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

We studied 1061 children with myelomeningocele, reviewing 3184 pelvic radiographs from 802 patients. Hip dislocation had occurred by the age of 11 years in 28% of children with a thoracic neurosegmental level, 30% of those with an L1/2 level, 36% of L3, 22% of L4, 7% of L5 and only 1% of those with sacral levels. Hip dislocation was not inevitable even when there was maximal muscle imbalance about the hip. The average hip flexion contracture in children aged 9 to 11 years was significantly greater in those with thoracic (22 degrees) and L1/2 (33 degrees) levels than in those with L4 (9 degrees), L5 (5 degrees) or sacral (4 degrees) levels. Our findings indicate that muscle imbalance is not a significant factor in the production of flexion deformity or dislocation of the hip; both are commonly seen in the absence of imbalance. The restoration of muscle balance should no longer be considered to be the principal aim of the management of the hip in children with myelomeningocele

High myelomeningocele lesions do not preclude an acceptable level of functional walking provided that an integrated programme of surgical treatment and bracing is adopted. Clinical analysis of 100 patients with myelomeningocele shows that the development of the "swivel walker" and "hip guidance orthosis" has been associated with an improved level of function. Over 30 per cent of patients with thoracic lesions and 68 per cent of those with lumbar lesions achieved independent walking. For this reason the criteria used at present by paediatricians to govern the selection of infants for non-active treatment may require reconsideration

We report the results of a prospective study of the surgical release of 45 knee flexion contractures in 28 patients with myelomeningocele. The neurosegmental level was thoracic in ten patients, L1/2 in one, L3/4 in 11, and L5/S1 in six. In walkers the indication for surgery was a fixed flexion contracture impeding walking, and in non-walking patients it was a flexion contracture impeding transfers or sitting balance, or likely to do so with increasing deformity. The mean age at surgery was 6.4 years (3 to 21) and the mean period of follow-up 13 years (4 to 20). The mean knee flexion contracture before surgery was 39° (25 to 70) which improved to 5° at maximum correction and to 13° at latest follow-up. We conclude that surgical release of knee flexion contractures in myelomeningocele improves gait in all children who walk, particularly those with low lumbar lesions. Recurrence of knee flexion contractures after surgical release is most common in those with thoracic lesions who do not achieve independent walking

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

We reviewed 55 patients with mid-lumbar myelomeningocele (L3 and L4) first seen over a 17-year period from 1970 to 1986 and followed up for an average of ten years. We assessed a number of factors which might affect hip stability and ability to walk, recording the natural history of clinical and radiological hip deformity. Two-thirds of the hips had become dislocated or subluxed by the end of the first year of life, involving 86% of hips in patients with an L3 level and 45% of those with an L4 level. All the hips that developed instability secondary to muscle imbalance did so within the first year. The neurological level was the most significant determinant of walking ability: all patients with L4 neurological levels could walk but only one-third of those with L3 lesions could do so. Hip stability, intelligence quotient and fixed deformity did not influence walking ability

We report five examples of physeal widening in four children with myelomeningocele. In all cases there was rapid clinical resolution with the use of the patients' normal orthoses and minor limitation of activity, and there was no evidence of early epiphyseal closure or growth disturbance. We suggest that recognition of the pathological process before fracture occurred may explain the rapid return to normal

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

1. A conservative approach to the varus deformity of anaesthetic feet in infants with myelomeningocele is advocated. Thirty-three of thirty-five varus feet treated by this method, supplemented when necessary by a minor operation to correct equinus, responded satisfactorily in this prospective study of twenty-four infants treated from birth. 2. A rotation flap incision has proved of considerable advantage in the rare instances when an extensive medial release is required

Hand function in 33 patients with myelomeningocele, aged from 4 to 17 years, was investigated by clinical examination and formal tests of co-ordination and dexterity. The average score for hand function was 59% of normal and only two children had clinically normal upper limbs. Twenty-eight patients (85%) had cerebellar ataxia, either alone or combined with other abnormal signs, most commonly upper motor neuron lesions. Hand function was significantly better in children of 11 years and older, in children under 11 with milder degrees of hydrocephalus, and in the younger children who were right-handed. Function was not shown to be better in those with lesions lower in the spine, nor in those with stable spines as has been previously reported. When co-ordination of the upper limb, manual dexterity and simultaneous movements of both arms were examined separately, these three were affected equally. Some abnormalities of hand function could be overcome by encouraging affected children to sit up and to practise skillful use of their hands as early in life as possible

