A retrospective study was performed in 18 patients with achondroplasia, who underwent bilateral humeral lengthening between 2001 and 2013, using monorail external fixators. The mean age was ten years (six to 15) and the mean follow-up was 40 months (12 to 104). . The mean disabilities of the arm, shoulder and hand (DASH) score fell from 32.3 (20 to 40) pre-operatively to 9.4 (6 to 14) post-operatively (p = 0.037). A mean lengthening of 60% (40% to 95%) was required to reach the goal of independent perineal hygiene. One patient developed early consolidation, and fractures occurred in the regenerate bone of four humeri in three patients. There were three transient radial nerve palsies. Humeral lengthening increases the independence of people with achondroplasia and is not just a cosmetic procedure. Cite this article: Bone Joint J 2015;97-B:1577–81

Aims. We aimed to evaluate the health-related quality of life (HRQoL) in children with supracondylar humeral fractures (SCHFs), who were treated following the recommendations of the Paediatric Comprehensive AO Classification, and to assess if HRQoL was associated with AO fracture classification, or fixation with a lateral external fixator compared with closed reduction and percutaneous pinning (CRPP). Methods. We were able to follow-up on 775 patients (395 girls, 380 boys) who sustained a SCHF from 2004 to 2017. Patients completed questionnaires including the Quick Disabilities of the Arm, Shoulder and Hand questionnaire (QuickDASH; primary outcome), and the Pediatric Quality of Life Inventory (PedsQL). Results. An AO type I SCHF was most frequent (327 children; type II: 143; type III: 150; type IV: 155 children). All children with type I fractures were treated nonoperatively. Two children with a type II fracture, 136 with a type III fracture, and 141 children with a type IV fracture underwent CRPP. In the remaining 27 children with type III or IV fractures, a lateral external fixator was necessary for closed reduction. There were no open reductions. After a mean follow-up of 6.3 years (SD 3.7), patients with a type I fracture had a mean QuickDASH of 2.0 (SD 5.2), at a scale of 0 to 100, with lower values representing better HRQoL (type II: 2.8 (SD 10.7); type III: 3.3 (SD 8.0); type IV: 1.8 (SD 4.6)). The mean function score of the PedsQL ranged from 97.4 (SD 8.0) for type I to 96.1 (SD 9.1) for type III fractures, at a scale of 0 to 100, with higher values representing better HRQoL. Conclusion. In this cohort of 775 patients in whom nonoperative treatment was chosen for AO type I and II fractures and CRPP or a lateral external fixator was used in AO type III and IV fractures, there was equally excellent mid- and long-term HRQoL when assessed by the QuickDASH and PedsQL. These results indicate that the treatment protocol followed in this study is unambiguous, avoids open reductions, and is associated with excellent treatment outcomes. Cite this article: Bone Joint J 2020;102-B(6):755–765

Aims. The treatment of tibial aplasia is controversial. Amputation represents the gold standard with good functional results, but is frequently refused by the families. In these patients, treatment with reconstructive limb salvage can be considered. Due to the complexity of the deformity, this remains challenging and should be staged. The present study evaluated the role of femoro-pedal distraction using a circular external fixator in reconstructive treatment of tibial aplasia. The purpose of femoro-pedal distraction is to realign the limb and achieve soft tissue lengthening to allow subsequent reconstructive surgery. Methods. This was a retrospective study involving ten patients (12 limbs) with tibial aplasia, who underwent staged reconstruction. During the first operation a circular hexapod external fixator was applied and femoro-pedal distraction was undertaken over several months. Subsequent surgery included reconstruction of the knee joint and alignment of the foot. Results. The mean follow-up was 7.1 years (2 to 10). The mean age of the patients at the time of the application of the fixator was 2.3 years (1.1 to 5.0). The mean time under distraction was 139.7 days (81.0 to 177.0). A mean fibular distalization of 38.7 mm (14.0 to 67.0) was achieved. Pin infections occurred in four limbs (33.3%) and osteitis in one. A femoral fracture occurred in one patient. Premature removal of the frame was not required in any patient. Sufficient realignment of the leg as well as soft tissue lengthening was achieved in all patients, allowing subsequent reconstruction. All patients were able to mobilize fully weight bearing after reconstruction. Functional outcome was limited in all limbs, and five patients (50.0%) required additional reconstructive operations. Conclusion. Regarding the functional results in the treatment of tibial aplasia, amputation remains superior to limb salvage. The latter procedure should only be performed in patients whose parents refuse amputation. Femoro-pedal distraction efficiently prepares the limb by realigning the leg and soft tissue lengthening. Minor complications are frequent, but usually do not hinder the continuation of distraction. Even though a fully weight-bearing limb is achieved, the functional outcome of reconstructive treatment remains limited. Recurrent deformities frequently occur and may require further operations. Cite this article: Bone Joint J 2020;102-B(9):1248–1255

