scholarly journals Surgical treatment of Chiari I malformation: indications and approaches

2001 ◽  
Vol 11 (1) ◽  
pp. 1-5 ◽  
Author(s):  
Tord D. Alden ◽  
Jeffrey G. Ojemann ◽  
T. S. Park

Chiari I malformation is a well-described entity characterized by hindbrain herniation through the foramen magnum. Although the exact origin of congenital Chiari I malformation is unknown, it appears to be caused by a mismatch between the volume of the posterior fossa neural elements and the posterior fossa cranial content. Several theories have been proposed to describe the resultant pathophysiology of this mismatch. It is clear, however, that abnormal cerebrospinal fluid flow and velocity play a role in the symptoms and signs associated with this disorder. The authors will review the pathophysiology, clinical presentation, and treatment options for patients with Chiari I malformation.

Neurosurgery ◽  
2011 ◽  
Vol 68 (2) ◽  
pp. 443-449 ◽  
Author(s):  
Nathaniel F. Watson ◽  
Dedra Buchwald ◽  
Jack Goldberg ◽  
Kenneth R. Maravilla ◽  
Carolyn Noonan ◽  
...  

Abstract BACKGROUND: The symptoms of Chiari I Malformation (CIM) and fibromyalgia (FM) overlap. Some FM patients have been surgically treated for presumed CIM-type pathology. OBJECTIVE: To determine whether CIM is more common among FM patients than pain- and fatigue-free controls. METHODS: One hundred seventy-six participants with FM and 67 pain- and fatigue-free control subjects underwent magnetic resonance imaging of the brain and upper cervical spine. Posterior fossa cerebrospinal fluid flow was assessed with cardiac gated cine phase-contrast imaging at the craniocervical region. CIM was defined as inferior extension of cerebellar tonsils ≥ 5 mm below the basion-opisthion line of the foramen magnum or tonsillar position 3 to 5 mm below the basion-opisthion line plus abnormalities of CSF flow, posterior fossa volume, or hindbrain or cervical spinal cord movement. Visual analog scales, questionnaires, and interviews were used to collect data on sleep quality, fatigue, pain, and headache. We used regression techniques to examine the association of outcome measures with disease status and the Fisher exact test to compare the CIM prevalence in the 2 groups. RESULTS: The FM group was older (mean age, 50 vs 40 years) and more likely to be white (89% vs 73%) and female (93% vs 54%; P < .01). Mean tonsillar position and the prevalence of CIM (2.8% vs 4.5%; P = .69) were similar in the FM and control groups. FM patients experienced more headaches, pain, fatigue, and sleep disturbances than control subjects (P < .01). CONCLUSION: Most patients with FM do not have CIM pathology. Future studies should focus on dynamic neuroimaging of craniocervical neuroanatomy in patients with FM.


2011 ◽  
Vol 7 (3) ◽  
pp. 257-260 ◽  
Author(s):  
Martin M. Mortazavi ◽  
R. Shane Tubbs ◽  
Maja Andrea Brockerhoff ◽  
Marios Loukas ◽  
W. Jerry Oakes

Few are familiar with the neurological contributions of the German pathologist Theodor Langhans. Even fewer are aware of his significant and early contributions to the study of what is now known as the Chiari I malformation. In at least 4 cases, Langhans described the association between tonsillar ectopia and syringomyelia. Moreover, this early pioneer speculated that there was a cause and effect with hindbrain herniation resulting in improper flow at the craniocervical junction and consequent development of syringomyelia. These cases were reported prior to Hans Chiari's descriptions, and Langhans' theory of impeded foramen magnum flow as a cause of syringomyelia was novel and preceded the current understanding of this mechanism by almost a century. The authors discuss the life of Langhans and translate excerpts from his 1881 work regarding tonsillar ectopia and syringomyelia.


Neurosurgery ◽  
2019 ◽  
Author(s):  
Andrea Bartoli ◽  
Jehuda Soleman ◽  
Assaf Berger ◽  
Jeffrey H Wisoff ◽  
Eveline Teresa Hidalgo ◽  
...  

