Sixty patients with congenital deformities of the spine were operated upon in the past fifteen years using a two-stage procedure. In the fifty patients with scoliosis half of the deformities were due to hemivertebrae and half to unilateral bars. The average correction of the deformity was 47 per cent. Early neurological signs observed in two patients with a diastematomyelia resolved. Of the ten patients with kyphosis nine had neurological signs of impending paraplegia and one was completely paraplegic before operation; all improved markedly. Posterior spinal fusion alone in the rapidly progressing congenital deformity may not prevent further progression, particularly in those cases iwth unilateral bars. Anterior resection of the vertebral body with later posterior fusion with Harrington instrumentation is safe and effective

A pronation deformity of the forearm following an obstetric brachial plexus injury causes functional and cosmetic disability. We evaluated the results of pronator teres transfer to correct their deformity in 14 children treated over a period of four years. The mean age at surgery was 7.6 years (5 to 15). The indication for surgery in each case was impairment of active supination in a forearm that could be passively supinated provided that there was no medial contracture of the shoulder and normal function of the hand. The median follow-up was 20.4 months (8 to 42). No patient was lost to follow-up. Qualitative results were also assessed. The median active supination improved from 5° (0° to 10°) to 75° (70° to 80°) with no loss of pronation. A passively correctible pronation contracture can be corrected safely and effectively by the transfer of pronator teres

Patients with spina bifida cystica commonly have significant disability from a combination of valgus deformity of the ankle and subtalar joints with lateral tibial torsion and plano-abduction deformity of the foot. These deformities can be corrected by a single procedure which combines a supramalleolar tibial osteotomy with a lateral inlay triple fusion. This procedure was carried out on 20 feet in 15 patients and the results were reviewed after an average of three years (range 18 months to 7 years). In 75 per cent of feet the combination of deformities was fully corrected, ulcers and callosities were eliminated in 95 per cent, the use of calipers minimised in 95 per cent, and in all patients the problem of shoe-wrecking was reduced. Complications included recurrent valgus deformity, delayed union of the tibial osteotomy and failure of midtarsal fusion

We determined the frequency, rate and extent of development of scoliosis (coronal plane deformity) in wheelchair-dependent patients with Duchenne muscular dystrophy (DMD) who were not receiving steroid treatment. We also assessed kyphosis and lordosis (sagittal plane deformity). The extent of scoliosis was assessed on sitting anteroposterior (AP) spinal radiographs in 88 consecutive non-ambulatory patients with DMD. Radiographs were studied from the time the patients became wheelchair-dependent until the time of spinal fusion, or the latest assessment if surgery was not undertaken. Progression was estimated using a longitudinal mixed-model regression analysis to handle repeated measurements. Scoliosis ≥ 10° occurred in 85 of 88 patients (97%), ≥ 20° in 78 of 88 (89%) and ≥ 30° in 66 of 88 patients (75%). The fitted longitudinal model revealed that time in a wheelchair was a highly significant predictor of the magnitude of the curve, independent of the age of the patient (p <  0.001). Scoliosis developed in virtually all DMD patients not receiving steroids once they became wheelchair-dependent, and the degree of deformity deteriorated over time. In general, scoliosis increased at a constant rate, beginning at the time of wheelchair-dependency (p < 0.001). In some there was no scoliosis for as long as three years after dependency, but scoliosis then developed and increased at a constant rate. Some patients showed a rapid increase in the rate of progression of the curve after a few years – the clinical phenomenon of a rapidly collapsing curve over a few months. A sagittal plane kyphotic deformity was seen in 37 of 60 patients (62%) with appropriate radiographs, with 23 (38%) showing lumbar lordosis (16 (27%) abnormal and seven (11%) normal). This study provides a baseline to assess the effects of steroids and other forms of treatment on the natural history of scoliosis in patients with DMD, and an approach to assessing spinal deformity in the coronal and sagittal planes in wheelchair-dependent patients with other neuromuscular disorders. Cite this article: Bone Joint J 2014;96-B:100–5

We reviewed 36 cases of forearm deformity caused by multiple osteochondromas in 30 patients and classified them into three types: Type I showed a combination of ulnar shortening and bowing of the radius secondary to osteochondromas of the distal ulna (22 forearms). Type II showed dislocation of the radial head, either with osteochondromas of the proximal radius (Type IIa, two forearms) or secondary to more distal involvement (Type IIb, five forearms). Type III had relative radial shortening due to osteochrondromas at the distal radius (seven forearms). Operations were performed on 16 forearms in 13 patients, with 92% of satisfactory results. For Type I deformity, excision of osteochondromas, immediate ulnar lengthening and corrective osteotomy of the radius are recommended. For Type IIa, excision of the radial head is necessary, and for Type IIb, we advise gradual lengthening of the ulna using an external fixator. Excision of osteochondromas alone gave good results in Type III deformity. Our classification gives a reliable indication of the prognosis and is a guide to the choice of surgical treatment

