Prevalence of Chiari I Malformation and Syringomyelia

Kahn, Elyne N; Muraszko, Karin M.; Maher, Cormac O.

doi:10.1016/j.nec.2015.06.006

Cited by 89 publications

(44 citation statements)

References 41 publications

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“…[1,5] The main limitation of these studies was that individuals undergoing MRI were not representative of the general population. [18] In our retrospective study, we found the prevalence as 4% on cervical MR images, higher compared to other studies in the literature. This higher prevalence was supposed to be due to definition of the disease as a herniation if more than 5 mm in some previous studies.…”

Section: Anatomy • Volume 10 / Issue 1 / April 2016contrasting

confidence: 46%

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Prevalence of Chiari type I malformation on cervical magnetic resonance imaging: a retrospective study

Oktem¹,

Dilli²,

Kürkçüoğlu³

et al. 2016

Anatomy

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Chiari malformation, also known as Arnold-Chiari malformation, is characterized by the caudal herniation of posterior fossa structures through the foramen magnum into the cervical spinal canal. It is a congenital malformation of the craniovertebral junction and hindbrain often associated with other abnormalities of the cerebrospinal axis. [1][2][3][4][5][6][7][8] Chiari malformation is classically classified into four types. Chiari type I is the caudal herniation of cerebellar tonsils through foramen magnum by no more than 3 or 5 mm. [1,3,4,6,7,[9][10][11] In Chiari type II, there is a caudal herniation of the brainstem and the fourth ventricle into upper cervical canal through foramen magnum, frequently associated with myelomeningocele, and rarely associated with spina bifida occulta. Chiari type III is the most severe form in which posterior fossa structures herniate into an occipitocervical meningocele sac. Severe cerebellar hypoplasia in absence of cerebellar herniation is observed in Chiari type AbstractObjectives: Chiari malformation is characterized by caudal herniation of cerebellar tonsils from foramen magnum into the cervical canal on radiological images. Type I Chiari malformation is the herniation of cerebellar tonsils more than 3 mm which does not cause any symptoms until adulthood. In this study, we aimed to investigate the prevalence of Chiari type I malformation. Methods:In this retrospective study, cervical magnetic resonance images taken between 2013 and 2015 were retrospectively analyzed. The patients were 18-70 year old males and females. Type I Chiari malformation was defined as an inferior displacement of the cerebellar tonsils more than 3 mm below the opisthion-basion line. The patients with intracranial lesions, hydrocephalus or previous craniotomy were excluded from the study. Results:The prevalence of Chiari type I malformation was found as 4% when both genders were taken into consideration. The prevalence was 3.3% in females and 0.7% in males. Chiari type I malformation was associated with syringomyelia in 0.5% of females and 0.2% of males. In the present study, concomitant syringomyelia was observed in 0.7% of the patients. On the other hand, incidence of Chiari type I malformation was found relatively higher in the patients with scoliosis (15.4%). The ratio of this co-occurrence was higher in females (11.4%) when compared to the males (4%). Conclusion:In the present study, the prevalence of Chiari type I malformation was investigated in a large population, consisting of 2480 individuals, and its association with syringomyelia and scoliosis.

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Section: Anatomy • Volume 10 / Issue 1 / April 2016contrasting

confidence: 46%

“…[3] Lower tonsil positions were also associated with syringomyelia. [18] Elam and Vaughn reported syringomyelia in approximately 25% of the patients with Chiari malformation type I. [1] [24] Banik et al (2006) [23] Patients with PTC > 5 mm 10…”

Section: Anatomy • Volume 10 / Issue 1 / April 2016mentioning

confidence: 99%

Prevalence of Chiari type I malformation on cervical magnetic resonance imaging: a retrospective study

Oktem¹,

Dilli²,

Kürkçüoğlu³

et al. 2016

Anatomy

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“…MRI studies of the brain and spinal cord are essential to confirm the degree of CIM and any associated intracranial or spinal cord abnormalities. Asymptomatic CIM is extremely common,14 and, since the natural history in the majority of such cases is favourable, most authorities would not recommend treatment on a prophylactic basis. At Great Ormond Street Hospital (GOSH), treatment is offered to patients with progressive syringomyelia or scoliosis or neurological symptoms that are attributable to the CIM (eg, Chiari-type headache).…”

Section: Introductionmentioning

confidence: 99%

Headache outcomes in children undergoing foramen magnum decompression for Chiari I malformation

et al. 2017

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“…Damage to cord tissue can result in pain and neurological deficits. Treatment options for syringomyelia are limited to surgical decompression or correction of deformity, arachnolysis or shunting procedures to facilitate drainage [4][5][6]. Surgical treatments are invasive, carry high risks of complication, and result in unsatisfactory long-term success rates in approximately 50% of cases [2].…”

Section: Introductionmentioning

confidence: 99%

Abnormalities in spinal cord ultrastructure in a rat model of post-traumatic syringomyelia

Berliner

Hemley

Najafi

et al. 2020

Fluids Barriers CNS

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Background: Syringomyelia is a serious complication of spinal cord trauma, occurring in approximately 28% of spinal cord injuries. Treatment options are limited and often produce unsatisfactory results. Post-traumatic syringomyelia (PTS) is presumably related to abnormalities of cerebrospinal fluid (CSF) and interstitial fluid hydrodynamics, but the exact mechanisms are unknown.Methods: Transmission electron microscopy (TEM) was used to investigate in detail the interfaces between fluid and tissue in the spinal cords of healthy Sprague-Dawley rats (n = 3) and in a rat model of PTS (n = 3). PTS was induced by computer-controlled impact (75 kDyn) to the spinal cord between C6 and C8, followed by a subarachnoid injection of kaolin to produce focal arachnoiditis. Control animals received a laminectomy only to C6 and C7 vertebrae. Animals were sacrificed 12 weeks post-surgery, and spinal cords were prepared for TEM. Ultra-thin spinal cord sections at the level of the injury were counterstained for structural anatomy. Results:Spinal cords from animals with PTS displayed several abnormalities including enlarged perivascular spaces, extracellular edema, cell death and loss of tissue integrity. Additionally, alterations to endothelial tight junctions and an abundance of pinocytotic vesicles, in tissue adjacent to syrinx, suggested perturbations to blood-spinal cord barrier (BSCB) function. Conclusions:These findings support the hypothesis that perivascular spaces are important pathways for CSF flow into and out of the spinal cord, but also suggest that fluid may enter the cord through vesicular transport and an altered BSCB.

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Prevalence of Chiari I Malformation and Syringomyelia

Cited by 89 publications

References 41 publications

Prevalence of Chiari type I malformation on cervical magnetic resonance imaging: a retrospective study

Prevalence of Chiari type I malformation on cervical magnetic resonance imaging: a retrospective study

Headache outcomes in children undergoing foramen magnum decompression for Chiari I malformation

Abnormalities in spinal cord ultrastructure in a rat model of post-traumatic syringomyelia

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