Results of surgical therapy of knee flexion contractures in patients with myelomeningocele]

This study reports the results of 98 operations for correcting knee-flexion-contractures, which were performed between 1972 and 1989 in 60 patients with myelomeningocele. 13 knees had hamstring lengthening and 85 knees had radical flexor release. In 4 patients, who had flexion-contractures of more than 50 degrees, the soft tissue release was combined with supracondylar extension osteotomy. 58 patients had additional hip-flexion-contractures and 39 patients had feet deformities. The average age at time of surgery was 8 years, 3 months. The average follow-up-period was 65 months with a minimum of 1 year and a maximum of 13 years. In 92 knees a permanent extension ability could be achieved. In 6 knees a recurrence of flexion-contracture occurred making a second surgery necessary. The main problem in the postoperative period were 13 skin necroses, which were seen mainly after an s-shaped incision. As a conclusion a perpendicular midline incision is recommended. After surgery 11 patients could be provided for the first time with an orthoses. In the remaining the upright body position or the erection of the orthoses could be improved.     

Experiences with surgical therapy of Budd-Chiari syndrome]

The obstruction of the hepatic venous outflow tract with or without involvement of the inferior vena cava results in the Budd-Chiari syndrome (BCS). With its very heterogenous etiology and variable epidemiology the rare disease either takes a chronic or an acute foudroyant clinical course. In general the prognosis is poor. Together with the clinical signs the diagnosis is based on radiological measures and the histology of the hepatic parenchyma. The exact etiological investigation of the BCS is of great significance. Typical findings are discussed and a diagnostic scheme is developed. Between 1979 and 1991, altogether 16 operations were carried out in 13 patients with a BCS. Predominantly there were undertaken a porto-systemic shunt procedure or an orthotopic liver transplantation, respectively, in 6 cases each. The need for an always individually tailored therapeutic strategy of the BCS is underlined by a case history. An overview analyzes the different therapeutic modalities of the BCS and their differential indications.     

Clinical and radiographic predictors of scoliosis in patients with myelomeningocele

BACKGROUND: The prevalence of scoliosis in patients with myelomeningocele has been reported to be as high as 80% to 90% in some studies. However, those studies included patients with both congenital and developmental curves. The variation in the patient population as well as the definition of scoliosis in those studies made it difficult to predict the true prevalence of scoliosis in these patients. The purpose of the present study was to identify clinical and radiographic factors that may predict the onset of developmental scoliosis in patients with myelomeningocele. METHODS: A retrospective review of the charts and radiographs of all patients with a diagnosis of myelomeningocele who were seen in our clinic between 1990 and 1995 was performed. The criteria for inclusion in the study included a diagnosis of myelomeningocele or lipomeningocele, an age of more than ten years at the time of the review, serial documentation of motor power, and radiographic documentation of spinal deformity primarily in the coronal plane. The radiographs were examined to assess the degree of scoliosis and to document the last intact laminar arch. The relationship between the degree of scoliosis and the last intact laminar arch was evaluated. Statistical analysis was performed to assess the association between scoliosis and the clinical motor level, the ambulatory status, spasticity, motor asymmetry, and hip instability. RESULTS: One hundred and forty-one patients satisfied the criteria for inclusion in the study. Seventy-four patients (52%) had scoliosis. The average duration of follow-up was 9.4 years (range, three to thirty years), and the average age of the patients was nineteen years (range, ten to forty-two years). Scoliosis developed before the age of nine years in forty-three patients and after the age of nine years in thirty-one patients, with new curves continuing to develop until the age of fifteen years. Curves of <20 degrees degrees often resolved. The clinical motor level, ambulatory status, and last intact laminar arch were all found to be predictive factors for the development of scoliosis in these patients. CONCLUSIONS: In the population of patients with myelomeningocele, the term scoliosis should be reserved for curves of >20 degrees. New curves may continue to develop until the age of fifteen years. The level of the last intact laminar arch is a useful early predictor of the development of scoliosis in these patients.     

Anterior only fusion for scoliosis in patients with myelomeningocele.

