Slipped capital femoral epiphysis (SCFE) is uncommon in India and we routinely look for associated metabolic or endocrine abnormalities. In this study we investigated a possible association between vitamin D deficiency and SCFE. All children presenting with SCFE during the study period had their 25-hydroxyvitamin D levels measured as part of an overall metabolic, renal and endocrine status evaluation, which included measurement of body mass index (BMI). Vitamin D status was compared with age-, gender- and habitat-matched controls with acute trauma or sepsis presenting to our emergency department. A total of 15 children (12 boys and three girls) with a mean age of 13 years (. sd. 1.81; 10 to 16) presented for treatment for SCFE during a two-year period beginning in January 2010. Renal and thyroid function was within the normal range in all cases. The mean BMI was 24.9 kg/m. 2. (17.0 to 33.8), which was significantly higher than that of the controls (p = 0.006). There was a statistically significant difference between the mean values of 25-hydroxyvitamin D in the children with SCFE and the controls (11.78 ng/ml (. sd. 5.4) versus 27.06 ng/ml (. sd. 5.53), respectively; p < 0.001). We concluded that, along with high BMI, there is a significant association of vitamin D deficiency and SCFE in India. Cite this article: Bone Joint J 2013;95-B:851–4

Heritable thrombophilic disorders have been proposed as one of the causes for Legg-Calvé-Perthes disease. A total of 62 patients diagnosed with this disease between 1988 and 1997 and 50 controls were screened for thrombophilia. The incidence and relationship of thrombophilia to the severity of the disease were evaluated. One patient and none of the controls had protein S deficiency. One of the control group and one of the patients had protein C deficiency with the latter child also having a combined deficiency with a mutant factor V gene. The number of children with a mutant factor V gene, protein C deficiency, who were homozygous for the C 677T polymorphism of methylenetetra-hydrofolate reductase or were heterozygous for mutant G20210A prothrombin did not differ statistically in the study and the control groups. No patient had antithrombin deficiency or positive lupus anticoagulant. We found no correlation between thrombophilia and the extent of the disease. The most common risk factors for arteriovenous thromboembolism showed no statistical significance in our patients compared with the control group or with the general population. These data do not confirm an aetiological role for thrombophilia in Perthes’ disease

Aims

A national screening programme has existed in the UK for the diagnosis of developmental dysplasia of the hip (DDH) since 1969. However, every aspect of screening and treatment remains controversial. Screening programmes throughout the world vary enormously, and in the UK there is significant variation in screening practice and treatment pathways. We report the results of an attempt by the British Society for Children’s Orthopaedic Surgery (BSCOS) to identify a nationwide consensus for the management of DDH in order to unify treatment and suggest an approach for screening.

Aims

The aim of this retrospective cohort study was to assess and investigate the safety and efficacy of using a distal tibial osteotomy compared to proximal osteotomy for limb lengthening in children.

Recent reports have suggested an association between Perthes’ disease and an underlying thrombophilic or hypofibrinolytic tendency. In Northern Ireland there is a high incidence of Perthes’ disease (11.7 per 100 000 or 1 in 607 children) in a stable paediatric population. We reviewed 139 children with Perthes’ disease and compared them with a control group of 220 aged- and gender-matched healthy primary schoolchildren with similar racial and ethnic backgrounds. There were no significant deficiencies of antithrombotic factors protein C, protein S, antithrombin III or resistance to activated protein C. A total of 53 (38.1%) of the children with Perthes’ disease had a prolonged activated partial thromboplastin time (> 38) compared with 13 (5.9%) of the control group (p < 0.001). Our findings have shown that using standard assays, thrombophilia secondary to antithrombotic factor deficiency or resistance to activated protein does not appear to be an aetiological factor for Perthes’ disease. The cause of the prolonged activated partial thromboplastin time, usually associated with a clotting factor deficiency, is under further investigation

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

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

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.

After open reduction for developmental dysplasia of the hip (DDH), a pelvic or femoral osteotomy may be required to maintain a stable concentric reduction. We report the clinical and radiological outcome in 82 children (95 hips) with DDH treated by open reduction through an anterior approach in which a test of stability was used to assess the need for a concomitant osteotomy. The mean age at the time of surgery was 28 months (9 to 79) and at the latest follow-up, 17 years (12 to 25). All patients have been followed up until closure of the triradiate cartilage with a mean period of 15 years (8 to 23). At the time of open reduction before closure of the joint capsule, the position of maximum stability was assessed. A hip which required flexion with abduction for stability was considered to need an innominate osteotomy. If only internal rotation and abduction were required, an upper femoral derotational and varus osteotomy was carried out. For a ‘double-diameter’ acetabulum with anterolateral deficiency, a Pemberton-type osteotomy was used. A hip which was stable in the neutral position required no concomitant osteotomy. Overall, 86% of the patients have had a satisfactory radiological outcome (Severin groups I and II) with an incidence of 7% of secondary procedures for persistent dysplasia including one hip which redislocated. The results were better (p = 0.04) in children under the age of two years. Increased leg length on the affected side was associated with poor acetabular development and recurrence of joint dysplasia (p = 0.01). The incidence of postoperative avascular necrosis was 7%. In a further 18%, premature physeal arrest was noted during the adolescent growth spurt (Kalamchi-MacEwen types II and III). Both of these complications were also associated with recurrence of joint dysplasia (p = 0.01). Studies with a shorter follow-up are therefore likely to underestimate the proportion of poor radiological results