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

In 21 children with myelomeningocele who underwent kyphectomy for congenital kyphosis of the lumbar spine, aortography revealed no case in which the aorta followed the spinal curvature. Many anomalies of the intercostal and segmental arteries were demonstrated which were only in part associated with deformities of the respective vertebral bodies. The kidneys, which were frequently malformed, often lay within the kyphosis and were therefore at risk of operative damage. We conclude that the aorta is not at risk and that aortography is not usually necessary before kyphectomy, except in patients who have undergone prior abdominal surgery. Non-invasive methods (ultrasound, CT or MRI) should be used to detect malpositions and malformations of the kidneys

We analysed the cases of lumbar kyphosis in 151 (21%) of a series of 719 patients with myelomeningocele. Three different types were distinguished: paralytic, sharp-angled and congenital. In a cross-sectional and partly longitudinal study the size and magnitude of the kyphosis, the apex of the curve and the level of paralysis of each group were recorded and statistically analysed. Paralytic kyphosis (less than 90° at birth) occurred in 44.4% and increased linearly during further development. Sharp-angled kyphosis (90° or more at birth) was present in 38.4% and also showed a linear progression. In both types, progression seemed to depend also on the level of paralysis. Congenital kyphosis occurred in 13.9% and we could find no significant factor which correlated with progression

1. The management of severe kyphosis of the lumbar spine in association with myelomeningocele is discussed. 2. Neonatal spinal osteotomy-resection has been performed in six patients with partial correction of the deformity and a greatly improved ease of closure and healing of the skin defect. The severity of lower limb paralysis has been diminished compared with the complete paraplegia that almost always results from conservative management of closure of the defect without osteotomy. 3. In an older child who has not had the benefit of neonatal osteotomy and who has complete lower limb paralysis, transverse spinal osteotomy or excision of the prominent laminae and pedicles on each side of the midline makes possible the fitting of apparatus for walking and diminishes the liability to recurrent ulceration of the skin

1. Deformities of the foot in children with myelomeningocele are described and classified. The results of a policy of operative correction of deformity in 148 patients all of whom had had at least one operation on the foot between 1947 and 1965 are described. 2. In 241 feet in which there were deformities 433 operations were performed, including tenotomies, soft-tissue divisions, tendon transfers and bony procedures. At the time of review successful correction of deformity had been obtained in 81 per cent with a plantigrade foot that could bear weight safely, and with a distribution of muscle activity that required minimal external support and presented the least liability to recurrent deformity. 3. The management of individual deformities is described and the causes of failure are analysed and discussed

1. Kyphosis and kyphoscoliosis associated with myelomeningocele are discussed. 2. It is suggested that the condition be treated by osteotomy of the spine, with removal of one or more vertebral bodies. 3. Sixteen patients treated by this method of spinal osteotomy are presented. 4. Although almost a third of the patients died, a high operative and post-operative mortality in this condition is considered acceptable

An operative technique for correction of the severe kyphosis that may develop in cases of myelomeningocele is described. It includes excision of the apical vertebrae, correction of the full length of the kyphotic segment and of rigid compensatory curves, and extensive fusion aided by internal fixation with longitudinal threaded wires. The technique evolved during the management of eleven children. Of these, in the first two the internal fixation employed was inadequate; two others died; and in the remainder good correction was maintained up to the time of review one to six years later

Operation on the hips of patients with myelomeningocele is often followed by stiffness or ankylosis. Excision of the upper end of the femur for this condition frequently fails because of extensive new bone formation. Twelve excisions in eight patients are reported. Six of these patients had recently had spinal fusion operations; the consequent loss of ability to compensate for hip stiffness by spinal flexion had made sitting difficult or impossible. Excision or even re-excision of the upper femur failed in most cases. Careful assessment of hip mobility before spinal operations in these patients is essential and osteotomy rather than excision of the upper femur is advised

