The purpose of this study was to evaluate the long-term outcome of adults with spina bifida cystica (SBC) who had been treated either operatively or non-operatively for scoliosis during childhood. We reviewed 45 patients with a SBC scoliosis (Cobb angle ≥ 50º) who had been treated at one of two children’s hospitals between 1991 and 2007. Of these, 34 (75.6%) had been treated operatively and 11 (24.4%) non-operatively. After a mean follow-up of 14.1 years (standard deviation (. sd. ) 4.3) clinical, radiological and health-related quality of life (HRQOL) outcomes were evaluated using the Spina Bifida Spine Questionnaire (SBSQ) and the 36-Item Short Form Health Survey (SF-36). Although patients in the two groups were demographically similar, those who had undergone surgery had a larger mean Cobb angle (88.0º (. sd. 20.5; 50.0 to 122.0); versus 65.7º (. sd. 22.0; 51.0 to 115.0); p < 0.01) and a larger mean clavicle–rib intersection difference (12.3 mm; (. sd. 8.5; 1 to 37); versus 4.1 mm, (. sd. 5.9; 0 to 16); p = 0.01) than those treated non-operatively. Both groups were statistically similar at follow-up with respect to walking capacity, neurological motor level, sitting balance and health-related quality of life (HRQOL) outcomes. Spinal fusion in SBC scoliosis corrects coronal deformity and stops progression of the curve but has no clear effect on HRQOL. Cite this article: Bone Joint J 2014; 96-B:1244–51

We report the outcome of 28 patients with spina bifida who between 1989 and 2006 underwent 43 lower extremity deformity corrections using the Ilizarov technique. The indications were a flexion deformity of the knee in 13 limbs, tibial rotational deformity in 11 and foot deformity in 19. The mean age at operation was 12.3 years (5.2 to 20.6). Patients had a mean of 1.6 previous operations (0 to 5) on the affected limb. The mean duration of treatment with a frame was 9.4 weeks (3 to 26) and the mean follow-up was 4.4 years (1 to 9). There were 12 problems (27.9%), five obstacles (11.6%) and 13 complications (30.2%) in the 43 procedures. Further operations were needed in seven patients. Three knees had significant recurrence of deformity. Two tibiae required further surgery for recurrence. All feet were plantigrade and braceable. We conclude that the Ilizarov technique offers a refreshing approach to the complex lower-limb deformity in spina bifida

1. Fifteen patients with spina bifida occulta are described in whom the fifth lumbar spinous process was pressing on the fibrous membrane that closes the spina bifida, or on the bony stumps of the defective lamina. 2. That this is a cause of low back pain, with or without pain referred to the lower limbs, is supported by the findings at operation and the results of treatment. 3. Eleven patients were treated by operation; in each the spinous process of the fifth lumbar vertebra pressed against the spina bifida, either directly or through the vestigial remnant of the first sacral spinous process. 4. In eight cases the fibrous membrane was adherent to the dura mater; in one case the nerve roots were adherent. 5. Excision of the fifth lumbar spinous process and the membrane across the breach of the spina bifida was an effective method of treatment

In spina bifida the femoral neck can develop either the well-known coxa valga or the hitherto unreported coxa vara. Twenty-three cases of coxa vara in spina bifida are reported. These result from spontaneous separation of the upper femoral epiphysis (10 cases), spontaneous fracture of the femoral neck (three cases) and iatrogenic avascular necrosis of the upper femoral epiphysis (10 cases)

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

We reviewed 20 patients with spina bifida who had had surgical management of tibial torsion. Eight had had bilateral procedures and 12 a unilateral procedure, giving a total of 28 limbs for analysis. We performed closed osteoclasis on seven limbs and tibial osteotomy on 21. In the closed osteoclasis group six limbs (85%) had a good result after an average follow-up of nine years (2 to 22). All limbs developed postoperative anteromedial bowing of the tibia which later remodelled. In the tibial osteotomy group 19 (90%) had a good result. The average follow-up was nine years (2 to 28). Complications occurred in seven limbs (33%). We recommend closed osteoclasis of the tibia for the young patient with spina bifida in whom walking is impeded by excessive internal tibial torsion, and supramalleolar tibial osteotomy in the older patient with excessive external tibial torsion and a planovalgus foot

