There is a close link between the embryological development of the musculoskeletal system and all other main organ systems. We report a prospective series of 202 patients with congenital vertebral abnormalities and document the associated abnormalities in other systems. There were 100 boys and 102 girls. In 153 there were 460 associated abnormalities, a mean of 2.27 abnormalities for each patient. Intravenous pyelography was carried out on 173 patients (85.6%) and ultrasonography on the remaining 29 (14.4%). Patients with genitourinary anomalies were more likely to have musculoskeletal (p = 0.002), gastrointestinal (p = 0.02) and cardiac abnormalities (p = 0.008) than those without genitourinary involvement. A total of 54 (26.7%) had at least one genitourinary abnormality, the most frequent being unilateral renal agenesis. There was urinary obstruction in six (3%). There was no association between genitourinary abnormality and the place of birth, parental age, birth order, level of spinal curvature, or the number, type and side of spinal anomaly. There was, however, a statistically significant association (p = 0.04) between costal and genitourinary abnormalities. The incidence of genitourinary abnormalities (26.7%) was similar to that of previously reported series. The diagnosis of a congenital vertebral abnormality should alert the clinician to a wide spectrum of possible associated anomalies most of which are of clinical importance

Aims. The aim of this study was to gain an agreement on the management of idiopathic congenital talipes equinovarus (CTEV) up to walking age in order to provide a benchmark for practitioners and guide consistent, high-quality care for children with CTEV. Methods. The consensus process followed an established Delphi approach with a predetermined degree of agreement. The process included the following steps: establishing a steering group; steering group meetings, generating statements, and checking them against the literature; a two-round Delphi survey; and final consensus meeting. The steering group members and Delphi survey participants were all British Society of Children’s Orthopaedic Surgery (BSCOS) members. Descriptive statistics were used for analysis of the Delphi survey results. The Appraisal of Guidelines for Research & Evaluation checklist was followed for reporting of the results. Results. The BSCOS-selected steering group, the steering group meetings, the Delphi survey, and the final consensus meeting all followed the pre-agreed protocol. A total of 153/243 members voted in round 1 Delphi (63%) and 132 voted in round 2 (86%). Out of 61 statements presented to round 1 Delphi, 43 reached ‘consensus in’, no statements reached ‘consensus out’, and 18 reached ‘no consensus’. Four statements were deleted and one new statement added following suggestions from round 1. Out of 15 statements presented to round 2, 12 reached ‘consensus in’, no statements reached ‘consensus out’, and three reached ‘no consensus’ and were discussed and included following the final consensus meeting. Two statements were combined for simplicity. The final consensus document includes 57 statements allocated into six successive stages. Conclusion. We have produced a consensus document for the treatment of idiopathic CTEV up to walking age. This will provide a benchmark for standard of care in the UK and will help to reduce geographical variability in treatment and outcomes. Appropriate dissemination and implementation will be key to its success. Cite this article: Bone Joint J 2022;104-B(6):758–764

Aims. To identify the minimum set of outcomes that should be collected in clinical practice and reported in research related to the care of children with idiopathic congenital talipes equinovarus (CTEV). Methods. A list of outcome measurement tools (OMTs) was obtained from the literature through a systematic review. Further outcomes were collected from patients and families through a questionnaire and interview process. The combined list, as well as the appropriate follow-up timepoint, was rated for importance in a two-round Delphi process that included an international group of orthopaedic surgeons, physiotherapists, nurse practitioners, patients, and families. Outcomes that reached no consensus during the Delphi process were further discussed and scored for inclusion/exclusion in a final consensus meeting involving international stakeholder representatives of practitioners, families, and patient charities. Results. In total, 39 OMTs were included from the systematic review. Two additional OMTs were identified from the interviews and questionnaires, and four were added after round one Delphi. Overall, 22 OMTs reached ‘consensus in’ during the Delphi and two reached ‘consensus out’; 21 OMTs reached ‘no consensus’ and were included in the final consensus meeting. In all, 21 participants attended the consensus meeting, including a wide diversity of clubfoot practitioners, parent/patient representative, and an independent chair. A total of 21 outcomes were discussed and voted upon; six were voted ‘in’ and 15 were voted ‘out’. The final COS document includes nine OMTs and two existing outcome scores with a total of 31 outcome parameters to be collected after a minimum follow-up of five years. It incorporates static and dynamic clinical findings, patient-reported outcome measures, and a definition of CTEV relapse. Conclusion. We have defined a minimum set of outcomes to draw comparisons between centres and studies in the treatment of CTEV. With the use of these outcomes, we hope to allow more meaningful research and a better clinical management of CTEV. Cite this article: Bone Jt Open 2022;3(1):98–106

