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. Congenital pseudarthrosis of the tibia (CPT) has traditionally been a difficult condition to treat, with high complication rates, including nonunion, refractures, malalignment, and leg length discrepancy. Surgical approaches to treatment of CPT include intramedullary rodding, external fixation, combined intramedullary rodding and external fixation, vascularized fibular graft, and most recently cross-union. The current study aims to compare the outcomes and complication rates of cross-union versus other surgical approaches as an index surgery for the management of CPT. Our hypothesis was that a good index surgery for CPT achieves union and minimizes complications such as refractures and limb length discrepancy. Methods. A multicentre study was conducted involving two institutions in Singapore and China. All patients with CPT who were surgically managed between January 2009 and December 2021 were included. The patients were divided based on their index surgery. Group 1 included patients who underwent excision of hamartoma, cross-union of the tibia and fibula, autogenic iliac bone grafting, and internal fixation for their index surgery. Group 2 included patients who underwent all other surgical procedures for their index surgery, including excision of hamartoma, intramedullary rodding, and/or external fixation, without cross-union of the tibia and fibula. Comparisons of the rates of union, refracture, limb length discrepancy, reoperations, and other complications were performed between the two groups. Results. A total of 36 patients were included in the study. Group 1 comprised 13 patients, while Group 2 comprised 23 patients. The mean age at index surgery was four years (1 to 13). The mean duration of follow-up was 4.85 years (1.75 to 14). All patients in Group 1 achieved bony union at a mean of three months (1.5 to 4), but ten of 23 patients in Group 2 had nonunion of the pseudarthrosis (p = 0.006). None of the patients in Group 1 had a refracture, while seven of 13 patients who achieved bony union in Group 2 suffered a refracture (p = 0.005). None of the patients in Group 1 had a limb length discrepancy of more than 2 cm, while ten of 23 patients in Group 2 have a limb length discrepancy of more than 2 cm (p = 0.006). In Group 1, four of 13 patients had a complication, while 16 of 23 patients in Group 2 had a complication (p = 0.004). Excluding removal of implants, four of 13 patients in Group 1 had to undergo additional surgery, while 18 of 23 patients in Group 2 had to undergo additional surgery following the index surgery (p = 0.011). Conclusion. A good index surgery of excision of hamartoma, cross-union of the tibia and fibula, autogenic iliac bone grafting, and internal fixation for CPT achieves union and minimizes complications such as refractures, limb length discrepancy, and need for additional surgeries

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

We have evaluated the clinical outcomes of simple excision, ulnar lengthening and the Sauvé-Kapandji procedure in the treatment of deformities of the forearm in patients with multiple hereditary osteochondromas. The medical records of 29 patients (33 forearms) were reviewed; 22 patients (22 forearms) underwent simple excision (four with ulnar lengthening) and seven the Sauvé-Kapandji procedure. Simple excision increased the mean supination of the forearm from 63.2° to 75.0° (p = 0.049). Ulnar lengthening did not significantly affect the clinical outcome. The Sauvé-Kapandji technique improved the mean pronation from 33.6° to 55.0° (p = 0.047) and supination from 70.0° to 81.4° (p = 0.045). Simple excision may improve the range of movement of the forearm but will not halt the progression of disease, particularly in younger patients. No discernable clinical or radiological improvement was noted with ulnar lengthening. The Sauvé-Kapandji procedure combined with simple excision of osteochondromas can improve stability of the wrist, movement of the forearm and the radiological appearance

Aims

Multiple secondary surgical procedures of the shoulder, such as soft-tissue releases, tendon transfers, and osteotomies, are described in brachial plexus birth palsy (BPBP) patients. The long-term functional outcomes of these procedures described in the literature are inconclusive. We aimed to analyze the literature looking for a consensus on treatment options.

