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

Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve. This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations. We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings, histology and upper-limb function. In these children the carpal tunnel syndrome differs from that seen in adults. Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common. At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons. Most patients had some definite nerve constriction with a thickened epineurium. Functional improvement was seen after early decompression, with some benefit from simultaneous tendon release. Regular physiotherapy helped to maintain increased hand movement. We describe our assessment protocol, the physiotherapy and operative regime and the standard functional review which helps to maximise function in the hands and upper limbs of these children

Morbid obesity and its association with obstructive sleep apnoea syndrome have been increasingly recognised in children. Orthopaedic surgeons are often the primary medical contact for older children with tibia vara, which has long been associated with obesity, but are unfamiliar with the evaluation and treatment of sleep apnoea in children. We reviewed all children with tibia vara treated surgically at one of our institutions over a period of five years. Thirty-seven patients were identified; 18 were nine years of age or older and 13 of these (72%) had morbid obesity and a history of snoring. Eleven children were diagnosed as having sleep apnoea on polysomnography. The incidence of this syndrome in the 18 children aged nine years or older with tibia vara, was 61%. All these patients required pre-operative non-invasive positive-pressure ventilation; tonsillectomy and adenoidectomy were necessary in five (45%). No peri-operative complications related to the airway occurred. There is a high incidence of sleep apnoea in morbidly obese patients with tibia vara. These patients should be screened for snoring and, if present, should be further evaluated for sleep apnoea before corrective surgery is undertaken

We describe ten patients with Turner’s syndrome (karyotype 45, XO) who had leg lengthening for short stature. A high incidence of postoperative complications was encountered and many patients required intramedullary fixation as a salvage procedure. We discuss the reasons for this and highlight the differences between our findings and those of a similar series recently reported. In view of the considerable difficulties encountered, we do not recommend leg lengthening in Turner’s syndrome

In 17 patients (eleven males, six females) with Morquio-Brailsford syndrome (mucopolysaccharidosis IV) we have used onlay femoral and tibial autografts placed posteriorly and secured to the laminae of C1 and C2 to obtain satisfactory occipito-C1/C2 posterior fusion. They were immobilised postoperatively in a halo-plaster body jacket for four months. The age at operation varied between three and 28 years. Those with myelopathic symptoms of recent onset made some recovery, but severely myelopathic patients showed little or no recovery. We advise prophylactic occipitocervical fusion in these patients since the cartilaginous dens is not strong enough to ensure atlanto-axial mechanical stability

The association between vitamin-D-resistant rickets and linear sebaceous naevus syndrome is extremely rare. Only eight cases have been described in the English literature and in none were the skeletal aspects addressed. We present three new cases and describe the musculoskeletal features. The details and outcome of surgery for correction of the deformities are discussed. The disturbances of metabolism of vitamin D and the effects of pharmacological treatment are also described

Aims. To assess if congenital foot deformity is a risk factor for developmental dysplasia of the hip (DDH). Methods. Between 1996 and 2012, 60,844 children were born in Sør-Trøndelag county in Norway. In this cohort study, children with risk factors for DDH were examined using ultrasound. The risk factors evaluated were clinical hip instability, breech delivery, a family history of DDH, a foot deformity, and some syndromes. As the aim of the study was to examine the risk for DDH and foot deformity in the general population, children with syndromes were excluded. The information has been prospectively registered and retrospectively analyzed. Results. Overall, 494 children (0.8%) had DDH, and 1,132 (1.9%) a foot deformity. Of the children with a foot deformity, 49 (4.3%) also demonstrated DDH. There was a statistically significant increased association between DDH and foot deformity (p < 0.001). The risk of DDH was highest for talipes calcaneovalgus (6.1%) and club foot (3.5%), whereas metatarsus adductus (1.5%) had a marginal increased risk of DDH. Conclusion. Compared with the general population, children with a congenital foot deformity had a significantly increased risk for DDH and therefore we regard foot deformity as a true risk factor for DDH. Cite this article: Bone Joint J 2020;102-B(11):1582–1586

The aim of this retrospective multicentre study was to report the continued occurrence of compartment syndrome secondary to paediatric supracondylar humeral fractures in the period 1995 to 2005. The inclusion criteria were children with a closed, low-energy supracondylar fracture with no associated fractures or vascular compromise, who subsequently developed compartment syndrome. There were 11 patients (seven girls and four boys) identified from eight hospitals in three countries. Ten patients with severe elbow swelling documented at presentation had a mean delay before surgery of 22 hours (6 to 64). One patient without severe swelling documented at presentation suffered arterial entrapment following reduction, with a subsequent compartment syndrome requiring fasciotomy 25 hours after the index procedure. This series is noteworthy, as all patients had low-energy injuries and presented with an intact radial pulse. Significant swelling at presentation and delay in fracture reduction may be important warning signs for the development of a compartment syndrome in children with supracondylar fractures of the humerus

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The aim of this retrospective cohort study was to assess and investigate the safety and efficacy of using a distal tibial osteotomy compared to proximal osteotomy for limb lengthening in children.

