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. 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.Aims
Methods
Type BI rotationplasty is currently indicated for children with
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
Eight-plates are used to correct varus-valgus deformity (VVD) or limb-length discrepancy (LLD) in children and adolescents. It was reported that these implants might create a bony deformity within the knee joint by change of the roof angle (RA) after epiphysiodesis of the proximal tibia following a radiological assessment limited to anteroposterior (AP) radiographs. The aim of this study was to analyze the RA, complemented with lateral knee radiographs, with focus on the tibial slope (TS) and the degree of deformity correction. A retrospective, single-centre study was conducted. The treatment group (n = 64 knees in 44 patients) was subclassified according to the implant location in two groups: 1) medial hemiepiphysiodesis; and 2) lateral hemiepiphysiodesis. A third control group consisted of 25 untreated knees. The limb axes and RA were measured on long standing AP leg radiographs. Lateral radiographs of 40 knees were available for TS analysis. The mean age of the patients was 10.6 years (4 to 15) in the treatment group and 8.4 years (4 to 14) in the control group. Implants were removed after a mean 1.2 years (0.5 to 3).Aims
Methods
To describe and analyze the mid-term functional outcomes of a large series of patients who underwent the Hoffer procedure for brachial plexus birth palsy (BPBP). All patients who underwent the Hoffer procedure with minimum two-year follow-up were retrospectively reviewed. Active shoulder range of movement (ROM), aggregate modified Mallet classification scores, Hospital for Sick Children Active Movement Scale (AMS) scores, and/or Toronto Test Scores were used to assess functional outcomes. Subgroup analysis based on age and level of injury was performed. Risk factors for subsequent humeral derotational osteotomy and other complications were also assessed. A total of 107 patients, average age 3.9 years (1.6 to 13) and 59% female, were included in the study with mean 68 months (24 to 194) follow-up.Aims
Methods
Using 90% of final height as a benchmark, we sought to develop
a quick, quantitative and reproducible method of estimating skeletal
maturity based on topographical changes in the distal femoral physis. Serial radiographs of the distal femoral physis three years prior
to, during, and two years following the chronological age associated
with 90% of final height were analyzed in 81 healthy children. The
distance from the tip of the central peak of the distal femoral
physis to a line drawn across the physis was normalized to the physeal width.Aims
Patients and Methods
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. 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).Aims
Patients and Methods
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. Between 2003 and June 2014, 50 children (51 prostheses) had a
non-invasive growing prosthesis implanted for a primary bone sarcoma.
The minimum follow-up was 24 months for those who survived. Their
mean age was 10.4 years (6 to 14). The incidence of complications
and further surgery was documented.Aims
Patients and Methods
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.
Septicaemia resulting from meningococcal infection is a devastating illness affecting children. Those who survive can develop late orthopaedic sequelae from growth plate arrests, with resultant complex deformities. Our aim in this study was to review the case histories of a series of patients with late orthopaedic sequelae, all treated by the senior author (CFB). We also describe a treatment strategy to address the multiple deformities that may occur in these patients. Between 1997 and 2009, ten patients (seven girls and three boys) were treated for late orthopaedic sequelae following meningococcal septicaemia. All had involvement of the lower limbs, and one also had involvement of the upper limbs. Each patient had a median of three operations (one to nine). Methods of treatment included a combination of angular deformity correction, limb lengthening and epiphysiodesis. All patients were skeletally mature at the final follow-up. One patient with bilateral below-knee amputations had satisfactory correction of her right amputation stump deformity, and has complete ablation of both her proximal tibial growth plates. In eight patients length discrepancy in the lower limb was corrected to within 1 cm, with normalisation of the mechanical axis of the lower limb. Meningococcal septicaemia can lead to late orthopaedic sequelae due to growth plate arrests. Central growth plate arrests lead to limb-length discrepancy and the need for lengthening procedures, and peripheral growth plate arrests lead to angular deformities requiring corrective osteotomies and ablation of the damaged physis. In addition, limb amputations may be necessary and there may be altered growth of the stump requiring further surgery. Long-term follow-up of these patients is essential to recognise and treat any recurrence of deformity.
The crucial differentiation between septic arthritis and transient synovitis of the hip in children can be difficult. In 1999, Kocher et al introduced four clinical predictors which were highly predictive (99.6%) of septic arthritis. These included fever (temperature ≥ 38.5°C), inability to bear weight, white blood-cell count >
12.0 × 109 cells/L and ESR ≥ 40 mm/hr; CRP ≥ 20 mg/L was later added as a fifth predictor. We retrospectively evaluated these predictors to differentiate septic arthritis from transient synovitis of the hip in children over a four-year period in a primary referral general hospital. When all five were positive, the predicted probability of septic arthritis in this study was only 59.9%, with fever being the best predictor. When applied to low-prevalence diseases, even highly specific tests yield a high number of false positives and the predictive value is thereby diminished. Clinical predictors should be applied with caution when assessing a child with an irritable hip, and a high index of suspicion, and close observation of patients at risk should be maintained.
There were 47 patients with congenital muscular torticollis who underwent operative release. After a mean follow-up of 74 months (60 to 90), they were divided into two groups, one aged one to four years (group 1) and the other aged five to 16 years (group 2). The outcomes were assessed by evaluating the following parameters: deficits of lateral flexion and rotation, craniofacial asymmetry, surgical scarring, residual contracture, subjective evaluation and degree of head tilt. The craniofacial asymmetry, residual contracture, subjective evaluation and overall scores were similar in both groups. However, group 2 showed superior results to group 1 in terms of the deficits of movement, surgical scarring and degree of head tilt. It is recommended that operative treatment for congenital muscular torticollis is postponed until the patient can comply successfully with post-operative bracing and an exercise programme.
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.
The Ilizarov method for leg lengthening was used for cosmetic reasons in 54 patients with constitutional short stature. A mean lengthening of 7 cm with a low rate of complications produced an excellent or good outcome in all the patients, including improvement in psychological disturbances related to short stature. Those who undergo the procedure must be highly motivated, fully informed and understand the technique and possible complications. We suggest that the Ilizarov method for cosmetic limb lengthening is a technique without major complications. However, it requires careful follow-up, and the involvement of orthopaedic surgeons who are familiar with use of the circular frame and are experienced in limb lengthening and correction of deformity for pathological conditions.
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.