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

Difficulties posed in managing developmental dysplasia of the hip diagnosed late include a high-placed femoral head, contracted soft tissues and a dysplastic acetabulum. A combination of open reduction with femoral shortening of untreated congenital dislocations is a well-established practice. Femoral shortening prevents excessive pressure on the located femoral head which can cause avascular necrosis. Instability due to a coexisting dysplastic shallow acetabulum is common, and so a pelvic osteotomy is performed to achieve a stable and concentric hip reduction. We retrospectively reviewed 15 patients (18 hips) presenting with developmental dysplasia of the hip aged four years and above who were treated by a one-stage combined procedure performed by the senior author. The mean age at operation was five years and nine months (4 years to 11 years). The mean follow-up was six years ten months (2 years and 8 months to 8 years and 8 months). All patients were followed up clinically and radiologically in accordance with McKay’s criteria and the modified Severin classification. According to the McKay criteria, 12 hips were rated excellent and six were good. All but one had a full range of movement. Eight had a limb-length discrepancy of about 1 cm. All were Trendelenburg negative. The modified Severin classification demonstrated four hips of grade IA, six of grade IB, and eight of grade II. One patient had avascular necrosis and one an early subluxation requiring revision. One-stage correction of congenital dislocation of the hip in an older child is a safe and effective treatment with good results in the short to medium term

In five children, six forearms with a fixed pronation deformity secondary to congenital radioulnar synostosis were treated by a derotation osteotomy of the distal radius and the midshaft of the ulna. There were three boys and two girls with a mean age of 4.9 years (3.5 to 8.25) who were followed up for a mean of 29 months (18 to 43). The position of the forearm was improved from a mean pronation deformity of 68° (40° to 80°) to a pre-planned position of 10° of supination in all cases. Bony union was achieved by 6.3 weeks with no loss of correction. There was one major complication involving a distal radial osteotomy which required exploration for a possible compartment syndrome

There is controversy whether congenital foot abnormalities are true risk factors for pathological dysplasia of the hip. Previous United Kingdom screening guidelines considered congenital talipes equinovarus (CTEV) to be a risk factor for hip dysplasia, but present guidelines do not. We assessed the potential relationship between pathological dysplasia of the hip and fixed idiopathic CTEV. We present a single-centre 21-year prospective longitudinal observational study. All fixed idiopathic CTEV cases were classified (Harrold and Walker Types 1 to 3) and the hips clinically and sonographically assessed. Sonographic Graf Type III, IV and radiological irreducible hip dislocation were considered to be pathological hip dysplasia. Over 21 years there were 139 children with 199 cases of fixed idiopathic CTEV feet. Sonographically, there were 259 normal hips, 18 Graf Type II hips, 1 Graf Type III hip and 0 Graf Type IV hip. There were no cases of radiological or sonographic irreducible hip dislocation. Fixed idiopathic CTEV should not be considered as a significant risk factor for pathological hip dysplasia. This conclusion is in keeping with the current newborn and infant physical examination guidelines in which the only risk factors routinely screened are family history and breech presentation. Our findings suggest CTEV should not be considered a significant risk factor in pathological dysplasia of the hip. Cite this article: Bone Joint J 2014;96-B:1553–5

Children with congenital vertical talus (CVT) have been treated with extensive soft-tissue releases, with a high rate of complications. Recently, reverse Ponseti-type casting followed by percutaneous reduction and fixation has been described, with excellent results in separate cohorts of children with CVT, of either idiopathic or teratological aetiology. There are currently no studies that compare the outcome in these two types. We present a prospective cohort of 13 children (21 feet) with CVT of both idiopathic and teratological aetiology, in which this technique has been used. Clinical, radiological and parent-reported outcomes were obtained at a mean follow-up of 36 months (8 to 57). Six children (nine feet) had associated neuromuscular conditions or syndromes; the condition was idiopathic in seven children (12 feet). Initial correction was achieved in all children, with significant improvement in all radiological parameters. Recurrence was seen in ten feet. Modification of the technique to include limited capsulotomy at the initial operation may reduce the risk of recurrence. The reverse Ponseti-type technique is effective in the initial correction of CVT of both idiopathic and teratological aetiology. Recurrence is a problem in both these groups, with higher rates than first reported in the original paper. However, these rates are less than those reported after open surgical release. Cite this article: Bone Joint J 2014;96-B:274–8

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 tumour. Excision biopsy confirmed this to be a local recurrence, although the lesion was less cellular with no appreciable mitotic activity. Congenital haemangiopericytoma is a rare cause of a soft-tissue mass in children. Most tumours are benign, and recurrence is uncommon. The treatment is controversial, but most centres recommend the use of adjuvant chemotherapy, combined with complete excision. We recommend treatment with doxorubicin. Orthopaedic surgeons should be familiar with this tumour since 30% to 50% of cases occur in the limbs

Only two cases have been reported of congenital dislocation of the hip in infants born after extrauterine pregnancies. We report a further two and discuss the management and the variable outcome. These cases seem to confirm that congenital dislocation of the hip is associated with moulding forces rather than being a teratological abnormality

In this study we evaluated the results of midtarsal release and open reduction for the treatment of children with convex congenital foot (CCF) (vertical talus) and compared them with the published results of peritalar release. Between 1977 and 2009, a total of 22 children (31 feet) underwent this procedure. In 15 children (48%) the CCF was isolated and in the remainder it was not (seven with arthrogryposis, two with spinal dysraphism, one with a polymalformative syndrome and six with an undefined neurological disorder).

