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.

Aims

We aimed to address the question on whether there is a place for shoulder stabilization surgery in patients who had voluntary posterior instability starting in childhood and adolescence, and later becoming involuntary and uncontrollable.

Aims

Anterior cruciate ligament (ACL) surgery in children and the adolescent population has increased steadily over recent years. We used a national database to look at trends in ACL reconstruction and rates of serious complications, growth disturbance, and revision surgery, over 20 years.

Aims. Preserving growth following limb-salvage surgery of the upper limb in children remains a challenge. Vascularized autografts may provide rapid biological incorporation with the potential for growth and longevity. In this study, we aimed to describe the outcomes following proximal humeral reconstruction with a vascularized fibular epiphyseal transfer in children with a primary sarcoma of bone. We also aimed to quantify the hypertrophy of the graft and the annual growth, and to determine the functional outcomes of the neoglenofibular joint. Patients and Methods. We retrospectively analyzed 11 patients who underwent this procedure for a primary bone tumour of the proximal humerus between 2004 and 2015. Six had Ewing’s sarcoma and five had osteosarcoma. Their mean age at the time of surgery was five years (two to eight). The mean follow-up was 5.2 years (1 to 12.2). Results. The overall survival at five and ten years was 91% (confidence interval (CI) 95% 75% to 100%). At the time of the final review, ten patients were alive. One with local recurrence and metastasis died one-year post-operatively. Complications included seven fractures, four transient nerve palsies, and two patients developed avascular necrosis of the graft. All the fractures presented within the first postoperative year and united with conservative management. One patient had two further operations for a slipped fibular epiphysis of the autograft, and a hemi-epiphysiodesis for lateral tibial physeal arrest. Hypertrophy and axial growth were evident in nine patients who did not have avascular necrosis of the graft. The mean hypertrophy index was 65% (55% to 82%), and the mean growth was 4.6 mm per annum (2.4 to 7.6) in these nine grafts. At final follow-up, the mean modified functional Musculoskeletal Tumour Society score was 77% (63% to 83%) and the mean Toronto Extremity Salvage Score (TESS) was 84% (65% to 94%). Conclusion. Vascularized fibular epiphyseal transfer preserves function and growth in young children following excision of the proximal humerus for a malignant bone tumour. Function compares favourably to other limb-salvage procedures in children. Longer term analysis is required to determine if this technique proves to be durable into adulthood. Cite this article: Bone Joint J 2018;100-B:535–41

Aims. To compare the structural durability of hydroxyapatite-tricalcium phosphate (HATCP) to autologous iliac crest bone graft in calcaneal lengthening osteotomy (CLO) for pes planovalgus in childhood. Patients and Methods. We present the interim results of ten patients (HATCP, n = 6 and autograft, n = 5) with a mean age of 11.5 years (8.2 to 14.2) from a randomised controlled non-inferiority trial with six months follow-up. The primary outcome was the stability of the osteotomy as measured by radiostereometric analysis. A non-inferiority margin of ≤ 2 mm osteotomy compression was set. Results. At six months the data showed that the osteotomy had been compressed by a mean 1.97 mm (99.8% confidence interval -1.65 to 5.60) more in the HATCP group than in the autograft group. Migration of the CLO grafted with HATCP stabilised at six months rather than at six weeks with autograft. Conclusion. This is the first randomised trial to compare the efficacy of HATCP graft with autograft in terms of stability of CLO in children. Because of problems with the HATCP the trial was stopped. We do not recommend HATCP graft in its current structure for use in unfixed CLOs. Cite this article: Bone Joint J 2016;98-B:1554–62

Congenital pseudarthrosis of the tibia (CPT) is a rare but well recognised condition. Obtaining union of the pseudarthrosis in these children is often difficult and may require several surgical procedures. The treatment has changed significantly since the review by Hardinge in 1972, but controversies continue as to the best form of surgical treatment. This paper reviews these controversies.

Cite this article: Bone Joint J 2013;95-B:1027–34.

The use of recombinant human bone morphogenetic protein-2 (rhBMP-2) for the treatment of congenital pseudarthrosis of the tibia has been investigated in only one previous study, with promising results. The aim of this study was to determine whether rhBMP-2 might improve the outcome of this disorder. We reviewed the medical records of five patients with a mean age of 7.4 years (2.3 to 21) with congenital pseudarthrosis of the tibia who had been treated with rhBMP-2 and intramedullary rodding. Ilizarov external fixation was also used in four of these patients. Radiological union of the pseudarthrosis was evident in all of them at a mean of 3.5 months (3.2 to 4) post-operatively. The Ilizarov device was removed after a mean of 4.2 months (3.0 to 5.3). These results indicate that treatment of congenital pseudarthrosis of the tibia using rhBMP-2 in combination with intramedullary stabilisation and Ilizarov external fixation may improve the initial rate of union and reduce the time to union.

Further studies with more patients and longer follow-up are necessary to determine whether this surgial procedure may significantly enhance the outcome of congenital pseudarthrosis of the tibia, considering the refracture rate (two of five patients) in this small case series.

Nonunion of the humerus with bone loss and shortening due to osteomyelitis is rare but difficult to treat. We describe our experience with a callus distraction technique using a monolateral external fixator for the treatment of this condition.

Between October 1994 and January 2004, 11 patients were treated. There were seven males and four females, with a mean age of 14 years (10 to 17). The mean bone loss was 1.9 cm (1 to 2.7) and the mean length discrepancy in the upper limb was 5.6 cm (3.5 to 8.0).

The mean follow-up was for 106 months (54 to 166). The mean external fixation index was 34.8 days/cm (29.8 to 40.5). The mean lengthening was 9.5 cm (5.5 to 13.4). There were seven excellent results, three good and one poor. There were nine excellent functional results and two good.

The treatment of humeral nonunion with bone loss and shortening due to osteomyelitis by callus distraction is a safe and effective means of improving function and cosmesis.

We investigated patterns of refracture and their risk factors in patients with congenital pseudarthrosis of the tibia after Ilizarov osteosynthesis. We studied 43 cases in 23 patients. Temporal and spatial patterns of refracture and refracture-free survival were analysed in each case. The refracture-free rate of cumulative survival was 47% at five years and did not change thereafter. Refracture occurred at the previous pseudarthrosis in 16 of 19 cases of refracture. The risk of refracture was significantly higher when osteosynthesis was performed below the age of four years, when the tibial cross-sectional area was narrow, and when associated with persistent fibular pseudarthrosis. Refracture occurs frequently after successful osteosynthesis in these patients. Delaying osteosynthesis, maximising the tibial cross-sectional area and stabilising the fibula may reduce the risk of refracture.

We present a retrospective study of 27 patients treated by callus distraction using a unilateral external fixator of our own design for nonunion with bone loss and shortening of the femur caused by suppurative osteomyelitis. The unilateral external fixator was used either alone or in combination with an intramedullary nail. The mean age of the patients was 13.6 years (8 to 18). The fixator was used alone in 13 patients and with an intramedullary nail in 14. The bone results at a mean follow-up of 88 months (37 to 144) were excellent in 16 patients and good in 11.

The functional results were excellent in 18 patients and good in nine. However, four patients still had draining sinuses at the latest follow-up. A residual deformity greater than 7° was present in seven femora, but this did not adversely affect function or require further treatment.

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

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