Potential association among posterior fossa bony volume and crowdedness, tonsillar hernia, syringomyelia, and CSF dynamics at the craniocervical junction in Chiari malformation type I

Wang, Shengxi; Zhang, Dongao; Wu, Kun; Fan, Wayne; Fan, Tao

doi:10.3389/fneur.2023.1069861

Cited by 3 publications

(1 citation statement)

References 33 publications

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“…CSF hydrodynamic parameters have been increasingly recognized as a predictive factor for the surgical effect and prognosis. Previous studies have revealed that the normalization of CSF dynamic parameters was related to the improvement in clinical symptoms, and patients with greater abnormal preoperative CSF flow had a better clinical response to suboccipital decompression (14,(32)(33)(34). Fakhri et al (15) and Shaffer et al (35) showed that normalization of CSF dynamic parameters could be seen in MRI of symptomatic CM patients after operation.…”

Section: Discussionmentioning

confidence: 98%

A long-term follow-up study of adults with Chiari malformation type I combined with syringomyelia

Hu,

Zhang,

Duan

et al. 2023

Front. Neurol.

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BackgroundThere is a considerable amount of controversy regarding the treatment and prognosis of adult patients with Chiari malformation type I (CM-I) at home and abroad; furthermore, no large-sample, long-term, follow-up studies have examined CM-I patients with syringomyelia (SM) comparing posterior fossa decompression with resection of tonsils (PFDRT) vs. posterior fossa decompression with duraplasty (PFDD).ObjectiveThis study retrospectively analyzed the factors affecting the treatment and long-term prognosis of adults with CM-I combined with SM.MethodsWe retrospectively analyzed data from 158 adult CM-I patients combined with SM who underwent PFDRT or PFDD, including 68 patients in group PFDRT and 90 patients in group PFDD. We examined the clinical manifestations, imaging features, and follow-up data of patients. Clinical outcomes were assessed using the Chicago Chiari Outcomes Scale (CCOS), and radiographic outcomes were indicated by the syrinx remission rate. Multivariate logistic regression analysis and multiple linear regression analysis were used to explore the relevant factors affecting the long-term prognosis of patients.ResultsThis study showed that compared with preoperative patients in the PFDRT group and PFDD group, the sensory impairment, cough-related headache, and movement disorder were significantly improved (p < 0.01); meanwhile, the diameter of the syrinx and the volume of the syrinx decreased significantly (p < 0.001). Additionally, the study found that there were significant differences in the syrinx remission rate (p = 0.032) and the clinical cure rates (p = 0.003) between the two groups. Multivariate logistic regression analysis showed that age (p = 0.021), cerebellar-related symptoms (p = 0.044), preoperative cisterna magna volume (p = 0.043), and peak systolic velocity (p = 0.036) were independent factors for clinical outcomes. Multiple linear regression analysis showed that different surgical procedures were positively correlated with the syrinx remission rate (p = 0.014), while preoperative syrinx diameter (p = 0.018) and age (p = 0.002) were negatively correlated with the syrinx remission rate.ConclusionIn conclusion, this study suggested that, in a long-term follow-up, although both surgical procedures are effective in treating patients with CM-I and SM, PFDRT is better than PFDD; age and cerebellar-related signs independently affect the patient’s prognosis. Additionally, an effective prognosis evaluation index can be developed for patients, which is based on imaging characteristics, such as preoperative cisterna magna volume, preoperative syrinx diameter, and preoperative cerebrospinal fluid (CSF) hydrodynamic parameters to guide clinical work.

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Section: Discussionmentioning

confidence: 98%

A long-term follow-up study of adults with Chiari malformation type I combined with syringomyelia

Hu,

Zhang,

Duan

et al. 2023

Front. Neurol.

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Morphometric analysis of posterior cranial fossa and foramen magnum and it’s clinical implications in craniovertebral junction malformations: a computed tomography based institutional study in a tertiary care hospital of northern part of India

Yadav,

Prasad,

Sahu

et al. 2024

Egypt J Neurosurg

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Background The posterior cranial fossa (PCF) and the foramen magnum (FM) are the critical anatomical components of the craniovertebral junction region, which comprise and transmit numerous vital neurovascular structures. So, a fundamental knowledge of the basic radiological anatomy of PCF and FM is of paramount importance in the evaluation of associated pathologies and approaching these areas surgically. The aim of this study is to describe different linear and angular craniometric parameters of PCF, FM and surrounding territory based on reconstructed computed tomography (CT) images. Material and methods This study was conducted in our tertiary care hospital in northern India from the period of January 2023 to June 2023 on 120 patients, and CT screening was done for the head and spine region following a history of head injury. Results In this study, 120 patients were included, of whom 50.83% (n = 61) were females and 49.17% (n = 59) were males. Age ranged from 18 to 70 years with mean age of 43.5 ± 14.08 years. The mean values for linear craniometric parameters of PCF were statistically nonsignificant for different age groups. Statistically significant differences were found for twinning line (TL) (p < 0.0001), McRae’s line (< 0.0001), clivus length (< p < 0.0001), internal occipital protuberence -opisthion line (p = 0.01), Klaus’ index (p < 0.0001), height of posterior fossa (h) (p < 0.0001), h/TL (p = 0.028), when these values were compared for the genders. The measurements of FM transverse diameter, anteroposterior diameter and area were 27.12 ± 1.42 mm (range 23.6–30.1 mm), 30.99 ± 2.23 mm (range 27.6–35.8 mm) and 691.32 ± 30.35 mm2 (range 632.7–777.7 mm2). The values of clivus canal angle (p = 0.038) and clivoodontoid angle (p = 0.012) were statistically significant when compared for different age groups. The values of Boogard’s angle (p = 0.021) and tentorial slope (p = 0.031) were statistically significant when these were compared for the genders. Conclusions This study described almost all the linear and angular craniometric parameters used in the morphometric analysis of PCF and FM. The findings of this study provide valuable data regarding linear and angular craniometric parameters of PCF and FM which can redefine reference values.

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The Genetics of Chiari 1 Malformation

Yan,

Chae,

Dahmane

et al. 2024

JCM

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Chiari malformation type 1 (CM1) is a structural defect that involves the herniation of the cerebellar tonsils through the foramen magnum, causing mild to severe neurological symptoms. Little is known about the molecular and developmental mechanisms leading to its pathogenesis, prompting current efforts to elucidate genetic drivers. Inherited genetic disorders are reported in 2–3% of CM1 patients; however, CM1, including familial forms, is predominantly non-syndromic. Recent work has focused on identifying CM1-asscoiated variants through the study of both familial cases and de novo mutations using exome sequencing. This article aims to review the current understanding of the genetics of CM1. We discuss three broad classes of CM1 based on anatomy and link them with genetic lesions, including posterior fossa-linked, macrocephaly-linked, and connective tissue disorder-linked CM1. Although the genetics of CM1 are only beginning to be understood, we anticipate that additional studies with diverse patient populations, tissue types, and profiling technologies will reveal new insights in the coming years.

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Potential association among posterior fossa bony volume and crowdedness, tonsillar hernia, syringomyelia, and CSF dynamics at the craniocervical junction in Chiari malformation type I

Cited by 3 publications

References 33 publications

A long-term follow-up study of adults with Chiari malformation type I combined with syringomyelia

A long-term follow-up study of adults with Chiari malformation type I combined with syringomyelia

Morphometric analysis of posterior cranial fossa and foramen magnum and it’s clinical implications in craniovertebral junction malformations: a computed tomography based institutional study in a tertiary care hospital of northern part of India

The Genetics of Chiari 1 Malformation

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