Pre-operatively, the mean tibiotalar angle was 150.2° (106° to 175°) and the mean calcaneal pitch angle was -19.3° (-72° to 4°). The procedure included talonavicular and calcaneocuboid joint capsulotomies, lengthening of tendons of tibialis anterior and the extensors of the toes, allowing reduction of the midtarsal joints. Lengthening of the Achilles tendon was necessary in 23 feet (74%).

The mean follow-up was 11 years (2 to 21). The results, as assessed by the Adelaar score, were good in 24 feet (77.4%), fair in six (19.3%) and poor in one foot (3.3%), with no difference between those with isolated CCF and those without. The mean American Orthopaedic Foot and Ankle Society midfoot score was 89.9 (54 to 100) and 77.8 (36 to 93) for those with isolated CCF and those without, respectively. At the final follow-up, the mean tibiotalar (120°; 90 to 152) and calcaneal pitch angles (4°; -13 to 22) had improved significantly (p < 0.0001). Dislocation of the talonavicular and calcaneocuboid joints was completely reduced in 22 (70.9%) and 29 (93.6%) of feet, respectively. Three children (five feet) underwent further surgery at a mean of 8.5 years post-operatively, three with pes planovalgus and two in whom the deformity had been undercorrected. No child developed avascular necrosis of the talus.

Midtarsal joint release and open reduction is a satisfactory procedure, which may provide better results than peritalar release. Complications include the development of pes planovalgus and persistent dorsal subluxation of the talonavicular joint.

Cite this article: Bone Joint J 2014;96-B:837–44.

Congenital unilateral anterolateral tibial bowing in combination with a bifid ipsilateral great toe is a very rare deformity which resembles the anterolateral tibial bowing that occurs in association with congenital pseudarthrosis of the tibia. However, spontaneous resolution of the deformity without operative treatment and with a continuously straight fibula has been described in all previously reported cases. We report three additional cases and discuss the options for treatment. We suggest that this is a specific entity within the field of anterolateral bowing of the tibia and conclude that it has a much better prognosis than congenital pseudarthrosis of the tibia, although conservative treatment alone may not be sufficient

A total of 38 relapsed congenital clubfeet (16 stiff, 22 partially correctable) underwent revision of soft-tissue surgery, with or without a bony procedure, and transfer of the tendon of tibialis anterior at a mean age of 4.8 years (2.0 to 10.1). The tendon was transferred to the third cuneiform in five cases, to the base of the third metatarsal in ten and to the base of the fourth in 23. The patients were reviewed at a mean follow-up of 24.8 years (10.8 to 35.6). A total of 11 feet were regarded as failures (one a tendon failure, five with a subtalar fusion due to over-correction, and five with a triple arthrodesis due to under-correction or relapse). In the remaining feet the clinical outcome was excellent or good in 20 and fair or poor in seven. The mean Laaveg-Ponseti score was 81.6 of 100 points (52 to 92). Stiffness was mild in four feet and moderate or severe in 23. Comparison between the post-operative and follow-up radiographs showed statistically significant variations of the talo-first metatarsal angle towards abduction. Variations of the talocalcaneal angles and of the overlap ratio were not significant. Extensive surgery for relapsed clubfoot has a high rate of poor long-term results. The addition of transfer of the tendon of tibialis anterior can restore balance and may provide some improvement of forefoot adduction. However, it has a considerable complication rate, including failure of transfer, over-correction, and weakening of dorsiflexion. The procedure should be reserved for those limited cases in which muscle imbalance is a causative or contributing factor

Congenital pseudarthrosis of the tibia (CPT) is a rare but well recognised condition. Obtaining union of the pseudarthrosis in these children is often difficult and may require several surgical procedures. The treatment has changed significantly since the review by Hardinge in 1972, but controversies continue as to the best form of surgical treatment. This paper reviews these controversies.

Cite this article: Bone Joint J 2013;95-B:1027–34.

We reviewed 33 patients (35 hips) after open reduction of congenital dislocation of the hip using Ludloff’s medial approach. The mean age at the time of operation was 14 months (5 to 29) and at the time of final follow-up 20.1 years (15 to 24) giving a mean duration of follow-up of 19.4 years (14 to 23). We evaluated the radiological results by the Severin classification and the extent of avascular necrosis using the criteria of Kalamchi. At the latest review 16 hips (45.7%) were in Severin groups I or II (acceptable results) and 19 (54.3%) in Severin groups III, IV or V (unacceptable results). There was evidence of avascular necrosis in 15 hips (42.9%). The radiological results began to become worse at about the age of ten years when type-II avascular changes presented. We conclude that Ludloff’s medial approach for open reduction is unsatisfactory for the treatment of congenital dislocation since about half of our patients required additional operations