Aims. To report the outcome of spinal deformity correction through anterior spinal fusion in wheelchair-bound patients with myelomeningocele. Methods. We reviewed 12 consecutive patients (7M:5F; mean age 12.4 years (9.2 to 16.8)) including demographic details, spinopelvic parameters, surgical correction, and perioperative data. We assessed the impact of surgery on patient outcomes using the Spina Bifida Spine Questionnaire and a qualitative questionnaire. Results. The mean follow-up was 5.4 years (2 to 14.9). Nine patients had kyphoscoliosis, two lordoscoliosis, and one kyphosis. All patients had a thoracolumbar deformity. Mean scoliosis corrected from 89.6° (47° to 151°) to 46.5° (17° to 85°; p < 0.001). Mean kyphosis corrected from 79.5° (40° to 135°) to 49° (36° to 65°; p < 0.001). Mean pelvic obliquity corrected from 19.5° (8° to 46°) to 9.8° (0° to 20°; p < 0.001). Coronal and sagittal balance restored to normal. Complication rate was 58.3% (seven patients) with no neurological deficits, implant failure, or revision surgery. The degree of preoperative spinal deformity, especially kyphosis and lordosis, correlated with increased blood loss and prolonged hospital/intensive care unit stay. The patients reported improvement in function, physical appearance, and pain after surgery. The parents reported decrease in need for everyday care. Conclusion. Anterior spinal fusion achieved satisfactory deformity correction with high perioperative complication rates, but no long-term sequelae among children with high level myelomeningocele. This resulted in physical and functional improvement and high reported satisfaction. Cite this article: Bone Joint J 2021;103-B(6):1133–1141

A study was made of knee stiffness after fractures around the knee in patients with spina bifida. Thirty-one patients with 45 fractures were followed up for 2 to 15 years after the fracture. Knee stiffness was found in 67% of patients; this amounted to loss of up to half the normal range of movement. The stiffness appeared at two months from the time of the fracture and was established by six months. However, in all patients it had resolved by three years, so that their mobility was not affected in the long term. It is concluded that though stiffness is common after fractures in patients with spina bifida, it should be treated expectantly as it will resolve within three years without specific treatment

Valgus deformity of the hindfoot can occur at the subtalar joint, the ankle joint, or at both sites. In children suffering from spina bifida, the ankle is often the main site of deformity. Thirty-five ankles with valgus deformity of the hindfoot were studied in 23 children with spina bifida. A radiological triad was observed in all patients: shortening of the fibula, lateral wedging of the distal tibial epiphysis, and lateral tilt of the talus at the ankle mortise . There was a definite correlation between the severity of wedging and the degree of talar tilt, and a fair correlation between the severity of wedging and the extent of fibular shortening. The results of operation in 12 feet are presented. It is concluded that any operations performed below the ankle on these patients (subtalar fusion or triple arthrodesis) is unlikely to succeed; the deformity needs to be corrected above the ankle (by epiphysiodesis or supramalleolar osteotomy). Radiological assessment of the ankle by taking weight-bearing films in the anteroposterior plane is essential to determine the true extent of the deformity before undertaking any operation

In a consecutive series of 124 children with spina bifida we found that 220 (89%) of the 248 feet were deformed: 70 had a calcaneus deformity; 126 were in equinus; 16 were in valgus; 3 were in varus; and 5 had convex pes valgus. Operations were performed on 171 (78%) of the deformed feet. Spasticity of the muscles controlling the foot was detected in 36 (51%) of the 70 calcaneus feet and in 22 (17%) of the 126 equinus feet. The deformities were symmetrical in 94 children. There is a high incidence of foot deformity in patients with spina bifida who have no voluntary activity in the motors of the feet

1. A review of 193 African and Indian children suffering from spina bifida has been made. Forty-three were seen on the first day of life and the remainder during subsequent weeks of life. 2. For the baby with mild or moderate paralysis and an open spinal lesion early closure was of value in preventing progressive neural damage. 3. For the baby with severe paralysis and an open myelomeningocele early operation was not of value in preventing further neural damage, and all remained severely paralysed. immediate operation to close the spinal lesion is not justified in babies with severe paralysis: survivors may be treated by later operation to prevent recurrent meningitis