Aims

The aim of this study was to utilize a national paediatric inpatient database to determine whether obesity influences the operative management and inpatient outcomes of paediatric limb fractures.

Aims

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.

Aims

We wished to examine the effectiveness of tibial lengthening using a two ring Ilizarov frame in skeletally immature patients. This is a potentially biomechanically unstable construct which risks the loss of axial control.

We describe the technique and results of medial submuscular plating of the femur in paediatric patients and discuss its indications and limitations. Specifically, the technique is used as part of a plate-after-lengthening strategy, where the period of external fixation is reduced and the plate introduced by avoiding direct contact with the lateral entry wounds of the external fixator pins. The technique emphasises that vastus medialis is interposed between the plate and the vascular structures.

A total of 16 patients (11 male and five female, mean age 9.6 years (5 to 17)), had medial submuscular plating of the femur. All underwent distraction osteogenesis of the femur with a mean lengthening of 4.99 cm (3.2 to 12) prior to plating. All patients achieved consolidation of the regenerate without deformity. The mean follow-up was 10.5 months (7 to 15) after plating for those with plates still in situ, and 16.3 months (1 to 39) for those who subsequently had their plates removed. None developed a deep infection. In two patients a proximal screw fractured without loss of alignment; one patient sustained a traumatic fracture six months after removal of the plate.

Placing the plate on the medial side is advantageous when the external fixator is present on the lateral side, and is biomechanically optimal in the presence of a femoral defect. We conclude that medial femoral submuscular plating is a useful technique for specific indications and can be performed safely with a prior understanding of the regional anatomy.

Cite this article: Bone Joint J 2014;96-B:137–42.

We undertook a retrospective analysis of 306 procedures on 233 patients, with a mean age of 12 years (1 to 21), in order to evaluate the use of somatosensory evoked potential (SSEP) monitoring for the early detection of nerve compromise during external fixation procedures for limb lengthening and correction of deformity. Significant SSEP changes were identified during 58 procedures (19%). In 32 instances (10.5%) the changes were transient, and resolved once the surgical cause had been removed. The remaining 26 (8.5%) were analysed in two groups, depending on whether or not corrective action had been performed in response to critical changes in the SSEP recordings. In 16 cases in which no corrective action was taken, 13 (81.2%, 4.2% overall) developed a post-operative neurological deficit, six of which were permanent and seven temporary, persisting for five to 18 months. In the ten procedures in which corrective action was taken, four patients (40%, 1.3% overall) had a temporary (one to eight months) post-operative neuropathy and six had no deficit.

After appropriate intervention in response to SSEP changes, the incidence and severity of neurological deficits were significantly reduced, with no cases of permanent neuropathy. SSEP monitoring showed 100% sensitivity and 91% specificity for the detection of nerve injury during external fixation. It is an excellent diagnostic technique for identifying nerve lesions when they are still highly reversible.

We carried out a morphometric analysis of the acetabulum following Dega osteotomy in patients with cerebral palsy using three-dimensional CT. We assessed 17 acetabula in 12 patients with instability of the hip. A Dega osteotomy and varus derotation femoral osteotomy were performed in all 17 hips. Three-dimensional CT scans were taken before and approximately one year after operation. Acetabular cover was evaluated using anterosuperior, superolateral and posterosuperior acetabular indices, and the change in the acetabular volume was calculated. Inter- and intra-observer reliability was assessed using the intraclass correlation coefficient. After the osteotomy, the anterosuperior, superolateral and posterosuperior cover had improved significantly towards the value seen in a control group. The mean acetabular volume increased by 68%.

Several authors have suggested that the final five weeks of gestation are a critical period for the development of the hip. In order to test the hypothesis that gestational age at birth may influence the development of the hip joint, we analysed the sonographic findings in 1992 hips (in 996 term newborns) with no risk factor for developmental dysplasia of the hip. The 996 infants were born at a mean gestational age of 39 weeks (37 to 41).

The mean bony roof angle (α), cartilage roof angle (β) and the distribution of the type of hip were compared between the 37th, 38th, 39th, 40th and 41st birth week groups. There was a significant difference in the distribution of type of hip between the different birth week groups (p < 0.001), but no significant difference between the α angles of all groups (p = 0.32). There was no correlation between birth week and roof angle (p = 0.407 and p = 0.291, respectively) and no significant correlation between birth weight and roof angle (p = 0.735 and p = 0.132, respectively).