We reviewed 13 patients with congenital insensitivity to pain. A quantitative sweat test was carried out in five and an intradermal histamine test in ten. DNA examination showed specific mutations in four patients. There were three clinical presentations: type A, in which multiple infections occurred (five patients); type B, with fractures, growth disturbances and avascular necrosis (three patients); and type C, with Charcot arthropathies and joint dislocations, as well as fractures and infections (five patients, four with mental retardation). Patient education, shoeware and periods of non-weight-bearing are important in the prevention and early treatment of decubitus ulcers. The differentiation between fractures and infections should be based on aspiration and cultures to prevent unnecessary surgery. Established infections should be treated by wide surgical debridement. Deformities can be managed by corrective osteotomies, and shortening by shoe raises or epiphysiodesis. Joint dislocations are best treated conservatively

We studied 24 children (40 feet) to demonstrate that a physiotherapist-delivered Ponseti service is as successful as a medically-led programme in obtaining correction of an idiopathic congenital talipes equinovarus deformity. The median Pirani score at the start of treatment was 5.5 (mean 4.75; 2 to 6). A Pirani score of ≥5 predicted the need for tenotomy (p < 0.01). Of the 40 feet studied, 39 (97.5%) achieved correction of deformity. The remaining foot required surgical correction. A total of 25 (62.5%) of the feet underwent an Achilles tenotomy, which was performed by a surgeon in the physiotherapy clinic. There was full compliance with the foot abduction orthoses in 36 (90%) feet. Continuity of care was assured, as one practitioner was responsible for all patient contact. This was rated highly by the patient satisfaction survey. We believe that the Ponseti technique is suitable for use by non-medical personnel, but a holistic approach and good continuity of care are essential to the success of the programme

To determine the effect of cordotomy on the function of the bladder during surgical correction of congenital kyphosis in myelomeningocele, we reviewed 13 patients who had this procedure between 1981 and 1996. The mean age of the patients at operation was 8.9 years (3.7 to 16) and the mean follow-up was 4.8 years (1.3 to 10.8). Bladder function before and after operation was assessed clinically and quantitatively by urodynamics. The mean preoperative kyphosis was 117° (52 to 175) and decreased to 49° (1 to 89) immediately after surgery. At the latest follow-up, a mean correction of 52% had been achieved. Only one patient showed deterioration in bladder function after operation. Eight out of the nine patients who had urodynamic assessment had improvement in bladder capacity and compliance, and five showed an increase in urethral pressure. One patient developed a spastic bladder and required subsequent surgical intervention. Cordotomy, at or below the level of the kyphosis, allows excellent correction of the structural deformity

We used the Ilizarov method in seven patients with severe congenital radial club hands who had had previous wrist surgery, to correct residual shortening and bowing of the ulna together with recurrent wrist deformity. The mean age at operation was 6.5 years. The mean ulnar shortening was 5.3 cm and the mean angular deformity 42°. The mean length gained was 51% of the original ulna. The mean healing index was 46.9 days (29.8 to 64.0). The ratio of the length of the lengthened ulna to the normal side improved on average from 64% to 95%. The angular deformity was initially completely corrected in six out of seven patients. The length ratio, however, decreased to 83% at the final follow-up. In four patients, the angular deformity partially recurred. We recommend correction of congenital radial club hand by staged procedures. The first is centralisation and stabilisation of the wrist and the second lengthening of the ulna and correction of the angular deformity using the Ilizarov method

We have assessed the reliability of four classification systems for club foot. Four observers evaluated nine children (18 feet) at different stages in the first six months of life, a total of 180 examinations. Each observer independently assessed all feet according to the classification systems described by Catterall, Diméglio et al, Harrold and Walker, and Ponseti and Smoley. The variation between observers was assessed using the kappa test which for no more agreement than chance has a value of 0, and for complete agreement between observers a value of 1. The kappa values varied between 0.14 and 0.77 depending on which classification system was used. The system of Diméglio et al was found to have the greatest reliability. Our findings suggest that current classification systems for the analysis of congenital talipes equinovarus are not entirely satisfactory

Congenital convex pes valgus (congenital vertical talus) is a rare condition. We reviewed ten feet in seven patients who had had surgical correction. All had been operated on by the senior author (JF) and the same surgical technique was used throughout, incorporating transfer of the tibialis anterior to the neck of the talus. The mean age at surgery was 31 months and the mean follow-up was nine years (6 to 14). All patients completed a questionnaire and had clinical, radiological and photographic evaluation performed by an independent examiner. None had required further surgery. All but one were satisfied with the result, and had no functional limitations. They all wore normal shoes. The mean ankle dorsiflexion was 17° and plantar flexion 21°. The mean arc of subtalar motion was 27°. All radiological parameters measured were within the normal range, although irregularity of the talonavicular joint was common. No avascular necrosis of the body of the talus was seen. We conclude that the medium-term results of this procedure are very satisfactory

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.

