Down’s syndrome is associated with a number of musculoskeletal abnormalities, some of which predispose patients to early symptomatic arthritis of the hip. The purpose of the present study was to review the general and hip-specific factors potentially compromising total hip replacement (THR) in patients with Down’s syndrome, as well as to summarise both the surgical techniques that may anticipate the potential adverse impact of these factors and the clinical results reported to date. A search of the literature was performed, and the findings further informed by the authors’ clinical experience, as well as that of the hip replacement in Down Syndrome study group. The general factors identified include a high incidence of ligamentous laxity, as well as associated muscle hypotonia and gait abnormalities. Hip-specific factors include: a high incidence of hip dysplasia, as well as a number of other acetabular, femoral and combined femoroacetabular anatomical variations. Four studies encompassing 42 hips, which reported the clinical outcomes of THR in patients with Down’s syndrome, were identified. All patients were successfully treated with standard acetabular and femoral components. The use of supplementary acetabular screw fixation to enhance component stability was frequently reported. The use of constrained liners to treat intra-operative instability occurred in eight hips. Survival rates of between 81% and 100% at a mean follow-up of 105 months (6 to 292) are encouraging. Overall, while THR in patients with Down’s syndrome does present some unique challenges, the overall clinical results are good, providing these patients with reliable pain relief and good function. Cite this article: Bone Joint J 2013;95-B, Supple A:41–5

We treated eight dysplastic acetabula in six skeletally mature patients with Down’s syndrome by a modified Bernese periacetabular osteotomy. The mean age at the time of surgery was 16.5 years (12.8 to 28.5). Mean length of follow-up was five years (2 to 10.4). Pre-operatively the mean (Tönnis) acetabular angle was 28°, the centre-edge angle was −9°, and the extrusion index was 60%; post-operatively they were 3°, 37°, and 17%, respectively. Two patients with post-operative (Tönnis) acetabular angles > 10° developed subluxation post-operatively and required secondary varus derotation femoral osteotomies. Another patient developed a late labral tear which was treated arthroscopically. All eight hips remain clinically stable, and are either asymptomatic or symptomatically improved. These results suggest that the modified Bernese periacetabular osteotomy can be used successfully in the treatment of acetabular dysplasia in patients with Down’s syndrome

Hip osteoarthritis is prevalent in 8%–28% of patients with Down's Syndrome. Presence of disabling hip pain is increased along with prolonged life expectancy, suggesting total hip arthroplasty (THA). Seven consecutive patients (9 hips) with Down's syndrome had primary THA. Coxarthrosis was secondary to developmental hip dysplasia in 6 patients and slipped capital epiphysis in 1 patient. In 5 patients (7 hips) a previous hip surgery was performed. Average clinical and radiological follow up was 9.9 ± 6.4 years (range 2–22.5, median 9.3). Average age of patients at THA was 34.8 ± 7.5 years (range 25–47, median 35.4). In 2 patients (3 hips) a trochanteric slide was used for the surgical approach, while a lateral transgluteal approach was used in the remaining patients. One way ANOVA test was used to compare Harris Hip Scores (HHS) at post-operative follow-up. HHS improved significantly (p=0.008) improved from 4.1 ± 15.1 (range 18.5–65, median 45) to 84.3 ± 7.7 (range 70–93, median 85.8 at 4 year follow up. HHS (average 70.9 ± 6.2, range 66.5–80, median 68) remained essentially unchanged (p=0.43) at 8 year follow-up. Two patients required revision arthroplasty for stem loosening at 6 and 16 years post THA, respectively. The first patient is 7 years post revision and ambulates without aids. The second patient is 6.1 years post revision and ambulates with a walker. Six of the THAs required a constrained liner. No dislocations or deep infections were encountered. THA is reliable surgical intervention in patients with Down's Syndrome and symptomatic coxarthrosis

Hip disease occurs in between 8% and 28% of patients with Down’s syndrome, many of whom develop disabling pain. We have carried out total hip replacement in six adult patients (9 hips) with severe arthritis of the hip. The mean follow-up was 7.75 years (2 to 14). At the latest review, all had relief of pain and full hip function. Increasing longevity and a high incidence of hip disease in these patients suggest a greater role for total hip arthroplasty in the future

