Aims. 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. Methods. A retrospective review at three paediatric tertiary centres identified 101 cases of tibial OFD in 99 patients. The clinical records, radiological images, and histology were analyzed. Results. Mean age at presentation was 13.5 years (SD 12.4), and mean follow-up was 5.65 years (SD 5.51). At latest review, 62 lesions (61.4%) were in skeletally mature patients. The most common site of the tibial lesion was the anterior (76 lesions, 75.2%) cortex (63 lesions, 62.4%) of the middle third (52 lesions, 51.5%). Pain, swelling, and fracture were common presentations. Overall, 41 lesions (40.6%) presented with radiological deformity (> 10°): apex anterior in 97.6%. A total of 41 lesions (40.6%) were treated conservatively. Anterior bowing < 10° at presentation was found to be related to successful conservative management of OFD (p = 0.013, multivariable logistic regression). Intralesional excision was performed in 43 lesions (42.6%) and a wide excision of the lesion in 19 (18.8%). A high complication rate and surgical burden was found in those that underwent a wide excision regardless of technique employed. There was progression/recurrence in nine lesions (8.9%) but statistical analysis found no predictive factors. No OFD lesion transformed to adamantinoma. Conclusion. This study confirms OFD to be a benign bone condition with low rates of local progression and without malignant transformation. It is important to distinguish OFD from adamantinoma by a histological diagnosis. Focus should be on angular deformity, monitored with full-length tibial radiographs. Surgery is indicated in symptomatic patients and predicted by the severity of the initial angular deformity. Surgery should focus more on the deformity rather than the lesion. Cite this article: Bone Joint J 2022;104-B(2):302–308

Aims

The aim of this study is to evaluate the surgical treatment with the best healing rate for patients with proximal femoral unicameral bone cysts (UBCs) after initial surgery, and to determine which procedure has the lowest adverse event burden during follow-up.

Aims

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.

Aims. We present the clinical and radiographic outcome of 81 children with Gartland type I to III supracondylar humeral fractures at a minimum follow-up of ten years (mean 12.1 years; 10.3 to 16.1) following injury. Patients and Methods. The clinical and functional outcomes are compared with normal age- and gender-matched individuals. The population-based study setting was first identified from the institutional registries; the rate of participation was 76%. Controls were randomly selected from Finnish National Population Registry. Results. According to Flynn's criteria, most fractures (75.3%) resulted in a satisfactory (“good or excellent”) outcome. Satisfactory recovery was achieved in 75.0% of type I fractures treated by closed splinting (p = 0.013). Type II fractures were associated with both satisfactory (57.7%) and unsatisfactory (42.3%) results, regardless of the type of treatment, although the numbers were small in the sub groups. Most type III fractures were treated operatively, and most (76%) had a satisfactory outcome according to Flynn’s criteria (p = 0.015). Compared with none among the normal subjects, flexion of the elbow was reduced by >  10° at long-term follow-up in 20 cases (24.7%, p < 0.001) and 9 (11.1%) had a reduced flexion of > 15° (p = 0.004). In patients who had sustained a type III fracture, the carrying angle was decreased by 35.7% (from 9.8° to 6.3°; p = 0.048). All patients achieved an excellent Mayo Elbow Performance Score (mean 96.4 points). Conclusion. The long-term outcome of extension-type supracondylar humeral fractures is generally good, but not exclusively benign, with the potential for long-term pain and ulnar nerve sensitivity, and a decrease in grip strength and range of movement in type II and type III fractures. Bony remodelling cannot be relied upon to correct any residual deformity. In particular, type II fractures have impaired long-term recovery and justify individual consideration in their treatment. Cite this article: Bone Joint J 2016;98-B:1410–17

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The aims of this study is to report the clinical and radiological outcomes after pre-, central-, and postaxial polydactyly resection in children from a tertiary referral centre.