The Ponseti and French taping methods have reduced the incidence of major surgery in congenital idiopathic clubfoot but incur a significant burden of care, including heel-cord tenotomy. We developed a non-operative regime to reduce treatment intensity without affecting outcome. We treated 402 primary idiopathic clubfeet in patients aged < three months who presented between September 1991 and August 2008. Their Harrold and Walker grades were 6.0% mild, 25.6% moderate and 68.4% severe. All underwent a dynamic outpatient taping regime over five weeks based on Ponseti manipulation, modified Jones strapping and home exercises. Feet with residual equinus (six feet, 1.5%) or relapse within six months (83 feet, 20.9%) underwent one to three additional tapings. Correction was maintained with below-knee splints, exercises and shoes. The clinical outcome at three years of age (385 feet, 95.8% follow-up) showed that taping alone corrected 357 feet (92.7%, ‘good’). Late relapses or failure of taping required limited posterior release in 20 feet (5.2%, ‘fair’) or posteromedial release in eight feet (2.1%, ‘poor’). The long-term (> 10 years) outcomes in 44 feet (23.8% follow-up) were assessed by the Laaveg–Ponseti method as excellent (23 feet, 52.3%), good (17 feet, 38.6%), fair (three feet, 6.8%) or poor (one foot, 2.3%). These compare favourably with published long-term results of the Ponseti or French methods. This dynamic taping regime is a simple non-operative method that delivers improved medium-term and promising long-term results. Cite this article: Bone Joint J 2013;95-B:271–8

A six-year-old girl with congenital sensory neuropathy with anhidrosis (CSNA) presented with bilateral hip dysplasia and subluxation on the right side. Conservative treatment of the hips by closed reduction and a plaster cast was unsuccessful. When aged seven years the patient had an intertrochanteric varus rotation osteotomy on the right side, but subluxation was again evident after five months. A Salter-type pelvic osteotomy was carried out followed by immobilisation, but one year later subluxation was present in the right hip and dislocation in the left. At the age of nine years, the right femoral head resembled a Charcot joint, although walking ability was preserved. In patients with CSNA, surgery may not always be advisable

Congenital talipes equinovarus is a common anomaly which can now be diagnosed prenatally on a routine ultrasound scan at 20 weeks of gestation. Prenatal counselling is increasingly offered to parents with affected fetuses, but it is difficult to counsel parents if there is a chance that the fetus may not have talipes. Our study correlates the prenatal ultrasound findings of 14 infants diagnosed as having unilateral or bilateral talipes during their routine 20-week ultrasound scan with their clinical findings at birth and the treatment received. No feet diagnosed as talipes on the ultrasound scan were completely normal at birth and therefore there were no true false-positive results. One foot graded as normal at 20 weeks was found to have a mild grade-1 talipes at birth, but did not require treatment other than simple stretches. A total of 32% of feet required no treatment and so could be considered functional false-positive results on the scan. Serial casting was required by 13% of feet and surgical treatment by 55%. The severity of the talipes is difficult to establish before birth. A number of patients are likely to need surgical treatment, but a proportion will have talipes so mildly that no treatment will be required. In counselling parents at 20 weeks, orthopaedic surgeons need to know whether or not there is a small chance that the ultrasound diagnosis could be wrong and also that the talipes may be so mild that the foot will not require treatment

The aim of this study was to define objectively gait function in children with treated congenital talipes equinovarus (CTEV) and a good clinical result. The study also attempted an analysis of movement within the foot during gait. We compared 20 children with treated CTEV with 15 control subjects. Clinical assessment demonstrated good results from treatment. Three-dimensional gait analysis provided kinematic and kinetic data describing movement and moments at the joints of the lower limb during gait. A new method was used to study movement within the foot during gait. The data on gait showed significantly increased internal rotation of the foot during walking which was partially compensated for by external rotation at the hip. A mild foot drop and reduced plantar flexor power were also observed. Dorsiflexion at the midfoot was significantly increased, which probably compensated for reduced mobility at the hindfoot. Patients treated for CTEV with a good clinical result should be expected to have nearly normal gait and dynamic foot movement, but there may be residual intoeing, mild foot drop, loss of plantar flexor power with compensatory increased midfoot dorsiflexion and external hip rotation

We review the results of a modified quadricepsplasty in five children who developed stiffness of the knee after femoral lengthening for congenital short femur using an Ilizarov external fixator which spanned the knee. All had a full range of movement of the knee before lengthening was undertaken. Unifocal lengthening was carried out in the distal metaphysiodiaphyseal region of the distal femur with a mean gain of 6.5 cm. The mean percentage lengthening was 24%. At the end of one year after removal of the Ilizarov frame and despite intensive physiotherapy all patients had stiffness. Physiotherapy was continued after the quadricepsplasty and, at the latest follow-up (mean 27 months), the mean active flexion was 102° (80 to 130). The gain in movement ranged from 50° to 100°. One patient had a superficial wound infection which settled after a course of oral antibiotics. None developed an increased extension lag after surgery and all were very satisfied with the results. Quadricepsplasty is a useful procedure for stiffness of the knee after femoral lengthening which has not responded to physiotherapy

