Avascular necrosis (AVN) is a serious complication of high-dose chemotherapy for haematological malignancy in childhood. In order to describe its incidence and main risk factors and to evaluate the current treatment options, we reviewed 105 children with a mean age of 8.25 years (1 to 17.8) who had acute lymphoblastic or acute myeloid leukaemia, or a non-Hodgkin’s lymphoma. Overall, eight children (7.6%) developed AVN after a mean of 16.8 months (8 to 49). There were four boys and four girls with a mean age of 14.4 years (9.8 to 16.8) and a total of 18 involved sites, 12 of which were in the femoral head. All these children were aged > nine years (p < 0.001). All had received steroid treatment with a mean cumulative dose of prednisone of 5967 mg (4425 to 9599) compared with a mean of 3943 mg (0 to 18 585) for patients without AVN (p = 0.005). No difference existed between genders and no thrombophilic disorders were identified. Their initial treatment included 11 core decompressions and two bipolar hip replacements. Later, two salvage osteotomies were done and three patients (four hips) eventually needed a total joint replacement. We conclude that AVN mostly affects the weight-bearing epiphyses. Its risk increases with age and higher steroid doses. These high-risk patients may benefit from early screening for AVN.

Cite this article: Bone Joint J 2013;95-B:1708–13.

A combined anterior and posterior surgical approach is generally recommended in the treatment of severe congenital kyphosis, despite the fact that the anterior vascular supply of the spine and viscera are at risk during exposure. The aim of this study was to determine whether the surgical treatment of severe congenital thoracolumbar kyphosis through a single posterior approach is feasible, safe and effective.

We reviewed the records of ten patients with a mean age of 11.1 years (5.4 to 14.1) who underwent surgery either by pedicle subtraction osteotomy or by vertebral column resection with instrumented fusion through a single posterior approach.

The mean kyphotic deformity improved from 59.9° (45° to 110°) pre-operatively to 17.5° (3° to 40°) at a mean follow-up of 47.0 months (29 to 85). Spinal cord monitoring was used in all patients and there were no complications during surgery. These promising results indicate the possible advantages of the described technique over the established procedures. We believe that surgery should be performed in case of documented progression and before structural secondary curves develop. Our current strategy after documented progression is to recommend surgery at the age of five years and when 90% of the diameter of the spinal canal has already developed.

Cite this article: Bone Joint J 2013;95-B:1527–32.

The August 2013 Children’s orthopaedics Roundup³⁶⁰ looks at: a multilevel approach to equinus gait; whether screening leads to needless intervention; salvage of subcapital slipped epiphysis; growing prostheses for children’s oncology; flexible nailing revisited; ultrasound and the pink pulseless hand; and slipping forearm fractures.

We evaluated results at one year after surgical correction of internal rotation deformities in the shoulders of 270 patients with obstetric brachial plexus palsy. The mean age at surgery was 6.2 years (0.6 to 35). Two techniques were used: open subscapularis elongation and latissimus dorsi to infraspinatus transfer. In addition, open relocation was performed or attempted in all patients with subluxed or dislocated joints. A mixed effects model approach was used to evaluate the effects of surgery on internal and external rotation, abduction, flexion and Mallet score. Independent factors included operative status (pre- or post-operative), gender, age, the condition of the joint, and whether or not transfer was performed. The overall mean improvement in external rotation following surgery was 84.6° (95% confidence interval (CI) 80.2 to 89.1) and the mean Mallet score improved by 4.0 (95% CI 3.7 to 4.2). There was a mean decrease in internal rotation of between 27.6° and 34.4° in the relocated joint groups and 8.6° (95% CI 5.2 to 12.0) in the normal joint group. Abduction and flexion were unchanged following surgery. Adding a latissimus dorsi transfer did not result in greater improvement in the mean external rotation compared with elongation of the subscapularis alone.

Cite this article: Bone Joint J 2013;95-B:1432–8.

