Management of idiopathic pediatric syringohydromyelia

Rodriguez, Analiz; Kuhn, Elizabeth N.; Somasundaram, Aravind; Couture, Daniel E.

doi:10.3171/2015.3.peds14433

Cited by 14 publications

(6 citation statements)

References 19 publications

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“…The association between syringomyelia and scoliosis is well documented. Rodriguez et al [ 11 ] found the incidence of scoliosis to be 49.1% in patients with IS. The frequency of scoliosis in patients with CM1 and syringomyelia is as high as 80% [ 20 ].…”

Section: Discussionmentioning

confidence: 99%

“…The incidences of scoliosis in IS and CM1 with syringomyelia are about 14%-50% and 25%-85%, respectively. However, the incidence of scoliosis in patients with CM1 without syringomyelia is about 4%, which suggests the presence of alternate pathomechanisms [9][10][11][12]. Syringomyelia is the second commonest cord anomaly seen in congenital scoliosis.…”

Section: Introductionmentioning

confidence: 99%

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Pattern of Syringomyelia in Presumed Idiopathic and Congenital Scoliosis

et al. 2021

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Study Design: Retrospective study.Purpose: This study was undertaken to compare the patterns of syringomyelia in patients with presumed idiopathic and congenital scoliosis.Overview of Literature: The incidence of neuraxial anomalies presenting as idiopathic scoliosis ranges from 2% to 14%; the common ones are idiopathic syringomyelia (IS) and Chiari malformation type 1 (CM1) with syringomyelia. Some authors have speculated that scoliosis is caused by the asymmetrical compression of anterior horn cells by a syrinx, which causes an imbalance of the trunk musculature. In congenital scoliosis, syringomyelia is the second commonest cord anomaly, and the deformity progression depends upon the underlying vertebral abnormality, the location of the abnormality, and the age of patient, and is independent of the intraspinal anomaly.Methods: We analyzed the radiological records of 44 consecutive patients with scoliosis and syringomyelia. Of these 44 patients, 13 had IS, 12 had CM1, and 19 had congenital scoliosis. The radiographs were evaluated to determine the curve magnitude, sagittal alignment, side of convexity, and type of vertebral anomaly, if any. T1- and T2-weighted magnetic resonance imaging of the whole spine was analyzed to determine the presence of craniovertebral anomalies, syrinx length, syrinx diameter, and syrinx-cord ratio (SCR).Results: The frequency of left convex curves was 26.1%, with no significant differences across the three groups. The mean length of the syrinx was 7.2±4.9 vertebral levels, and the mean SCR was 0.39±0.2. The mean syrinx length was significantly higher in patients with CM1 and IS, compared to patients with congenital scoliosis. The mean SCR was highest in patients with CM1. In congenital scoliosis, syringomyelia was seen most frequently in patients with a failure of formation, and 63.2% had concomitant cord anomalies.Conclusions: Syrinxes were smaller in size and length in patients with congenital scoliosis, and are distinct from those seen in IS and CM1.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pattern of Syringomyelia in Presumed Idiopathic and Congenital Scoliosis

et al. 2021

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“…As a common type of neuromuscular scoliosis, the rate of scoliosis in syringomyelia patients ranges from 25 to 74.4% [16]. Rodriguez et al [17] found that 49.1% of cases were "idiopathic" scoliosis among IS patients. Many studies have reported that syringomyelia associated scoliosis was unintentionally found in "presumed" idiopathic scoliosis [18,19].…”

Section: Discussionmentioning

confidence: 99%

Selective thoracolumbar/lumbar fusion for Syringomyelia-associated scoliosis: a case-control study with Lenke 5C adolescent idiopathic scoliosis