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

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

Deformity of the forearm due to growth disturbance of the ulna occurs in a number of conditions such as ulnar deficiency, multiple exostoses, and neurofibromatosis. We report a previously unrecognised form, caused by focal cortical indentation. We have treated five children with this condition, three girls and two boys; the mean age at presentation was 5 years (2 to 8). The deformity was first recognised about the age of two years, and progressed gradually. The radiological findings were the same in all cases. The focal cortical indentation was seen at the distal end of the ulna with anteromedial bowing and dysplasia. The radial head was dislocated posterolaterally. In one patient the histological findings at the site of indentation were of a fold of tissue resembling periosteum, which interfered with enchondral ossification. Treatment by ulnar lengthening using an external fixator and osteotomy which corrected both the ulnar deformity and reduced the dislocated radial head in two cases gave the best results

We performed limb lengthening and correction of deformity of nine long bones of the lower limb in six children (mean age, 14.7 years) with osteogenesis imperfecta (OI). All had femoral lengthening and three also had ipsilateral tibial lengthening. Angular deformities were corrected simultaneously. Five limb segments were treated using a monolateral external fixator and four with the Ilizarov frame. In three children, lengthening was done over previously inserted femoral intramedullary rods. The mean lengthening achieved was 6.26 cm (mean healing index, 33.25 days/cm). Significant complications included one deep infection, one fracture of the femur and one anterior angulation deformity of the tibia. The abnormal bone of OI tolerated the external fixators throughout the period of lengthening without any episodes of migration of wires or pins through the soft bone. The regenerate bone formed within the time which is normally expected in limb-lengthening procedures performed for other conditions. We conclude that despite the abnormal bone characteristics, distraction osteogenesis to correct limb-length discrepancy and angular deformity can be performed safely in children with OI

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

We reviewed retrospectively 45 patients (46 procedures) with bladder exstrophy treated by bilateral oblique pelvic osteotomy in conjunction with genitourinary repair. The operative technique and post-operative management with or without external fixation are described. A total of 21 patients attended a special follow-up clinic and 24 were interviewed by telephone. The mean follow-up time was 57 months (24 to 108). Of the 45 patients, 42 reported no pain or functional disability, although six had a waddling gait and two had marked external rotation of the hip. Complications included three cases of infection and loosening of the external fixator requiring early removal with no deleterious effect. Mid-line closure failed in one neonate managed in plaster. This patient underwent a successful revision procedure several months later using repeat osteotomies and external fixation. The percentage pubic approximation was measured on anteroposterior radiographs pre-operatively, post-operatively and at final follow-up. The mean approximation was 37% (12% to 76%). It varied markedly with age and was better when external fixation was used. The wide range reflects the inability of the anterior segment to develop naturally in spite of close approximation at operation. We conclude that bilateral oblique pelvic osteotomy with or without external fixation is useful in the management of difficult primary closure in bladder exstrophy, failed primary closure and secondary reconstruction

Aims

Given the possible radiation damage and inaccuracy of radiological investigations, particularly in children, ultrasound and superb microvascular imaging (SMI) may offer alternative methods of evaluating new bone formation when limb lengthening is undertaken in paediatric patients. The aim of this study was to assess the use of ultrasound combined with SMI in monitoring new bone formation during limb lengthening in children.