The maturity of the infant hip, as assessed sonographically, does not appear to be affected by gestational age, and the fetal development of the acetabular roof appears to plateau from 37 weeks.

Cite this article: Bone Joint J 2013;95-B:266–70.

The role of heritable thrombophilic risk factors in the pathogenesis of the Perthes’ disease is controversial. The clinical and radiological findings of Perthes’ disease may be indistinguishable from those of Gaucher’s disease, and the most common Jewish N370S Gaucher mutation is threefold greater in patients with Perthes’ disease. Familial osteonecrosis of the femoral head is associated with variant mutations of collagen type II (COL2A1 mutations). We therefore studied the potential role of genetic thrombophilia and the Gaucher and COL2A1 mutations in children with Perthes’ disease.

Genomic DNA of 119 children with radiologically-confirmed Perthes’ disease diagnosed between 1986 and 2005 was analysed for the thrombophilic polymorphisms Factor V Leiden, 677T-MTHFR and FIIG20210A. The results were compared with those of a group of 276 children without Perthes’ disease. DNA was also analysed for the Gaucher mutations N370S, G insertion (84GG), L444P, Intron 2 (IVS2+1G> A) and R496H. Enzymic assays confirmed the Gaucher disease status. Collagen (COL2A1) mutations of the 12q13 gene were also analysed. The prevalence of thrombophilic markers was similar among the 119 patients with Perthes’ disease and the 276 control subjects. The prevalence of the Gaucher mutation was consistent with Israeli population carriership data and did not confirm an earlier-claimed association with Perthes’ disease. All 199 patients were negative for the studied COL2A1 mutations.

We found no genetic association between Perthes’ disease and either Gaucher’s disease or COL2A1 mutations or increased genetic thrombophilia among our patients compared with the control group. A systematic review of case-control studies suggested that there was a positive association between Perthes’ disease and Factor V Leiden. The impact of this association upon the disease, although not consistent across the studies, remains unclear.

At our institution surgical correction of symptomatic flat foot deformities in children has been guided by a paradigm in which radiographs and pedobarography are used in the assessment of outcome following treatment. Retrospective review of children with symptomatic flat feet who had undergone surgical correction was performed to assess the outcome and establish the relationship between the static alignment and the dynamic loading of the foot.

A total of 17 children (21 feet) were assessed before and after correction of soft-tissue contractures and lateral column lengthening, using standardised radiological and pedobarographic techniques for which normative data were available.

We found significantly improved static segmental alignment of the foot, significantly improved mediolateral dimension foot loading, and worsened fore-aft foot loading, following surgical treatment. Only four significant associations were found between radiological measures of static segmental alignment and dynamic loading of the foot.

Weakness of the plantar flexors of the ankle was a common post-operative finding. Surgeons should be judicious in the magnitude of lengthening of the plantar flexors that is undertaken and use techniques that minimise subsequent weakening of this muscle group.

Cite this article: Bone Joint J 2013;95-B:706–13.

We describe three cases of infantile tibia vara resulting from an atraumatic slip of the proximal tibial epiphysis upon the metaphysis. There appears to be an association between this condition and severe obesity. Radiologically, the condition is characterised by a dome-shaped metaphysis, an open growth plate and disruption of the continuity between the lateral borders of the epiphysis and metaphysis, with inferomedial translation of the proximal tibial epiphysis. All patients were treated by realignment of the proximal tibia by distraction osteogenesis with an external circulator fixator, and it is suggested that this is the optimal method for correction of this complex deformity. There are differences in the radiological features and management between conventional infantile Blount’s disease and this ‘slipped upper tibial epiphysis’ variant.

We reviewed the long-term radiological outcome, complications and revision operations in 19 children with quadriplegic cerebral palsy and hip dysplasia who underwent combined peri-iliac osteotomy and femoral varus derotation osteotomy. They had a mean age of 7.5 years (1.6 to 10.9) and comprised 22 hip dislocations and subluxations. We also studied the outcome for the contralateral hip. At a mean follow-up of 11.7 years (10 to 15.1) the Melbourne cerebral palsy (CP) hip classification was grade 2 in 16 hips, grade 3 in five, and grade 5 in one. There were five complications seen in four hips (21%, four patients), including one dislocation, one subluxation, one coxa vara with adduction deformity, one subtrochanteric fracture and one infection. A recurrent soft-tissue contracture occurred in five hips and ten required revision surgery.

In pre-adolescent children with quadriplegic cerebral palsy good long-term outcomes can be achieved after reconstruction of the hip; regular follow-up is required.

Cite this article: Bone Joint J 2013;95-B:259–65.