A series of patients with single major scoliosis curvatures attributable to spina bifida treated by anterior only spinal fusion was studied for 2 years to determine whether the infection rate could be decreased, adequate correction and pelvic balance could be provided, and posterior surgery could be avoided in these patients. Anterior surgery alone was performed for thoracolumbar scoliosis greater than 45 degrees if the compensatory thoracic curve was less than 40 degrees and there was no significant junctional kyphosis. Fourteen patients were treated at a mean age of 11.9 years (range, 7-16 years), with a mean curve of 64 degrees (range, 51 degrees-85 degrees), and motor levels distributed from T10-L4. Thirteen patients had prior neurosurgery for tether, syrinx, or Arnold-Chiari malformation. The spine was fused over a mean of seven vertebrae. A 3/16 inch Texas Scottish Rite Hospital rod was used most commonly (10 patients). Blood loss averaged 1100 cc. The mean curve correction was 57% at 40 months after surgery. Loss of correction occurred primarily by adding on outside the instrumented area. Mean pelvic obliquity was improved from 16 degrees to 9 degrees. There was one superficial infection. Results were good in five patients, fair in four, and poor in five. Failures were attributable to proximal decompensation in two patients who required revision surgery (two), neurologic deterioration in two, and screw pullout in one. Both patients with decompensation had syringomyelia. Both patients with neurologic deterioration had large curves (> 75 degrees). Both patients recovered after rod removal. Retrospectively, by eliminating patients with syrinx or with a curve greater than 75 degrees, all poor results would be eliminated. Anterior only fusion and instrumentation may have significant advantages, but only for selected patients with thoracolumbar curves less than 75 degrees, compensatory curves less than 40 degrees, no increased kyphosis, and no syrinx. Quadriceps function should be monitored. On the basis of this preliminary experience, continued use of this approach using stricter selection seems warranted.     

Effect of scoliosis surgery on pulmonary function in patients with myelomeningocele

Thirteen patients with myelomeningocele who required operation for scoliosis were tested for changes in pulmonary function 1 month before and an average of 13 months after spinal stabilization. The average age at the time of operation was 12 years 11 months. Ten patients had restrictive changes in pulmonary function preoperatively. Owing to the severity of scoliosis, an anterior approach to the spine including resection of ribs and division of the diaphragm was necessary for eight children. Postoperatively, despite these procedures, eight patients had an increase in vital capacity and six patients had an increased forced expiratory volume in 1 s.     

Progressive spasticity and scoliosis in children with myelomeningocele. Radiological investigation and surgical treatment

Seventeen myelodysplastic patients with progressive extremity spasticity and scoliosis underwent radiological evaluation and surgical treatment. All but one were under 18 years of age at the time of surgical treatment. Duration of the clinical presentation ranged from 1 1/2 to 7 years. Metrizamide was instilled into the subarachnoid space in 12 patients, the lateral ventricle in two, and the hydromyelic cavity in three. Sequential computerized tomography scanning after intrathecal instillation of the contrast material clearly demonstrated hydromyelia in nine patients and compression of the brain stem in five. Posterior fossa decompression with plugging of the obex was performed in 12 patients, posterior fossa decompression alone in three, and ventriculoperitoneal (VP) shunting procedures in two. Of the 12 patients who underwent the obex plugging procedures, eight have shown partial or complete resolution of spasticity and an increase in motor strength with no significant postoperative complications. In contrast, posterior fossa decompression or VP shunting procedures alone have not led to a favorable neurological outcome. Hydromyelia may occur more commonly among myelodysplastic patients than previously recognized and may be treated most effectively by the obex plugging procedure.     