We describe three sisters in a family who presented with a clinical form of osteomesopycnosis which is a rare, benign osteosclerotic bone disorder limited to the axial skeleton. It must be distinguished from other osteosclerotic conditions which carry a worse prognosis. This is the first report of the condition in a Japanese family, and we believe it to be the first to be identified in Asian races

A five-day-old boy was referred with a soft-tissue mass in his right upper arm. Plain radiographs and ultrasound demonstrated a lesion extending from the axilla to the elbow on the posterolateral aspect of the humerus. Open biopsy confirmed the diagnosis of congenital haemangiopericytoma. After MRI and selective angiography, excision biopsy was carried out, but no adjuvant therapy was administered. At further examination, four years and ten months later, he was noted to have three small nodules at the site of the original tumour. Excision biopsy confirmed this to be a local recurrence, although the lesion was less cellular with no appreciable mitotic activity. Congenital haemangiopericytoma is a rare cause of a soft-tissue mass in children. Most tumours are benign, and recurrence is uncommon. The treatment is controversial, but most centres recommend the use of adjuvant chemotherapy, combined with complete excision. We recommend treatment with doxorubicin. Orthopaedic surgeons should be familiar with this tumour since 30% to 50% of cases occur in the limbs

Between 1990 and 1998 we saw 21 children with primary subacute haematogenous osteomyelitis. Pain, swelling and a limp had been present for two to 12 weeks with little functional impairment. Laboratory tests were non-contributory. The lesions were classified radiologically into metaphyseal, diaphyseal, epiphyseal and vertebral. There were 24 sites involved, with most (20) being in the tibia; 17 lesions were in the diaphysis, five in the metaphysis and two in the epiphysis. The diagnosis was confirmed histologically in all cases. Staphylococcus aureus was cultured in six patients. Healing occurred in all patients after treatment with antibiotics for six weeks and radiological improvement was seen after three to six months. Subacute osteomyelitis develops as a result of increased host resistance and decreased bacterial virulence. The radiological features can mimic various benign or malignant bone tumours and non-pyogenic infections. Histological confirmation is necessary to avoid a delay in diagnosis

We investigated the epidemiology, assessment and outcome of acute atraumatic limp in 243 children under the age of 14 years presenting to a paediatric accident and emergency department (AED) over a period of six months. Data were collected at presentation and medical notes were re-examined after 18 to 21 months. The incidence of limp was 1.8 per thousand. The male:female ratio was 1.7:1 and the median age 4.35 years. Limp was mainly right-sided (54%) and painful (80%); 33.7% of the children had localised pain in the hip. A preceding illness was found in 40%. The main diagnosis was ‘irritable hip’/transient synovitis (39.5%); Perthes’ disease accounted for 2%. Most patients (77%) were managed entirely in the AED. Acute atraumatic limp is a common problem in children presenting to the AED. Most can be safely managed there if guidelines are followed and will have a benign outcome. Further studies are needed to identify the role of preceding illness in the aetiology of acute atraumatic limp

We have reviewed the incidence of bacteriologically or radiologically confirmed acute haematogenous osteomyelitis in children under 13 years of age resident in the area of the Greater Glasgow Health Board between 1990 and 1997. In this period there was a fall of 44% in the incidence of both acute and subacute osteomyelitis, mainly involving the acute form (p = 0.005). This mirrors the decline of just over 50% previously reported in the same population between 1970 and 1990. Using multiple regression analysis a decline in incidence of 0.185 cases per 100 000 population per year was calculated for the 28-year period (p > 0.001). Staphylococcus was the most commonly isolated pathogen (70%). Only 20% of patients required surgery and there was a low rate of complications (10%). In general, patients with a subacute presentation followed a benign course and there were no complications or long-term sequelae in this group. Haematogenous osteomyelitis in children in this area is becoming a rare disease with an annual incidence of 2.9 new cases per 100 000 population per year

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The aims of this study were to describe the course of non-operatively managed, bilateral Perthes’ disease, and to determine specific prognostic factors for the radiographic and clinical outcome.

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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.

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There is increasing evidence that flexible flatfoot (FF) can lead to symptoms and impairment in health-related quality of life. As such we undertook an observational study investigating the aetiology of this condition, to help inform management. The hypothesis was that as well as increased body mass index (BMI) and increased flexibility of the lower limb, an absent anterior subtalar articulation would be associated with a flatter foot posture.

Controversy remains whether the contralateral hip should be fixed in patients presenting with unilateral slipped capital femoral epiphysis (SCFE). This retrospective study compares the outcomes and cost of those patients who had prophylactic fixation with those who did not.