The feet of 13 spina bifida patients who had undergone triple arthrodesis in adolescence were reviewed at an average of 10 years after operation. Fifteen of 18 feet were considered satisfactory (83%); of the remaining three, two had recurrent planovalgus deformities and one a painful pseudarthrosis. Three feet had required revision of the triple arthrodesis, and there was one postoperative infection. No patient had lost ambulatory status as a result of foot problems and eight of the 10 patients who previously needed calipers were able to discard them or to use lighter ones

We reviewed 16 patients with spina bifida and unilateral dislocation of the hip at an average age of 17 years. Nine had a high neurological level (thoracic to L3) and seven a low lesion (L4 to sacral). We assessed the influence of unilateral dislocation of the hip on leg-length discrepancy, hip pain, hip stiffness and pressure sores of the ischial tuberosity. In non-walking patients with high-level lesions, unilateral dislocation gave little functional disability and did not appear to require reduction. In walking patients with low-level lesions, leg-length discrepancy led to a poor gait and functional problems which could be prevented by reduction of the dislocation. In all patients with low lesions, surgery was successful in maintaining reduction; in two of five patients with high lesions it was unsuccessful

1. An enterogenous cyst lying in the cauda equina opposite the third lumbar vertebra, and associated with spina bifida occulta of the fifth lumbar vertebra and spondylolisthesis of the fifth lumbar on the first sacral vertebra, is described in a man aged thirty-five suffering from chronic low back pain and sciatica. 2. Current embryological theories concerning the formation of intraspinal enterogenous cysts from primitive gut cells are further substantiated by the features of this case

1. Paralytic dislocation of the hip in spina bifida generally requires reduction and iliopsoas transfer. The muscle transfer ensures that the hip remains reduced, lessens the need for calipers and prevents progressive flexion deformity. In addition, varus and rotation osteotomy of the femur and innominate osteotomy are sometimes required. Varus and rotation osteotomy alone is indicated if the psoas is not strong enough for transfer. The age for reduction of dislocated hips and muscle transfer has been reduced to eight months as experience and confidence has been gained. 2. Flexion deformity is best prevented by early iliopsoas transfer. An anterior release operation is occasionally indicated when there is flexion deformity and a weak psoas muscle. This procedure, alone or supplemented by extension osteotomy, may be necessary to correct severe flexion deformity in children seen late. 3. Lateral rotation deformity of the hip may be caused by unbalanced iliopsoas action or the unbalanced action, or contracture, of the short lateral rotator muscles. If the deformity is sufficient to make the gait bizarre, or if it is difficult to fit a caliper, then soft-tissue operations as described are of value. 4. Abduction deformity can be corrected by division of the unopposed or contracted abductors

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. A specially designed splint is described with which it is possible to maintain the reduction of a paralytic dislocation in a child with spina bifida cystica. The results of its use in a series of thirteen cases are recorded. 2. It is suggested that all such children presenting in the first year of life, in whom the power of the flexor and adductor muscle groups is preserved, should be treated initially in this way until the prognosis for the individual can be accurately assessed. 3. The theoretical implications of the findings are discussed

We reviewed 52 children, born between 1974 and 1985 with spina bifida affecting L3 and L4, who had dislocated hips. Their motor function was stable and they were able to walk at the time of dislocation. They were interviewed and examined physically and radiologically. Physical function was measured by the Rand Health Insurance Study questionnaire (HIS), the Childhood Health Assessment questionnaire (CHAQ), and by determining the functional level of ambulation according to Hoffer et al (1973). In a subgroup of 12 patients with L4 level of involvement from both treatment groups we measured the metabolic energy consumption while walking. Thirty patients (49 hips) had been treated by operative relocation and 22 conservatively. Ten of the hips treated by operation subluxated or redislocated. The function in the two groups (conservative v operated) was similar (HIS score 7.8 v 8.0, p = 0.45; CHAQ 14 v 13, p = 0.2; level of mobility 0.61 v 0.63, p = 0.5). Patients in whom operation had failed had worse function than did those with successful surgery (HIS score 8.8 v 6.1, p = 0.025) and those with successful surgery had better function than patients treated conservatively (HIS score 8.8 v 8.0, p = 0.15). Function in patients with failed operations, however, was worse than in those who did not have surgical treatment (HIS score 6.6 v 7.8, p = 0.07). In the 12 so examined the operated group had a 30% more energy-efficient gait (0.271 v 0.361 ml O. 2. kg/m, p = 0.05). Patients with failed operations had worse function than those who were not operated on. The benefit of surgical relocation of the dislocated hips was marginal

Aims

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.

