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 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 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).

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.

Slipped upper femoral epiphysis (SUFE) is the most common hip disorder to affect adolescents. Controversy exists over the optimal treatment of severe slips, with a continuing debate between in situ fixation versus corrective surgery. We present our experience in a series of 57 patients presenting with severe unilateral SUFE (defined > 50°) managed with a subcapital cuneiform osteotomy.

Between 2001 and 2011, 57 patients (35 male, 22 female) with a mean age of 13.1 years (9.6 to 20.3, SD 2.3) were referred to our tertiary referral institution with a severe slip. The affected limb was rested in slings and springs before corrective surgery which was performed via an anterior Smith-Petersen approach. Radiographic analysis confirmed an improvement in mean head–shaft slip angle from 53.8^o (standard deviation (sd) 3.2) pre-operatively to 9.1^o (sd 3.1) post-operatively, with minimal associated femoral neck shortening. In total 50 (88%) patients were complication free at a mean follow-up of seven years (2.8 to 13.9 years, sd 3). Their mean Oxford hip score was 44 (37 to 48) and median visual analogue pain score was 0 out of 10 (interquartile range 0 to 4). A total of six patients (10.5%) developed avascular necrosis requiring further surgery and one (1.8%) patient developed chondrolysis but declined further intervention.

This is a technically demanding operation with variable outcomes reported in the literature. We have demonstrated good results in our tertiary centre.

Cite this article: Bone Joint J 2015;97-B:1718–25.

Transarticular screw fixation with autograft is an established procedure for the surgical treatment of atlantoaxial instability. Removal of the posterior arch of C1 may affect the rate of fusion. This study assessed the rate of atlantoaxial fusion using transarticular screws with or without removal of the posterior arch of C1. We reviewed 30 consecutive patients who underwent atlantoaxial fusion with a minimum follow-up of two years. In 25 patients (group A) the posterior arch of C1 was not excised (group A) and in five it was (group B). Fusion was assessed on static and dynamic radiographs. In selected patients CT imaging was also used to assess fusion and the position of the screws. There were 15 men and 15 women with a mean age of 51.2 years (23 to 77) and a mean follow-up of 7.7 years (2 to 11.6). Stable union with a solid fusion or a stable fibrous union was achieved in 29 patients (97%). In Group A, 20 patients (80%) achieved a solid fusion, four (16%) a stable fibrous union and one (4%) a nonunion. In Group B, stable union was achieved in all patients, three having a solid fusion and two a stable fibrous union. There was no statistically significant difference between the status of fusion in the two groups. Complications were noted in 12 patients (40%); these were mainly related to the screws, and included malpositioning and breakage. The presence of an intact or removed posterior arch of C1 did not affect the rate of fusion in patients with atlantoaxial instability undergoing C1/C2 fusion using transarticular screws and autograft.

Cite this article: Bone Joint J 2013;95-B:972–6.

A nationwide study of Perthes’ disease in Norway was undertaken over a five-year period from January 1996. There were 425 patients registered, which represents a mean annual incidence of 9.2 per 100 000 in subjects under 15 years of age, and an occurrence rate of 1:714 for the country as a whole. There were marked regional variations. The lowest incidence was found in the northern region (5.4 per 100 000 per year) and the highest in the central and western regions (10.8 and 11.3 per 100 000 per year, respectively). There was a trend towards a higher incidence in urban (9.5 per 100 000 per year) compared with rural areas (8.9 per 100 000 per year). The mean age at onset was 5.8 years (1.3 to 15.2) and the male:female ratio was 3.3:1.

We compared 402 patients with a matched control group of non-affected children (n = 1 025 952) from the Norwegian Medical Birth Registry and analysed maternal data (age at delivery, parity, duration of pregnancy), birth length and weight, birth presentation, head circumference, ponderal index and the presence of congenital anomalies. Children with Perthes’ disease were significantly shorter at birth and had an increased frequency of congenital anomalies.

Applying Sartwell’s log-normal model of incubation periods to the distribution of age at onset of Perthes’ disease showed a good fit to the log-normal curve. Our findings point toward a single cause, either genetic or environmental, acting prenatally in the aetiology of Perthes’ disease.