A follow-up study, on average four years after operation, of seventy-six cildren with myelomeningocele who had surgical treatment for flexion contracture of the knee, is reported. After a single operation sixty-eight knees (55 per cent) had a contracture of less than 20 degrees, which was the main criterion of success. About half of the cases of transplantation of the hamstrings to the femur, division or elongation of the hamstrings, or supracondylar osteotomy had successful results. Transfer of the hamstring tendons to the patella was successful in nine of twelve instances, so giving the lowest rate of failure. Forty-five patients were active walkers at follow-up as compared to eighteen before operation. However, only one of the eleven patients over twelve years of age with a motor level above T.12 was an active walker at review. Several conclusions are drawn. A flexion contracture of the hip greater than 30 degress should be corrected before operation of the knee. Supracondylar osteotomy as a primary procedure should only be used to correct flexion contracture of the knee secondary to malunited fractures. Faradic stimulation is useful in the evaluation of muslce function before operation. An intelligence quotient below 65 and weakness of the arms are relative contraindications for correction of the deformity of the knee

1. A controlled trial of ninety-nine patients with myelomeningocele has shown that immediate closure did not result in any significant (at P<0.05) reduction in mortality or alteration in muscle power. 2. If required, closure of the back may be delayed for forty-eight hours after birth or longer to allow a detailed clinical assessment of the infant. 3. The muscle power in the lower limbs is a useful guide to prognosis both in relation to mortality and the incidence of hydrocephalus. 4. Mortality is greatest in the first year of life. 5. In the urinary tract the commonest anomaly is neurogenic bladder. Other congenital anomalies were not more common than in children generally

1 . The indications, technique and results of supra-malleolar wedge osteotomy of the tibia in the management of valgus or varus deformity of the ankle in children with myelomeningocele are described. 2. This operation should not be performed until as much correction as possible has been obtained by soft-tissue release and muscle balance has been restored by tendon transfer. 3. In sixteen feet satisfactory correction was obtained and maintained in fourteen, one of which had required revision for over-correction. 4. A closing-wedge osteotomy is recommended ; the two failures occurred after opening-wedge osteotomies. 5. In the fourteen feet with satisfactory correction the complications were negligible, deformity has not recurred and epiphysial growth has been well sustained

Seventy-two posterior psoas transfer operations performed in forty-four children with lumbar myelomeningocele were reviewed one to eight years after operation in an attempt to assess its value. Muscle charting, an objective recording of the child's walking ability, and radiographic examination of the hips were done. Hip stability was improved: 49 per cent were stable at the time of psoas transfer and 94 per cent at review. Functional results depended mainly on the level of neurological activity present: 57 per cent of the children had an acceptable functional result. Usually, posterior psoas transfer should be done as soon after the age of nine months as the child's condition will allow. Over the age of two years it should be restricted to children with activity in the third and fourth segments of the lumbar cord

We have reviewed the results of operations to stabilise the paralytic hip and to correct and stabilise the deformed spine of children with myelomeningocele. Despite a high complication rate the spinal operations were frequently successful. The hip operations were less satisfactory, with stabilisation of the hip eventually achieved in 69 per cent of the patients and improved walking in only 27 per cent. We do not recommend that such operations be undertaken on the hips of children with a level of paralysis at L3 or above. If stabilisation is indicated multiple combined procedures produce the best result

1. The etiology and natural progress of lumbar kyphosis in children from three to twelve years of age with myelomeningocele are reviewed. 2. The indications for operation have included intractable or recurrent skin ulceration, inability to wear calipers for walking, inability to sit in a wheel-chair and inability to perform ileal conduit operations. 3. The technique of osteotomy-excision of lumbar vertebrae used in eighteen cases is described. 4. The results in fourteen children are described. The primary aims of operation have been achieved in all patients. 5. A comparison is made with the results of neonatal osteotomy-excision of the spine in the newborn. Recurrence of deformity, but at a much reduced rate, must be anticipated after either operation

Since 1987, 22 children with myelomeningocele have been fitted with reciprocating orthoses. The level of the spinal lesions ranged from T10 to L4 and 13 had associated spinal deformities. Twelve of the patients currently use a Reciprocating Gait Orthosis, seven use a Hip Guidance Orthosis or Parawalker, one has progressed to a Knee Ankle Foot Orthosis, one has died and one has been lost to follow-up. The reciprocating orthoses are worn for a mean of 3.5 hours per day (1 to 6.5); daily usage by girls is almost twice that by boys. The mean daily usage by community walkers is 4.2 hours (13 children) as against 2.8 hours by household ambulators (8 children). Active hip flexion is not essential and fixed-flexion contractures up to 35 degrees can be accommodated. The average breakdown rate is 0.45 per year with an average of 1.5 adjustments each year. The average annual cost of a reciprocating orthosis is Aus$750 (375 pounds, US$570); this includes fabrication, adjustments and repairs

