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