We reviewed the long-term radiological outcome, complications and revision operations in 19 children with quadriplegic cerebral palsy and hip dysplasia who underwent combined peri-iliac osteotomy and femoral varus derotation osteotomy. They had a mean age of 7.5 years (1.6 to 10.9) and comprised 22 hip dislocations and subluxations. We also studied the outcome for the contralateral hip. At a mean follow-up of 11.7 years (10 to 15.1) the Melbourne cerebral palsy (CP) hip classification was grade 2 in 16 hips, grade 3 in five, and grade 5 in one. There were five complications seen in four hips (21%, four patients), including one dislocation, one subluxation, one coxa vara with adduction deformity, one subtrochanteric fracture and one infection. A recurrent soft-tissue contracture occurred in five hips and ten required revision surgery.

In pre-adolescent children with quadriplegic cerebral palsy good long-term outcomes can be achieved after reconstruction of the hip; regular follow-up is required.

Cite this article: Bone Joint J 2013;95-B:259–65.

Slipped upper femoral epiphysis (SUFE) with an open physis is rare in an adult and the condition may present without prior diagnosis of an underlying medical condition. We have treated a 29-year-old man with bilateral SUFE associated with autoimmune hypothyroidism. The management was delayed and complicated by co-existing autoimmune chronic active hepatitis. He underwent thyroxine therapy and bilateral pinning in situ with a single ASNIS screw. Closure of the physis occurred after five months on the right side. The left side required a further corrective intertrochanteric osteotomy, and it was only after 13 months that complete fusion of this physis was seen. The case highlights the need to consider endocrine and metabolic conditions in atypical presentation of SUFE.

This prospective cohort study aimed to investigate the relationship between developmental dysplasia of the hip and mode of delivery in 571 consecutive breech infants using a modified Graf’s static morphological method to grade the severity of dysplasia.

In this group, 262 infants were born by planned Caesarian section, 223 by emergency section and 86 vaginally. Taking all grades of hip dysplasia into account (Graf types II, III and IV), there was no statistical difference in the incidence of dysplasia between the groups (elective section 8.4%, emergency section 8.1% and vaginal delivery 7.0%). However, when cases with Graf type II dysplasia, which may represent physiological immaturity, were excluded, the rate of type III and IV hips, which we consider to be clinically relevant, increased in the vaginally delivered group (4.7%) compared with the elective section group (1.1%), with a relative risk of approximately 1:4 (95% confidence interval 1.03 to 15.91). No difference was observed between the emergency and elective section groups, or between the emergency section and vaginally delivered groups.

This study supports previous published work, with the added value that the diagnoses were all confirmed by ultrasound.

Avascular necrosis is a serious complication of slipped capital femoral epiphysis and is difficult to treat. The reported incidence varies from 3% to 47% of patients. The aims of treatment are to maintain the range of movement of the hip and to prevent collapse of the femoral head. At present there are no clear guidelines for the management of this condition and treatment can be difficult and unrewarding.

We have used examination under anaesthesia and dynamic arthrography to investigate avascular necrosis and to determine the appropriate method of treatment. We present 20 consecutive cases of avascular necrosis in patients presenting with slipped capital femoral epiphysis and describe the results of treatment with a mean follow-up of over eight years (71 to 121 months). In patients who were suitable for joint preservation (14), we report a ten-year survivorship of the hip joint of 75% and a mean Harris hip score of 82 (44 to 98).

Our aim was to evaluate the results of triple arthrodesis, performed without the use of supplementary bone graft. We carried out a retrospective review of 100 consecutive triple arthrodeses. All the operations had been performed by the senior author (TSS) using a standard technique. Only local bone graft from the excised joint surfaces had been used, thereby avoiding complications at the donor site.

The mean age of the patients at surgery was 58 years (18 to 84). The mean time to union was 5.1 months (3 to 17). There were 75 good, 20 fair and five poor results. There were four cases of nonunion.

Our study has shown that comparable rates of union are achieved without the need for supplementary bone graft from the iliac crest or other donor site.