Craniovertebral Junction Instability in the Setting of Chiari I Malformation

2015 ◽  
Vol 26 (4) ◽  
pp. 561-569 ◽  
Author(s):  
Hannah E. Goldstein ◽  
Richard C.E. Anderson
Keyword(s):  
Craniovertebral Junction ◽  
Chiari I Malformation ◽  
Chiari I ◽  
Craniovertebral Junction Instability

scholarly journals Endoscopic Endonasal Odontoidectomy Preserving Atlantoaxial Stability: a Pediatric Case

2020 ◽  
Author(s):  
Pierlorenzo Veiceschi ◽  
Fabio Pozzi ◽  
Francesco Restelli ◽  
Tommaso Alfiero ◽  
Paolo Castelnuovo ◽  
...  
Keyword(s):  
Craniovertebral Junction ◽  
Basilar Invagination ◽  
Chiari I Malformation ◽  
Lower Limbs ◽  
University Hospital ◽  
Third Ventriculostomy ◽  
Posterior Fixation ◽  
Endoscopic Endonasal ◽  
Postoperative Mri ◽  
Chiari I

Abstract Objectives We illustrate endoscopic endonasal odontoidectomy for the Chiari-I malformation respecting craniovertebral junction (CVJ) stability. Design Case report of a 12-year-old girl affected by the Chiari-I malformation. Magnetic resonance imaging (MRI) showed tonsillar herniation, basilar invagination, and dental retroversion, causing angulation and compression of the bulbomedullary junction. Patient underwent endoscopic third ventriculostomy (ETV) with reduction of ventricular size and resolution of gait disturbances, but she complained the Valsalva-induced headaches, hiccup, and dysesthesias in the lower limbs. Endoscopic endonasal odontoidectomy was chosen to decompress the cervicomedullary junction. Setting The research was conducted at University Hospital “Ospedale di Circolo,” Department of Neurosurgery at Varese in Italy. Participants Patients were from neurosurgical and ENT (ear, nose, and throat) skull base team. Main Outcome Measures A bilateral paraseptal approach was performed, using a four-hand technique. After resection of posterior edge of the nasal septum, the choana is entered and a rhinopharynx muscle–mucosal flap is dissected subperiosteal and transposed in oral cavity. The CVJ is exposed and, using neuronavigation and neuromonitoring, odontoidectomy is fulfilled until dura is reached, preserving the anterior arch of C1. Reconstruction is obtained suturing the flap previously harvested. Results Postoperative course was unremarkable and the patient experienced improvement of symptoms. Postoperative MRI documented the appearance of tight cerebrospinal fluid (CSF) film anterior to bulbomedullary junction and in retrotonsillar spaces, opening of the bulbomedullary angle, and slight tonsils reduction. No CVJ instability was occurred with any need of posterior fixation. Conclusion Endoscopic endonasal odontoidectomy is a feasible approach for CVJ malformation. In this case, bulbar decompression was achieved preserving CVJ stability and avoiding posterior fixation.The link to the video can be found at: https://youtu.be/VIobocHfCuc.

scholarly journals Successful non-operative management for atlanto-occipital dislocation resulting in spinal cord contusion in a patient with atlanto-occipital assimilation and severe Chiari I malformation

2020 ◽  
Vol 11 ◽  
pp. 338
Author(s):  
Jordan R. Davis ◽  
Matthew L. Kluckman ◽  
Grant W. Mallory ◽  
John L. Ritter
Keyword(s):  
Spinal Cord ◽  
Operative Management ◽  
Craniovertebral Junction ◽  
High Energy ◽  
Basilar Invagination ◽  
Chiari I Malformation ◽  
Severity Of Injury ◽  
Cord Injury ◽  
Chiari I ◽  
Non Operative Management

Background: Atlanto-occipital dislocation (AOD) is a rare, highly morbid, and highly lethal injury that results from high-energy trauma and almost universally requires operative management for satisfactory outcomes. It can be difficult to identify the severity of injury at the time of presentation, and when diagnosis is delayed outcomes worsen significantly. Anatomic anomalies of the craniovertebral junction may further complicate its detection. When such anomalies are present either singly or in combination, they are known to cause space constraints which may increase the likelihood of spinal cord injury. Given that such anomalies and AOD are rare, few examples of patients with both are reported in the literature. Furthermore, it is not clear in what way patient management may be impacted in this context. Case Description: We will present a unique case of an 18-year-old patient with traumatic AOD and an intact neurologic examination who was found to have atlanto-occipital assimilation (AOA), platybasia, basilar invagination, and severe Chiari I malformation, who was treated effectively with non-operative management. Conclusion: Our case demonstrates the successful application of a non-operative treatment strategy in a carefully selected patient with AOD in the context of AOA.

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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