Neurosurgery ◽  
2010 ◽  
Vol 67 (6) ◽  
pp. E1826-E1830 ◽  
Author(s):  
Nitin Mukerji ◽  
Peter Newman ◽  
Fred P Nath

Abstract BACKGROUND AND IMPORTANCE: Hemifacial spasm has rarely been described as one of the presenting features of Chiari I malformation. We present a case in which we found an association between the two in the absence of a basilar impression. CLINICAL PRESENTATION: A case of a 39-year-old man who presented with a disabling hemifacial spasm and was found to have Chiari I malformation as the possible cause is described. A foramen magnum decompression successfully relieved the hemifacial spasm. The patient remains symptom free at 20 months after the operation. CONCLUSION: Neurosurgeons may want to consider foramen magnum decompression as first-line surgical treatment for hemifacial spasm when it coexists with Chiari I malformation. Chiari I malformation should be considered one of the rare causes of hemifacial spasm.


2015 ◽  
Vol 122 (5) ◽  
pp. 1068-1075 ◽  
Author(s):  
Aaron E. Bond ◽  
John A. Jane ◽  
Kenneth C. Liu ◽  
Edward H. Oldfield

OBJECT The authors completed a prospective, institutional review board–approved study using intraoperative MRI (iMRI) in patients undergoing posterior fossa decompression (PFD) for Chiari I malformation. The purpose of the study was to examine the utility of iMRI in determining when an adequate decompression had been performed. METHODS Patients with symptomatic Chiari I malformations with imaging findings of obstruction of the CSF space at the foramen magnum, with or without syringomyelia, were considered candidates for surgery. All patients underwent complete T1, T2, and cine MRI studies in the supine position preoperatively as a baseline. After the patient was placed prone with the neck flexed in position for surgery, iMRI was performed. The patient then underwent a bone decompression of the foramen magnum and arch of C-1, and the MRI was repeated. If obstruction was still present, then in a stepwise fashion the patient underwent dural splitting, duraplasty, and coagulation of the tonsils, with an iMRI study performed after each step guiding the decision to proceed further. RESULTS Eighteen patients underwent PFD for Chiari I malformations between November 2011 and February 2013; 15 prone preincision iMRIs were performed. Fourteen of these patients (93%) demonstrated significant improvement of CSF flow through the foramen magnum dorsal to the tonsils with positioning only. This improvement was so notable that changes in CSF flow as a result of the bone decompression were difficult to discern. CONCLUSIONS The authors observed significant CSF flow changes when simply positioning the patient for surgery. These results put into question intraoperative flow assessments that suggest adequate decompression by PFD, whether by iMRI or intraoperative ultrasound. The use of intraoperative imaging during PFD for Chiari I malformation, whether by ultrasound or iMRI, is limited by CSF flow dynamics across the foramen magnum that change significantly when the patient is positioned for surgery.


2011 ◽  
Vol 7 (4) ◽  
pp. 369-374 ◽  
Author(s):  
Brian J. Dlouhy ◽  
Arnold H. Menezes

Osteopetrosis is a heterogeneous group of rare, inherited disorders of the skeleton that results in neurological manifestations due to restriction of growth of cranial foramina and calvarial thickening. A 25-year-old woman with a history of autosomal dominant osteopetrosis presented to the authors' institution with headache worsened by exertion and radiating from the occipital region forward with episodes of choking/coughing when eating and a loss of gag reflex on physical examination. On MR imaging, she was found to have severe posterior fossa calvarial thickening resulting in a small posterior fossa and tonsillar ectopia of 9 mm and compression and deformation of the brainstem. She underwent posterior fossa craniectomy, foramen magnum decompression, and partial C-1 laminectomy with external durotomy. The patient did well postoperatively with resolution of symptoms. This case describes a new neurological manifestation of autosomal dominant osteopetrosis. To the authors' knowledge, this report represents the first described case of extreme posterior fossa calvarial thickening from autosomal dominant osteopetrosis with associated Chiari I malformation (CM-I) requiring posterior fossa decompression and extradural decompression. Given previously published MR imaging data that demonstrate the association of osteopetrosis and CM may be more common than in this case alone, the authors discuss the need for further investigation of the incidence of CM-I in patients with autosomal dominant osteopetrosis. Additionally, they review osteopetrosis and other diagnoses of calvarial hyperostosis presenting as CM-I.


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