scholarly journals Is Chiari I Malformation Associated With Fibromyalgia?

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.

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.


2020 ◽  
Vol 26 (1) ◽  
pp. 45-52
Author(s):  
Gabe Haller ◽  
Brooke Sadler ◽  
Timothy Kuensting ◽  
Nivan Lakshman ◽  
Jacob K. Greenberg ◽  
...  

OBJECTIVEChiari I malformation (CM-I) has traditionally been defined by measuring the position of the cerebellar tonsils relative to the foramen magnum. The relationships of tonsillar position to clinical presentation, syringomyelia, scoliosis, and the use of posterior fossa decompression (PFD) surgery have been studied extensively and yielded inconsistent results. Obex position has been proposed as a useful adjunctive descriptor for CM-I and may be associated with clinical disease severity.METHODSA retrospective chart review was performed of 442 CM-I patients with MRI who presented for clinical evaluation between 2003 and 2018. Clinical and radiological variables were measured for all patients, including presence/location of headaches, Chiari Severity Index (CSI) grade, tonsil position, obex position, clival canal angle, pB-C2 distance, occipitalization of the atlas, basilar invagination, syringomyelia, syrinx diameter, scoliosis, and use of PFD. Radiological measurements were then used to predict clinical characteristics using regression and survival analyses, with performing PFD, the presence of a syrinx, and scoliosis as outcome variables.RESULTSAmong the radiological measurements, tonsil position, obex position, and syringomyelia were each independently associated with use of PFD. Together, obex position, tonsil position, and syringomyelia (area under the curve [AUC] 89%) or obex position and tonsil position (AUC 85.4%) were more strongly associated with use of PFD than tonsil position alone (AUC 76%) (Pdiff = 3.4 × 10−6 and 6 × 10−4, respectively) but were only slightly more associated than obex position alone (AUC 82%) (Pdiff = 0.01 and 0.18, respectively). Additionally, obex position was significantly associated with occipital headaches, CSI grade, syringomyelia, and scoliosis, independent of tonsil position. Tonsil position was associated with each of these traits when analyzed alone but did not remain significantly associated with use of PFD when included in multivariate analyses with obex position.CONCLUSIONSCompared with tonsil position alone, obex position is more strongly associated with symptomatic CM-I, as measured by presence of a syrinx, scoliosis, or use of PFD surgery. These results support the role of obex position as a useful radiological measurement to inform the evaluation and potentially the management of CM-I.


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. 319-328 ◽  
Author(s):  
Sunil V. Furtado ◽  
Sumit Thakar ◽  
Alangar S. Hegde

Abstract BACKGROUND: Chiari I malformation occurs because of an underdeveloped posterior fossa with reduced volume that cannot accommodate the normally developed hindbrain. OBJECTIVE: To study the clinical presentation and surgical outcome of pediatric Chiari I malformation and to correlate outcome with demographic and clinical factors and radiological changes in the syrinx, spinal cord, and preoperative intracranial, posterior fossa, and foramen magnum dimensions. METHODS: This retrospective study spanning 9 years included 20 symptomatic patients who underwent foramen magnum decompression, shrinkage of tonsils, and duraplasty. Improvement at follow-up was assessed with a modified Asgari scoring system. Mean differences in the dimensions of the syrinx and cord, foramen magnum morphometry, and intracranial and posterior fossa for 2 groups (with or without improvement) were analyzed with the independent-sample Student t test. Correlation of outcome in relation to change in radiological factors and influence of variables such as age, type and duration of symptoms, and presence of syrinx were evaluated with the Pearson χ2 test. RESULTS: Sixty percent of patients showed functional improvement at follow-up. Of various demographic and radiological factors assessed, there was significant difference in mean values of change in cord diameter for the entire cohort (P = .05) and for the subgroup with preoperative syringes (P = .03). There was no correlation between change in any of these factors and functional improvement (χ2 range, 0-4.673; P > .05). CONCLUSION: More than half the pediatric patients with Chiari I malformation improve after surgery. The age at presentation, duration and type of symptoms, cranial and foramen magnum morphometry, and syrinx-related changes have no bearing on outcome at short-term follow-up. The spinal cord diameter differs significantly in patients with and without functional improvement.


Neurosurgery ◽  
2005 ◽  
Vol 57 (2) ◽  
pp. E371-E371 ◽  
Author(s):  
Mustafa Efkan Colpan ◽  
Zeki Sekerci

ABSTRACT OBJECTIVE AND IMPORTANCE: We report on a patient with a Chiari I malformation presenting with right hemifacial spasm. Clinicians should consider the downward displacement of the hindbrain as a rare cause of hemifacial spasm in Chiari I malformation. CLINICAL PRESENTATION: An 18-year-old man was admitted with right hemifacial spasm. The results of the neurological examination were normal except for the facial spasm. Magnetic resonance imaging demonstrated a Chiari I malformation without syringomyelia. After surgery, the hemifacial spasm completely resolved. INTERVENTION: Posterior fossa decompression, C1 laminectomy, and duraplasty were performed. CONCLUSION: The hemifacial spasm could be attributed to compression and/or traction of the facial nerve because of downward displacement of the hindbrain in Chiari I malformation. Compression and/or traction might create irritation of the facial nerve that causes hemifacial spasm. Resolution of the hemifacial spasm after posterior fossa decompression could explain the facial nerve irritation in Chiari I malformation. Clinicians should consider Chiari malformation as a cause of hemifacial spasm and posterior fossa decompression as a potential treatment.