Patients with acetabular dysplasia commonly undergo peri-acetabular osteotomy after skeletal maturity to reduce the risk of the late development of osteoarthritis. Several studies have suggested that deformity of the femoral head influences the long-term outcome. We radiologically examined 224 hips in 112 patients with acetabular dysplasia and early-stage osteoarthritis. There were 103 women and nine men with a mean age of 37.6 years (18 to 49). A total of 201 hips were placed in the acetabular dysplasia group and 23 in a normal group. The centre–edge angle and acetabular head index were significantly smaller (both p < 0.001), and the acetabular angle, acetabular roof angle and roundness index were significantly greater in the acetabular dysplasia group than those in the normal group (all p < 0.001). There were significant correlations between the roundness index and other parameters. Femoral head shape may be influenced by the severity of the acetabular dysplasia. Cite this article: Bone Joint J 2013;95-B:1192–6

We reviewed the results of a selective à la carte soft-tissue release operation for recurrent or residual deformity after initial conservative treatment for idiopathic clubfoot by the Ponseti method. Recurrent or residual deformity occurred in 13 (19 feet) of 33 patients (48 feet; 40%). The mean age at surgery was 2.3 years (1.3 to 4) and the mean follow-up was 3.6 years (2 to 5.3). The mean Pirani score had improved from 2.8 to 1.1 points, and the clinical and radiological results were satisfactory in all patients. However, six of the 13 patients (9 of 19 feet) had required further surgery in the form of tibial derotation osteotomy, split anterior tibialis tendon transfer, split posterior tibialis transfer or a combination of these for recurrent deformity. We concluded that selective soft-tissue release can provide satisfactory early results after failure of initial treatment of clubfoot by the Ponseti method, but long-term follow-up to skeletal maturity will be necessary

Joint deformity secondary to extensive haemangiomatous involvement of the soft tissues has been well described and is easy to diagnose. If the haemangioma is small, localised and within the belly of a muscle the diagnosis is more difficult. In equinus deformity of obscure aetiology localised calf tenderness may be the only diagnostic sign. Three children with equinus deformity caused by a small haemangioma in the calf muscles were treated by simple excision with satisfactory results

We reviewed the results of anterior hip release for fixed flexion deformity in 57 hips in 38 children with spina bifida at an average follow-up of 8.9 years (2 to 22). The indication for this operation was a fixed flexion deformity of more than 30° which interfered with function. In 43 hips there was a good outcome in that the fixed flexion deformity remained less than 30° at follow-up. Four hips had a good initial result but deteriorated after an average of five years, and ten had a poor outcome with deformity of over 30°. Six hips required a repeated anterior hip release and two of these were successful. The success of anterior hip release could not be related to the neurological level or the age at operation. Successful surgery correlated with the walking ability of the child at the latest follow-up

1. Correction of equinus deformity in cerebral palsy either by elongation of the tendo calcaneus or by gastrocnemius recession gives satisfactory results without splintage or bracing after operation. 2. Gastrocnemius recession is the operation of choice in paraplegic spastic cerebral palsy, and wherever possible in tetraplegic cerebral palsy. In hemiplegia the whole of the triceps surae is usually involved, and elongation of the tendo calcaneus is almost always needed. 3. Correction at operation should aim to result in a mild degree of equinus deformity at the end of the growth period in hemiplegic spastic cerebral palsy. Over-enthusiastic gastrocnemius recession can lead to excessive weakness of the calf in some patients

We reviewed 24 feet in 15 patients who had undergone talectomy for recurrent equinovarus deformity; 21 were associated with arthrogryposis multiplex congenita, two with myelomeningocele and one with idiopathic congenital talipes equinovarus. The mean follow-up was 20 years. Good results were achieved in eight feet (33%) in which further surgery was not needed and walking was painless; a fair result was obtained in ten feet (42%) in which further surgery for recurrence of a hindfoot deformity had been necessary but walking was painless; the remaining six feet (25%) were poor, with pain on walking. All patients wore normal shoes and could walk independently, except one who was wheelchair-bound because of other joint problems. Recurrent deformity, the development of tibiocalcaneal arthritis and spontaneous fusion of the tibia to the calcaneum were all seen in these patients

We report a prospective case-controlled study which compared the outcome of knee replacement in seriously deformed and slightly deformed knees. There were 51 knees with varus or valgus deformity greater than 20 degrees matched for age, gender, disease, type of prosthesis and time of operation with a control group in which the alignment of the leg was within 5 degrees of normal. The clinical outcome at a mean 5.5 years was similar in the two groups. Some deformity persisted in 14 patients in the first group, 13 of whom were initially in valgus. These patients had a significantly poorer mean clinical outcome. Lateral dislocation or subluxation of the patella was found in four knees, all of which had had valgus deformity of 30 degrees or more