The purpose of this study was to describe the clinical course of patients with Down's syndrome (DS) and congentital talipes equinovarus (CTEV) treated with the Ponseti regimen. The members of the United Kingdom Ponseti Users Group were contacted to provide details of patients with DS and CTEV, whom they had treated using the Ponseti regimen. Nine patients (13 feet: 7 right, 6 left) were identified, and the case notes were reviewed. Six patients were male, 3 female. In all but one case, the DS was diagnosed postnatally. Co-morbidites included atrioventricular septal defect, hearing deficiencies and plagiocephaly. The initial mean Pirani score was 4.5 (range 3.0 to 6.0). Casting was commenced at a mean of 25 days (range 12–84 days). The mean number of casts required was 7 (range 3 to 12), taking a mean of 6.5 weeks (range 3–12) to achieve correction. 6 of the 13 feet (46%) required a tendoachilles tenotomy, and 2 of 13 (15%) required re-casting. No patients have required a tibialis anterior transfer, soft tissue releases or bony procedures, at a mean follow up of 44 months (9–65 months). The results of the Ponseti regimen have not been described in patients with DS. From this small series, we can conclude that all patients responded to the regime. A tendoachilles tenotomy was required in just under half, and further casting was required in only 15% of the treated feet. No patient has required further surgery. The tenotomy rate is lower than in most series, but otherwise, the results are comparable to those for idiopathic CTEV for which the Ponseti regimen has become the gold standard. Parents of children with DS can be reassured that in the short term their feet will respond well to Ponseti treatment

There is an increased incidence of dislocation, dysplasia, slipped epiphysis, Perthes’ disease, and avascular necrosis leading to degenerative arthritis which occurs in up to 28% of Down's syndrome patients. As the life expectancy for patients with Down's syndrome has increased, so has the presence of hip disease. Hip replacement has been shown to have good results in this population. Special considerations include a high risk of postoperative dislocation and leg length inequality which often require large head THR or dual mobility type reconstruction to reduce these risks. Numerous spine deformities including scoliosis and C1-2 subluxation need to be taken into account-anesthesia consult

In primary total hip replacements there are numerous options available for providing hip stability in difficult situations (i.e. Down's syndrome, Parkinson's disease). We have considered constrained liners in some of these cases. However, in the revision situation in general and in revision for recurrent dislocation situation specifically it is important to have all options available including tripolar constrained liners in order to optimise the potential for hip stability as well as function of the arthroplasty. Even with the newer options available dislocation rates of higher than 10–15% have been reported following revision surgery at institutions where high volumes of revision surgery are performed. Because of the deficient abductors, other soft tissue laxity and the requirement for large diameter cups revision cases will always have more potential for dislocation. In these situations in the lower demand patient, constraint has provided excellent success in terms of preventing dislocation and maintaining implant construct fixation to bone at intermediate- term follow-up. Hence in these situations tripolar constrained liners remains the option we utilise. We are also confident in using this device in cases with instability or laxity where there is a secure well- positioned acetabular shell. We cement a dual mobility constrained liner in these situations using the technique described below. Present indication for tripolar constrained liners: low demand patient, large outer diameter cups, instability with well-fixed shells that are adequately positioned, abductor muscle deficiency or soft tissue laxity, multiple operations for instability. Technique of cementing liner into shell: score acetabular shell if no holes, score liner in spider web configuration, all one or two millimeters of cement mantle. Results. Constrained Dual Mobility Liner. For Dislocation: 56 Hips, 10 yr average f/u, 7% failure of device, 5% femoral loosening, 4% acetabular loosening. For Difficult Revisions:101 hips, 10 yr average f/u, 6% failure of device, 4% femoral loosening, 4% acetabular loosening. Cementing Liner into Shell: 31 hips, 3.6 yr average f/u (2–10 years), 2 of 31 failures

