Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia

Godzik, Jakub; Kelly, Michael P.; Radmanesh, Alireza; Kim, David; Holekamp, Terrence F.; Smyth, Matthew D.; Lenke, Lawrence G.; Shimony, Joshua S.; Park, Tae Sung; Leonard, Jeffrey R.; Limbrick, David D.

doi:10.3171/2014.1.peds13105

Cited by 43 publications

(24 citation statements)

References 47 publications

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“…The radiological variables recorded on MRI included degree of tonsillar descent below the FM (Fig. 1A), 10,27 characteristics of the syrinx (diameter, levels, location, and type; i.e., central, enlarged, or deviated) (Fig. 1B-D), 20,21 presence or absence of scoliosis, maximum axial width of the fourth ventricle, 30 odontoid retroversion (angle between the base of C-2 and its intersection with a line from the odontoid tip) ( Fig.…”

Section: Preoperative Evaluationmentioning

confidence: 99%

A points-based algorithm for prognosticating clinical outcome of Chiari malformation Type I with syringomyelia: results from a predictive model analysis of 82 surgically managed adult patients

Thakar

Sivaraju

Jacob

et al. 2018

Journal of Neurosurgery: Spine

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OBJECTIVEAlthough various predictors of postoperative outcome have been previously identified in patients with Chiari malformation Type I (CMI) with syringomyelia, there is no known algorithm for predicting a multifactorial outcome measure in this widely studied disorder. Using one of the largest preoperative variable arrays used so far in CMI research, the authors attempted to generate a formula for predicting postoperative outcome.METHODSData from the clinical records of 82 symptomatic adult patients with CMI and altered hindbrain CSF flow who were managed with foramen magnum decompression, C-1 laminectomy, and duraplasty over an 8-year period were collected and analyzed. Various preoperative clinical and radiological variables in the 57 patients who formed the study cohort were assessed in a bivariate analysis to determine their ability to predict clinical outcome (as measured on the Chicago Chiari Outcome Scale [CCOS]) and the resolution of syrinx at the last follow-up. The variables that were significant in the bivariate analysis were further analyzed in a multiple linear regression analysis. Different regression models were tested, and the model with the best prediction of CCOS was identified and internally validated in a subcohort of 25 patients.RESULTSThere was no correlation between CCOS score and syrinx resolution (p = 0.24) at a mean ± SD follow-up of 40.29 ± 10.36 months. Multiple linear regression analysis revealed that the presence of gait instability, obex position, and the M-line–fourth ventricle vertex (FVV) distance correlated with CCOS score, while the presence of motor deficits was associated with poor syrinx resolution (p ≤ 0.05). The algorithm generated from the regression model demonstrated good diagnostic accuracy (area under curve 0.81), with a score of more than 128 points demonstrating 100% specificity for clinical improvement (CCOS score of 11 or greater). The model had excellent reliability (κ = 0.85) and was validated with fair accuracy in the validation cohort (area under the curve 0.75).CONCLUSIONSThe presence of gait imbalance and motor deficits independently predict worse clinical and radiological outcomes, respectively, after decompressive surgery for CMI with altered hindbrain CSF flow. Caudal displacement of the obex and a shorter M-line–FVV distance correlated with good CCOS scores, indicating that patients with a greater degree of hindbrain pathology respond better to surgery. The proposed points-based algorithm has good predictive value for postoperative multifactorial outcome in these patients.

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Section: Preoperative Evaluationmentioning

confidence: 99%

A points-based algorithm for prognosticating clinical outcome of Chiari malformation Type I with syringomyelia: results from a predictive model analysis of 82 surgically managed adult patients

Thakar

Sivaraju

Jacob

et al. 2018

Journal of Neurosurgery: Spine

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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%

“…3 There also is an association between increased syrinx size and presence of scoliosis in patients with Chiari Type I malformations. 4 Thirty-seven IS patients had concomitant scoliosis, and 89% of these patients had clinical follow-up. The majority of patients had idiopathic 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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“…13 In children, scoliosis is a common presenting symptom of syringomyelia, 7,8 and larger syrinx size appears to increase the risk of scoliosis. 5 The classic findings associated with cervical syrinx of loss of pain and temperature sensation in a cape-like distribution can be difficult to assess in a young child. More subtle signs, such as clumsiness, difficulty running, or torticollis may be the presenting features in children.…”

Section: Discussionmentioning

confidence: 99%

Severe holocord syrinx in a child with megalencephaly-capillary malformation syndrome

Segal¹,

Heary

Sabharwal

et al. 2016

PED

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The authors present the case of a child with megalencephaly-capillary malformation syndrome who developed a rapidly progressive holocord syringomyelia that was treated surgically. A 3-year-old boy with megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome presented with several months of right leg weakness, worsening scoliosis, and increased seizures. An MRI study of the brain demonstrated a Chiari I malformation and massively dilated syringomyelia extending from C-2 to the conus medullaris. The patient underwent an urgent suboccipital craniectomy with C1–3 laminectomies to relieve the CSF outflow obstruction with significant clinical improvement. Surgery was complicated by bleeding from intracranial vascular malformations. This report describes a very rapidly developing, massive holocord syringomyelia related to CSF obstruction due to an unusual congenital brain malformation and associated vascular overgrowth at the site. Serial, premorbid MRI studies demonstrated the very rapid progression from no Chiari malformation, to progressively greater cerebellar tonsillar herniation, to holocord syrinx. This complication has never been reported in MCAP syndrome and should be considered in any affected MCAP patient with a progressive neurological decline, even if previous spine imaging findings were normal. Surgical complications due to hemorrhage also need to be considered in this vascular brain malformation.

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Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation Type I with associated syringomyelia

Cited by 43 publications

References 47 publications

A points-based algorithm for prognosticating clinical outcome of Chiari malformation Type I with syringomyelia: results from a predictive model analysis of 82 surgically managed adult patients

A points-based algorithm for prognosticating clinical outcome of Chiari malformation Type I with syringomyelia: results from a predictive model analysis of 82 surgically managed adult patients

Management of idiopathic pediatric syringohydromyelia

Severe holocord syrinx in a child with megalencephaly-capillary malformation syndrome

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