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

Introduction: The management of deformities of the lower extremity in children with spina bifida is challenging. Surgery is fraught with high complication and recurrence rates. The Ilizarov technique has shown to be a successful tool in the management of complex lower limb deformity. There are few published series in the literature dealing specifically with Ilizarov correction of complex lower limb deformities in spina bifida. In this paper we present our experience of the Ilizarov technique for complex deformities in children with spina bifida. Methods: From 1989 to 2006, 33 patients with spina bifida underwent 48 corrections of their lower extremity deformity using the Ilizarov technique at one of three tertiary care centres (Royal Children’s Hospital, Melbourne, Australia, Musgrave Park Hospital, Belfast, Northern Ireland and Birmingham Children’s Hospital, Birmingham, UK). Notes were reviewed retrospectively. Patient demographics, indications for surgery, deformity analysis and the extent of surgery were recorded. Complications of the surgery and outcomes were noted. Results: Group I comprised of 12 fixed knee flexion deformities and one unstable neuropathic joint. Group II comprised 15 external tibial rotational deformities. Group III comprised 20 complex foot deformities. The mean age of the patients was 12.1 years (5.2–20.6 years). Prior to their treatment using the Ilizarov technique patients had undergone a mean of 1.6 previous surgeries (range: 0–5) on the affected limb. The mean duration of treatment in the frame was 9.3 weeks (range: 2–26 weeks). The mean follow-up is 4.0 years (range 0.3–9.0 years). Thirteen problems occurred in the 48 procedures (27.1%). Five obstacles occurred in the 48 procedures (10.4%). Thirteen complications occurred in the 48 procedures (27.1%). Further surgical procedures are either planned, or have been performed, in 8 of the 33 patients (24.2%). Following treatment, all feet were recorded to be plantigrade. All were comfortable in their respective orthoses where appropriate. Conclusion: Through the pitfalls in treating the complex lower limb deformity in spina bifida, the Ilizarov technique offers a refreshing approach. It offers distinct advantages throughout the treatment period. It modifies both the intraoperative and postoperative environment to address the morbidity of the significant complications associated with the traditional approach to the correction of the limb deformity in the child with spina bifida. Significance: This is a large series of complex limb reconstruction cases in spina bifida showing excellent results, an acceptable complication rate and recommendations for further use of this technique

The authors comment on the treatment of patients suffering from spina bifida. The indications to surgical-orthopaedic approach for lower limb correction in patients with spina bifida are outlined. According to the authors’ experience, the therapeutic approach should be aimed at treating deformities of the lower extremities in order to apply orthotic devices, particularly for higher levels of medullary lesion, and to obtain standing and/or gait with support. In the natural history of spina bifida, this purpose has become more and more important, since reduced complications, longer average life, and improved clinical, therapeutic and technological knowledge have radically changed the life expectancy of these patients. The analysis of a series of 71 patients with spina bifida, treated over 20 years, has enabled the authors to evaluate the main sites of deformities. They examined the surgical procedures carried out in relation to the levels of damage and observed the resulting functional autonomy. The foot was the site which offered the most frequent opportunities for treatment at all levels. Knee deformities were treated in a few cases. In particular, difficulties arose when treating L3-L4-L5 neurosegmental levels of the hip, with the result that today bone correction of this joint is performed in association with muscle transposition (external oblique abdominis m. pro medio gluteus m.) in order to ensure a dynamic correction. The authors emphasise the need for a more cautious surgical approach, since lower limb alignment, often achieved using orthopaedic aids and splints, is more important from a functional point of view than the anatomic and radiographic correction of the deformities. From a functional point of view, apparently positive neurosegmental levels for functional recovery are damaged by the lack of early training using splints. Actually, even severe clinical conditions can acquire increased functional autonomy if splints are early introduced to aid walking. Moreover, there are increased indications for spinal surgery in patients suffering from severe paraplegia in order to improve cardio-respiratory function. A correct alignment and an adequate application of orthotic devices reduce the frequency of pelvic obliquity, which favours spine deformities

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

Purpose of the study: In spina bifida, independently of limb paralysis, spinal deformation can cause significant static disorders (scoliosis, kyphosis, or hyperlordosis) which in turn cause significant disability. These deformations generally develop during growth. We wanted to determine the predictive value of a clinical classification based on the neurological examination at five years for risk of spinal deformation. Material: This retrospective study included 163 patients. Groups were defined on the basis of motor function determined by the neurological examination at five years: group I: L5 or below (all patients in this group had motor deficit leaving at least one L5 segment intact); group II: L3–L4; group III: L1–L2; group IV: T12 and above. Results: Results showed that group I was a factor predictive of an absence of future spinal deformation. Groups III and IV were predictive of presence of a future spinal deformation. Group IV was predictive of future kyphosis. Discussion: It is well known that the higher the neurological lesion in spina bifida, the higher the rate of spinal deformation. No work has however set the limits nor provided predictive rules useful in clinical practice. Our work demonstrated that this classification based on the motor function established by neurological examination at five years can predict which children have a risk of developing a spinal deformation and thus enabling early detection and treatment. Conclusion: This neurological classification can be used as a clinical tool for the prognostic evaluation of spina bifida

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

Background: The simultaneous occurrence of spina bifida occulta (SBO) and spondylolysis has been noted previously. The occurrence of SBO and spondylolysis are approximately 17% and 5% respectively. Aims: The aim of this study was to determine the incidence of SBO when a symptomatic spondylolysis has been proven. Method: Analysis of four years of lumbar spine CT scans was performed. Patients with spondylolysis or spondylolisthesis caused by a pars defect were identified. These patients’ CT images were then reviewed to establish the patients who also had SBO. Results: In this unit 650 CT scans of lumbar spines were performed in the last five years. Of these scans 100 (15%) were found to have a pars defect. The average age was 34.6. On CT 30 (30%) patients were shown to have SBO. Of the total 23 were under 18 years old and 13 (56%) of these patients had SBO associated with the spondylolysis. In the over 18 year olds 21 (27%) patients had SBO associated with spondylolysis. On review of the 550 other scans the average age was 51.9 years old. The incidence of SBO within this group is 10%. Only 6% of these patients were under 18. Out of these 33 patients 30.3% (10 patients) had SBO. Over 18 the incidence of SBO was only 8.5%. Conclusions: Our results indicate that the occurrence of SBO within the group with a spondylolysis is much higher than in the group where no spondylolysis is present. There is higher incidence of SBO in the younger patients with spondylolysis. 56% of paediatric patients with symptomatic spondylolysis will have SBO. Clinicians should be aware of this fact preoperatively to decide on the technique of repair of the symptomatic lysis

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