In primary total hip replacements there are numerous options available for providing hip stability in difficult situations (i.e. Down's syndrome, Parkinson's disease). However, in the revision situation in general and in revision for recurrent dislocation specifically, it is important to have all options available including dual mobility constrained liners in order to optimise the potential for hip stability as well as function of the arthroplasty. Even with the newer options, available dislocation rates of higher than 5% have been reported in the first two years following revision surgery at institutions where high volumes of revision surgery are performed. Because of the deficient abductors, other soft tissue laxity and the requirement for large diameter cups, revision cases will always have more potential for dislocation. In these situations in the lower demand patient and where, a complex acetabular reconstruction that requires time for ingrowth before optimal implant bone stability to occur isn't present, dual mobility with constraint has provided excellent success in terms of preventing dislocation and maintaining implant construct fixation to bone at intermediate term follow-up. Hence in these situations dual mobility with constraint remains the option we utilise. We are also confident in using this device in cases with instability or laxity where there is a secure well-positioned acetabular shell. We cement a dual mobility constrained liner in these situations using the technique described below. Present indication for dual mobility constrained liners: low demand patient, large outer diameter cups, instability with well-fixed shells that are adequately positioned, abductor muscle deficiency or soft tissue laxity, multiple operations for instability. Technique of cementing liner into shell: score acetabular shell if no holes, score liner in spider web configuration, all one or two millimeters of cement mantle. Results: Constrained Dual Mobility Liner – For Dislocation: 56 Hips, 10 year average follow-up, 7% failure of device, 5% femoral loosening, 4% acetabular loosening. For Difficult Revisions: 101 hips, 10 year average follow-up, 6% failure of device, 4% femoral loosening, 4% acetabular loosening. Cementing Liner into Shell: 31 hips, 3.6 year average follow-up (2–10 years), 2 of 31 failures

In primary total hip replacements there are numerous options available for providing hip stability in difficult situations i.e. Down's syndrome, Parkinson's disease. However, in the revision situation, in general, and in revision for recurrent dislocation situations specifically, it is important to have all options available including dual mobility constrained liners in order to optimise the potential for hip stability as well as function of the arthroplasty. Even with the newer options available dislocation rates of higher than 5% have been reported in the first two years following revision surgery at institutions where high volumes of revision surgery are performed [Della Valle, Sporer, Paprosky unpublished data]. Because of the deficient abductors, other soft tissue laxity and the requirement for large diameter cups, revision cases will always have more potential for dislocation. In these situations in the lower demand patient and where, a complex acetabular reconstruction that requires time for ingrowth before optimal implant bone stability to occur isn't present, dual mobility with constraint has provided excellent success in terms of preventing dislocation and maintaining implant construct fixation to bone at intermediate term follow-up. Hence in these situations dual mobility with constraint remains the option we utilise. We are also confident in using this device in cases with instability or laxity where there is a secure well-positioned acetabular shell. We cement a dual mobility constrained liner in these situations using the technique described below. Present indication for dual mobility constrained liners: low demand patient, abductor muscle deficiency or soft tissue laxity, large outer diameter cups, multiple operations for instability, and instability with well-fixed shells that are adequately positioned. Technique of cementing liner into shell: score acetabular shell if no holes; score liner in spider web configuration; all one or two millimeters of cement mantle. Results. Constrained Dual Mobility Liner. For Dislocation: 56 Hips 10 yr average f/u, 7% failure of device, 5% femoral loosening, 4% acetabular loosening. For Difficult Revisions: 101 hips 10 yr average f/u, 6% failure of device, 4% femoral loosening, 4% acetabular loosening. Cementing Liner into Shell: 31 hips 3.6 yr average f/u (2–10 years), 2 of 31 failures

