Scoliosis in children with Chiari I-related syringomyelia

Bhangoo, Ranjeev; Sgouros, Spyros

doi:10.1007/s00381-006-0090-y

Cited by 41 publications

(35 citation statements)

References 11 publications

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“…In addition, Bhangoo et al studied 13 patients with symptomatic CM I and clinically detected scoliosis who underwent primary cranio-vertebral decompression. The patients requiring scoliosis corrective surgery had a mean age of 158 months and a 76°Cobb angle [2]. All adolescent patients in our series presented large and predictable progressed scoliotic curves, therefore proper spinal deformity corrections and fusions were essential.…”

Section: Discussionmentioning

confidence: 91%

One-stage and posterior approach for correction of moderate to severe scoliosis in adolescents associated with Chiari I malformation: is a prior suboccipital decompression always necessary?

et al. 2011

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Priority of neurological decompression was regarded as necessary for scoliosis patients associated with Chiari I malformation in order to decrease the risk of spinal cord injury from scoliosis surgery. We report a retrospective series of scoliosis associated with Chiari I malformation in 13 adolescent patients and explore the effectiveness and safety of posterior scoliosis correction without suboccipital decompression. One-stage posterior approach total vertebral column resection was performed in seven patients with scoliosis or kyphosis curve [90°(average 100.1°s coliotic and 97.1°kyphotic curves) or presented with apparent neurological deficits, whereas the other six patients underwent posterior pedicle screw instrumentation for correction of spinal deformity alone (average 77.3°s coliotic and 44.0°kyphotic curves). The apex of the scoliosis curve was located at T7-T12. Mean operating time and intraoperative hemorrhage was 463 min and 5,190 ml in patients undergoing total vertebral column resection, with average correction rate of scoliosis and kyphosis being 63.3 and 71.1%, respectively. Mean operating time and intraoperative hemorrhage in patients undergoing instrumentation alone was 246 min and 1,450 ml, with the average correction rate of scoliosis and kyphosis being 60.8 and 53.4%, respectively. The mean follow-up duration was 32.2 months. No iatrogenic neurological deterioration had been encountered during the operation procedure and follow-up. After vertebral column resection, neurological dysfunctions such as relaxation of anal sphincter or hypermyotonia that occurred in three patients preoperatively improved gradually. In summary, suboccipital decompression prior to correction of spine deformity may not always be necessary for adolescent patients with scoliosis associated with Chiari I malformation. Particularly in patients with a severe and rigid curve or with significant neurological deficits, posterior approach total vertebral column resection is likely a good option, which could not only result in satisfactory correction of deformity, but also decrease the risk of neurological injury secondary to surgical intervention by shortening spine and reducing the tension of spinal cord.

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Section: Discussionmentioning

confidence: 91%

One-stage and posterior approach for correction of moderate to severe scoliosis in adolescents associated with Chiari I malformation: is a prior suboccipital decompression always necessary?

et al. 2011

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“…It is not surprising that the decrease in syrinx size correlates with scoliosis improvement, as the syrinx is likely to be the cause of the scoliotic curve, and previous studies have commonly noted the occurrence of scoliosis in up to 60% of patients with syrinx. 1,3,[6][7][8][9]12 In our scoliosis cohort there was a predominance of females, constituting 80% of our series. This was a natural variant of our population, as we did not include or exclude patients on the basis of sex.…”

Section: Discussionmentioning

confidence: 99%

Suboccipital decompression for Chiari malformation–associated scoliosis: risk factors and time course of deformity progression

