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

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 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

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.