Diastematomyelia is a rare congenital abnormality of the spinal cord. This paper summarises more than 30 years’ experience of treating this condition. Data were collected retrospectively on 138 patients with diastematomyelia (34 males, 104 females) who were treated at our hospital from May 1978 to April 2010. A total of 106 patients had double dural tubes (type 1 diastematomyelia), and 32 patients had single dural tubes (type 2 diastematomyelia). Radiographs, CT myelography, and MRI showed characteristic kyphoscoliosis, widening of the interpedicle distance, and bony, cartilaginous, and fibrous septum. The incidences of symptoms including characteristic changes of the dorsal skin, neurological disorders, and congenital spinal or foot deformity were significantly higher in type 1 than in type 2. Surgery is more effective for patients with type 1 diastematomyelia; patients without surgery showed no improvement.

Aims. We investigated the long-term performance of the Tripolar Trident acetabular component used for recurrent dislocation in revision total hip arthroplasty. We assessed: 1) rate of re-dislocation; 2) incidence of complications requiring re-operation; and 3) Western Ontario and McMaster Universities osteoarthritis index (WOMAC) pain and functional scores. Patients and Methods. We retrospectively identified 111 patients who had 113 revision tripolar constrained liners between 1994 and 2008. All patients had undergone revision hip arthroplasty before the constrained liner was used: 13 after the first revision, 17 after the second, 38 after the third, and 45 after more than three revisions. A total of 75 hips (73 patients) were treated with Tripolar liners due to recurrent instability with abductor deficiency, In addition, six patients had associated cerebral palsy, four had poliomyelitis, two had multiple sclerosis, two had spina bifida, two had spondyloepiphyseal dysplasia, one had previous reversal of an arthrodesis, and 21 had proximal femoral replacements. The mean age of patients at time of Tripolar insertions was 72 years (53 to 89); there were 69 female patients (two bilateral) and 42 male patients. All patients were followed up for a mean of 15 years (10 to 24). Overall, 55 patients (57 hips) died between April 2011 and February 2018, at a mean of 167 months (122 to 217) following their tripolar liner implantation. We extracted demographics, implant data, rate of dislocations, and incidence of other complications. Results. At ten years, the Kaplan–Meier survivorship for dislocation was 95.6% (95% confidence interval (CI) 90 to 98), with 101 patients at risk. At 20 years, the survivorship for dislocation was 90.6% (95% CI 81.0 to 95.5), with one patient at risk. Eight patients (7.2%) had a dislocation of their constrained liners. At ten years, the survival to any event was 89.4% (95% CI 82 to 93.8), with 96 patients at risk. At 20 years, the survival to any event was 82.5% (95% CI 71.9 to 89.3), with one patient at risk. Five hips (4.4%) had deep infection. Two patients (1.8%) developed dissociated constraining rings with pain but without dislocation, which required re-operation. Two patients (1.8%) had periprosthetic femoral fractures, without dislocation, that were treated by revision stems along with exchange of the well-functioning constrained liners. Conclusion. Constrained tripolar liners used at revision hip arthroplasty provided favourable results in the long term for treatment of recurrent dislocation and for patients at high risk of dislocation. Cite this article: Bone Joint J 2019;101-B(6 Supple B):123–126

1. It has been shown in fifty normal feet that the perpendicular static plantar pressures rise by about 35 per cent between the ages of five years and maturity. 2. It is very likely that similar increases in plantar pressures occur between two and five years of age. 3. The plantar pressures in seventy-eight feet of children with spina bifida were with a few exceptions substantially higher than those of normal children of the same age. It is likely that this is so, at least in part, because the feet of children with spina bifida are smaller than normal. 4. This increase in perpendicular static pressure during growth must be relevant in the production of trophic foot ulceration. 5. The existence and importance of "flail deformity" as a factor producing areas of increased pressure and ulceration in anaesthetic feet is postulated