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Children with spinal dysraphism can develop various musculoskeletal deformities, necessitating a range of orthopaedic interventions, causing significant morbidity, and making considerable demands on resources. This systematic review aimed to identify what outcome measures have been reported in the literature for children with spinal dysraphism who undergo orthopaedic interventions involving the lower limbs.

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.

Two consecutive cases of chronic dislocation of the head of the radius after missed Bado type-I Monteggia lesions are presented. Reduction was successfully achieved in both patients after ulnar corticotomy, gradual lengthening and angulation of the ulna using an external fixator. Open reduction or reconstruction of the radio-ulnar capitellar joint was not undertaken. The age at injury was seven years in the older and two years in the younger patient. The time from injury to treatment was five years in the older and three months in the younger child. At follow-up, nine years after completion of treatment in the older and eight months in the younger patient, both show satisfactory movement, function of the forearm and reduction of the head of the radius. This technique may be considered in missed Monteggia lesions before open procedures on the radio-ulnar capitellar joint are undertaken

We review the results of a modified quadricepsplasty in five children who developed stiffness of the knee after femoral lengthening for congenital short femur using an Ilizarov external fixator which spanned the knee. All had a full range of movement of the knee before lengthening was undertaken. Unifocal lengthening was carried out in the distal metaphysiodiaphyseal region of the distal femur with a mean gain of 6.5 cm. The mean percentage lengthening was 24%. At the end of one year after removal of the Ilizarov frame and despite intensive physiotherapy all patients had stiffness. Physiotherapy was continued after the quadricepsplasty and, at the latest follow-up (mean 27 months), the mean active flexion was 102° (80 to 130). The gain in movement ranged from 50° to 100°. One patient had a superficial wound infection which settled after a course of oral antibiotics. None developed an increased extension lag after surgery and all were very satisfied with the results. Quadricepsplasty is a useful procedure for stiffness of the knee after femoral lengthening which has not responded to physiotherapy

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.

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The aim of this study was to evaluate the epidemiology and treatment of Perthes’ disease of the hip.

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The aim of this study was to describe the epidemiology of closed isolated fractures of the femoral shaft in children, and to compare the treatment and length of stay (LOS) between major trauma centres (MTCs) and trauma units (TUs) in England.

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The aim of this study was to report the clinical, functional and radiological outcomes of children and adolescents with tibial fractures treated using the Ilizarov method.

Extensive limb lengthening may be indicated in achondroplastic patients who wish to achieve a height within the normal range for their population. However, increasing the magnitude of lengthening is associated with further complications particularly adjacent joint stiffness and fractures. We studied the relationship between the magnitude of femoral lengthening and callus pattern, adjacent joint stiffness and fracture of the regenerate bone in 40 femoral lengthenings in 20 achondroplastic patients. They were divided into two groups; group A had lengthening of less than 50% and group B of more than 50% of their initial femoral length. The patterns of radiological callus formation were classified according to shape, type and features. The incidence of callus features, knee stiffness and regenerate bone fracture were analysed in the two groups. Group B was associated with an increased incidence of concave, lateral and central callus shapes, adjacent joint and stiffness and fracture. Statistically, the incidence of stiffness in adjacent joints and regenerate bone fracture was significantly associated with the magnitude of lengthening.

We suggest that careful radiological assessment of the patterns of callus formation is a useful method for the evaluation and monitoring of regenerate bone.

We report our experience of the use of callus distraction with a monolateral fixator for the treatment of acquired radial club-hand deformity after osteomyelitis. Between 1994 and 2004, 13 patients with a mean age of eight years (4 to 15) were treated by callus distraction with a monolateral fixator after a preliminary period of at least four weeks in a corrective short-arm cast.

All patients achieved bony union and were satisfied with the functional and cosmetic outcome. There were no major complications, but three patients required cancellous bone grafting at the docking site for delayed union. Local treatment and oral antibiotics were required for pin-site infection in six patients. There were no deep infection or recurrence of osteomyelitis.