We operated on 111 patients with 159 congenital club feet with the aim of correcting the deformity and achieving dynamic muscle balance. Clinical and biomechanical assessment was undertaken at least six years after operation when the patient was more than 13 years of age. The mean follow-up was for 11 years 10 months (6 to 36 years). Good and excellent results were obtained in 91.8%. Patients with normal function of the calf had a better outcome than those with weak calf muscles. The radiological changes were assessed in relation to the clinical outcome. The distribution of pressure under the foot was measured for biomechanical assessment. Our results support the view that muscle imbalance is an aetiological factor in club foot. Early surgery seems to be preferable. It is suggested that operation should be undertaken as soon as possible after the age of six months, although it may be carried out up to the age of five years. The establishment of dynamic muscle balance appears to be an effective method of maintaining correction. Satisfactory long-term results can be achieved with adequate appearance and function

We report 12 consecutive cases of vertical scapular osteotomy to correct Sprengel’s deformity, performed during a 16-year period, with a mean follow-up of 10.4 years. The mean increase in abduction of the shoulder was 53°. The cosmetic appearance improved by a mean of 1.5 levels on the Cavendish scale. Neither function nor cosmesis deteriorated with time. We recommend the procedure for correction of moderate deformities with a functional deficit.

Between 1988 and 1995, we studied 91 club feet from a series of 120 recalcitrant feet in 86 patients requiring surgical treatment. There were 48 boys and 20 girls. The mean age at operation was 8.9 months. Surgery consisted of an initial plantar medial release followed two weeks later by a posterolateral release. This strategy was adopted specifically to address the problems of wound healing associated with single-stage surgery and to ascertain the rate of relapse after a two-stage procedure. Immobilisation in plaster was used for three months followed by night splintage. The feet were classified preoperatively and prospectively into four grades according to the system suggested by Dimeglio et al. Grade-1 feet were postural and did not require surgery. All wounds were closed primarily. One superficial wound infection occurred in a grade-4 foot and there were no cases of wound breakdown. The rate of relapse was 20.4% in grade-3 and 65.4% in grade-4 feet.

Two-stage surgery for the treatment of club foot seems to be effective in the reduction of wound problems but does not appear to give significantly better results in terms of relapse when performed for more severe deformities.

Aims. The Ponseti method is the gold standard treatment for congenital talipes equinovarus (CTEV), with the British Consensus Statement providing a benchmark for standard of care. Meeting these standards and providing expert care while maintaining geographical accessibility can pose a service delivery challenge. A novel ‘Hub and Spoke’ Shared Care model was initiated to deliver Ponseti treatment for CTEV, while addressing standard of care and resource allocation. The aim of this study was to assess feasibility and outcomes of the corrective phase of Ponseti service delivery using this model. Methods. Patients with idiopathic CTEV were seen in their local hospitals (‘Spokes’) for initial diagnosis and casting, followed by referral to the tertiary hospital (‘Hub’) for tenotomy. Non-idiopathic CTEV was managed solely by the Hub. Primary and secondary outcomes were achieving primary correction, and complication rates resulting in early transfer to the Hub, respectively. Consecutive data were prospectively collected and compared between patients allocated to Hub or Spokes. Mann-Whitney U test, Wilcoxon signed-rank test, or chi-squared tests were used for analysis (alpha-priori = 0.05, two-tailed significance). Results. Between 1 March 2020 and 31 March 2023, 92 patients (139 feet) were treated at the service (Hub 50%, n = 46; Spokes 50%, n = 46), of whom nine were non-idiopathic. All patients (n = 92), regardless of allocation, ultimately achieved primary correction, with idiopathic patients at the Hub requiring fewer casts than the Spokes (mean 4.0 (SD 1.4) vs 6.9 (SD 4.4); p < 0.001). Overall, 60.9% of Spokes’ patients (n = 28/46) required transfer to the Hub due to complications (cast slips Hub n = 2; Spokes n = 17; p < 0.001). These patients ultimately achieved full correction at the Hub. Conclusion. The Shared Care model was found to be feasible in terms of providing primary correction to all patients, with results comparable to other published services. Complication rates were higher at the Spokes, although these were correctable. Future research is needed to assess long-term outcomes, parents’ satisfaction, and cost-effectiveness. Cite this article: Bone Jt Open 2023;4(11):865–872

Aims. To assess if congenital foot deformity is a risk factor for developmental dysplasia of the hip (DDH). Methods. Between 1996 and 2012, 60,844 children were born in Sør-Trøndelag county in Norway. In this cohort study, children with risk factors for DDH were examined using ultrasound. The risk factors evaluated were clinical hip instability, breech delivery, a family history of DDH, a foot deformity, and some syndromes. As the aim of the study was to examine the risk for DDH and foot deformity in the general population, children with syndromes were excluded. The information has been prospectively registered and retrospectively analyzed. Results. Overall, 494 children (0.8%) had DDH, and 1,132 (1.9%) a foot deformity. Of the children with a foot deformity, 49 (4.3%) also demonstrated DDH. There was a statistically significant increased association between DDH and foot deformity (p < 0.001). The risk of DDH was highest for talipes calcaneovalgus (6.1%) and club foot (3.5%), whereas metatarsus adductus (1.5%) had a marginal increased risk of DDH. Conclusion. Compared with the general population, children with a congenital foot deformity had a significantly increased risk for DDH and therefore we regard foot deformity as a true risk factor for DDH. Cite this article: Bone Joint J 2020;102-B(11):1582–1586