Between January 2000 and December 2010 a total of 50 patients underwent unilateral fixation and 36 had prophylactic fixation of the contralateral hip. There were 54 males and 32 females with a mean age of 12.3 years (9 to 16). The rate of a subsequent slip without prophylactic fixation was 46%. The risk of complications was greater, the generic health measures (Short Form-12 physical (p < 0.001) and mental (p = 0.004) summary scores) were worse. Radiographic cam lesions in patients presenting with unilateral SCFE were only seen in patients who did not have prophylactic fixation. Furthermore, prophylactic fixation of the contralateral hip was found to be a cost-effective procedure, with a cost per quality adjusted life year gained of £1431 at the time of last follow-up.

Prophylactic fixation of the contralateral hip is a cost-effective operation that limits the morbidity from the complications of a further slip, and the diminished functional outcome associated with unilateral fixation.

Cite this article: Bone Joint J 2015;97-B:1428–34.

Over a 15-year prospective period, 201 infants with a clinically unstable hip at neonatal screening were subsequently reviewed in a ‘one stop’ clinic where they were assessed clinically and sonographically. Their mean age was 1.62 weeks (95% confidence interval (CI) 1.35 to 1.89). Clinical neonatal hip screening revealed a sensitivity of 62% (mean, 62.6 95%CI 50.9 to 74.3), specificity of 99.8% (mean, 99.8, 95% CI 99.7 to 99.8) and positive predictive value (PPV) of 24% (mean, 26.2, 95% CI 19.3 to 33.0). Static and dynamic sonography for Graf type IV dysplastic hips had a 15-year sensitivity of 77% (mean, 75.8 95% CI 66.9 to 84.6), specificity of 99.8% (mean, 99.8, 95% CI 99.8 to 99.8) and a PPV of 49% (mean, 55.1, 95% CI 41.6 to 68.5). There were 36 infants with an irreducible dislocation of the hip (0.57 per 1000 live births), including six that failed to resolve with neonatal splintage.

Most clinically unstable hips referred to a specialist clinic are female and stabilise spontaneously. Most irreducible dislocations are not identified from this neonatal instability group. There may be a small subgroup of females with instability of the hip which may be at risk of progression to irreducibility despite early treatment in a Pavlik harness.

A controlled study is required to assess the value of neonatal clinical screening programmes.

Cite this article: Bone Joint J 2015;97-B:265-9.

We undertook a retrospective comparative study of all patients with an unstable slipped capital femoral epiphysis presenting to a single centre between 1998 and 2011. There were 45 patients (46 hips; mean age 12.6 years; 9 to 14); 16 hips underwent intracapsular cuneiform osteotomy and 30 underwent pinning in situ, with varying degrees of serendipitous reduction. No patient in the osteotomy group was lost to follow-up, which was undertaken at a mean of 28 months (11 to 48); four patients in the pinning in situ group were lost to follow-up, which occurred at a mean of 30 months (10 to 50). Avascular necrosis (AVN) occurred in four hips (25%) following osteotomy and in 11 (42%) following pinning in situ. AVN was not seen in five hips for which osteotomy was undertaken > 13 days after presentation. AVN occurred in four of ten (40%) hips undergoing emergency pinning in situ, compared with four of 15 (47%) undergoing non-emergency pinning. The rate of AVN was 67% (four of six) in those undergoing pinning on the second or third day after presentation.

Pinning in situ following complete reduction led to AVN in four out of five cases (80%). In comparison, pinning in situ following incomplete reduction led to AVN in 7 of 21 cases (33%). The rate of development of AVN was significantly higher following pinning in situ with complete reduction than following intracapsular osteotomy (p = 0.048). Complete reduction was more frequent in those treated by emergency pinning and was strongly associated with AVN (p = 0.005).

Non-emergency intracapsular osteotomy may have a protective effect on the epiphyseal vasculature and should be undertaken with a delay of at least two weeks. The place of emergency pinning in situ in these patients needs to be re-evaluated, possibly in favour of an emergency open procedure or delayed intracapsular osteotomy. Non-emergency pinning in situ should be undertaken after a delay of at least five days, with the greatest risk at two and three days after presentation. Intracapsular osteotomy should be undertaken after a delay of at least 14 days. In our experience, closed epiphyseal reduction is harmful.

Cite this article: Bone Joint J 2015;97-B:412–19.