The outcome of displaced hips treated by Somerville and Scott’s method was assessed after more than 25 years. A total of 147 patients (191 displaced hips) was reviewed which represented an overall follow-up of 65.6%. The median age at the index operation was two years. During the first five years, 25 (13%) hips showed signs of avascular change.

The late development of valgus angulation of the neck, after ten years, was seen in 69 (36%) hips. Further operations were frequently necessary. Moderate to severe osteoarthritis developed at a young age in 40% of the hips. Total hip replacement or arthrodesis was necessary in 27 (14%) hips at a mean age of 36.5 years. Risk factors identified were high dislocation, open reduction, and age at the original operation. Two groups of patients were compared according to outcome. All the radiographic indices were different between the two groups after ten years, but most were similar before. It takes a generation to establish the prognosis, although some early indicators may help to predict outcome.

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.

We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay.

Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis.

Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.

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.

Hip arthrodesis remains a viable surgical technique in well selected patients, typically the young manual labourer with isolated unilateral hip disease. Despite this, its popularity with patients and surgeons has decreased due to the evolution of hip replacement, and is seldom chosen by young adult patients today. The surgeon is more likely to encounter a patient who requests conversion to total hip replacement (THR). The most common indications are a painful pseudarthrosis, back pain, ipsilateral knee pain or contralateral hip pain. Occasionally the patient will request conversion because of difficulty with activities of daily living, body image and perceived cosmesis. The technique of conversion and a discussion of the results are presented.

Cite this article: Bone Joint J 2013;95-B, Supple A:114–19.

We present the results of treatment of developmental dysplasia of the hip in infancy with the Pavlik harness using a United Kingdom screening programme with ultrasound-guided supervision. Initially, 128 consecutive hips in 77 patients were reviewed over a 40-month period; 123 of these were finally included in the study. The mean age of the patients at the start of treatment was five weeks (1 to 12). All hips were examined clinically and monitored with ultrasound scanning. Failure of treatment was defined as an inability to maintain reduction with the harness. All hips diagnosed with dysplasia or subluxation but not dislocation were managed successfully in the harness. There were 43 dislocated hips, of which 39 were reducible, but six failed treatment in the harness. There were four dislocated but irreducible hips which all failed treatment in the harness. One hip appeared to be successfully treated in the harness but showed persistent radiological dysplasia at 12 and 24 months. Grade 1 avascular necrosis was identified radiologically in three patients at 12 months.

This nationwide prospective study was designed to determine prognostic factors and evaluate the outcome of different treatments of Perthes’ disease.

A total of 28 hospitals in Norway were instructed to report all new cases of Perthes’ disease over a period of five years and 425 patients were reported and followed for five years. Of these, 368 with unilateral disease were included in the present study. The hips were classified radiologically according to a modified two-group Catterall classification and the lateral pillar classification. A total of 358 patients (97%) attended the five-year follow-up, when a modified three-group Stulberg classification was used as a radiological outcome measure. For patients over six years of age at diagnosis and with more than 50% necrosis of the femoral head (152 patients), the surgeons at the different hospitals had chosen one of three methods of treatment: physiotherapy (55 patients), the Scottish Rite abduction orthosis (26), and proximal femoral varus osteotomy (71). Of these hips, 146 (96%) were available for the five-year follow-up.

The strongest predictor of outcome was femoral head involvement of more or less than 50% (odds ratio (OR) = 7.76, 95% confidence interval (CI) 2.82 to 21.37), followed by age at diagnosis (OR = 0.98, 95% CI 0.92 to 0.99) and the lateral pillar classification (OR = 0.62, 95% CI 0.40 to 0.98). In children over six years at diagnosis with more than 50% of femoral head necrosis, proximal femoral varus osteotomy gave a significantly better outcome than orthosis (p = 0.001) or physiotherapy (p = 0.001). There was no significant difference between the physiotherapy and orthosis groups (p = 0.36), and we found no difference in outcome after any of the treatments in children under six years (p = 0.73).