Twenty-three patients with severe paralytic thoracolumbar scoliosis due to a myelomeningocele were treated by a two-stage procedure. Before operation the mean scoliosis was 98 degrees: after the first-stage procedure, an anterior spinal fusion and correction with Dwyer instrumentation, this was reduced to a mean of 45 degrees. Approximately two weeks later a posterior spinal fusion with Harrington instrumentation was performed, further reducing the scoliosis to a mean of 29 degrees. The pelvic obliquity also was reduced from a mean of 32 degrees to 6 degrees. Although such management carries risks (one patient died of cardiorespiratory failure after the first stage and one patient was made worse), 21 of the 23 patients had improved posture and function

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.

Patients with myelomeningocele who had had surgery to stabilise the hip were reviewed; the results of the 106 operations in 88 patients were assessed. In the earlier part of the series there were 55 children who had 64 iliopsoas transfers; later in the series 33 children had 42 varus-rotation osteotomies combined with adductor tenotomy, anterior obturator neurectomy and psoas division. The technical results of both operations were satisfactory: following iliopsoas transfer only 19% of the hips were either dislocated or subluxated; the corresponding figure for the osteotomy was 12%. Thus varus-rotation osteotomy with psoas division, adductor tenotomy and anterior obturator neurectomy was at least as effective in stabilising the hip as iliopsoas transfer. Nevertheless 80% of the latter and 61% of the osteotomy patients relied on wheelchairs for mobility

The hip problems in 116 children with myelomeningocele are discussed. The management described stresses the importance of selection of the type of operation; major surgery is appropriate only for those children who benefit significantly, and this generally means only those with strong quadriceps muscles on both sides. Some children with acetabular dysplasia gain immediate stability if acetabuloplasty is combined with other hip operations. Children who lack strong quadriceps muscles are best served by simpler procedures, such as tendon excision, designed to rid them of fixed deformity and to prevent recurrent deformity. The operations described are performed whenever possible under one anaesthetic and are combined with any other limb operation that may be necessary. It is suggested that the aim in management is not the treatment of paralytic dislocation of the hip but the correction of fixed deformity so that the child can stand with a stable posture. Subluxation and dislocation are treated incidentally to procedures designed to prevent or correct fixed deformity

Aims

The aim of this study is to define a core outcome set (COS) to allow consistency in outcome reporting amongst studies investigating the management of orthopaedic treatment in children with spinal dysraphism (SD).

One hundred and nine children with myelodysplasia were evaluated and classified according to the level and type of paralysis and its effect on functional ability. Thirty-one per cent of the patients were paralysed at the thoracic level, 26% at the upper lumbar level, 30% at the lower lumbar level and 13% at the sacral level. Fifty-four per cent of these patients demonstrated the classic flaccid paralysis in the lower limbs with normal upper limbs; 9% were flaccid in the lower limbs, but were spastic in the upper; 24% were spastic in the lower limbs; 13% were spastic in the upper and lower limbs. Patients with spastic lower limbs required more orthopaedic procedures, more days in hospital and in casts, and were less likely to walk than those with flaccid paralysis. Patients with spastic upper limbs were less likely to be independent in activities of daily living and were more likely to require special schools than patients with normal upper limbs. In addition to the spinal cord level of the lesion, the degree of spasticity is important in the evaluation, treatment and prognosis of myelodysplastic patients.

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.

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 results of vertebral column resection (VCR) for paediatric patients with spinal deformity. A total of 49 VCRs in paediatric patients from four university hospitals between 2005 and 2009 with a minimum two-year follow-up were retrospectively identified. After excluding single hemivertebral resections (n = 25) and VCRs performed for patients with myelomeningocele (n = 6), as well as spondylectomies performed for tumour (n = 4), there were 14 patients who had undergone full VCR at a mean age of 12.3 years (6.5 to 17.9). The aetiology was congenital scoliosis in five, neuromuscular scoliosis in three, congenital kyphosis in two, global kyphosis in two, adolescent idiopathic scoliosis in one and secondary scoliosis in one. A total of seven anteroposterior and seven posterolateral approaches were used. The mean major curve deformity was 86° (67° to 120°) pre-operatively and 37° (17° to 80°) at the two-year follow-up; correction was a mean of 54% (18% to 86%) in the anteroposterior and 60% (41% to 70%) in the posterolateral group at the two-year follow-up (p = 0.53). The mean Scoliosis Research Society-24 total scores were 100 (92 to 108) for the anteroposterior and 102 (95 to 105) for the posterolateral group. There was one paraparesis in the anteroposterior group necessitating urgent re-decompression, with a full recovery. Patients undergoing VCR are highly satisfied after a successful procedure

The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients’ quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal correction.