A radiographic suvey has been carried out of 147 first-degree relatives of forty-seven patients treated in Edinburgh for spondylolisthesis of the fifth lumbar vertebra; twelve patients had the dysplastic (congenital) type and thirty-five an isthmic defect. The survey identified 19 per cent of relatives with spondylolysis, and index patients with each type of spondylolisthesis had relatives with the opposite type. Index patients with the dysplastic form had a higher proportion of affected relatives (33 per cent) than had those with the isthmic type (15 per cent), but both figures were significantly in excess of the estimated frequency for the general population of under 1 per cent and 5 per cent respectively. Spina bifida occulta at the fifth lumbar or first sacral level or both, and lumbosacral segmental defects were commoner amongst all individuals with spondylolysis than amongst unaffected relatives (dysplastic form 94 per cent, isthmic type 32 per cent, unaffected relatives 7 per cent). However, there was no single instance of a neural tube defect (anencephaly, spina bifida with or without meningocele, other generalised vertebral anomalies or spinal dysraphism) amongst 826 first-, second- or third-degree relatives. It is concluded that the developmental defects of the vertebrae associated with spondylolysis are not aetiologically related to the neural tube defects. The one in three risk of spondylolysis to near relatives of patients with the dysplastic form of spondylolisthesis is emphasised in order that the deformity in their sibs and children can be recognised at any early age

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.

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.

1. The nature of paralytic deformity arising in poliomyelitis, cerebral palsy and spina bifida is considered and three types of deformity–acute contracture, postural contracture and deformity from muscle imbalance are described. 2. The place of physiotherapy, splintage and surgery in the management of these varieties of paralytic deformity is discussed and the overall results of treatment are reviewed

A method of triple arthrodesis is described which involves inlay of the subtalar and midtarsal joints. It is applicable to the undeformed and valgus foot as is encountered in poliomyelitis, spasmodic flat foot, cerebral palsy and spina bifida. The operation was successful in controlling deformity and pain. The only significant complication was failure of fusion of the midtarsal joint which occurred in three of eighty-five feet (3-5%)

Four children are described, each with spontaneous osteonecrosis affecting nearly one-third of the lateral femoral condyle. All the children had a motor and a sensory deficit in the affected limb: two had been previously treated for neuroblastoma of the spine, one for an infected lumbar dermoid cyst and one had spina bifida. We consider that these disorders, singly or in combination, may lead to repeated excessive loading of the lateral femoral condyle, which cannot be appreciated in a knee that is not protected by normal sensation

1. The problem of paralytic hip instability has been studied in a series of twenty-one patients brought to a spina bifida clinic. 2. Thirty iliopsoas tendon transfers were done in an attempt to reduce deformity and improve hip stability. 3. At the time of review, ten of these hips were stable and twenty were unstable. Ten hips were improved by operation, and one hip was worse. The other nineteen remained the same. 4. All of the children except one were capable of walking with an orthotic device. 5. Some of the complexities of the problem of paralytic hip instability in the patient with myelomeningocele are discussed

1. Nineteen patients with spina bifida, myelomeningocele or lipoma of the cauda equina have been reviewed. Convex pes valgus was found in twenty-five feet. All patients had a neuromuscular imbalance between the evertors and invertors of the foot. 2. Results of release procedures only and of those which combine release procedures with tendon transfers are compared; they show that there is no consistently satisfactory operation for correction of the deformity when it exists with neuromuscular imbalance. 3. An operation in which release procedures are combined with the transfer of the peroneus brevis to the tibialis posterior and of the tibialis anterior to the neck of the talus is described. It has given satisfactory results in two out of three patients with paralytic convex pes valgus

The lumbar spines of 485 skeletons of adult South African "Whites" and "Blacks" of both sexes were examined to determine the incidence and morphology of defects in the pars interarticularis. The overall incidence was 3.5%, without significant variation between races and sexes. The incidence of lumbar spina bifida in the whole sample was 1.9%, but was 11.8% in those skeletons with spondylolysis. Some instances of unilateral spondylolysis demonstrated obvious callus formation, suggesting a capability for normal repair. It is possible that the defects in bilateral cases represent established non-union of fractures of the pars interarticularis resulting from excessive mobility, and are not due to dysplasia of bone. It is noted, for the first time, that the superior facets of affected vertebrae are abnormally enlarged, and that the inferior facets of the separate neural arch are characteristically elliptical

