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

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.

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

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

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 histologically. Hips with tuberculosis were excluded. The children were reviewed in a dedicated clinic at a mean follow-up of 8.1 months (3 to 18). There were 40 hips with sepsis in 38 patients. Two patients were lost to follow-up. Nine (24%) had multi-focal sepsis. Overall, 13 hips (34%) had a full and uncomplicated clinical and radiological recovery and 25 (66%) had complications. All patients treated by arthrotomy and appropriate antibiotics within five days of the onset of symptoms had an uncomplicated recovery. Initial misdiagnosis was associated with a delay to arthrotomy. However, ‘deprivation’, consultation with a traditional healer, maternal educational attainment and distance to a primary health-care facility were not associated with delay to arthrotomy. The early correct diagnosis of this condition, common in the developing world, remains a significant factor in improving the clinical outcome

The anatomy and development of the growing acetabulum are not clearly understood. We dissected and studied histologically two acetabula from the pelvis of a three-month-old infant. Relative rates of growth at the different growth plates were assessed by comparing the height of the proliferative layer with that of the hypertrophic layer. The three bones which form the acetabulum are surrounded by growth plates on all sides except medially. These face towards the centre of the triradiate cartilage, the limbs of the triradiate cartilage and the articular surface and each may be divided into four distinct areas according to the orientation of its cell columns which reflect the direction of growth. Growth was particularly rapid at the ischial growth plates directed towards the centre and the articular cartilage, and on both sides of the anterior limb of the triradiate cartilage. These findings may explain the mechanism by which the acetabulum changes orientation and inclination with growth

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 histologically in all cases. Staphylococcus aureus was cultured in six patients. Healing occurred in all patients after treatment with antibiotics for six weeks and radiological improvement was seen after three to six months. Subacute osteomyelitis develops as a result of increased host resistance and decreased bacterial virulence. The radiological features can mimic various benign or malignant bone tumours and non-pyogenic infections. Histological confirmation is necessary to avoid a delay in diagnosis

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, histology and upper-limb function. In these children the carpal tunnel syndrome differs from that seen in adults. Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common. At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons. Most patients had some definite nerve constriction with a thickened epineurium. Functional improvement was seen after early decompression, with some benefit from simultaneous tendon release. Regular physiotherapy helped to maintain increased hand movement. We describe our assessment protocol, the physiotherapy and operative regime and the standard functional review which helps to maximise function in the hands and upper limbs of these children

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.

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.

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 (Nf1^+/−) mice and control mice. Fractures were open and featured periosteal stripping. All mice received 10 μg rhBMP-2 delivered in a carboxymethylcellulose carrier around the fracture as an anabolic stimulus. Bisphosphonate-treated mice also received five doses of 0.02 mg/kg zoledronic acid given by intraperitoneal injection.

When only rhBMP but no zoledronic acid was used to promote repair, 75% of fractures in Nf1^+/− mice remained ununited at three weeks compared with 7% of controls (p < 0.001). Systemic post-operative administration of zoledronic acid halved the rate of ununited fractures to 37.5% (p < 0.07).

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: Bone Joint J 2013;95-B:699–705.

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.