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Radiological residual acetabular dysplasia (RAD) has been reported in up to 30% of children who had successful brace treatment of infant developmental dysplasia of the hip (DDH). Predicting those who will resolve and those who may need corrective surgery is important to optimize follow-up protocols. In this study we have aimed to identify the prevalence and predictors of RAD at two years and five years post-bracing.

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Temporary hemiepiphysiodesis (HED) is applied to children and adolescents to correct angular deformities (ADs) in long bones through guided growth. Traditional Blount staples or two-hole plates are mainly used for this indication. Despite precise surgical techniques and attentive postoperative follow-up, implant-associated complications are frequently described. To address these pitfalls, a flexible staple was developed to combine the advantages of the established implants. This study provides the first results of guided growth using the new implant and compares these with the established two-hole plates and Blount staples.

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Brace treatment is the cornerstone of managing developmental dysplasia of the hip (DDH), yet there is a lack of evidence-based treatment protocols, which results in wide variations in practice. To resolve this, we have developed a comprehensive nonoperative treatment protocol conforming to published consensus principles, with well-defined a priori criteria for inclusion and successful treatment.

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The aim of this study was to gain an agreement on the management of idiopathic congenital talipes equinovarus (CTEV) up to walking age in order to provide a benchmark for practitioners and guide consistent, high-quality care for children with CTEV.

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

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Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD.

Children with congenital vertical talus (CVT) have been treated with extensive soft-tissue releases, with a high rate of complications. Recently, reverse Ponseti-type casting followed by percutaneous reduction and fixation has been described, with excellent results in separate cohorts of children with CVT, of either idiopathic or teratological aetiology. There are currently no studies that compare the outcome in these two types. We present a prospective cohort of 13 children (21 feet) with CVT of both idiopathic and teratological aetiology, in which this technique has been used. Clinical, radiological and parent-reported outcomes were obtained at a mean follow-up of 36 months (8 to 57). Six children (nine feet) had associated neuromuscular conditions or syndromes; the condition was idiopathic in seven children (12 feet). Initial correction was achieved in all children, with significant improvement in all radiological parameters. Recurrence was seen in ten feet. Modification of the technique to include limited capsulotomy at the initial operation may reduce the risk of recurrence. The reverse Ponseti-type technique is effective in the initial correction of CVT of both idiopathic and teratological aetiology. Recurrence is a problem in both these groups, with higher rates than first reported in the original paper. However, these rates are less than those reported after open surgical release. Cite this article: Bone Joint J 2014;96-B:274–8

In five children, six forearms with a fixed pronation deformity secondary to congenital radioulnar synostosis were treated by a derotation osteotomy of the distal radius and the midshaft of the ulna. There were three boys and two girls with a mean age of 4.9 years (3.5 to 8.25) who were followed up for a mean of 29 months (18 to 43). The position of the forearm was improved from a mean pronation deformity of 68° (40° to 80°) to a pre-planned position of 10° of supination in all cases. Bony union was achieved by 6.3 weeks with no loss of correction. There was one major complication involving a distal radial osteotomy which required exploration for a possible compartment syndrome

Our aim was to review the efficacy of the wound vacuum-assisted closure (VAC) system in the treatment of deep infection after extensive instrumentation and fusion for spinal deformity in children and adolescents. A total of 14 patients with early deep spinal infection were treated using this technique. Of these, 12 had neuromuscular or syndromic problems. Clinical and laboratory data were reviewed. The mean follow-up was 44 months (24 to 72). All wounds healed. Two patients required plastic surgery to speed up the process. In no patient was the hardware removed and there was no loss of correction or recurrent infection. We believe that the wound VAC system is a useful tool in the armamentarium of the spinal surgeon dealing with patients susceptible to wound infections, especially those with neuromuscular diseases. It allows for the retention of the instrumentation and the maintenance of spinal correction. It is reliable and easy to use

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Open tibial fractures are limb-threatening injuries. While limb loss is rare in children, deep infection and nonunion rates of up to 15% and 8% are reported, respectively. We manage these injuries in a similar manner to those in adults, with a combined orthoplastic approach, often involving the use of vascularised free flaps. We report the orthopaedic and plastic surgical outcomes of a consecutive series of patients over a five-year period, which includes the largest cohort of free flaps for trauma in children to date.