We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity. We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a pseudarthrosis

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Given the possible radiation damage and inaccuracy of radiological investigations, particularly in children, ultrasound and superb microvascular imaging (SMI) may offer alternative methods of evaluating new bone formation when limb lengthening is undertaken in paediatric patients. The aim of this study was to assess the use of ultrasound combined with SMI in monitoring new bone formation during limb lengthening in children.

Since September 1964, neonates born in New Plymouth have undergone clinical examination for instability of the hip in a structured clinical screening programme. Of the 41 563 babies born during this period, 1639 were diagnosed as having unstable hips and 663 (1.6%) with persisting instability were splinted, five of which failed. Also, three unsplinted hips progressed to congenital dislocation, and there were four late-presenting (walking) cases, giving an overall failure rate of 0.29 per 1000 live births, with an incidence of late-walking congenital dislocation of the hip of 0.1 per 1000 live births. This study confirms that clinical screening for neonatal instability of the hip by experienced orthopaedic examiners significantly reduces the incidence of late-presenting (walking) congenital dislocation of the hip

The aetiology of congenital club foot is unclear. Although studies on populations, families and twins suggest a genetic component, the mode of inheritance does not comply with distinctive patterns. The Odense-based Danish Twin Registry contains data on all 73 000 twin pairs born in Denmark over the last 130 years. In 2002 all 46 418 twins born between 1931 and 1982 received a 17-page questionnaire, one question of which was ‘Were you born with club foot?’ A total of 94 twins answered ‘Yes’, giving an overall self-reported prevalence of congenital club foot of 0.0027 (95% confidence interval (CI) 0.0022 to 0.0034). We identified 55 complete twin pairs, representing 12 monozygotic, 22 dizygotic same sex (DZ. ss. ), 18 dizygotic other sex (DZ. os. ) and three unclassified. Two monozygotic and 2 DZ. ss. pairs were concordant. The pairwise concordance was 0.17 (95% CI 0.02 to 0.48) for monozygotic, 0.09 (95% CI 0.01 to 0.32) for DZ. ss. and 0.05 (95% CI 0.006 to 0.18) for all dizygotic (DZ. tot. ) twins. We have found evidence of a genetic component in congenital club foot, although non-genetic factors must play a predominant role

Congenital Talipes Equinovarus (CTEV) is one of the most common congenital limb deformities. We reviewed the records of infants who had received treatment for structural CTEV between 1 January 2007 and 30 November 2012. This was cross-referenced with the prenatal scans of mothers over a corresponding period of time. We investigated the sensitivity, specificity, and positive and negative predictive values of the fetal anomaly scan for the detection of CTEV and explored whether the publication of Fetal Anomaly Screening Programme guidelines in 2010 affected the rate of detection. During the study period there were 95 532 prenatal scans and 34 373 live births at our hospital. A total of 37 fetuses with findings suggestive of CTEV were included in the study, of whom 30 were found to have structural CTEV at birth. The sensitivity of screening for CTEV was 71.4% and the positive predictive value was 81.1%. The negative predictive value and specificity were more than 99.5%. There was no significant difference between the rates of detection before and after publication of the guidelines (p = 0.5). We conclude that a prenatal fetal anomaly ultrasound screening diagnosis of CTEV has a good positive predictive value enabling prenatal counselling. The change in screening guidance has not affected the proportion of missed cases. This information will aid counselling parents about the effectiveness and accuracy of prenatal ultrasound in diagnosing CTEV. Cite this article: Bone Joint J 2014;96-B:984–8

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Early detection of developmental dysplasia of the hip (DDH) is associated with improved outcomes of conservative treatment. Therefore, we aimed to evaluate a novel screening programme that included both the primary risk factors of breech presentation and family history, and the secondary risk factors of oligohydramnios and foot deformities.

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Children with spinal dysraphism can develop various musculoskeletal deformities, necessitating a range of orthopaedic interventions, causing significant morbidity, and making considerable demands on resources. This systematic review aimed to identify what outcome measures have been reported in the literature for children with spinal dysraphism who undergo orthopaedic interventions involving the lower limbs.

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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

Developmental dysplasia of the hip (DDH) can be managed effectively with non-surgical interventions when diagnosed early. However, the likelihood of surgical intervention increases with a late presentation. Therefore, an effective screening programme is essential to prevent late diagnosis and reduce surgical morbidity in the population.