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

Aim. To review the natural history of upper limb osteochondromas and assess their functional effect. Materials. We performed a retrospective casenote review of a consecutive patient cohort presenting between 1997–2012 with upper limb osteochondromas. Indications for surgical intervention were noted and considered to be cosmetic, functional (including pain relief) and ‘prophylactic’ in terms of deformity prevention. All patients were invited to complete questionnaires for the PODCI, DASH, OSS and MHS scores. Results. We identified 102 patients (62 male: 40 female; mean age = 13.3 years; range 3–31 years). 84 patients had multiple exostoses whilst 18 had a solitary lesion. 52 patients had shoulder girdle involvement (scapula, clavicle and proximal humerus), 51 forearm (Masada I (n=31) Masada II (n=9) Masada III (n=11)), and 38 hand involvement. 46/102 patients had concurrent lower limb lesions. 56 operative procedures were performed primarily for functional benefit. Shoulder girdle procedures (n=21) improved pressure related pain, scapular pseudowinging/dyskinesia and cuff impingement. Forearm procedures (n=35) were performed for functional and prophylactic reasons and involved excision with ulnar lengthening and radial deformity correction (n=15, Masada I), realignment osteotomy or radial head excision for subluxation (n=7, Masada II) and excision with internal fixation of concomitant osteotomy (n=13, Masada I/III). No hand surgery was performed. Radial head dislocations are associated with large ulnar lesions causing shortening > 0.15 total ulnar length. Osteochondromas of the upper limb are generally well tolerated: functional effects were most commonly present in lesions involving the forearm but significant patient benefit was noted following shoulder girdle procedures. The scoring systems used failed to discriminate well between the various procedures used and the perceived benefit. Conclusion:. Patient outcomes are related to surgical indications. Currently available PROMs are either inappropriate Qs (DASH) and/or non-validated (OSS, MHS) and/or non-specific (PODCI*) only 8 parameters for the upper extremity. Better-validated measures may be required. Level of evidence: IV

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

Aims

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.

The results of further soft-tissue release of 79 feet in 60 children with recurrent idiopathic congenital talipes equinovarus were evaluated. The mean age of the children at the time of re-operation was 5.8 years (15 months to 14.5 years). Soft-tissue release was performed in all 79 feet and combined with distal calcaneal excision in 52 feet. The mean follow-up was 12 years (4 to 32). At the latest follow-up the result was excellent or good in 61 feet (77%) according to the Ghanem and Seringe scoring system. The results was considered as fair in 14 feet (18%), all of whom had functional problems and eight had anatomical abnormalities. Four feet (5%) were graded as poor on both functional and anatomical grounds. The results were independent of the age at which revision was undertaken

The outcome of 56 children (61 shoulders) treated surgically at the Rizzoli Institute between April 1975 and June 2010 for congenital elevation of the scapula is reported. There were 31 girls and 25 boys with a mean age at surgery of 6.4 years (2 to 15). The deformity involved the right shoulder in 20 cases, the left in 31 and was bilateral in five. The degree of the deformity was graded clinically and radiologically according to the classifications of Cavendish and Rigault, respectively. All patients underwent a modified Green procedure combined, in selected cases, with resection of the superomedial portion of the scapula and excision of any omovertebral connection. After a mean follow-up of 10.9 years (1 to 29.3), there was cosmetic improvement by at least one Cavendish grade in 54 shoulders (88.5%). The mean abduction of the shoulder improved from 92° (50° to 155°) to 112° (90° to 170°) and the mean flexion improved from 121° (80° to 160°) to 155° (120° to 175°). The unsatisfactory cosmetic result in seven shoulders was due to coexistent scoliosis in two cases and insufficient reduction of the scapular elevation in the other five. An incomplete upper brachial plexus palsy occurred post-operatively in three patients but resolved within seven months. We suggest that a modified Green procedure combined with resection of the superomedial portion of the scapula provides good cosmetic and functional results in patients with Sprengel’s shoulder

The management of chronic osteomyelitis requires the excision of necrotic and infected material followed by the prolonged administration of antibiotics. Sequestrectomy may be required before an involucrum has formed, resulting in a longitudinal bone defect. This can be difficult to fill. Vascularised grafts are complicated by a high rate of recurrent infection and thrombosis. We have managed defects of long bones in children after sequestrectomy by the use of non-vascularised fibular grafts harvested subperiosteally and held by an intramedullary Kirschner wire. Eight children underwent this procedure. In six the tibia was involved and in one each the humerus and radius. One patient was lost to follow-up. Six grafts united at both ends within 12 weeks. The seventh developed an infected nonunion distally which united after further debridement. One patient required a further sequestrectomy which did not compromise union. We have found this to be a straightforward technique with reliable results and were able to salvage the limb in all the seven patients who were reviewed

Aims

Osteonecrosis (ON) can cause considerable morbidity in young people who undergo treatment for acute lymphoblastic leukaemia (ALL). The aims of this study were to determine the operations undertaken for ON in this population in the UK, along with the timing of these operations and any sequential procedures that are used in different joints. We also explored the outcomes of those patients treated by core decompression (CD), and compared this with conservative management, in both the pre- or post-collapse stages of ON.