Feng

Shen

Chen

et al. 2020

BMC Musculoskelet Disord

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Background Selective thoracolumbar/lumbar fusion technique was introduced to treat adolescent idiopathic scoliosis (AIS) patients with major thoracolumbar/lumbar curves. Theoretically, this surgical strategy could also be applied to syringomyelia patients. No previous study has specifically addressed the effectiveness of selective thoracolumbar/lumbar fusion for patients with syringomyelia-associated scoliosis. The aim of the study was to investigate the effectiveness of selective thoracolumbar/lumbar fusion for the surgical treatment of patients with syringomyelia-associated scoliosis. Methods From February 2010 to September 2016, 14 syringomyelia-associated patients with major thoracolumbar/lumbar curves were retrospectively reviewed. Besides, 30 Lenke 5C AIS patients were enrolled as a control group. Posterior selective thoracolumbar/lumbar fusion was performed for both groups. Patients’ demographic, operative, radiological, and quality of life data were reviewed with follow-up. Intragroup comparisons were performed for each parameter. Results The two groups were matched by age, gender, curve characteristics, duration of follow-up, and all preoperative radiographic parameters except for thoracic kyphosis. After surgery, the average correction rate of the major thoracolumbar/lumbar curve was 82.2 ± 7.8% in the syringomyelia group, which was not significantly different from that of AIS group (82.5 ± 10.6%, P = 0.47). A similar improvement of unfused thoracic curve was observed between the two groups (50.1 ± 16.5% vs. 48.5 ± 26.9%, P = 0.29). During the follow-up, the correction effect of scoliosis was well maintained, without aggravation of the original neural symptoms or fresh permanent neurological deficits. Of note, the number of fusion levels was significantly larger in syringomyelia group than that in AIS group (7.6 ± 1.4 vs. 6.5 ± 1.2, P < 0.01). The average follow up was 47.6 months (36–81 months). Conclusion Similar to AIS cases, syringomyelia-associated scoliosis can be effectively and safely corrected by selective thoracolumbar/lumbar fusion with satisfactory surgical outcomes. However, the syringomyelia group, on average, required an additional fused segment for treatment as compared to the AIS group (7.6 versus 6.5 in the AIS group).

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“…A number of speculations and hypotheses have been suggested that evaluate the pathogenesis and treatment of this vexed clinical problem. [ 3 4 5 6 7 ] In the year 2000, we classified syringomyelia into three groups depending on the identified etiological factor. [ 12 ] Group 1 cases included those where no definite cause could be identified.…”

Section: Discussionmentioning

confidence: 99%

“…[ 1 2 ] Factors like trauma, infections, tumors and craniovertebral junctional abnormalities have been incriminated to be causative. However, there is a significant subgroup where no cause is identified and syringomyelia in such patients is labeled to be “idiopathic.”[ 3 4 5 6 7 ] In general, in the later group of patients no clear direction regarding pathogenesis or treatment has yet been specified in the literature. We evaluated nine patients, who were earlier diagnosed on the basis of standard discussed parameters to have “idiopathic” syringomyelia.…”

Section: Introductionmentioning

confidence: 99%

Treatment of “idiopathic” syrinx by atlantoaxial fixation: Report of an experience with nine cases

Shah

Sathe

Patil

et al. 2017

J Craniovert Jun Spine

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Objective:The authors evaluate the significance of atlantoaxial instability in the management of idiopathic syringomyelia.Background:We recently observed that atlantoaxial dislocation can be present even when the atlantodental interval was within normal range. Atlantoaxial instability can be identified on the basis of facetal mal-alignment or even by direct observation of status of joint during surgery. Our observations are discussed in nine patients where we identified and treated atlantoaxial instability in cases that would otherwise be considered as having “idiopathic” syrinx.Materials and Methods:The authors report experience with nine cases that were diagnosed to have “idiopathic” syrinx. The main bulk of the syrinx was located in the cervico-dorsal spinal region in all cases. One patient had been treated earlier by syringo-subarachnoid shunt surgery and one patient had undergone foramen magnum decompression.Results:On radiological evaluation, eight patients had posterior atlantoaxial facetal (Type B) dislocation. In one patient there was no facetal mal-alignment and was labeled to have axial or central (Type C) facetal instability. All patients were treated by atlantoaxial fixation. All patients improved symptomatically in the immediate postoperative period and the improvement was progressive and sustained on follow-up. In one case, the size of syrinx reduced in the immediate postoperative imaging. In the period of follow-up (range 6–42 months - average 19 months), reduction in the size of syrinx was demonstrated on imaging in three cases.Conclusions:The positive clinical outcome suggests that atlantoaxial instability may be the defining phenomenon in development of previously considered “idiopathic” syringomyelia.

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Management of idiopathic pediatric syringohydromyelia

Cited by 14 publications

References 19 publications

Pattern of Syringomyelia in Presumed Idiopathic and Congenital Scoliosis

Pattern of Syringomyelia in Presumed Idiopathic and Congenital Scoliosis

Selective thoracolumbar/lumbar fusion for Syringomyelia-associated scoliosis: a case-control study with Lenke 5C adolescent idiopathic scoliosis

Treatment of “idiopathic” syrinx by atlantoaxial fixation: Report of an experience with nine cases

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