In a prospective study over 11 years we assessed the relationship between neonatal deformities of the foot and the presence of ultrasonographic developmental dysplasia of the hip (DDH). Between 1 January 1996 and 31 December 2006, 614 infants with deformities of the foot were referred for clinical and ultrasonographic evaluation. There were 436 cases of postural talipes equinovarus deformity (TEV), 60 of fixed congenital talipes equinovarus (CTEV), 93 of congenital talipes calcaneovalgus (CTCV) and 25 of metatarsus adductus. The overall risk of ultrasonographic dysplasia or instability was 1:27 in postural TEV, 1:8.6 in CTEV, 1:5.2 in CTCV and 1:25 in metatarsus adductus. The risk of type-IV instability of the hip or irreducible dislocation was 1:436 (0.2%) in postural TEV, 1:15.4 (6.5%) in CTCV and 1:25 (4%) in metatarsus adductus. There were no cases of hip instability (type IV) or of irreducible dislocation in the CTEV group. Routine screening for DDH in cases of postural TEV and CTEV is no longer advocated. The former is poorly defined, leading to the over-diagnosis of a possibly spurious condition. Ultrasonographic imaging and surveillance of hips in infants with CTCV and possibly those with metatarsus adductus should continue

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To investigate the risk factors for unsuccessful radial head reduction (RHR) in children with chronic Monteggia fractures (CMFs) treated surgically.

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There is a lack of high-quality research investigating outcomes of Ponseti-treated idiopathic clubfeet and correlation with relapse. This study assessed clinical and quality of life (QoL) outcomes using a standardized core outcome set (COS), comparing children with and without relapse.

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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.

A nationwide study of Perthes’ disease in Norway was undertaken over a five-year period from January 1996. There were 425 patients registered, which represents a mean annual incidence of 9.2 per 100 000 in subjects under 15 years of age, and an occurrence rate of 1:714 for the country as a whole. There were marked regional variations. The lowest incidence was found in the northern region (5.4 per 100 000 per year) and the highest in the central and western regions (10.8 and 11.3 per 100 000 per year, respectively). There was a trend towards a higher incidence in urban (9.5 per 100 000 per year) compared with rural areas (8.9 per 100 000 per year). The mean age at onset was 5.8 years (1.3 to 15.2) and the male:female ratio was 3.3:1. We compared 402 patients with a matched control group of non-affected children (n = 1 025 952) from the Norwegian Medical Birth Registry and analysed maternal data (age at delivery, parity, duration of pregnancy), birth length and weight, birth presentation, head circumference, ponderal index and the presence of congenital anomalies. Children with Perthes’ disease were significantly shorter at birth and had an increased frequency of congenital anomalies. Applying Sartwell’s log-normal model of incubation periods to the distribution of age at onset of Perthes’ disease showed a good fit to the log-normal curve. Our findings point toward a single cause, either genetic or environmental, acting prenatally in the aetiology of Perthes’ disease

Aims. After the initial correction of congenital talipes equinovarus (CTEV) using the Ponseti method, a subsequent dynamic deformity is often managed by transfer of the tendon of tibialis anterior (TATT) to the lateral cuneiform. Many surgeons believe the lateral cuneiform should be ossified before surgery is undertaken. This study quantifies the ossification process of the lateral cuneiform in children with CTEV between one and three years of age. . Patients and Methods. The length, width and height of the lateral cuneiform were measured in 43 consecutive patients with unilateral CTEV who had been treated using the Ponseti method. Measurements were taken by two independent observers on standardised anteroposterior and lateral radiographs of both feet taken at one, two and three years of age. Results. All dimensions of the lateral cuneiform on the affected side increased annually but remained smaller than the corresponding dimensions of the unaffected foot (p < 0.01). The lateral cuneiform resembled a 9 mm cube at two years and an 11 mm cube at three years. Conclusion. At one and two years, the ossification centre of the lateral cuneiform may not be large enough to accommodate a drill hole for tendon transfer. However, by three years, it has undergone sufficient ossification to do so. Cite this article: Bone Joint J 2017;99-B:1109–14

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Within healthcare, several measures are used to quantify and compare the severity of health conditions. Two common measures are disability weight (DW), a context-independent value representing severity of a health state, and utility weight (UW), a context-dependent measure of health-related quality of life. Neither of these measures have previously been determined for developmental dysplasia of the hip (DDH). The aim of this study is to determine the DW and country-specific UWs for DDH.

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Temporary hemiepiphysiodesis (HED) is applied to children and adolescents to correct angular deformities (ADs) in long bones through guided growth. Traditional Blount staples or two-hole plates are mainly used for this indication. Despite precise surgical techniques and attentive postoperative follow-up, implant-associated complications are frequently described. To address these pitfalls, a flexible staple was developed to combine the advantages of the established implants. This study provides the first results of guided growth using the new implant and compares these with the established two-hole plates and Blount staples.

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The gold standard for percutaneous Achilles tendon tenotomy during the Ponseti treatment for idiopathic clubfoot is a tenotomy with a No. 15 blade. This trial aims to establish the technique where the tenotomy is performed with a large-bore needle as noninferior to the gold standard.