Osteofibrous dysplasia is an unusual developmental condition of childhood, which almost exclusively affects the tibia. It is thought to follow a slowly progressive course and to stabilise after skeletal maturity. The possible link with adamantinoma is controversial and some authors believe that they are part of one histological process.

We retrospectively reviewed 16 patients who were diagnosed as having osteofibrous dysplasia initially or on the final histological examination. Their management was diverse, depending on the severity of symptoms and the extent of the lesion. Definitive (extraperiosteal) surgery was localised ‘shark-bite’ excision for small lesions in five patients. Extensive lesions were treated by segmental excision and fibular autograft in six patients, external fixation and bone transport in four and proximal tibial replacement in one. One patient who had a fibular autograft required further excision and bone transport for recurrence. Six initially underwent curettage and all had recurrence. There were no recurrences after localised extraperiosteal excision or bone transport. There were three confirmed cases of adamantinoma.

The relevant literature is reviewed. We recommend extraperiosteal excision in all cases of osteofibrous dysplasia, with segmental excision and reconstruction in more extensive lesions.

Of 48 consecutive children with Gartland III supracondylar fractures, 11 (23%) had evidence of vascular injury, with an absent radial pulse. The hand was pink and warm in eight and white and cold in the other three patients. They underwent colour-coded duplex scanning (CCDS) and ultrasound velocimetry (UV) to investigate the patency of the brachial artery and arterial blood flow. In seven patients with a pink pulseless hand, CCDS showed a displaced, kinked and spastic brachial artery and a thrombosis was present in the other. In all cases UV showed reduced blood flow in the hand. In three patients with a white pulseless hand, scanning demonstrated a laceration in the brachial artery and/or thrombosis. In all cases, the fracture was reduced under general anaesthesia and fixed with Kirschner wires. Of the seven patients with a pink pulseless hand without thrombosis, the radial pulse returned after reduction in four cases. The remaining three underwent exploration, along with the patients with laceration in the brachial artery and/or thrombosis.

We believe that the traditional strategy of watchful waiting in children in whom the radial pulse remains absent in spite of good peripheral perfusion should be revisited. Vascular investigation using these non-invasive techniques that are quick and reliable is recommended in the management of these patients.

Cite this article: Bone Joint J 2013;95-B:694–98.

The outcome of one-stage bilateral open reduction through a medial approach for the treatment of developmental dysplasia of the hip in children under 18 months was studied in 23 children, 18 girls and five boys. Their mean age at operation was 10.1 months (6 to 17) and the mean follow-up was 5.4 years (3 to 8).

Acceptable clinical and radiological results were achieved in 44 (95.7%) and 43 (93.5%) of 46 hips, respectively. Excellent results were significantly evident in patients younger than 12 months, those who did not require acetabuloplasty, those whose ossific nucleus had appeared, and in those who did not develop avascular necrosis.

One-stage bilateral medial open reduction avoids the need for separate procedures on the hips and has the advantages of accelerated management and shorter immobilisation and rehabilitation than staged operations.

Between 2005 and 2010 ten consecutive children with high-energy open diaphyseal tibial fractures were treated by early reduction and application of a programmable circular external fixator. They were all male with a mean age of 11.5 years (5.2 to 15.4), and they were followed for a mean of 34.5 months (6 to 77). Full weight-bearing was allowed immediately post-operatively. The mean time from application to removal of the frame was 16 weeks (12 to 21). The mean deformity following removal of the frame was 0.15° (0° to 1.5°) of coronal angulation, 0.2° (0° to 2°) sagittal angulation, 1.1 mm (0 to 10) coronal translation, and 0.5 mm (0 to 2) sagittal translation. All patients achieved consolidated bony union and satisfactory wound healing. There were no cases of delayed or nonunion, compartment syndrome or neurovascular injury. Four patients had a mild superficial pin site infection; all settled with a single course of oral antibiotics. No patient had a deep infection or re-fracture following removal of the frame. The time to union was comparable with, or better than, other published methods of stabilisation for these injuries. The stable fixator configuration not only facilitates management of the accompanying soft-tissue injury but enables anatomical post-injury alignment, which is important in view of the limited remodelling potential of the tibia in children aged > ten years. Where appropriate expertise exists, we recommend this technique for the management of high-energy open tibial fractures in children.