Complex Chiari Malformations in Children

Brockmeyer, Douglas L.; Spader, Heather S.

doi:10.1016/j.nec.2015.06.002

Cited by 29 publications

(14 citation statements)

References 13 publications

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“…It is generally agreed that some of these patients are more likely to require craniocervical fusion. 18,19…”

Section: Complex Chiarimentioning

confidence: 99%

“…13 Occipitocervical fusion may be indicated in patients with craniocervical instability. 19 The most common complication is leakage of CSF from the incision and ranges from 4.7% to 9.6%. 6,46 Contained leakage in the form of pseudomeningocele often is not regarded as a complication, although evidence has shown that such pseudomeningocele may act as a compliance reservoir and thereby impair resolution of a syrinx cavity.…”

Section: Adult Chiari Surgerymentioning

confidence: 99%

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Chiari malformation and syringomyelia

Holly¹,

Batzdorf²

2019

Journal of Neurosurgery: Spine

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Chiari malformation was first described over a century ago, and consists of posterior fossa anomalies that generally share the feature of cerebellar tonsillar descent through the foramen magnum. Our understanding of this disorder was initially based on autopsy studies, and has been greatly enhanced by the advent of MRI. The surgical management of Chiari anomalies has also evolved in a parallel fashion. Although the exact surgical technique varies among individual surgeons, the goals of surgery remain constant and consist of relieving brainstem compression and cranial nerve distortion, restoring the normal flow of CSF across the foramen magnum, and reducing the size of any associated syrinx cavity. Syrinx cavities are most commonly associated with Chiari anomalies, yet primary spinal syringomyelia (PSS) can be caused by traumatic, infectious, degenerative, and other etiologies that cause at least a partial CSF flow obstruction in the spinal subarachnoid space. As with syringomyelia associated with Chiari anomalies, the main goal of PSS surgery is to reestablish CSF flow across the area of obstruction. In addition to MRI, myelography with CT can be very helpful in the evaluation and management of these patients by identifying focal regions of CSF obstruction that may be amenable to surgical intervention. Future directions for the treatment of Chiari anomalies and syringomyelia include the application of advanced imaging techniques, more widespread use of genetic evaluation, large-scale outcome studies, and the further refinement of surgical technique.

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“…It is generally agreed that some of these patients are more likely to require craniocervical fusion. 18,19…”

Section: Complex Chiarimentioning

confidence: 99%

Section: Adult Chiari Surgerymentioning

confidence: 99%

Chiari malformation and syringomyelia

Holly¹,

Batzdorf²

2019

Journal of Neurosurgery: Spine

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“…1 Questioning the utility of this definition, the relationship between tonsil position and measures of disease severity, such as syringomyelia and surgical treatment with posterior fossa decompression (PFD), has been inconsistently reported in the CM-I literature. [2][3][4][5][6][7][8][9][10][11][12][13][14][15] These results emphasize the need for improved understanding of the association between radiological craniovertebral junction (CVJ) parameters and clinical disease in cases of CM-I.…”

mentioning

confidence: 99%

“…16 Obex position is one CVJ measure that has been investigated in CM-I, and an obex position below the level of the foramen magnum, in combination with tonsillar ectopia, has been classified by some authors as a further disease subset, Chiari 1.5 (CM-1.5). 4,16 However, an obex position is inconsistently reported in the CM-I literature, and the degree to which CM-1.5 represents a distinct disease subset is unclear. 4,17 While limited evidence in adult CM-I patients suggests obex position may predict symptom resolution after PFD, 15 the clinical importance of obex position in children remains to be elucidated.…”

mentioning

confidence: 99%

“…4,16 However, an obex position is inconsistently reported in the CM-I literature, and the degree to which CM-1.5 represents a distinct disease subset is unclear. 4,17 While limited evidence in adult CM-I patients suggests obex position may predict symptom resolution after PFD, 15 the clinical importance of obex position in children remains to be elucidated. Given this gap in the existing literature, the objective of this study was to investigate the association of obex position, tonsillar position, and other CVJ morphometric measurements with measures of clinical disease severity in children with CM-I.…”

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confidence: 99%

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Obex position is associated with syringomyelia and use of posterior fossa decompression among patients with Chiari I malformation

Haller

Sadler

Kuensting

et al. 2020

Journal of Neurosurgery: Pediatrics

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OBJECTIVEChiari I malformation (CM-I) has traditionally been defined by measuring the position of the cerebellar tonsils relative to the foramen magnum. The relationships of tonsillar position to clinical presentation, syringomyelia, scoliosis, and the use of posterior fossa decompression (PFD) surgery have been studied extensively and yielded inconsistent results. Obex position has been proposed as a useful adjunctive descriptor for CM-I and may be associated with clinical disease severity.METHODSA retrospective chart review was performed of 442 CM-I patients with MRI who presented for clinical evaluation between 2003 and 2018. Clinical and radiological variables were measured for all patients, including presence/location of headaches, Chiari Severity Index (CSI) grade, tonsil position, obex position, clival canal angle, pB-C2 distance, occipitalization of the atlas, basilar invagination, syringomyelia, syrinx diameter, scoliosis, and use of PFD. Radiological measurements were then used to predict clinical characteristics using regression and survival analyses, with performing PFD, the presence of a syrinx, and scoliosis as outcome variables.RESULTSAmong the radiological measurements, tonsil position, obex position, and syringomyelia were each independently associated with use of PFD. Together, obex position, tonsil position, and syringomyelia (area under the curve [AUC] 89%) or obex position and tonsil position (AUC 85.4%) were more strongly associated with use of PFD than tonsil position alone (AUC 76%) (Pdiff = 3.4 × 10−6 and 6 × 10−4, respectively) but were only slightly more associated than obex position alone (AUC 82%) (Pdiff = 0.01 and 0.18, respectively). Additionally, obex position was significantly associated with occipital headaches, CSI grade, syringomyelia, and scoliosis, independent of tonsil position. Tonsil position was associated with each of these traits when analyzed alone but did not remain significantly associated with use of PFD when included in multivariate analyses with obex position.CONCLUSIONSCompared with tonsil position alone, obex position is more strongly associated with symptomatic CM-I, as measured by presence of a syrinx, scoliosis, or use of PFD surgery. These results support the role of obex position as a useful radiological measurement to inform the evaluation and potentially the management of CM-I.