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

1. A syndrome resulting from congenital lesions affecting the spinal cord and cauda equina, associated with spina bifida occulta, is described. 2. The syndrome consists of a progressive deformity of the lower limbs in children. One foot and the same leg grow less rapidly than the other. The foot develops a progressive deformity which begins as a cavo-varus and becomes a valgus one. Both lower limbs may be affected. There may be progression to sensory loss, trophic ulceration, disturbance of function of bowel and bladder and even paraplegia. 3. Methods of investigation including myelography are described. 4. Exploration of the spinal cord has been undertaken in twenty-four patients so affected. Extrinsic congenital lesions causing traction or pressure or a combination of traction and pressure on the spinal cord have been found in twenty-two of these. 5. In two-thirds of the patients some degree of improvement has followed operation

Of 47 patients with spina bifida who had had transplantation of the iliopsoas more than 10 years previously, 32 (68%) were community walkers, 3 were household walkers and 12 were non-walkers. Comparison with other published reports showed that, at the very least, the patients reviewed had not had their walking ability jeopardized by the inevitable loss of hip flexor power. Furthermore, all but three of the community walkers were able to climb and descend stairs. There was a high proportion of non-walkers in those patients whose operation had been performed in the first year of life and such early surgery is no longer recommended. We also found that the pre-operative assessment of muscle power had, in some patients, been inaccurate. Finally, we found that, at review, the power of the transferred muscles was poor, suggesting that transplantation is beneficial because it achieves permanent and major reduction in hip flexor power; this usually prevents recurrent hip flexion deformity and dislocation

This is a study of children who first attended as infants with either progressive infantile idiopathic scoliosis or congenital scoliosis. All had a pattern of scoliosis in which early and damaging deterioration is inevitable. The infants were treated from before the age of three, initially by plaster casts and then a Milwaukee brace, followed at about the age of ten by correction and fusion. The cases were then observed to the end of growth or near that point. In the main study there were twelve cases, six of progressive infantile idiopathic scoliosis and six of congenital scoliosis, which were followed through this long period. Only one of the twelve had a curve worse at the end of growth compared with the initial radiograph as an infant; this one curve had increased only 16 degrees in almost as many years. Although small, the series does show that it is nearly always possible to control even the most serious scoliosis in an infant, if it is tackled early and unremittingly. There are supportive studies of children who have partially completed this regime, and interim results in a newer group of children with spina bifida and scoliosis

The orthopaedic surgeon is often the first consultant to whom a patient with syringomyelia is referred. The disease is not as rare as he may suppose, but its early presenting features are very variable; if he relies solely on such familiar features as pes cavus and scoliosis, he may well miss the diagnosis. The commonest presenting symptom is pain in the head, neck, trunk or limbs; headache or neckache made worse by straining is particularly significant. A history of birth injury also may suggest the possibility of syringomyelia, especially if any spasticity subsequently worsens. Neurological features which may be diagnostic include nystagmus, dissociated sensory loss, muscle wasting, spasticity of the lower limbs or Charcot's joints. Radiographic features include erosion of the bodies of cervical vertebrae and widening of the spinal canal; if, at C5, the size of the canal exceeds that of the body by 6 millimetres in the adult, pathological dilatation is present. The presence of basilar invagination or other abnormalities of the foramen magnum, of spina bifida occulta and of scoliosis are further pointers. Thermography is a useful way of showing asymmetrical sympathetic involvement in early cases. A greater awareness of the prevalence of syringomyelia may lead to earlier diagnosis and to early operation, which appears to hold out the best hope of arresting what is all too commonly a severely disabling and progressive condition

Aims

The study was undertaken to compare the efficacy of Woodcast splints and plaster-of-Paris casts in maintaining correction following sequential manipulation of idiopathic clubfeet.