Complications of scoliosis surgery in children with myelomeningocele

The purpose of the present study was to evaluate whether the high incidence of complications in scoliosis surgery in myelomeningocele (MMC) could be attributed to the surgical technique and whether improvements were possible. Between 1984 and 1996, 77 patients with MMC and scoliosis were treated surgically. The clinical and radiological follow-up ranged from 1 to 10 years with a mean follow-up of 3.6 years. The mean age at time of surgery was 12 years 8 months. The average preoperative scoliosis measured 90.20° and was corrected by 47%. The first four patients were stabilized with Harrington rods after anterior correction with a Zielke device (group 1). Twenty-five patients were operated only from posterior, using Cotrel-Dubousset (CD) instrumentation (group 2). In 13 patients an anterior release and discectomy was performed prior to CD posterior instrumentation (group 3). In 26 patients (group 4) this was combined with an anterior instrumentation. The 9 patients of group 5 had congenital vertebral malformations which made a special treatment necessary. Complications could be divided into hardware problems, such as implant failure, dislocation or pseudarthrosis, infections, anesthetic, and neurologic complications. Hardware problems were seen in 29% of all patients. More hardware problems were seen with the Harrington rod (75%) and after solitary posterior instrumentation (30%). The occurrence of pseudarthrosis was dependent on the surgical technique, the extent of posterior spondylodesis, and lumbosacral fusion. Patients with hardware problems had a mean loss of correction of 49% compared to 13% in the other patients. Depending on the different surgical techniques a loss of more than 30% was seen in 12–75% of the cases. Early postoperative shunt failure occurred in four cases; delayed failure – after more than 1 year – in three cases. One patient died within 1 day due to an acute hydrocephalus, another died after 21/2 years because of chronic shunt insufficiency with herniation. Wound problems were not dependent on the surgical technique, but on the extent of posterior spondylodesis and the lumbosacral fusion. Based on this analysis we believe our current practice of instrumented anterior and posterior fusion is justified. Further, we are very careful to check shunt function prior to acute correction of spinal deformity. Received: 27 February 1998 Revised: 19 August 1998 Accepted: 15 September 1998     

Development of paralytic scoliosis in myelomeningocele

Among other things the probability of developing a scoliosis in myelomeningocele depends on the patients age, the neurological level of lesion and the localisation of the vertebral arch defect. This study tries to analyse the influence of these factors in 465 patients with MMC and paralytic scoliosis. The results show, that the patients age and the neurological level of lesion are the most important factors for the development of paralytic scoliosis. The statistical evaluation based on the data found permits the determination of regression lines, which allow an estimation of further progression of paralytic scoliosis with respect to the neurological level of lesion and the patients age. At levels of paralysis between Th 3 and Th 12 an average progression of 3.5 degrees per year can be expected. Between L 1 and L 3 the progression rate can be estimated to be 2.5 degrees per year. At levels of paralysis distal to L 3 one can expect to find no significant development of scoliosis. In cases, which exceed the prediction interval, additional intraspinal anomalies, i.e. syringomyelia, must be considered and further examinations including MRI are required.     

Surgical management of paralytic scoliosis in myelomeningocele

A retrospective analysis of 54 patients with paralytic scoliosis due to myelomeningocele, who underwent surgical treatment, was performed. The aim of this study was to compare different surgical techniques and to identify clinical parameters influencing primary and midterm results. Three surgical techniques were used: 1) group I, posterior fusion/instrumentation; 2) group II, anterior fusion/no instrumentation combined with posterior fusion/instrumentation; and 3) group III, anterior and posterior fusion/instrumentation. Average age at surgery was 13.1 years. A preoperative scoliosis angle of 90 degrees [interquartile range (25th-75th percentile) (IQR), 76-106 degrees] was primarily reduced to 38 degrees (IQR, 30-50 degrees). At final follow-up (mean, 3.3 years), correction deteriorated to 44 degrees (IQR, 38-65 degrees). The group III procedure resulted in a better midterm correction of scoliosis compared with group I (P = 0.02). The extension of anterior fusion correlated with primary and midterm correction of scoliosis (P < 0.03). Patients with a thoracic level of paralysis had a higher relative loss of correction compared with patients with a lumbar level (P < 0.06). This finding can be attributed mostly to group I patients (P = 0.011). Hardware complications occurred in 16 patients (30%). Relative loss of correction among these patients was high (P < 0.01) and relative midterm correction low (P = 0.001). We recommend anterior and posterior fusion, each with instrumentation for the treatment of paralytic scoliosis in myelomeningocele. In patients with a thoracic level of paralysis, the two-stage procedure is mandatory to reduce the risk of hardware complications and subsequent major loss of correction.     

