Aims

The Ponseti method is the benchmark treatment for the correction of clubfoot. The primary rate of correction is very high, but outcome further down the treatment pathway is less predictable. Several methods of assessing severity at presentation have been reported. Classification later in the course of treatment is more challenging. This systematic review considers the outcome of the Ponseti method in terms of relapse and determines how clubfoot is assessed at presentation, correction, and relapse.

Our aim was to evaluate the effect of adding inhibitory casting to the treatment of young children with cerebral palsy who received injections of botulinum neurotoxin A (BoNT-A) to gastrocnemius for equinus gait. Of the 20 patients in the series, 11 in group A had inhibitory casts applied on the day of the first set of BoNT-A injections and nine in group B did not have casting. Both groups received another BoNT-A injection four months later. The patients were followed for eight months and examined at five intervals.

Both groups showed significant improvement in gait parameters and function (p < 0.0001) and selective motor control (p = 0.041, − 0.036) throughout the study. Group A had significantly better passive dorsiflexion of the ankle (p = 0.029), observational gait score (p = 0.006) and selective motor control (p = 0.036). We conclude that the addition of inhibitory casting enhances and prolongs the results of treatment and mainly influences the passive range of movement, while BoNT-A mostly influences the dynamic motion. The second injection further improved the results of most parameters. The gross motor function measure, the selective motor control test and the modified Tardieu scale correlated well with the results of treatment.

We recommend the use of inhibitory casting whenever augmentation of the effect of treatment with BoNT-A is needed.

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.

A congenital, unilateral, fixed flexion deformity in a neonate was diagnosed as a congenital absence of the knee. A single cartilage mass, with fusion of the lower femoral and upper tibial ossification centres, was demonstrated by imaging studies. This condition has been reported in the literature only once before. Surgery on our patient, which was performed at the age of two years, consisted of separation of the fused cartilaginous anlage and gradual correction of the deformity using an Ilizarov frame.

Club foot can be diagnosed by ultrasound of the fetus in more than 60% of cases. We have correlated the accuracy of the prenatal findings in 281 ultrasound surveys with the physical findings after birth and the subsequent treatment in 147 children who were born with club foot.

The earliest week of gestation in which the condition was diagnosed with a high degree of confidence was the 12th and the latest was the 32nd. Not all patients were diagnosed at an early stage. In 29% of fetuses the first ultrasound examination failed to detect the deformity which subsequently became obvious at a later examination. Club foot was diagnosed between 12 and 23 weeks of gestation in 86% of children and between 24 and 32 weeks of gestation in the remaining 14%. Therefore it can be considered to be an early event in gestation (45% identified by the 17th week), a late event (45% detected between 18th and 24th weeks) or a very late event (10% recognised between 25th and 32nd weeks). We cannot exclude, however, the possibility that the late-onset groups may have been diagnosed late because earlier scans were false-negative results. The prenatal ultrasonographic findings were correlated with the physical findings after birth and showed that bilateral involvement was more common than unilateral. There was no significant relationship between the prenatal diagnosis and the postnatal therapeutic approach (i.e., conservative or surgical), or the degree of rigidity of the affected foot.

The conventional osteotomies used to treat infantile tibia vara (Blount’s disease) may require internal fixation and its subsequent removal. These techniques, which carry the risk of traction injury, and potential problems of stability and consolidation, do not always succeed in correcting the rotational deformity which accompanies the angular deformity.

We have used a new surgical approach, the serrated W/M osteotomy of the proximal tibia, to correct infantile tibia vara in 15 knees of 11 patients. We present the results in 13 knees of nine patients who have been followed up for a mean of eight years. The mean angular correction achieved after operation was 18 ± 5.8°. The mean femorotibial shaft angle was corrected from 14.2 ± 3.7° of varus to 4.6 ± 4.4° of valgus. At the last follow-up, the mean angular correction had reduced to 1.3 ± 4.9° of valgus without compromising the rotational correction and the overall good clinical results. All the patients and parents were satisfied, rating the result as excellent or good. There were no major postoperative complications and no reoperations. Eight patients were free from pain and able to perform physical activities suitable for their age. One complained of occasional pain. This procedure has the advantage of allowing both angular and rotational correction with a high degree of success without the need for internal fixation.

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.

Osteomyelitis is a rare manifestation of cat-scratch disease in patients who do not have AIDS. The clinical presentation and non-specific subacute course of the disease make diagnosis difficult.

We present a child with osteomyelitis of a metacarpal following a dog scratch. Bartonella henselae was found to be the aetiological agent. The bone healed after treatment with antibiotics. Increased awareness and a comprehensive medical history are needed to identify patients with suspected Bartonella henselae osteomyelitis.

We describe a five-year-old child with neurofibromatosis type I who developed a Charcot knee. Infiltration of the joint by tissue associated with the disease caused damage to the proprioceptive mechanism and resulted in severe joint instability, accelerated destruction and development of neuropathic arthropathy.

Primary subacute haematogenous osteomyelitis (PSHO) of the small bones of the foot is a rare and infrequently considered cause of a limp in children. We describe 11 patients with PSHO, of whom nine were under three years of age, who had a limp with few symptoms. The talus was involved in 36%. Bone scans were positive in all patients and led to localisation of the lesion in two. The radiological features included soft-tissue swelling, an osteolytic lesion in the talus and the calcaneus and a sclerotic appearance of the cuboid and the navicular bones. All patients except one were cured with antibiotics.

We prospectively evaluated the percutaneous injection of autogenous bone marrow for the treatment of active simple bone cysts in ten consecutive children with cysts in the proximal humerus, proximal femur or tibia. The treatment included percutaneous biopsy, aspiration of fluid and the injection of autogenous bone marrow aspirated from the iliac crest.