The August 2024 Children’s orthopaedics Roundup³⁶⁰ looks at: Antibiotic prophylaxis and infection rates in paediatric supracondylar humerus fractures; Clinical consensus recommendations for the non-surgical treatment of children with Perthes’ disease in the UK; Health-related quality of life in idiopathic toe walkers: a multicentre prospective cross-sectional study; Children with spinal dysraphism: a systematic review of reported outcomes; No delay in age of crawling, standing, or walking with Pavlik harness treatment: a prospective cohort study; No value found with routine early postoperative radiographs after implant removal in paediatric patients; What do we know about the natural history of spastic hip dysplasia and pain in total-involvement cerebral palsy?; Evaluating the efficacy and safety of preoperative gallows traction for hip open reduction in infants

1. The pathological anatomy in a case of convex pes valgus in a patient with myelomeningocele is described. 2. A neuromuscular imbalance between the tibialis posterior and the evertors of the foot is suggested as the underlying cause of this type of foot deformity

Children with flail feet who have control of their hips and knees are able to walk but are unable to stand still and find it difficult to walk slowly. These problems have been overcome in fifty-three children with flail feet due to myelomeningocele by fitting them with below-knee orthoses which provide maximum stability and yet allow normal walking. The biomechanical principles and the development of the orthoses are discussed

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

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

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

Arthrogryposis multiplex congenita is believed to be a specific clinical entity which is aetiologically unrelated to the "arthrogryposis-like" deformities of known neurological diseases such as myelomeningocele and myelodysplasia. The observation that the condition appeared to be three times as common in Melbourne, Australia, as in four centres in the United Kingdom (Wynne-Davies and Lloyd-Roberts 1976), prompted this survey of 132 patients: 73 from the United Kingdom, 34 from Australia and 25 from Wilmington, Delaware, USA. The survey aimed to established the same criteria for diagnosis in the three countries and to search for prenatal and genetic aetiological factors. It was shown that all centers treated more newborn children with this disorder during the 1960s than either before or after that period. All cases were sporadic and there was no family association with talipes equinovarus, congenital dislocation of the hip or hereditary neuromuscular disease. "Environmental" findings from all three centers were similar and it was concluded that arthrogryposis multiplex cogenita is a non-genetic disease of early pregnancy, associated with a variety of unfavourable intra-uterine factors. In addition, an unknown but possibly viral environmental agent may have been present to a significant extent only during recent decades and is now declining

Aims

It is uncertain whether instrumented spinal fixation in nonambulatory children with neuromuscular scoliosis should finish at L5 or be extended to the pelvis. Pelvic fixation has been shown to be associated with up to 30% complication rates, but is regarded by some as the standard for correction of deformity in these conditions. The incidence of failure when comparing the most caudal level of instrumentation, either L5 or the pelvis, using all-pedicle screw instrumentation has not previously been reported. In this retrospective study, we compared nonambulatory patients undergoing surgery at two centres: one that routinely instrumented to L5 and the other to the pelvis.

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.

Acute angulation at the thoracolumbar junction with segmental subluxation of the spine occurring at the level above an anteriorly hypoplastic vertebra in otherwise normal children is a rare condition described as infantile developmental thoracolumbar kyphosis. Three patient series with total of 18 children have been reported in the literature. We report five children who presented with thoracolumbar kyphosis and discuss the treatment algorithm. We reviewed the medical records and spinal imaging at initial clinical presentation and at minimum two-year follow-up. The mean age at presentation was eight months (two to 12). All five children had L2 anterior vertebral body hypoplasia. The kyphosis improved spontaneously in three children kept under monitoring. In contrast, the deformity was progressive in two patients who were treated with bracing. The kyphosis and segmental subluxation corrected at latest follow-up (mean age 52 months; 48 to 60) in all patients with near complete reconstitution of the anomalous vertebra. The deformity and radiological imaging on a young child can cause anxiety to both parents and treating physicians. Diagnostic workup and treatment algorithm in the management of infantile developmental thoracolumbar kyphosis is proposed. Observation is indicated for non-progressive kyphosis and bracing if there is evidence of kyphosis and segmental subluxation deterioration beyond walking age. Surgical stabilisation of the spine can be reserved for severe progressive deformities unresponsive to conservative treatment.