We recommend proximal femoral varus osteotomy in children aged six years and over at the time of diagnosis with hips having more than 50% femoral head necrosis. The abduction orthosis should be abandoned in Perthes’ disease.

Permanent growth arrest of the longer bone is an option in the treatment of minor leg-length discrepancies. The use of a tension band plating technique to produce a temporary epiphysiodesis is appealing as it avoids the need for accurate timing of the procedure in relation to remaining growth. We performed an animal study to establish if control of growth in a long bone is possible with tension band plating. Animals (pigs) were randomised to temporary epiphysiodesis on either the right or left tibia. Implants were removed after ten weeks. Both tibiae were examined using MRI at baseline, and after ten and 15 weeks. The median interphyseal distance was significantly shorter on the treated tibiae after both ten weeks (p = 0.04) and 15 weeks (p = 0.04). On T₁-weighted images the metaphyseal water content was significantly reduced after ten weeks on the treated side (p = 0.04) but returned to values comparable with the untreated side at 15 weeks (p = 0.14). Return of growth was observed in all animals after removal of implants.

Temporary epiphysiodesis can be obtained using tension band plating. The technique is not yet in common clinical practice but might avoid the need for the accurate timing of epiphysiodesis.

Cite this article: Bone Joint J 2013;95-B:855–60.

We present the long-term results of pectoralis major transfer to restore elbow flexion in seven patients (ten procedures). The early results in all the patients were encouraging but with longer follow-up a gradual and progressive flexion deformity was observed with a decrease in the arc of flexion in eight elbows, reaching ≥ 90° in all cases.

The results of pectoralis major transfer deteriorate with time due to the development of a recalcitrant flexion deformity of the elbow. With bilateral involvement we now recommend that the procedure be undertaken on one side only to allow the hand to reach the mouth for feeding, while the opposite side remains in extension for perineal toilet.

Dupuytren’s disease of the hand has only been rarely reported in children and is rarer still in infants. We report a case in a six-month-old infant who required surgery when aged ten months. Histology confirmed the diagnosis of Dupuytren’s disease.

Background

Symptoms of obstetric brachial plexus injury (OBPI) vary widely over the course of time and from individual to individual and can include various degrees of denervation, muscle weakness, contractures, bone deformities and functional limitations. To date, no universally accepted overall framework is available to assess the outcome of patients with OBPI. The objective of this paper is to outline the proposed process for the development of International Classification of Functioning, Disability and Health (ICF) Core Sets for patients with an OBPI.

The use of joint-preserving surgery of the hip has been largely abandoned since the introduction of total hip replacement. However, with the modification of such techniques as pelvic osteotomy, and the introduction of intracapsular procedures such as surgical hip dislocation and arthroscopy, previously unexpected options for the surgical treatment of sequelae of childhood conditions, including developmental dysplasia of the hip, slipped upper femoral epiphysis and Perthes’ disease, have become available. Moreover, femoroacetabular impingement has been identified as a significant aetiological factor in the development of osteoarthritis in many hips previously considered to suffer from primary osteoarthritis.

As mechanical causes of degenerative joint disease are now recognised earlier in the disease process, these techniques may be used to decelerate or even prevent progression to osteoarthritis. We review the recent development of these concepts and the associated surgical techniques.

Cite this article: Bone Joint J 2014;96-B:5–18.

The April 2013 Children’s orthopaedics Roundup³⁶⁰ looks at: improving stress distribution in dysplastic hips; the dangers of fashion; the natural history of supracondylar fractures; ankles that perform well as knees; intra-articular hip pathology at osteotomy; the safe removal of flexible nails; supracondylar fracture fixation; and talipes.

Talipes equinovarus is one of the more common congenital abnormalities affecting the lower limb and can be challenging to manage. This review provides a comprehensive update on idiopathic congenital talipes equinovarus with emphasis on the initial treatment. Current management is moving away from operative towards a more conservative treatment using the Ponseti regime. The long-term results of surgical correction and the recent results of conservative treatment will be discussed.