Aims

To our knowledge, there is currently no information available about the rate of venous thromboembolism (VTE) or recommendations regarding chemoprophylaxis for patients whose lower limb is immobilized in a plaster cast. We report a retrospective case series assessing the rate of symptomatic VTE in patients treated with a lower limb cast. Given the complex, heterogeneous nature of this group of patients, with many risk factors for VTE, we hypothesized that the rate of VTE would be higher than in the general population.

Aims

We reviewed 34 consecutive patients (18 female-16 male) with isthmic spondylolysis and grade I to II lumbosacral spondylolisthesis who underwent in situ posterolateral arthodesis between the L5 transverse processes and the sacral ala with the use of iliac crest autograft. Ten patients had an associated scoliosis which required surgical correction at a later stage only in two patients with idiopathic curves unrelated to the spondylolisthesis.

Aims

The primary aim of this study was to evaluate the performance and safety of magnetically controlled growth rods in the treatment of early onset scoliosis. Secondary aims were to evaluate the clinical outcome, the rate of further surgery, the rate of complications, and the durability of correction.

Aims

The purpose of this study was to analyze the incidence of the different ultrasound phenotypes of developmental dysplasia of the hip (DDH), and to determine their subsequent course.

Patients with neuromuscular imbalance who require total hip arthroplasty (THA) present particular technical problems due to altered anatomy, abnormal bone stock, muscular imbalance and problems of rehabilitation.

In this systematic review, we studied articles dealing with THA in patients with neuromuscular imbalance, published before April 2017. We recorded the demographics of the patients and the type of neuromuscular pathology, the indication for surgery, surgical approach, concomitant soft-tissue releases, the type of implant and bearing, pain and functional outcome as well as complications and survival.

Recent advances in THA technology allow for successful outcomes in these patients. Our review suggests excellent benefits for pain relief and good functional outcome might be expected with a modest risk of complication.

Cite this article: Bone Joint J 2018;100-B(1 Supple A):17–21.

A review of the current literature shows that there is a lack of consensus regarding the treatment of spondylolysis and spondylolisthesis in children and adolescents. Most of the views and recommendations provided in various reports are weakly supported by evidence. There is a limited amount of information about the natural history of the condition, making it difficult to compare the effectiveness of various conservative and operative treatments. This systematic review summarises the current knowledge on spondylolysis and spondylolisthesis and attempts to present a rational approach to the evaluation and management of this condition in children and adolescents.

Lumbar disc herniation (LDH) is uncommon in youth and few cases are treated surgically. Very few outcome studies exist for LDH surgery in this age group. Our aim was to explore differences in gender in pre-operative level of disability and outcome of surgery for LDH in patients aged ≤ 20 years using prospectively collected data.

From the national Swedish SweSpine register we identified 180 patients with one-year and 108 with two-year follow-up data ≤ 20 years of age, who between the years 2000 and 2010 had a primary operation for LDH.

Both male and female patients reported pronounced impairment before the operation in all patient reported outcome measures, with female patients experiencing significantly greater back pain, having greater analgesic requirements and reporting significantly inferior scores in EuroQol (EQ-5D-index), EQ-visual analogue scale, most aspects of Short Form-36 and Oswestry Disabilities Index, when compared with male patients. Surgery conferred a statistically significant improvement in all registered parameters, with few gender discrepancies. Quality of life at one year following surgery normalised in both males and females and only eight patients (4.5%) were dissatisfied with the outcome. Virtually all parameters were stable between the one- and two-year follow-up examination.

LDH surgery leads to normal health and a favourable outcome in both male and female patients aged 20 years or younger, who failed to recover after non-operative management.

Cite this article: Bone Joint J 2015;97-B:1675–82.

A combination of hemivertebrae and diastematomyelia is rare. We have identified 12 such patients seen during a period of 11 years in the orthopaedic, spinal and neurosurgical units in Nottingham and analysed their treatment and outcome.

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.

