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

Introduction: The incidence of scoliosis in patients with myelomeningocele has been reported to be as high as 80 to 90% in some studies. However these studies included patients with both congenital and developmental curves. The purpose of this study is to identify clinical and radiological factors, which may predict the development of scoliosis in patients with myelomeningocele. Methods: A retrospective review of the charts and radiographs of all patients with myelomeningocele seen in our clinic between 1990 and 1995 was performed. Selection criteria for the study included: a diagnosis of myelo-meningocele or lipomeningocele, age greater than 10 years, serial documentation of motor power testing, and a radiographic documentation of spinal deformity primarily in the coronal plane. Statistical analysis was performed to obtain predictive values, specificity and sensitivity for each of the following factors: clinical motor level, functional status, motor asymmetry and hip instability. Radiographs were examined to obtain the last intact laminar arch in these patients. The relationship between the last intact laminar arch and scoliosis was evaluated. Results: 141 patients satisfied the inclusion criteria. Seventy-four patients (53%) developed scoliosis. The mean follow-up was 9.4 years (range 3–30 years). The average age of the patient population was 19 (range 10–42 years). Forty-three patients developed scoliosis before nine years of age. New curves continued to develop until 15 years of age. Curves less than 20° often resolved. Clinical motor level, functional status, motor asymmetry and the last intact laminar arch were all found to be predictive for scoliosis in these patients. The presence of spasticity and hip instability had no definite influence on the development of scoliosis. Conclusion: The term scoliosis should be reserved for curves greater than 20° in patients with myelomeningocele. New curves may continue to develop until 15 years of age. The last laminar arch is a useful early indicator of scoliosis in these patients

Introduction: Since 1989 vertebral resection with modified Luque fixation has been the procedure of choice for correction of myelomeningocele kyphotic deformity at this institution. The purpose of this study was to evaluate long-term results with this technique. Treatment or congenital kyphosis in myelomeningocele is a difficult problem. Current thinking supports kyphectomy and post-operative internal fixation. The majority of authors agree that kyphotic deformity in myelomeningocele should be treated with vertebral resection. There is less uniform consensus as to postoperative fixation. Literature reports appear to support fixation with modified segmental instrumentation. Methods and Results: 16 patients, followed for an average of 57.2 months (36–94 months), underwent vertebral resection from the proximal aspect of the apical vertebra cephalad into the compensatory lordotic curve. Fixation was segmental instrumentation wired to the thoracic spine and anterior to the sacrum. The average blood loss was 1121 cc (450–2580 cc.). Pre-operative kyphotic deformity averaged 111° (75–157°), postoperative 15° (−18° –36°) and latest follow-up of 20° (−17° –83°), with loss of correction of 6° (0–27°). Post-operative immobilisation was with a TLSO for 18 months. Complications occurred in eight of the 16 patients: (1) transient headache (2), superficial wound breakdown (2), supracondylar femur fractures (2), and one late infection secondary to skin breakdown necessitating early rod removal and some loss of correction. Conclusions: Kyphectomy is an excellent method of correcting rigid kyphotic deformity in the myelodys-plastic patient. Segmental spinal instrumentation provides three distinct advantages: rigidity of the construct, greater correction of the deformity and low-profile instrumentation

