Aims. To determine the normal values and usefulness of the C1/4 space available for spinal cord (SAC) ratio and C1 inclination angle, which are new radiological parameters for assessing atlantoaxial instability in children with Down syndrome. Patients and Methods. We recruited 272 children with Down syndrome (including 14 who underwent surgical treatment), and 141 children in the control group. All were aged between two and 11 years. The C1/4 SAC ratio, C1 inclination angle, atlas-dens interval (ADI), and SAC were measured in those with Down syndrome, and the C1/4 SAC ratio and C1 inclination angle were measured in the control group. Results. The mean C1/4 SAC ratio in those requiring surgery with Down syndrome, those with Down syndrome not requiring surgery and controls were 0.63 (standard deviation (. sd. ) 0.1), 1.15 (. sd . 0.13) and 1.29 (. sd. 0.14), respectively, and the mean C1 inclination angles were -3.1° (. sd.  10.7°), 15.8° (. sd. 7.3) and 17.2° (. sd. 7.3), in these three groups, respectively. The mean ADI and SAC in those with Down syndrome requiring surgery and those with Down syndrome not requiring surgery were 9.8 mm (. sd. 2.8) and 4.3 mm (. sd. 1.0), and 11.1 mm (. sd. 2.6) and 18.5 mm (. sd. 2.4), respectively. Conclusion. The normal values of the C1/4 SAC ratio and the C1 inclination angle were found to be about 1.2° and 15º, respectively. Cite this article: Bone Joint J 2016;98-B:1704–10

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

Aims

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

Aims

Although atlantoaxial rotatory fixation (AARF) is a common cause of torticollis in children, the diagnosis may be delayed. The condition is characterised by a lack of rotation at the atlantoaxial joint which becomes fixed in a rotated and subluxed position. The management of children with a delayed presentation of this condition is controversial. This is a retrospective study of a group of such children.

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.

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.

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.

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).