Introduction. Through the paediatric LCP Hip plating system, the highly successful technique of the locking compression plate used in adult surgery, has been incorporated in a system dedicated to paediatrics. The purpose of this study was to review the outcome of the paediatric LCP Hip plate use in children, both with and without neuromuscular disease, for fixation of proximal femoral osteotomy for a variety of indications. Materials and methods. We retrospectively reviewed the notes and radiographs of all those children who have had Paediatric LCP Hip Plate for the fixation of proximal femoral osteotomy and proximal femur fractures in our institution, between October 2007 and July 2010, for their clinical progress, mobilization status, radiological healing and any complications. Results. Forty-three Paediatric LCP hip plates were used in forty patients for the fixation of proximal femoral osteotomies (n=40) and proximal femur fractures (n=3). The osteotomies were performed for a variety of indications including Perthes disease, DDH, Cerebral Palsy, Down's syndrome, coxa vara, Leg length discrepancy and previous failed treatment of SUFE. Twenty-five children were allowed touch to full weight bearing post operatively. Two were kept non-weight bearing for 6 weeks. The remaining 13 children were treated in hip spica due to simultaneous pelvic osteotomy or multilevel surgery for cerebral palsy. All osteotomies and fractures radiologically healed within 6 months (majority [n=40] within 3 months). There was no statistically significant difference (p = 0.45) in the neck shaft angle between the immediately postoperative and final x-rays after completion of bone healing. There were no implant related complications. Conclusion. The Paediatric LCP Hip Plate provides a stable and reliable fixation of the proximal femoral osteotomy performed for a variety of paediatric hip conditions in children with and without neuromuscular disease

Patients with Neuromuscular disorders who have an arthritic hip joint present unique challenges. Due to inadequate bone stock, bony deformity and muscle imbalance there is a potentially increased risk of dislocation. These patients who have undergone conventional total hip replacement in the past, have been immobilised post-operatively in a hip spica to prevent post-op dislocation. Large heads are inherently more stable and adequately treat these patients, without the need for post-operative immobilisation. We present our experience with large head replacement in this group of patients. Eight patients, five with cerebral palsy, two with Down’s Syndrome and one with Cornelia de large Syndrome, who underwent total hip replacement with a large head prosthesis were reviewed. Their average age was 44.5 years and patients were followed up for maximum 51 months. Patients were assessed with respect to pre- and post-op pain, mobility and function. Hip scores have not been found to be useful in assessing these patients. Two Birmingham Surface replacements were used and six large heads on conventional stems, with large uncemented cups. In all patients there was improvement in pain and function. Four patients had improvement in their ambulatory status. In none did this worsen. Despite no post-operative immobilisation, there was only on dislocation. This was due to error in cup positioning, an error in the early learning curve. Despite the small numbers and short follow up, we feel that large head replacement is a viable alternative to conventional hip replacement in these difficult cases

Through the paediatric LCP Hip plating system (Synthes GmBH Eimattstrasse 3 CH- 4436 Oberdorff), the highly successful technique of the locking compression plate used in adult surgery, has been incorporated in a system dedicated to pediatrics. We are presenting the outcome of the paediatric LCP hip plating system used for a variety of indications in our institution. We retrospectively reviewed the notes and radiographs of all those children who have had Paediatric LCP Plate for the fixation of proximal femoral osteotomy and proximal femur fractures in our institution, between October 2007 and July 2010, for their clinical progress, mobilization status, radiological healing and any complications. Forty-three Paediatric LCP hip plates were used in forty patients (24 males and 13 females) for the fixation of proximal femoral osteotomies (n=40) and proximal femur fractures (n=3). The osteotomies were performed for a variety of indications including Perthes disease, developmental dysplasia of hip, Cerebral Palsy, Down's syndrome, coxa vara, Leg length discrepancy and previous failed treatment of Slipped Upper Femoral Epiphysis. Twenty-five children were allowed touch to full weight bearing post operatively. Two were kept non-weight bearing for 6 weeks. The remaining 13 children were treated in hip spica due to simultaneous pelvic osteotomy or multilevel surgery for cerebral palsy. All osteotomies and fractures radiologically healed within 6 months (majority [n=40] within 3 months). There was no statistically significant difference (p= 0.45) in the neck shaft angle between the immediately postoperative and final x-rays after completion of bone healing. Among the children treated without hip spica, 1 child suffered a periprosthetic fracture. Of the children treated in hip spica, 2 had pressure sores, 3 had osteoporotic distal femur fractures and 2 had posterior subluxations requiring further intervention. There were no implant related complications. The Paediatric LCP Hip Plate provides a stable and reliable fixation of the proximal femoral osteotomy performed for a variety of paediatric orthopaedic conditions

