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
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. This multicentre retrospective study was conducted in 20 tertiary paediatric hospitals in France, Belgium, and Switzerland, and included patients aged < 16 years admitted for UBC treatment in the proximal femur from January 1995 to December 2017. UBCs were divided into seven groups based on the index treatment, which included elastic stable intramedullary nail (ESIN) insertion with or without percutaneous injection or grafting, percutaneous injection alone, curettage and grafting alone, and insertion of other orthopaedic hardware with or without curettage.Aims
Methods
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
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
Between September 2004 and December 2005 we carried out a prospective study of all cases of sepsis of the hip in childhood at a South African regional hospital with a large local population, and which also took referrals from nine rural hospitals. The clinical, radiological, ultrasound and bacteriological features were assessed. All the hips were drained by arthrotomy and the diagnosis was confirmed microbiologically and
The anatomy and development of the growing acetabulum are not clearly understood. We dissected and studied
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
Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve. This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations. We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings,
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
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. 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.Aims
Patients and Methods
Congenital pseudarthrosis of the tibia is an uncommon manifestation of neurofibromatosis type 1 (NF1), but one that remains difficult to treat due to anabolic deficiency and catabolic excess. Bone grafting and more recently recombinant human bone morphogenetic proteins (rhBMPs) have been identified as pro-anabolic stimuli with the potential to improve the outcome after surgery. As an additional pharmaceutical intervention, we describe the combined use of rhBMP-2 and the bisphosphonate zoledronic acid in a mouse model of NF1-deficient fracture repair. Fractures were generated in the distal tibiae of neurofibromatosis type 1-deficient ( When only rhBMP but no zoledronic acid was used to promote repair, 75% of fractures in These data support the concept that preventing bone loss in combination with anabolic stimulation may improve the outcome following surgical treatment for children with congenital pseudarthoris of the tibia and NF1.
We investigated the predictive value of intra-operative
neurophysiological investigations in obstetric brachial plexus injuries.
Between January 2005 and June 2011 a total of 32 infants of 206
referred to our unit underwent exploration of the plexus, including
neurolysis. The findings from intra-operative electromyography,
sensory evoked potentials across the lesion and gross muscular response
to stimulation were evaluated. A total of 22 infants underwent neurolysis
alone and ten had microsurgical reconstruction. Of the former, one
was lost to follow-up, one had glenoplasty and three had subsequent
nerve reconstructions. Of the remaining 17 infants with neurolysis,
13 (76%) achieved a modified Mallet score >
13 at a mean age of
3.5 years (0.75 to 6.25). Subluxation or dislocation of the shoulder
is a major confounding factor. The positive predictive value and
sensitivity of the intra-operative EMG for C5 were 100% and 85.7%,
respectively, in infants without concurrent shoulder pathology.
The positive and negative predictive values, sensitivity and specificity
of the three investigations combined were 77%, 100%, 100% and 57%, respectively. In all, 20 infants underwent neurolysis alone for C6 and three
had reconstruction. All of the former and one of the latter achieved
biceps function of Raimondi grade 5. The positive and negative predictive
values, sensitivity and specificity of electromyography for C6 were
65%, 71%, 87% and 42%, respectively. Our method is effective in evaluating the prognosis of C5 lesion.
Neurolysis is preferred for C6 lesions. Cite this article:
We retrospectively studied the possibility that direct trauma to the biceps muscle might be the cause of poor elbow flexion and supination in 18 consecutive children with birth lesions of the brachial plexus who had delayed or impaired biceps recovery despite neurophysiological evidence of reinnervation. All had good shoulder and hand function at three months of age. Eight recovered a strong biceps after six months, but nine required a pectoralis minor to biceps transfer to augment elbow flexion and supination. One had a delayed but good recovery of the biceps after microsurgical reconstruction of the plexus. All had a clinical ‘pseudotumour’ in the biceps muscle, which was biopsied during pectoralis minor transfer in two patients and showed rupture and degeneration of muscle fibres with a fibro-fatty infiltrate, suggesting previous muscle trauma. Direct muscle trauma is an uncommon but important cause of delayed or impaired biceps recovery after brachial plexus birth injuries. Surgery to reinnervate the biceps muscle will not work if substantial muscle damage is present when a suitable muscle transfer should be considered.
The outcome of displaced hips treated by Somerville and Scott’s method was assessed after more than 25 years. A total of 147 patients (191 displaced hips) was reviewed which represented an overall follow-up of 65.6%. The median age at the index operation was two years. During the first five years, 25 (13%) hips showed signs of avascular change. The late development of valgus angulation of the neck, after ten years, was seen in 69 (36%) hips. Further operations were frequently necessary. Moderate to severe osteoarthritis developed at a young age in 40% of the hips. Total hip replacement or arthrodesis was necessary in 27 (14%) hips at a mean age of 36.5 years. Risk factors identified were high dislocation, open reduction, and age at the original operation. Two groups of patients were compared according to outcome. All the radiographic indices were different between the two groups after ten years, but most were similar before. It takes a generation to establish the prognosis, although some early indicators may help to predict outcome.