Introduction and Objectives: This is a retrospective clinical and radiographic study of 19 patients affected by paralytic scoliosis secondary to myelomeningocele who were treated surgically with double anterior fusion (without anterior instrumentation) and instrumented posterior fusion from T2 to the sacrum. Materials and Methods: The study involved 19 patients with scoliosis secondary to myelomeningocele treated surgically by instrumented posterior fusion (3 cases) or double anterior fusion (non-instrumented) with instrumented posterior fusion from T2 to the sacrum (10 cases). Instrumentation consisted of two rods shaped in physiologic kyphosis and lordosis, sublaminar wiring, and anchoring to the pelvis using the classic Galveston technique (7 patients); two rods fixed to the spine by means of sublaminar wires and pedicle screws on the convexity of the curve in the lumbar region and an iliac screw in the pelvis (11 cases); and in one case, anchoring to the pelvis was achieved using an iliosacral screw. Results: Age of the patients ranged from 9 to 16 years, with a mean of 12 years. There were 11 females and 8 males. Maximum time of patient review was 9 years, and minimum time was 2 years. The level of spinal cord involvement was thoracic in 1 case, upper lumber (L1–L2) in 6 cases, mid-lumbar (L3) in 7 cases, and lower lumber (L3–L4) in 5 cases. Curve patterns were thoracolumbar (apex at T12–L1) in 14 cases and lumbar in 5 cases. Pre-operative curve angles ranged from 60° to 133° with a mean of 93°. Postoperative curve ranged from 15° to 60° with a mean of 42°. There were 6 patients with thoracolumbar kyphosis ranging from 24° to 92° (mean 49°) and 15 patients with pelvic obliquity ranging from 14° to 42° (mean 28°). Decompensation of the trunk with respect to the pelvis ranged from 26 to 0 cm, and postoperatively ranged from 13 to 0 cm (mean 5.5 cm). Of the 19 patients, 9 (50%) experienced complications. One patient presented with non-union and a subsequent delayed infection requiring four operations and removal of material. There were 3 patients that experienced postoperative infections requiring surgical drainage, with 2 cases of leakage of CSF, which resolved with postural therapy. In 5 cases, there was necrosis of the vertex of the triradiate incision, and in one case there was failure of the iliac screw in the pelvis with no clinical consequences. Discussion and Conclusions: The use of a triradiate incision avoiding a direct approach to the sacral dura, a double anterior and posterior approach in cases of rigid curve (radiographic examination in traction) and saggital deformities (thoracolumbar kyphosis), and the use of pedicle screws in the distal vertebrae and in the iliac wing allow the correction of frontal and sagital deformities and pelvic obliquity in patients with myelomeningocele

Background:. Severe kyphosis in myelomeningocoele patients results in seating problems, early satiety and ultimately pressure sores over the prominence. Kyphectomy and sagittal correction can improve these morbidities. Aim:. To evaluate the outcome of kyphectomy surgery in meningomyelocoele children. Methods:. A retrospective review was performed of a single surgeon series of paediatric myelomeningocele patients who underwent kyphectomy surgery. All the patients had posterior fusion, employing pedicle screws and sub laminar wiring. All the posterior fusions extended to the pelvis either to include S1 or the ilium. Prior to surgery, three children had open wounds over the apex of the deformity. Despite prone nursing, these failed to heal. These were closed primarily intra-operatively without the requirement of flaps. Results:. Seven children (four males, three females) were identified with an average age of 9.5 (8–13) years. The kyphosis was corrected from a mean range of 110° (88°–180°) to post-operative range of 5°–45°. The operative time averaged 240 min (165–284 min) with an estimated blood loss average of 957 ml (500–2550 ml). All the patients recovered well and no short-term complications were experienced except one transient CSF leak intra operatively. Two patients presented with sub-acute delayed infection requiring removal of instrumentation once the spine had fused. There was one case of instrumentation failure. All patients had improved wheel chair seating. Conclusion:. Kyphectomy in myelomeningocele is an infrequently required procedure which is effective in sagittal correction and improved seating with an acceptable complication rate

Purpose: To give the review of the foot deformities in the patients with myelomeningocele (MMC), their relationship to the level of the neurological lesion and therapeutic possibilities. Material & Methods: Since 1998 till 2004 there were 20 patients with myelomeningocele treated in our clinic, in 19 patients was noted the foot deformity (total 34 feet). The deformity was unilateral in 4 patients, asymmetric in 2 patients (equinovarus + calcaneovalgus or planovalgus). The most frequent was equinovarus deformity (16 feet, 47%), next planovalgus deformity (9 feet, 26,5%), calcaneovalgus (7 feet, 20,6%) and equinovalgus (2 feet, 5,9%). In 28 feet the surgery was done. For the correction of the various deformities of the feet were used soft tissue releases or tendons transfer, in the older patient or after failed soft tissue release the bony operation was done (tarsal osteotomy, talo-calcaneal stabilisation, artrodesis). Results: The aim of every type of surgery was to achieve the acceptable foot shape, with plantigrade step and possibility of weigh bearing, with minimal risk of the pressure necrosis. In the most patients the purpose was achieved, although some of them passed several surgeries. In 18 deformities only one surgery obtained the correction of the deformity, the but for the 10 feet subsequent surgery was required because of the residual or relapsing deformity. In 3 patients was noted the pressure necrosis, in 1 patient appeared the fracture of the distal tibia (epiphyseolysis) after the removal the postoperative plaster. Conclusion: The orthopaedic care about the patients with MMC is only a part of the interdisciplinary approach of several specialists. The procedures for the correction of the neurogenic deformities of the feet, belong to the delicate surgery and is necessary very careful indication in relation with the type of deformity, which is dependent on the high of the lesion, and with the expectancy of walking. In the patients with good prognosis of walking is necessary to choose the procedure which guarantee weight-bearing and plantigrade step without the risk of ischemic skin defects