We describe a successful modified operative procedure at an average 19 months follow-up in 3 patients with congenital dislocation of the patella and compare its merits to the other procedures already reported in the literature. Congenital dislocation of the patella may be associated with other congenital conditions or syndromes i.e. Down’s syndrome, congenital vertical talus and cerebral palsy. Numerous operative techniques have been described in the literature which may be divided into 3 basic groups. A modification of the Langenskiold & Ritsila procedure is described. The original procedure describes an extensive lateral release with detachment and medial transfer of the patellar tendon through a curved incision. The tendon is routed through a fold of synovium medially and fixed distally to bone with sutures through drill holes in the proximal tibia. We found at surgery this synovium was too fragile to hold the transferred tendon and the use of drill holes unnecessary. The main alterations include a limited and straight anterior skin incision, a fashioning of a ‘buckle’ of the transferred distal patellar tendon to a distally based flap which avoids drill holes in the growing bone. This modification of the Langenskiold procedure was used successfully in 3 cases, including a revision of a failed Goldthwaite- Roux procedure in a mentally handicapped child. The results at average 19 months follow-up are successful. The early results confirm that the patella remains located and tracks normally. The valgus and flexion deformities have significantly improved. This modification of the Langenskiold & Ritsila procedure requires less dissection than other operations, with no bony surgery and a cosmetic scar. The Langen-skiold & Ritsila procedure has been successful and we feel that this modification simplifies and improves on the original technique

The advent of modular porous metal augments has ushered in a new form of treatment for acetabular bone loss. The function of an augment can be seen as reducing the size of a defect or reconstituting the anterosuperior/posteroinferior columns and/or allowing supplementary fixation. Depending on the function of the augment, the surgeon can decide on the sequence of introduction of the hemispherical shell, before or after the augment. Augments should always, however, be used with cement to form a unit with the acetabular component. Given their versatility, augments also allow the use of a hemispherical shell in a position that restores the centre of rotation and biomechanics of the hip. Progressive shedding or the appearance of metal debris is a particular finding with augments and, with other radiological signs of failure, should be recognized on serial radiographs. Mid- to long-term outcomes in studies reporting the use of augments with hemispherical shells in revision total hip arthroplasty have shown rates of survival of > 90%. However, a higher risk of failure has been reported when augments have been used for patients with chronic pelvic discontinuity.

Cite this article: Bone Joint J 2024;106-B(4):312–318.

Aims

The aims of this study were to characterize the incidence and risk factors associated with stress fractures following periacetabular osteotomy, and to determine their effect on osteotomy union.

Aims

The aim of this study was to inform the epidemiology and treatment of slipped capital femoral epiphysis (SCFE).

Aims

The COVID-19 pandemic drastically affected elective orthopaedic services globally as routine orthopaedic activity was largely halted to combat this global threat. Our institution (University College London Hospital, UK) previously showed that during the first peak, a large proportion of patients were hesitant to be listed for their elective lower limb procedure. The aim of this study is to assess if there is a patient perception change towards having elective surgery now that we have passed the peak of the second wave of the pandemic.

Aims

Prior studies have identified that malseating of a modular dual mobility liner can occur, with previous reported incidences between 5.8% and 16.4%. The aim of this study was to determine the incidence of malseating in dual mobility implants at our institution, assess for risk factors for liner malseating, and investigate whether liner malseating has any impact on clinical outcomes after surgery.

Aims

Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service.

We describe our experience with the ‘four-in-one’ procedure for habitual dislocation of the patella in five children (six knees). All the patients presented with severe generalised ligamentous laxity and aplasia of the trochlear groove. All had a lateral release, proximal ‘tube’ realignment of the patella, semitendinosus tenodesis and transfer of the patellar tendon. The mean age at the time of the operation was 6.1 years (4.9 to 6.9), and the patients were followed up for a mean of 54.5 months (31 to 66). The clinical results were evaluated using the Kujala score.

There has been no recurrence of dislocation. All the patients have returned to full activities and the parents and children were satisfied with the clinical results. The mean Kujala score was 95.3 (88 to 98). Two patients had marginal skin necrosis which healed after debridement and secondary closure. These early results in this small group have shown that the ‘four-in-one’ procedure is effective in the treatment of obligatory dislocation of the patella in children with severe ligamentous laxity and trochlear aplasia.