Results of the surgical therapy of scoliosis

In the years 1981 to 1985 84 patients with scoliosis were mainly treated by the proceeding of Harrington respectively Harrington-Luque. By using the combination Harrington-Luque-wires its getting an evident better result in correction (63%) as by only using the Harrington-instruments (55%).     

Prevalence of scoliosis in children with myelomeningocele in western Sweden.

In a cross-sectional study of the prevalence of scoliosis in patients with myelomeningocele, 131 patients 5-20 years of age were investigated with clinical examination and radiograms. Sixty-nine percent of the myelomeningocele patients had scoliosis, this prevalence being present already at a young age (6 years). The occurrence of scoliosis increased drastically at high levels of dysraphism, being 20% in patients with sacral myelomeningocele but 94% in patients with thoracic MMC level. Between ages 5 and 10, the mean scoliosis increased successively from 15 to 33 degrees. After this age there was no significant further increase. The ambulatory status of the patients was strongly correlated to the scoliosis incidence. Forty-nine percent of the patients had a uni- or bilateral hip dislocation. There was no correlation between the side of the scoliosis convexity and the side of hip dislocation.     

Development in conservatively treated scoliosis in patients with myelomeningocele (patients of the years 1964-1977)]

The advances in urological and neurosurgical management in myelomeningocoele patients have led to an increased survival rate. The extremely complex spinal deformity presents a major treatment challenge. Clinical and radiological evolution of scoliosis treated conservatively for at least 10 years was studied. Eighty-nine myelomeningocoele patients born between 1964 and 1977 were reviewed. Thirty-one (35%) were noted to have scoliosis (10 congenital type, 21 developmental type). All congenital forms showed rapid progression despite brace treatment, often to curvatures in excess of 100 degrees. Among the developmental type, curves detected prior to 10 years (10 cases before 5 years, 6 cases between 6 and 10 years) were rapidly progressive beyond 70 degrees. Curves detected after 10 years (5 patients) never exceeded 30 degrees. Because of this natural history, conservative treatment should be limited to developmental forms less than 50 degrees. Spinal stabilization is indicated in all curvatures over 50 degrees without awaiting adulthood.     

Experiences with surgical fusion of the lumbar spine]

Authors report on the results of the operative fusion of the lumbar spine performed by them between 1 March 1987 through 31 December 1990. Based on the assessment of 243 cases they call attention to the importance of the correct indication and operative technique and of the close supervision. It is stated that the use of the internal fixation improves the results of the operative fusions.     

Posterior spinal fusion in paralytic scoliosis and myelomeningocele

The Shriners Hospital, Lexington, KY, experience with posterior spinal fusion for scoliosis in 50 patients with myelomeningocele was reviewed. Six patients were fused with Harrington rods and 47 of 50 patients were treated with some form of segmental fixation. The complication rate was 48% (24/50) resulting in 1.48 procedures per patient. The deep infection rate was 8% (4/50) and the pseudarthrosis rate was 16% (8/50). The use of modern segmental instrumentation systems has improved the pseudarthrosis rates for posterior spinal fusion in scoliosis associated with myelomeningocele but not to the point of displacing the current approach of an anterior and posterior spinal fusion.     

Efficacy of surgical management for scoliosis in myelomeningocele: correction of deformity and alteration of functional status

The effect of spinal fusion for paralytic scoliosis in 49 patients with myelomeningocele was studied. Improved sitting balance was seen in 70% of the patients following anterior and posterior fusions, 67% following posterior fusion alone, and 28% after anterior fusion alone. The ability to ambulate was adversely affected in 67% of the patients who had undergone combined anterior and posterior fusions, in 57% following anterior fusion alone, and in 27% following posterior fusion alone. Sitting is likely to be improved but ambulation may be more difficult following spinal correction and fusion.