scholarly journals Incidence of Cerebellar Tonsillar Ectopia in Idiopathic Intracranial Hypertension: A Mimic of the Chiari I Malformation

2012 ◽  
Vol 33 (10) ◽  
pp. 1901-1906 ◽  
Author(s):  
A.H. Aiken ◽  
J.A. Hoots ◽  
A.M. Saindane ◽  
P.A. Hudgins
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Chiari I Malformation ◽  
Tonsillar Ectopia ◽  
Chiari I

Idiopathic intracranial hypertension in a patient with Chiari I malformation

2015 ◽  
Vol 90 (4) ◽  
pp. 190-194
Author(s):  
E. Santos-Bueso ◽  
J. Porta-Etessam ◽  
D. Díaz-Valle ◽  
J.M. Benítez-del-Castillo ◽  
J.A. Gegúndez-Fernández ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Idiopathic Intracranial Hypertension ◽  
Chiari I Malformation ◽  
Chiari I

scholarly journals Posterior odontoid process angulation in pediatric Chiari I malformation: an MRI morphometric external validation study

2015 ◽  
Vol 16 (2) ◽  
pp. 138-145 ◽  
Author(s):  
Travis R. Ladner ◽  
Michael C. Dewan ◽  
Matthew A. Day ◽  
Chevis N. Shannon ◽  
Luke Tomycz ◽  
...  
Keyword(s):  
External Validation ◽  
Age Groups ◽  
Odontoid Process ◽  
Line Length ◽  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Tonsillar Ectopia ◽  
Chiari I

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.

Chiari I malformation without hydrocephalus: acute intracranial hypertension managed with endoscopic third ventriculostomy (ETV)

2008 ◽  
Vol 24 (12) ◽  
pp. 1493-1497 ◽  
Author(s):  
Jothy Kandasamy ◽  
Rachel Kneen ◽  
Melissa Gladstone ◽  
William Newman ◽  
Tawil Mohamed ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Endoscopic Third Ventriculostomy ◽  
Chiari I Malformation ◽  
Third Ventriculostomy ◽  
Chiari I

scholarly journals Chiari I - Idiopathic Intracranial Hypertension Association After Failed Posterior Fossa Surgery. Case Report

2019 ◽  
Vol 30 (3) ◽  
pp. 252-260
Author(s):  
Felix Pastor Escartín ◽  
Vicent Quilis Quesada ◽  
Pau Capilla Guasch ◽  
Diego Tabarés Palacín ◽  
Esteban Vega Torres ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Lumbar Puncture ◽  
Posterior Fossa ◽  
Idiopathic Intracranial Hypertension ◽  
Decompression Surgery ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Posterior Fossa Surgery ◽  
Opening Pressure ◽  
Chiari I

Objectives: Several papers have been published relating the Idiopathic Intracranial Hypertension Syndrome (HTII) to the Arnold Chiari type I malformation (AC1M). Both entities have clinical and demographic similarities, a poorly defined etiology and, sometimes common therapeutic posibilities. A correlation between both entities has been suggested, especially in a subgroup of patients in whom posterior fossa decompression surgery fails. With regard to a case, we reviewed the literature and proposed our hypothesis about the origin of Chiari-HTII syndrome and its therapeutic possibilities. Case presentation: A 41year-old patient with mild obesity, menstrual abnormalities and empty Sella Turcicae, was operated on with an AC1M associating basilar impression and syringomyelia causing all together a centromedullary syndrome. After posterior fossa decompression surgery and successful arthrodesis, she improved in the immediate postoperative period. Nevertheless, she soon developed symptoms of intracranial hypertension (ICH), and showed increased opening pressure in lumbar puncture compatible with HTII syndrome. A ventriculoperitoneal shunt (VPS) was implanted with clinical improvement and 12 months later the syringomyelia was absent on in the magnetic resonance (MRI). Conclusion: The Chiari I-HTII syndrome is described as the coexistence of ICH symptoms after failed posterior fossa surgery, in patients with no flow MRI anomalies, and increased opening pressure at the lumbar puncture. In our experience, both entities seem to overlap in a common syndrome and must be taken into account, especially in patients with atypical onset of symptoms or patients in whom conservative treatment fails.

Sagittal MRI often overestimates the degree of cerebellar tonsillar ectopia: a potential for misdiagnosis of the Chiari I malformation

2016 ◽  
Vol 32 (7) ◽  
pp. 1245-1248 ◽  
Author(s):  
R. Shane Tubbs ◽  
Huang Yan ◽  
Amin Demerdash ◽  
Joshua J. Chern ◽  
Fabian N. Fries ◽  
...  
Keyword(s):  
Chiari I Malformation ◽  
Tonsillar Ectopia ◽  
Chiari I

Otto Mennicke (1876–) and the first description of skull base anomalies causing cerebellar tonsillar ectopia: one of the first mentions of the Chiari I malformation

2017 ◽  
Vol 33 (5) ◽  
pp. 825-827 ◽  
Author(s):  
Fabian N. Fries ◽  
Philipp Hendrix ◽  
Titus J. Brinker ◽  
Marios Loukas ◽  
R. Shane Tubbs
Keyword(s):  
Skull Base ◽  
Chiari I Malformation ◽  
Tonsillar Ectopia ◽  
Chiari I

The first description of Chiari I malformation with intuitive correlation between tonsillar ectopia and syringomyelia

2011 ◽  
Vol 7 (3) ◽  
pp. 257-260 ◽  
Author(s):  
Martin M. Mortazavi ◽  
R. Shane Tubbs ◽  
Maja Andrea Brockerhoff ◽  
Marios Loukas ◽  
W. Jerry Oakes
Keyword(s):  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Current Understanding ◽  
Tonsillar Ectopia ◽  
Cause And Effect ◽  
Hindbrain Herniation ◽  
Chiari I

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.

Tonsillar ectopia and Chiari malformations: monozygotic triplets

1995 ◽  
Vol 82 (3) ◽  
pp. 497-500 ◽  
Author(s):  
Richard K. Cavender ◽  
John H. Schmidt
Keyword(s):  
Magnetic Resonance ◽  
Chiari I Malformation ◽  
Unique Case ◽  
Content Type ◽  
Tonsillar Ectopia ◽  
Chiari I ◽  
Disease Entities ◽  
Criteria For Diagnosis ◽  
Chiari Malformations ◽  
Fine Print

✓ A unique case of monozygotic triplets, each of whom exhibits variable degrees of tonsillar ectopia, is reported. Patient X presented with a Chiari I malformation and associated syringomyelia; examination of patients Y and Z showed 4 mm and 2.5 mm of tonsillar ectopia, respectively. No such case has been reported in the literature. The discussion defines the current magnetic resonance criteria for diagnosis of hindbrain malformations and addresses the question of whether these disorders represent a spectrum or separate disease entities, with specific emphasis on genetic predisposition. Due to the 100% concordance in this case the presence of a common hereditary factor in the etiology of these malformations is highly suggested.

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