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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.

Aims

The purpose of this study was to compare the prevalence of hip displacement and dislocation in a total population of children with cerebral palsy (CP) in Scotland before and after the initiation of a hip surveillance programme.

Aim. Debate remains over the optimal treatment for severe unstable SCFE. AVN is the principle problem; current thinking suggests this can be minimized by emergent reduction and fixation within 24 hours. If emergent treatment is not possible, open osteotomy with a variable delay of 10–21 days has been advocated. We present our experience of delayed intracapsular cuneiform osteotomy (ICO). Methods. SCFE cases were identified through ICD-10 coding and theatre records. Unstable slips were identified and reviewed retrospectively. When ICO was performed, the hip was accessed via anterior approach without hip dislocation. A cuneiform shortening osteotomy of the neck with physeal excision was undertaken. The epiphysis was carefully reduced and stabilized with a single screw. Results. 46 unstable slips were treated during the study period. 16 hips underwent ICO, while the remaining cases were treated by closed pinning in situ (PIS) without capsulotomy. The rate of AVN following ICO in 16 hips was 25% in comparison to the PIS group, in which 40% developed AVN. AVN was not seen in those cases treated with ICO with a delay of at least 14 days. In contrast, AVN occurred regardless of the timing of PIS, including a rate of 33% in those treated emergently. Sub analysis of the PIS group revealed that AVN occurred more frequently (66.7% vs. 33.3%) when complete epiphyseal reduction occurred during patient positioning. While these results are not statistically significant, they are striking. Patients who developed AVN had a poor outcome in both groups, with 75% requiring further surgical intervention. There were 2 cases of heterotrophic ossification and meralgia paraesthetica respectively. Hardware failure occurred once. Conclusion. Timing and method of treatment for unstable SCFE remains controversial. This series suggests delayed intracapsular cuneiform osteotomy may result in a lower rate of AVN than closed reduction and pinning within 24 hours

We studied the natural history of Perthes’ disease in 62 children in whom the onset of symptoms was in adolescence. Three patterns of disease were noted, namely, late-onset pattern, segmental collapse, or destructive with failure of revascularisation. In the late-onset pattern, the disease followed the sequence of healing seen in younger children, but adequate epiphyseal remodelling did not occur. Consequently, the femoral head was never spherical after revascularisation. With segmental collapse, early and irreversible collapse of part of the epiphysis occurred with gross deformation of the femoral head. The destructive pattern was characterised by a failure of revascularisation and repair of the avascular epiphysis. The radiological outcome was poor in all three patterns. The poorest clinical results were found in the destructive type which was frequently associated with incapacitating pain requiring arthrodesis or excision arthroplasty within three years of onset of the disease

Aims

Extendible endoprostheses have been available for more than 30 years and have become more sophisticated with time. The latest generation is ‘non-invasive’ and can be lengthened with an external magnetic force. Early results have shown a worryingly high rate of complications such as infection. This study investigates the incidence of complications and the need for further surgery in a cohort of patients with a non-invasive growing endoprosthesis.

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 aims of this study were to report functional outcomes of salvage procedures for patients with cerebral palsy (CP) who have chronic dislocation of the hip using validated scoring systems, and to compare the results of three surgical techniques.

We reviewed 37 patients retrospectively. The mean age at the time of surgery was 12.2 years (8 to 22) and the mean follow-up was 56 months (24 to 114). Patients were divided into three groups: 14 who underwent proximal femoral resection arthroplasty (PFRA group 1), ten who underwent subtrochanteric valgus osteotomy (SVO group 2), and 13 who underwent subtrochanteric valgus osteotomy with resection of the femoral head (SVO with FHR group 3). All patients were evaluated using the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) and the Pediatric Quality of Life Inventory (PedsQL).

Significant improvements occurred in most CPCHILD and PedsQL subsection scores following surgery in all patients, without significant differences between the groups. There were 12 post-operative complications. Less severe complications were seen in group 1 than in groups 2 and 3.

Salvage surgery appears to provide pain relief in patients with CP who have painful chronic dislocation of the hip. The three salvage procedures produced similar results, however, we recommend the use of PFRA as the complications are less severe.

Take home message: Salvage surgery can be of benefit to patients with CP with chronic painful hip dislocation, but should be limited to selected patients considering complications.

Cite this article: Bone Joint J 2016;98-B:137–43.