Noise generation has been reported with ceramic-on-ceramic articulations in total hip replacement (THR). This study evaluated 208 consecutive Delta Motion THRs at a mean follow-up of 21 months (12 to 35). There were 141 women and 67 men with a mean age of 59 years (22 to 84). Patients were reviewed clinically and radiologically, and the incidence of noise was determined using a newly described assessment method. Noise production was examined against range of movement, ligamentous laxity, patient-reported outcome scores, activity level and orientation of the acetabular component. There were 143 silent hips (69%), 22 (11%) with noises other than squeaking, 17 (8%) with unreproducible squeaking and 26 (13%) with reproducible squeaking. Hips with reproducible squeaking had a greater mean range of movement (p < 0.001) and mean ligament laxity (p = 0.004), smaller median head size (p = 0.01) and decreased mean acetabular component inclination (p = 0.02) and anteversion angle (p = 0.02) compared with the other groups. There was no relationship between squeaking and age (p = 0.13), height (p = 0.263), weight (p = 0.333), body mass index (p = 0.643), gender (p = 0.07) or patient outcome score (p = 0.422). There were no revisions during follow-up. Despite the surprisingly high incidence of squeaking, all patients remain satisfied with their hip replacement.

Cite this article: Bone Joint J 2013;95-B:160–5.

The incidence of acute and chronic conditions of the tendo Achillis appear to be increasing. Causation is multifactorial but the role of inherited genetic elements and the influence of environmental factors altering gene expression are increasingly being recognised. Certain individuals’ tendons carry specific variations of genetic sequence that may make them more susceptible to injury. Alterations in the structure or relative amounts of the components of tendon and fine control of activity within the extracellular matrix affect the response of the tendon to loading with failure in certain cases.

This review summarises present knowledge of the influence of genetic patterns on the pathology of the tendo Achillis, with a focus on the possible biological mechanisms by which genetic factors are involved in the aetiology of tendon pathology. Finally, we assess potential future developments with both the opportunities and risks that they may carry.

Cite this article: Bone Joint J 2013;95-B:305–13.

Neurological conditions affecting the hip pose a considerable challenge in replacement surgery since poor and imbalanced muscle tone predisposes to dislocation and loosening. Consequently, total hip replacement (THR) is rarely performed in such patients. In a systematic review of the literature concerning THR in neurological conditions, we found only 13 studies which described the outcome. We have reviewed the evidence and discussed the technical challenges of this procedure in patients with cerebral palsy, Parkinson’s disease, poliomyelitis and following a cerebrovascular accident, spinal injury or development of a Charcot joint. Contrary to traditional perceptions, THR can give a good outcome in these often severly disabled patients.

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.

We reviewed retrospectively 45 patients (46 procedures) with bladder exstrophy treated by bilateral oblique pelvic osteotomy in conjunction with genitourinary repair.

The operative technique and post-operative management with or without external fixation are described. A total of 21 patients attended a special follow-up clinic and 24 were interviewed by telephone. The mean follow-up time was 57 months (24 to 108).

Of the 45 patients, 42 reported no pain or functional disability, although six had a waddling gait and two had marked external rotation of the hip. Complications included three cases of infection and loosening of the external fixator requiring early removal with no deleterious effect. Mid-line closure failed in one neonate managed in plaster. This patient underwent a successful revision procedure several months later using repeat osteotomies and external fixation.

The percentage pubic approximation was measured on anteroposterior radiographs pre-operatively, post-operatively and at final follow-up. The mean approximation was 37% (12% to 76%). It varied markedly with age and was better when external fixation was used. The wide range reflects the inability of the anterior segment to develop naturally in spite of close approximation at operation.

We conclude that bilateral oblique pelvic osteotomy with or without external fixation is useful in the management of difficult primary closure in bladder exstrophy, failed primary closure and secondary reconstruction.

Subluxation of the hip is common in patients with intermediate spinal muscular atrophy. This retrospective study aimed to investigate the influence of surgery on pain and function, as well as the natural history of subluxed hips which were treated conservatively. Thirty patients were assessed clinically and radiologically. Of the nine who underwent surgery only one reported satisfaction and four had recurrent subluxation. Of the 21 patients who had no surgery, 18 had subluxation at the latest follow-up, but only one reported pain in the hip. We conclude that surgery for subluxation of the hip in these patients is not justified.

Talipes equinovarus is one of the more common congenital abnormalities affecting the lower limb and can be challenging to manage. This review provides a comprehensive update on idiopathic congenital talipes equinovarus with emphasis on the initial treatment. Current management is moving away from operative towards a more conservative treatment using the Ponseti regime. The long-term results of surgical correction and the recent results of conservative treatment will be discussed.