Attenello¹,

McGirt²,

Atiba³

et al. 2008

PED

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Object Chiari malformation Type I (CM-I) is often associated with scoliosis. It remains unclear which subgroups of patients are most likely to experience progression of spinal deformity after cervicomedullary decompression. The authors' goal was to determine the time frame of curvature progression and assess which patient subgroups are at greatest risk for progression of spinal deformity after surgery. Methods The authors retrospectively reviewed the records of all pediatric patients with significant scoliosis in whom suboccipital decompression was performed to treat for CM-I during a 10-year period at a single academic institution. Clinical, radiological, and operative variables were assessed as independent factors for failure (worsening of scoliosis) by using a univariate regression analysis. Results Twenty-one children (mean age 9 ± 3 years; 4 male) underwent hindbrain decompression for CM-I–associated scoliosis and were followed for a mean of 39 months. All patients harbored a syrinx. Eight patients (38%) experienced improvement in scoliosis curvature, whereas 10 (48%) suffered a progression. Thoracolumbar junction scoliosis (p = 0.04) and failure of the syrinx to improve (p = 0.05) were associated with 5- and 4-fold respective increases in the likelihood of deformity progression. Each increasing degree of preoperative Cobb angle was associated with an 11% increase in the likelihood of scoliotic curve progression (p < 0.05). Conclusions Over one third of patients with CM-I–associated scoliosis will improve after cervicomedullary decompression alone. Cervicomedullary decompression is a good first-line option, particularly in children with concordant posterior fossa symptoms. Patients presenting with more severe scoliosis (increasing Cobb angle) or scoliosis that crosses the thoracolumbar junction may benefit from earlier orthopedic involvement and should be monitored regularly for curvature progression after cervicomedullary decompression. In cases in which there is a failure of the syrinx to show improvement after suboccipital decompression, the patients are also more likely to develop curvature progression.

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“…Median clinical follow-up was 20.5 months, and median radiological follow-up was 13 months. The median maximum syrinx diameter was 2 mm (range 0.5-17 mm) and the median number of vertebral levels spanned was 5 (range [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. Eighty-one of the patients had a syrinx diameter equal to or greater than 2 mm.…”

Section: Resultsmentioning

confidence: 99%

“…Scoliosis has been associated with syringomyelia and can be a sign of developing syringomyelia. 1 Controversy exists on the best management of these patients. There are studies which indicate that treatment of the syrinx does not improve the scoliosis.…”

Section: Discussionmentioning

confidence: 99%

Management of idiopathic pediatric syringohydromyelia

Rodriguez

Kuhn

Somasundaram

et al. 2015

PED

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OBJECT Syringohydromyelia is frequently identified on spinal imaging. The literature provides little guidance to decision making regarding the need for follow-up or treatment. The purpose of this study was to review the authors' experience in managing pediatric syringohydromyelia of unknown cause. METHODS A single-institution retrospective review of all cases involving pediatric patients who underwent spinal MRI from 2002 to 2012 was conducted. Patients with idiopathic syringohydromyelia (IS) were identified and categorized into 2 subgroups: uncomplicated idiopathic syrinx and IS associated with scoliosis. Clinical and radiological course were analyzed. RESULTS Ninety-eight patients (50 female, 48 male) met the inclusion criteria. Median age at diagnosis of syrinx was 11.9 years. Median maximum syrinx size was 2 mm (range 0.5–17 mm) and spanned 5 vertebral levels (range 1–20 vertebral levels). Thirty-seven patients had scoliosis. The most common presenting complaint was back pain (26%). Clinical follow-up was available for 78 patients (80%), with a median follow-up of 20.5 months (range 1–143 months). A neurological deficit existed at presentation in 36% of the patients; this was either stable or improved at last follow-up in 64% of cases. Radiological follow-up was available for 38 patients (39%), with a median duration of 13 months (range 2–83 months). There was no change in syrinx size in 76% of patients, while 16% had a decrease and 8% had an increase in syrinx size. Thirty-six patients had both clinical and radiological follow-up. There was concordance between clinical and radiological course in 14 patients (39%), with 11 patients (31%) showing no change and 3 patients (8%) showing clinical and radiological improvement. No patients had concurrent deterioration in clinical and radiological course. One patient with scoliosis and muscular dystrophy underwent direct surgical treatment of the syrinx and subsequently had a deteriorated clinical course and decreased syrinx size. CONCLUSIONS There remains a paucity of data regarding the management of pediatric IS. IS in association with scoliosis can complicate neurosurgical decision making. There was no concordance between radiological syrinx size increase and clinical deterioration in this cohort, indicating that surgical decision making should reflect clinical course as opposed to radiological course.

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Scoliosis in children with Chiari I-related syringomyelia

Abstract: Cranio-vertebral decompression for Chiari I may prevent the need for corrective scoliosis surgery when performed before the age of ten and below a Cobb angle of 30 degrees.

Cited by 41 publications

References 11 publications

One-stage and posterior approach for correction of moderate to severe scoliosis in adolescents associated with Chiari I malformation: is a prior suboccipital decompression always necessary?

One-stage and posterior approach for correction of moderate to severe scoliosis in adolescents associated with Chiari I malformation: is a prior suboccipital decompression always necessary?

Suboccipital decompression for Chiari malformation–associated scoliosis: risk factors and time course of deformity progression

Management of idiopathic pediatric syringohydromyelia

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