2017 ◽  
Vol 10 ◽  
pp. 150-154 ◽  
Author(s):  
Tetsuryu Mitsuyama ◽  
Yasuo Aihara ◽  
Takaomi Taira ◽  
Seiichiro Eguchi ◽  
Kentaro Chiba ◽  
...  

2021 ◽  
Author(s):  
Amir Kershenovich

Abstract BACKGROUND Different conditions of the posterior fossa such as Chiari malformations, tumors, and arachnoid cysts require surgery through a suboccipital approach, for which a typical midline vertical linear incision is used. Curvilinear incisions have been carried in all other scalp regions other than the sub region for better cosmetic outcomes; a vertical curvilinear incision in the occipital and suboccipital region has not been reported. OBJECTIVE To evaluate the cosmetic value and safety of the “3 on a stick” vertical suboccipital curvilinear incision. METHODS We compared curvilinear to linear incisions, considering the scar's width, color, how conspicuous, and how well the scar could be covered by hair naturally. RESULTS Between 2010 and 2016, 68 children with Chiari I malformation were surgically intervened. The curvilinear incision was performed in 56 (82.4%) while a linear incision in 12 (17.6%) children. There were only 2 (2.9%) wound related complications (superficial dehiscences) in the curvilinear group and 1 additional dehiscence in a linear incision case. There were no neural or vascular complications. Scars were very similar among the 2 groups; both were equally conspicuous but curvilinear ones seemed to get covered better by hair. CONCLUSION The “3 on a stick” curvilinear incision of the suboccipital region is safe and allows for better hair coverage of the scar. It can be used for multiple conditions requiring a midline suboccipital or even occipital approach, such as Chiari malformations, tumors, and cysts.


2015 ◽  
Vol 16 (2) ◽  
pp. 138-145 ◽  
Author(s):  
Travis R. Ladner ◽  
Michael C. Dewan ◽  
Matthew A. Day ◽  
Chevis N. Shannon ◽  
Luke Tomycz ◽  
...  

OBJECT Osseous anomalies of the craniocervical junction are hypothesized to precipitate the hindbrain herniation observed in Chiari I malformation (CM-I). Previous work by Tubbs et al. showed that posterior angulation of the odontoid process is more prevalent in children with CM-I than in healthy controls. The present study is an external validation of that report. The goals of our study were 3-fold: 1) to externally validate the results of Tubbs et al. in a different patient population; 2) to compare how morphometric parameters vary with age, sex, and symptomatology; and 3) to develop a correlative model for tonsillar ectopia in CM-I based on these measurements. METHODS The authors performed a retrospective review of 119 patients who underwent posterior fossa decompression with duraplasty at the Monroe Carell Jr. Children’s Hospital at Vanderbilt University; 78 of these patients had imaging available for review. Demographic and clinical variables were collected. A neuroradiologist retrospectively evaluated preoperative MRI examinations in these 78 patients and recorded the following measurements: McRae line length; obex displacement length; odontoid process parameters (height, angle of retroflexion, and angle of retroversion); perpendicular distance to the basion-C2 line (pB–C2 line); length of cerebellar tonsillar ectopia; caudal extent of the cerebellar tonsils; and presence, location, and size of syringomyelia. Odontoid retroflexion grade was classified as Grade 0, > 90°; Grade I,85°–89°; Grade II, 80°–84°; and Grade III, < 80°. Age groups were defined as 0–6 years, 7–12 years, and 13–17 years at the time of surgery. Univariate and multivariate linear regression analyses, Kruskal-Wallis 1-way ANOVA, and Fisher’s exact test were performed to assess the relationship between age, sex, and symptomatology with these craniometric variables. RESULTS The prevalence of posterior odontoid angulation was 81%, which is almost identical to that in the previous report (84%). With increasing age, the odontoid height (p < 0.001) and pB–C2 length (p < 0.001) increased, while the odontoid process became more posteriorly inclined (p = 0.010). The pB–C2 line was significantly longer in girls (p = 0.006). These measurements did not significantly correlate with symptomatology. Length of tonsillar ectopia in pediatric CM-I correlated with an enlarged foramen magnum (p = 0.023), increasing obex displacement (p = 0.020), and increasing odontoid retroflexion (p < 0.001). CONCLUSIONS Anomalous bony development of the craniocervical junction is a consistent feature of CM-I in children. The authors found that the population at their center was characterized by posterior angulation of the odontoid process in 81% of cases, similar to findings by Tubbs et al. (84%). The odontoid process appeared to lengthen and become more posteriorly inclined with age. Increased tonsillar ectopia was associated with more posterior odontoid angulation, a widened foramen magnum, and an inferiorly displaced obex.


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