Chiari malformation: Has the dilemma ended?

Shah, Abhidha; Dhar, Arjun; Elsanafiry, Mohamed Said Mohamed; Goel, Atul

doi:10.4103/jcvjs.jcvjs_138_17

Cited by 17 publications

(19 citation statements)

References 33 publications

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“…Much has been written about the history of CIM and the development in understanding through the years, regarding the nature of this condition. 35,44,45,52 As recently as the 1970s, standard neurology texts did not mention CIM and grouped syringomyelia in the degenerative section. 41 Surgery was thought to have no value at the time and radiation therapy is instead noted as a treatment option.…”

Section: Background and Historical Contextmentioning

confidence: 99%

The Chiari I malformation

McClugage¹,

Oakes²

2019

Journal of Neurosurgery: Pediatrics

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As with many pathologies, the course of our understanding of the Chiari I malformation (CIM) has developed extensively over time. The early descriptions of the Chiari malformations by Hans Chiari in 1891 opened the door for future classification and research on this topic. However, even over a long timeframe, our understanding of the pathophysiology and, more importantly, treatment, remained in its infancy. As recently as the 1970s, CIM was not discussed in popular neurology textbooks. Syringomyelia is listed as a degenerative disorder with no satisfactory treatment. Radiation therapy was considered an option in treatment, and surgery was thought to play no role. During the last 40 years, equivalent to the duration of a neurosurgical career, our understanding of the pathophysiology and natural history of CIM, coupled with modern MRI, has improved the treatment paradigm for this patient population. More importantly, it has given us evidence confirming that CIM is a disorder responsive to surgical intervention, giving patients once thought to be destined for lifelong disability a comparatively normal life after treatment. The purpose of this article is to offer a review of CIM and its important associated entities. The authors will discuss the evolution in understanding of the Chiari malformation and, importantly, distinguish between symptomatic CIM and asymptomatic tonsillar ectopia, based on imaging and presenting symptomatology. They will discuss techniques for surgical intervention, expected outcomes, and complications after surgery. Proper patient selection for surgery based on appropriate symptomatology is tantamount to achieving good surgical outcomes in this population, separating those who can be helped by surgery from those who are unlikely to improve. While our knowledge of the Chiari malformations continues to improve through the efforts of clinical and basic science researchers, surgeons, and patients, our current understanding of these entities represents a monumental improvement in patient care over a relatively short time period.

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Section: Background and Historical Contextmentioning

confidence: 99%

The Chiari I malformation

McClugage¹,

Oakes²

2019

Journal of Neurosurgery: Pediatrics

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“…CM-1 is characterized by a narrow posterior fossa leading to the caudal displacement of the tonsils (> 5 mm) and a CSF circulation disruption at the level of the FM. [1][2][3][4] Syringomyelia often coexists and symptoms can be myriad. CM-1 may or may not be associated with a bony instability like AAD or BI.…”

Section: Discussionmentioning

confidence: 99%

“…CM-1 may or may not be associated with a bony instability like AAD or BI. [1][2][3][4][5][17][18][29][30][31] When CM occurs without a bony instability, the condition can be termed as a "pure CM" and traditionally, PFD has remained their treatment of choice. 9,[26][27][28][29][30][31][32][33][34][35][36][37] We found that nearly 31% of the patients had some bony anomaly like basilar impression, platybasia, assimilation of atlas, and C2-3 congenital fusion.…”

Section: Discussionmentioning

confidence: 99%

“…Type 1 Chiari malformation (CM-1) is characterized by a caudal displacement of the cerebellar tonsils due to an over-crowded posterior fossa. 1,2 This often leads to a true or a functional blockade of cerebrospinal fluid (CSF) circulation across the foramen magnum (FM) leading to a plethora of symptomatology. Nearly two-thirds of such patients develop syringomy-…”

Section: Introductionmentioning

confidence: 99%

“…The treatment principle of CM-1 is to re-establish the altered CSF flow across the FM and addressal of the bony instability is usually sufficient for this purpose in cases accompanied by an AAD or BI [ 5 - 7 ]. On the other hand, pure CM-1, that exists without any associated bony instability, is generally treated by posterior fossa decompression (PFD) [ 1 - 4 ]. There is some controversy in the literature regarding the extent of PFD.…”

Section: Introductionmentioning

confidence: 99%

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Type I Chiari Malformation Without Concomitant Bony Instability: Assessment of Different Surgical Procedures and Outcomes in 73 Patients

et al. 2021

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Posterior fossa decompression is the treatment of choice in type 1 Chiari malformation (CM-1) without bony instability. Although surgical fixation has been recommended by a few authors recently, comparative studies to evaluate these treatment strategies using objective outcome tools are lacking. Methods: Seventy-three patients with pure CM-1 (posterior fossa bony decompression [PFBD], n = 21; posterior fossa bony and dural decompression [PFBDD], n = 40; and posterior fixation [PF], n = 12) underwent a postoperative outcome assessment using Chicago Chiari Outcome Score (CCOS). Logistic regression analysis detected predictors of an unfavorable outcome. Results: Minimally symptomatic patients generally underwent a PFBD while most of the clinically severe patients underwent a PFBDD (p = 0.049). The mean CCOS score at discharge was highest in the PF (12.0 ± 1.41) and lowest in PFBDD group (10.98 ± 1.73, p = 0.087). Patients with minimal preoperative clinical disease severity (adjusted odds ratio [AOR], 4.58; 95% confidence interval [CI], 1.29-16.31) and PFBDD (AOR, 7.56; 95% CI, 1.70-33.68) represented risks for an unfavorable short-term postoperative outcome. Though long-term outcomes (CCOS) did not differ among the 3 groups (p = 0.615), PFBD group showed the best long-term improvements (mean follow-up CCOS, 13.71 ± 0.95), PFBDD group improved to a comparable degree despite a poorer short-term outcome while PF had the lowest scores. Late deteriorations (n = 3, 4.1%) occurred in the PFBDD group. Conclusion:Minimally symptomatic patients and PFBDD predict a poor short-term postoperative outcome. PFBD appears to be a durable procedure while PFBDD group is marred by complications and late deteriorations. PF does not provide any better results than posterior fossa decompression alone in the long run.

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