All the patients became painfree after a mean of two weeks and resumed full activities within six weeks. All ten cysts consolidated radiologically and showed remarkable remodelling within four months. Review at 12 to 48 months showed satisfactory healing without complications. Percutaneous injection of autologous bone marrow appears to be an effective treatment for active simple bone cysts.

We performed electrophysiological studies on both legs of 52 children, aged from 3 months to 15 years, with idiopathic club foot. In only nine (17%) was no abnormality found. Isolated peroneal nerve damage was seen in 14 (27%). Abnormality of both peroneal and posterior tibial nerves was found in five (10%). Four patients (8%) had evidence of isolated spinal-cord dysfunction, whereas combined spinal-cord and peripheral-nerve lesions were seen in 14 (27%). Six patients (11%) had variable neurogenic electrophysiological patterns. In 13 patients in whom the studies were repeated neither progression nor improvement of the electrophysiological parameters was observed. Pathological electrophysiological findings were found in 66% of conservatively-treated patients. In the 43 patients treated surgically, all 16 with fair and poor results had pathological electrophysiological findings and 12 required further operations. Multiplicity of the pathological findings was related to the severity of the deformity of the foot; normal studies represent a good prognostic sign. Electrophysiological studies are useful in idiopathic club foot with residual deformities after conservative or operative treatment. Our findings support the theory that muscle imbalance is an aetiological factor in idiopathic club foot.

We made a prospective longitudinal clinical and radiological study of 18 children diagnosed as having dysplasia epiphysealis capitis femoris. Half the cases were bilateral. Boys were affected five times more often than girls. There were no symptoms or clinical signs in most but some of the bilateral cases had an inconsistent waddling gait. The imaging studies suggest that the cartilaginous proximal femoral epiphysis is hypoplastic, with delayed appearance of single or multiple ossification centres. Progressive improvement occurred and at an average age of five years and six months, there was complete fusion of all the ossific nuclei and normal density and texture of the epiphyseal bone. The end result was a round epiphysis with a slightly diminished height. The dysplasia is attributed to focal hypoplasia of the proximal femoral epiphysis.

Forty-four feet in 28 children previously treated by a one-stage posteromedial release operation (the Turco procedure) were reviewed clinically and radiologically to determine the cause of residual adduction of the forefoot. In 21 clinically adducted feet (48%) the main cause of residual deformity was metatarsus varus alone or metatarsus varus in spite of talonavicular overcorrection; in five feet the cause was talonavicular subluxation. There was no residual adduction in 23 feet (52%) but only 12 had normal radiographic measurements. In the remaining feet, various forms of spurious correction of metatarsus varus and talonavicular subluxation or both were seen, resulting in normal-looking feet. Recession of the origin of abductor hallucis and release of the short plantar muscles and fascia at the time of posteromedial release is recommended. The forefoot adduction was satisfactorily corrected in 91% of the feet subsequently operated on using this modified procedure.

Three amputated legs with tibial dysplasia were studied by radiography, arteriography and anatomical dissection. The radiographic appearances were the same as the Type 1b tibial dysplasia described by Jones, Barnes and Lloyd-Roberts (1978) in that the tibiae were absent but the lower femoral epiphyses were normal. However, our anatomical findings differed from those of Jones et al. since no bony or cartilaginous anlage of the proximal tibia was found in any of the three legs. The pattern of vascular anomaly was identical in the three legs and similar to the findings of Hootnick et al. (1980) in congenital short fibula. Congenital fusion of the subtalar joint was a constant finding. These results support the hypothesis that the arterial and skeletal systems are vulnerable to a teratogenic insult in the fifth week of embryonic life. The bony and arterial anomalies should be borne in mind by the surgeon attempting reconstructive surgery for this condition.

Stereoradiography with a base shift of the source of illumination was used to produce pairs of radiographs to be measured by stereophotogrammetric techniques. The direction of shift was parallel with the longitudinal axis of the body, so that each radiograph in the stereopair could be used for other clinical purposes. A base shift of 10 centimetres with a distance of 100 centimetres between the focus and the film gave acceptable value of stereoscopic parallax. The radiographs were measured using a Hilger and Watts medical stereometer. This method was checked with test specimens, namely an osteotomised pelvis in which one acetabulum could be rotated and an osteotomised femur in which the whole upper portion could be rotated against the shaft. Measurements made on the acetabulum and its radiographs showed a correlation coefficient of 0.9838 over the range 0 to 30 degrees of anteversion, with a mean error +2.54 degrees and a standard deviation of +/- 1.52 degrees (n = 21). For the femoral neck, over the range from 10 degrees of retroversion to 80 degrees of anteversion, the correlation coefficient was 0.9979, the mean error +2.46 and the standard deviation +/- 1.48 degrees (n = 30).

The sagging rope sign is the term used to describe the radiographic appearances which sometimes occur after Perthes' disease. It is severe examples of that disease and indicates damage to the growth plate with a marked metaphysial reaction. The same appearance follows severe epiphysitis after forcible reduction of a congenitally dislocated hip, and certain rare epiphysial dysplasias. The origin and significance of the sign are discussed.

We compared 63 hips (Catterall Groups 3 and 4) contained by femoral osteotomy with 85 untreated hips and found that 50.7 per cent of treated patients developed congruous spherical femoral heads in contrast to 14.1 per cent of those untreated. We have also considered certain other features relevant to the outcome. We suggest that the indications should not be modified on the grounds of early age of onset. Relief from weight-bearing does not appear to improve the results of containment. We have assessed the shortening which follows femoral osteotomy and conclude that this is only significant when there is growth disturbance at the capital epiphysis. These changes are at least as frequent in untreated patients.