Equinus deformity of the ankle is one of the serious orthopaedic problems associated with Duchenne muscular dystrophy. Sixty-nine patients (age range 4 to 17 years) were treated, 43 conservatively and 26 operatively. They were followed up at six-monthly intervals for a minimum of two years and a maximum of six years. The patients were divided into three groups: independently mobile, mobile in calipers, and wheelchair-bound. It was found that conservative treatment could at best only minimise progression of the deformity. The indications for surgery, the operative procedure and the postoperative management are described; all varied according to the stage of the disease. The postoperative follow-up suggests that, though the deformity recurs, the patients have several years of benefit from the procedure

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

Segmental vessel ligation during anterior spinal surgery has been associated with paraplegia. However, the incidence and risk factors for this devastating complication are debated. We reviewed 346 consecutive paediatric and adolescent patients ranging in age from three to 18 years who underwent surgery for anterior spinal deformity through a thoracic or thoracoabdominal approach, during which 2651 segmental vessels were ligated. There were 173 patients with idiopathic scoliosis, 80 with congenital scoliosis or kyphosis, 43 with neuromuscular and 31 with syndromic scoliosis, 12 with a scoliosis associated with intraspinal abnormalities, and seven with a kyphosis. There was only one neurological complication, which occurred in a patient with a 127° congenital thoracic scoliosis due to a unilateral unsegmented bar with contralateral hemivertebrae at the same level associated with a thoracic diastematomyelia and tethered cord. This patient was operated upon early in the series, when intra-operative spinal cord monitoring was not available. Intra-operative spinal cord monitoring with the use of somatosensory evoked potentials alone or with motor evoked potentials was performed in 331 patients. This showed no evidence of signal change after ligation of the segmental vessels. In our experience, unilateral segmental vessel ligation carries no risk of neurological damage to the spinal cord unless performed in patients with complex congenital spinal deformities occurring primarily in the thoracic spine and associated with intraspinal anomalies at the same level, where the vascular supply to the cord may be abnormal

We performed limb lengthening and correction of deformity of nine long bones of the lower limb in six children (mean age, 14.7 years) with osteogenesis imperfecta (OI). All had femoral lengthening and three also had ipsilateral tibial lengthening. Angular deformities were corrected simultaneously. Five limb segments were treated using a monolateral external fixator and four with the Ilizarov frame. In three children, lengthening was done over previously inserted femoral intramedullary rods. The mean lengthening achieved was 6.26 cm (mean healing index, 33.25 days/cm). Significant complications included one deep infection, one fracture of the femur and one anterior angulation deformity of the tibia. The abnormal bone of OI tolerated the external fixators throughout the period of lengthening without any episodes of migration of wires or pins through the soft bone. The regenerate bone formed within the time which is normally expected in limb-lengthening procedures performed for other conditions. We conclude that despite the abnormal bone characteristics, distraction osteogenesis to correct limb-length discrepancy and angular deformity can be performed safely in children with OI

1. The indications for talectomy in the treatment of equinovarus deformity in arthrogryposis multiplex congenita and spina bifida are discussed. 2. The technique of the operation is described, with variations which may be necessary in special circumstances. 3. The results of forty-one operations are analysed. 4. It is concluded that the operation has a useful place in the management of equinovarus deformity in arthrogryposis multiplex congenita and spina bifida, especially between the ages of one and five years

Spasm or contracture of gastrocnemius causes an equinus deformity of the ankle in both cerebral palsy and hemiplegia. Its release is therefore required in the treatment of those patients who do not respond to conservative measures. The Vulpius procedure is a simple and effective method for the release of gastrocnemius and is particularly indicated when long periods of immobilisation of the foot and ankle are not desirable. We have used this procedure with good results to correct an equinus deformity in 230 adults with a cerebrovascular accident and various associated medical conditions. It is not only effective in cerebral palsy, but should be considered at an early stage in all adult patients with deformity of the ankle and foot in whom spasm of gastrocnemius is the major cause

We describe three patients who developed gross calcaneus deformity following surgery for talipes equinovarus. One also had an associated valgus deformity and another had supination of the forefoot; all had intractable problems with footwear. Operation for transfer of the tibialis anterior to the heel, with correction of the associated deformities, was successful and improved both their gait and the shoe problems

We reviewed our experience of tibialis anterior transfer and anterior release for calcaneus deformity in 46 feet of 26 ambulant patients with myelomeningocele. At an average follow-up of 8.4 years (2 to 17.6) there were 89% who had satisfactory results; 64% of the patients having tibialis anterior transfers were able to stand on their toes. Hip abductor power was a good predictor of a functional transfer. Pre-operative trophic ulceration of the heel increased from 3.2% to 33% if surgery was delayed. Secondary deformities, two-thirds of them into valgus, developed in 76% of feet