Cite this article: Bone Joint J 2015;97-B:982–7.

External fixation is widely used in orthopaedic and trauma surgery. Infections around pin or wire sites, which are usually localised, non-invasive, and are easily managed, are common. Occasionally, more serious invasive complications such as necrotising fasciitis (NF) and toxic shock syndrome (TSS) may occur.

We retrospectively reviewed all patients who underwent external fixation between 1997 and 2012 in our limb lengthening and reconstruction programme. A total of eight patients (seven female and one male) with a mean age of 20 years (5 to 45) in which pin/wire track infections became limb- or life-threatening were identified. Of these, four were due to TSS and four to NF. Their management is described. A satisfactory outcome was obtained with early diagnosis and aggressive medical and surgical treatment.

Clinicians caring for patients who have external fixation and in whom infection has developed should be aware of the possibility of these more serious complications. Early diagnosis and aggressive treatment are required in order to obtain a satisfactory outcome.

Cite this article: Bone Joint J 2015;97-B:1296–1300.

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.

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.

Our aim was to review the efficacy of the wound vacuum-assisted closure (VAC) system in the treatment of deep infection after extensive instrumentation and fusion for spinal deformity in children and adolescents.

A total of 14 patients with early deep spinal infection were treated using this technique. Of these, 12 had neuromuscular or syndromic problems. Clinical and laboratory data were reviewed. The mean follow-up was 44 months (24 to 72). All wounds healed. Two patients required plastic surgery to speed up the process. In no patient was the hardware removed and there was no loss of correction or recurrent infection.

We believe that the wound VAC system is a useful tool in the armamentarium of the spinal surgeon dealing with patients susceptible to wound infections, especially those with neuromuscular diseases. It allows for the retention of the instrumentation and the maintenance of spinal correction. It is reliable and easy to use.

In developed countries, children with cerebral palsy are treated from the time of diagnosis. This is usually not the case in developing countries where such patients often present at an age when it is traditionally believed that if walking has not already commenced, it is unlikely to. This study reports the outcome of the surgical treatment of 85 spastic diplegic patients at a mean of 8.5 years (5 to 12). All presented as untreated non-walkers and had achieved sitting balance by the age of five to six years. They underwent single-event multilevel surgery followed by physiotherapy and orthotic support. For outcome assessment, a modified functional walking scale was used at a mean of 3.5 years (2 to 5) post-operatively.

At all levels, static joint contractures had resolved almost completely. All patients improved and became walkers, 18 (21.2%) as exercise, 39 (45.9%) as household and 28 (33%) as community walkers.

This study shows that children with cerebral palsy who cannot walk and have not been treated can be helped by single-event multilevel surgery, provided that inclusion criteria are followed and a structural, supervised rehabilitation programme is in place.

Club foot was diagnosed by ultrasonography in 91 feet (52 fetuses) at a mean gestational age of 22.1 weeks (14 to 35.6). Outcome was obtained by chart review in 26 women or telephone interview in 26. Feet were classified as normal, positional deformity, isolated club foot or complex club foot.

At initial diagnosis, 69 feet (40 fetuses) were classified as isolated club foot and 22 feet (12 fetuses) as complex club foot. The diagnosis was changed after follow-up ultrasound scan in 13 fetuses (25%), and the final ultrasound diagnosis was normal in one fetus, isolated club foot in 31 fetuses, and complex club foot in 20 fetuses.

At birth, club foot was found in 79 feet in 43 infants for a positive predictive value of 83%. Accuracy of the specific diagnosis of isolated club foot or complex club foot was lower; 63% at the initial ultrasound scan and 73% at the final scan. The difference in diagnostic accuracy between isolated and complex club foot was not statistically significant. In no case was postnatal complex club foot undiagnosed on fetal ultrasound and all inaccuracies were overdiagnoses. Karyotyping was performed in 25 cases. Abnormalities were noted in three fetuses, all with complex club foot and with additional findings on ultrasound.