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

Due to the increasing rate of relapses and the morbidity degree that this implies, we report our experience and results in the treatment of clubfoot in patients with myelomeningocele. Between February 1996 and February 2001 12 patients with myelomeningocele (16 feet with clubfoot deformity and 4 bilateral cases) underwent surgical treatment. 5 were boys and 7 were girls. 3 relapsed cases were referred to our institution, 1 of them had a bilateral involvement. The average age at time of surgery was 27 months (range 7 months–5.3 years). Levels of functional involvement were recorded according to Caneo (Argentina Chapter of Neuroorthopaedics) classification: Caneo 0: 2 patients, Caneo 1: 4 patients, Caneo 2: 8 patients, Caneo 3: 1 patient. Relapses occurred in 3 cases; 2 with tendon lengthening technique and 1 tibialis posterior transfer to lateral peroneus brevis, split tibialis anterior tendon transfer. The complications were postoperative infection in 3 cases with wound dehiscence, tibia fracture after cast removal in 1 case and residual tibia intrarotation in 1 case. AFO were used in patients older than 2 years old with Caneo type 2 and 3 and RGO in patients with Caneo type 0 and 1. The final results after solving all the complications were: 6 plantigrade feet, 1 intrarotated plantigrade foot and 1 relapsed inverse foot undergoing release of filum terminale with tethered spinal cord. In conclusion, we consider the most effective technique the one that presents the lower rate of relapses and with efficient functional outcomes. We agree with Luciano Dias opinion that regional resection of all the tendinous elements is the best option to fulfill our goals. We strongly advice a tendon lengthening or transfer in patients belonging to Caneo classification type 3

Treatment of congenital kyphosis with severe angular dysplastic spine in children with myelomeningocele (MMC) is one of the most difficult spinal procedures. Most of the surgeons support kyphectomy with long segmental spinal instrumentation and postoperative immobilization by thoracolumbosacral orthosis. Several spinal deformities are seen frequently in patients who have MMC. The deformity may be congenital or paralytic. Congenital lumbar kyphosis is less common, but most difficult in patients with MMC, occurring in 10–20% of patients. Most curves are congenital and rigid, often more than 80° at birth, and rapidly progresses. With progression of kyphotic deformity, patients experience recurrent skin breakdown over the apex of the kyphos; impaired sitting balance; the necessity of using their hands for support; collapsing spine and decreasing of lumbar height reduce the capacity of the abdominal cavity and resulting in reduced respiratory capacity and malnutrition. The poor posture and short abdomen make it difficult to manage the patients’ urological needs. A severe deformity raises difficulties in social and psychological development. Non-operative treatment with spinal orthoses may provide only temporary correction of a kyphotic deformity, but does not prevent progression and skin breakdown. The goal of surgical treatment is correction of spinal deformity by long segmental instrumentation and achievement of a solid spine fusion in order to allow a balanced sitting position and to prevent complications. From 1983 to 2001, 6 patients with thoracic level myelomeningocele and severe kyphotic deformity were referred for surgical correction. There were 5 males and 1 female patients with average age at the time of surgery of 8.3 years (range 4.3–13 years). All patients suffered from severe kyphosis, range 90° to 130°, average – 108°. All of them underwent posterior ligation of spinal cord during resection of lordotic segment of the kyphos, and segmental spinal fixation of the deformity from the thoracic spine to the sacrum. In all cases following the resection of the vertebrae it was possible to correct the deformity. All patients were available for follow-up with range of 6–216 months, average 85 months. All of them were satisfied with the surgical outcome and presented in their final clinical examination with balanced and comfortable sitting, without soft tissue complications. In all cases a significant correction of the deformity was achieved (15°–30°) and enabled comfortable and stable sitting. Two patients suffered post-operative complications, one from surgical wound infection which required surgical debridement followed by soft tissue covering, and the other suffered from distal migration of the rod which was shortened later on. Discussion: Kyphotic deformity in a patient who has MMC is a challenge for the orthopaedic surgeon and requires major surgical intervention. Resection of the kyphos with posterior instrumentation and fusion may solve patient’s functional problems

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