Identification of optimal surgical intervention for Chiari I malformation

2019 ◽  
Author(s):  
Luca Mesin ◽  
Forough Mokabberi ◽  
Christian Francesco Carlino
Keyword(s):  
Surgical Intervention ◽  
Chiari I Malformation ◽  
Chiari I

Spontaneous resolution of a Chiari malformation with syringomyelia

2021 ◽  
Vol 14 (6) ◽  
pp. e241789
Author(s):  
Hadleigh Cuthbert ◽  
Joshua Pepper ◽  
Rupert Price
Keyword(s):  
Natural History ◽  
Literature Review ◽  
Chiari Malformation ◽  
Surgical Intervention ◽  
Chiari I Malformation ◽  
Spontaneous Resolution ◽  
Conservative Approach ◽  
Resonance Imaging ◽  
History Of ◽  
Chiari I

The Chiari I malformation (CM-I) is characterised by overcrowding of the posterior fossa and descent of the cerebellar tonsils and is associated with syringomyelia. With the increasing availability of magnetic resonance imaging, CM-I is placing a growing burden on neurosurgical services. However, its natural history remains poorly understood, and the timing and nature of surgical intervention is controversial. We present a case of a significant, symptomatic CM-I with associated syrinx which underwent complete spontaneous resolution over a 4-year period. Spontaneous regression of Chiari malformation and syringomyelia is exceedingly rare; a literature review reveals 15 other cases and only one case which underwent complete resolution. The present case and literature review suggest a more benign natural history of CM-I and support a more conservative approach to its management. Further studies are required to determine whether any factors can predict resolution for certain patient cohorts.

scholarly journals The Chiari I malformation

2019 ◽  
Vol 24 (3) ◽  
pp. 217-226 ◽  
Author(s):  
Samuel G. McClugage ◽  
W. Jerry Oakes
Keyword(s):  
Surgical Intervention ◽  
Chiari I Malformation ◽  
Proper Patient Selection ◽  
Time Period ◽  
Treatment Paradigm ◽  
History Of ◽  
Chiari I ◽  
Degenerative Disorder ◽  
Short Time ◽  
Chiari Malformations

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.

scholarly journals Spontaneous syrinx resolution in patient with Chiari I malformation: illustrative case

2021 ◽  
Vol 1 (26) ◽  
Author(s):  
Elizabeth Gallo ◽  
Gazanfar Rahmathulla ◽  
Dinesh Rao ◽  
Kourosh Tavanaiepour ◽  
Daryoush Tavanaiepour
Keyword(s):  
Chiari Malformation ◽  
Surgical Intervention ◽  
Chiari I Malformation ◽  
Spontaneous Resolution ◽  
Illustrative Case ◽  
Type I ◽  
Conservative Approach ◽  
Chiari Malformation Type I ◽  
Hindbrain Herniation ◽  
Chiari I

BACKGROUIND Chiari malformations include a spectrum of congenital hindbrain herniation syndromes. In patients with the most common subtype, Chiari malformation Type I, 50% to 75% develop a syrinx. The pathogenesis of syringomyelia is not well understood, with multiple theories outlined in the literature. Although the presence of a syrinx in a patient with Chiari malformation is generally accepted as an indication for surgical intervention, there are documented cases of spontaneous resolution that support a more conservative approach to management. OBSERVATIONS The authors reported a case of spontaneous resolution of a cervical syrinx in an adult with an unchanged Chiari malformation. LESSONS Given the possibility of spontaneous resolution over time, the authors believe a more conservative approach of observation with periodic surveillance, magnetic resonance imaging, and neurological examination should be considered in the management of a patient with a Chiari malformation and associated syringomyelia.

Chiari I malformation in patients with RASopathies

2021 ◽  
Author(s):  
Yong Han ◽  
Min Chen ◽  
Hangzhou Wang
Keyword(s):  
Chiari I Malformation ◽  
Chiari I

Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases

1998 ◽  
Vol 88 (2) ◽  
pp. 237-242 ◽  
Author(s):  
John L. D. Atkinson ◽  
Brian G. Weinshenker ◽  
Gary M. Miller ◽  
David G. Piepgras ◽  
Bahram Mokri
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Chiari I Malformation ◽  
Csf Leak ◽  
Cerebrospinal Fluid Leakage ◽  
Content Type ◽  
Intracranial Hypotension Syndrome ◽  
Csf Leakage ◽  
Chiari I ◽  
Fine Print

Object. Spontaneous spinal cerebrospinal fluid (CSF) leakage with development of the intracranial hypotension syndrome and acquired Chiari I malformation due to lumbar spinal CSF diversion procedures have both been well described. However, concomitant presentation of both syndromes has rarely been reported. The object of this paper is to present data in seven cases in which both syndromes were present. Three illustrative cases are reported in detail. Methods. The authors describe seven symptomatic cases of spontaneous spinal CSF leakage with chronic intracranial hypotension syndrome in which magnetic resonance (MR) images depicted dural enhancement, brain sagging, loss of CSF cisterns, and acquired Chiari I malformation. Conclusions. This subtype of intracranial hypotension syndrome probably results from chronic spinal drainage of CSF or high-flow CSF shunting and subsequent loss of brain buoyancy that results in brain settling and herniation of hindbrain structures through the foramen magnum. Of 35 cases of spontaneous spinal CSF leakage identified in the authors' practice over the last decade, MR imaging evidence of acquired Chiari I malformation has been shown in seven. Not to be confused with idiopathic Chiari I malformation, ideal therapy requires recognition of the syndrome and treatment directed to the site of the spinal CSF leak.

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