To justify its use, SRA must demonstrate comparable component survival and some clinical advantages. We therefore performed a systematic literature review to investigate the differences in complication rates, patient-reported outcomes, stress shielding, and hip biomechanics between SRA and THA. Materials and Methods. A systematic review of the literature was completed using MEDLINE and EMBASE search engines. Inclusion criteria were level I to level III articles that reported clinical outcomes following primary SRA compared with THA. An initial search yielded 2503 potential articles for inclusion. Exclusion criteria included review articles, level IV or level V evidence, less than one year’s follow-up, and previously reported data. In total, 27 articles with 4182 patients were available to analyze. Results. Fracture and infection rates were similar between SRA and THA, while dislocation rates were lower in SRA compared with THA. SRA demonstrated equivalent patient-reported outcome scores with greater activity scores and a return to high-level activities compared with THA. SRA more reliably restored native hip joint biomechanics and decreased stress shielding of the proximal femur compared with THA. Conclusion. In young active men with osteoarthritis, there is evidence that SRA offers some potential advantages over THA, including: improved return to high level activities and sport, restoration of native hip biomechanics, and decreased proximal femoral stress shielding. Continued long-term follow up is required to assess ultimate survivorship of SRA

Aims. The aim of this study was to evaluate blood metal ion levels, leucocyte profiles, and serum cytokines in patients with a total hip arthroplasty (THA) involving modular dual-mobility components. Patients and Methods. A total of 39 patients were recruited, with clinical follow-up of up to two years. Outcome was assessed using the Harris Hip Score (HHS, the 12-Item Short-Form Health Survey (SF-12), the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), and a visual analogue scale (VAS) for pain. Blood concentrations of cobalt (Co), chromium (Cr), and serum cytokines were measured. Subpopulations of leucocytes were analyzed by flow cytometry. Results. The clinical performance was good. Blood Co levels (ref 1.0 µg/l) were mildly elevated in seven patients at three months, and two patients at two years’ follow-up. The preoperative Cr levels were normal except for one patient with a detectable Cr (1.2 µg/l). Cr levels were detectable in three patients at three months, two patients at one year, and three patients at two years’ follow-up. No patients had symptoms suggestive of failure. Although flow cytometry showed constant circulating leucocyte profiles, there was a significant reduction of serum interleukin (IL)-4, IL-5, and interferon gamma (IFNγ) postoperatively compared with the preoperative levels (p < 0.05). Conclusion. These results suggest that THA using modular dual-mobility components is safe. This allows an opportunity to use a large femoral head and a thick polyethylene bearing surface, which is especially useful in revision procedures or high-risk situations when added stability is required. Cite this article: Bone Joint J 2019;101-B:1035–1041

Aims

Perthes’ disease (PD) often results in femoral head deformity and leg length discrepancy (LLD). Our objective was to analyze femoral morphology in PD patients at skeletal maturity to assess where the LLD originates, and evaluate the effect of contralateral epiphysiodesis for length equalization on proximal and subtrochanteric femoral lengths.

Total hip replacement in patients with Gaucher’s disease with symptomatic osteonecrosis of the femoral head is controversial because of the high early failure rates. We describe four patients who had an uncemented total hip replacement following enzyme replacement therapy for a median of two years and one month (1 to 9.8 years) prior to surgery, and who remained on treatment. At operation, the bone had a normal appearance and consistency. Histopathological examination showed that, compared with previous biopsies of untreated Gaucher’s disease, the Gaucher cell infiltrate had decreased progressively with therapy, being replaced by normal adipose tissue. The surfaces of viable bone beyond the osteonecrotic areas showed osteoblasts, indicating remodelling. In one case acetabular revision was carried out after 11 years and eight months. The three remaining patients had a mean follow-up of six years and four months (3.3 to 12 years). We recommend initiating enzyme replacement therapy at least one to two years prior to total hip replacement to facilitate bone remodelling and to allow implantation of uncemented components in these young patients