scholarly journals Breaking Barriers for Cerebrospinal Fluid Flow in Chiari Malformation Type I: “What and How Much Is Enough?” A Retrospective Analysis of 74 Cases

2018 ◽  
Vol 07 (03) ◽  
pp. 196-208
Author(s):  
Manish Jaiswal ◽  
Amit Patil ◽  
Radhey Mittal
Keyword(s):  
Cerebrospinal Fluid ◽  
Natural History ◽  
Retrospective Analysis ◽  
Chiari Malformation ◽  
Foramen Magnum ◽  
Common Finding ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Compression Syndrome ◽  
History Of

Abstract Introduction: Chiari malformation type I is a collection of hindbrain abnormalities, for which natural history of the disease process is not clear. The challenge is to identify which patients will benefit most from posterior fossa decompression. Objectives: To identify important surgical implications that most likely benefit patients with Chiari malformation type I by analyzing and reviewing various operative interventions in these patients with appropriate symptoms and then following their course. Subjects & Methods: Retrospective analysis of 74 operated Chiari malformation type I with syrinx adult patients was done. Results: No definite pattern of progression in natural history of disease was noted. Most of the patients who were symptomatically stable for months to years presented with recent rapid progression. The most common symptom was suboccipital pain. The most common finding was lower extremity weakness. On clinical presentation basis, patients were divided into three categories: foramen magnum compression syndrome, central cord syndrome, and cerebellar syndrome. Most patients in our study fall in first category. Foramen magnum decompression with atlas posterior arch removal and sometimes partial C2 laminectomy depending on extent of tonsillar descent as well as augmentation duraplasty was done in most patients. Improvement was seen in foramen magnum compression syndrome group more significantly. Conclusions: Individualized surgical techniques for breaking the barriers of cerebrospinal fluid (CSF) flow in Chiari malformation type I with syrinx to restore normal CSF dynamics across craniocervical junction provide the pragmatic solution. The trend is toward balance between optimum wide decompression as compared with long craniocaudal decompression and preserving normal integrity.

Natural history of Chiari malformation Type I following decision for conservative treatment

2011 ◽  
Vol 8 (2) ◽  
pp. 214-221 ◽  
Author(s):  
Jennifer Strahle ◽  
Karin M. Muraszko ◽  
Joseph Kapurch ◽  
J. Rajiv Bapuraj ◽  
Hugh J. L. Garton ◽  
...  
Keyword(s):  
Natural History ◽  
Mr Imaging ◽  
Foramen Magnum ◽  
Type I ◽  
Csf Flow ◽  
Chiari Malformation Type I ◽  
Nonsurgical Management ◽  
Cerebellar Tonsillar Herniation ◽  
History Of

Object The natural history of the Chiari malformation Type I (CM-I) is incompletely understood. The authors report on the outcome of a large group of patients with CM-I that were initially selected for nonsurgical management. Methods The authors identified 147 patients in whom CM-I was diagnosed on MR imaging, who were not offered surgery at the time of diagnosis, and in whom at least 1 year of clinical and MR imaging follow-up was available after the initial CM-I diagnosis. These patients were included in an outcome analysis. Results Patients were followed clinically and by MR imaging for a mean duration of 4.6 and 3.8 years, respectively. Of the 147 patients, 9 had new symptoms attributed to the CM-I during the follow-up interval. During this time, development of a spinal cord syrinx occurred in 8 patients; 5 of these patients had a prior diagnosis of a presyrinx state or a dilated central canal. Spontaneous resolution of a syrinx occurred in 3 patients. Multiple CSF flow studies were obtained in 74 patients. Of these patients, 23 had improvement in CSF flow, 39 had no change, and 12 showed worsening CSF flow at the foramen magnum. There was no significant change in the mean amount of cerebellar tonsillar herniation over the follow-up period. Fourteen patients underwent surgical treatment for CM-I. There were no differences in initial cerebellar tonsillar herniation or CSF flow at the foramen magnum in those who ultimately underwent surgery compared with those who did not. Conclusions In patients with CM-Is that are selected for nonsurgical management, the natural history is usually benign, although spontaneous improvement and worsening are occasionally seen.

Association Between Resistance to Cerebrospinal Fluid Flow Near the Foramen Magnum and Cough-Associated Headache in Adult Chiari Malformation Type I

2021 ◽  
Author(s):  
Alaaddin Ibrahimy ◽  
Chi-Wen Christina Huang ◽  
Abraham F. Bezuidenhout ◽  
Phillip A. Allen ◽  
Rafeeque A. Bhadelia ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Roc Curve ◽  
Spinal Canal ◽  
Chiari Malformation ◽  
Foramen Magnum ◽  
Patient Specific ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Cerebrospinal Fluid Flow ◽  
Longitudinal Impedance

Abstract Background and Purpose: Cough-associated headaches (CAH) are thought to be distinctive for Chiari malformation type I (CMI) patients and have been shown to be related to the motion of cerebrospinal fluid (CSF) near the foramen magnum. We used computational fluid dynamics (CFD) to compute patient-specific resistance to CSF motion in the spinal canal for CMI patients to determine its accuracy in predicting CAH. Methods: 51 symptomatic CMI patients with cerebellar tonsillar position (CTP) = 5 mm were included in this study. The patients were divided into two groups based on their symptoms (CAH and non-CAH) by review of the neurosurgical records. CFD was utilized to simulate CSF motion and the integrated longitudinal impedance (ILI) was calculated for all patients. A receiver operating characteristic (ROC) curve was evaluated for its accuracy in predicting CAH. Results: The ILI for CMI patients with CAH (776 dyn/cm5, 288-1444 dyn/cm5; median, inter-quartile range) was significantly larger compared to non-CAH (285 dyn/cm5, 187-450 dyn/cm5; p = .001). The ILI was more accurate in predicting CAH in CMI patients than the CTP when the comparison was made using the area under the ROC curve (0.77 and 0.70, for ILI and CTP, respectively). ILI = 750 dyn/cm5 had a sensitivity of 50% and a specificity of 95% in predicting CAH. Conclusions: ILI is a parameter, used to assess CSF blockage in the spinal canal and can predict patients with and without CAH with greater accuracy than CTP.

Prognostic significance of C1–C2 facet malalignment after surgical decompression in adult Chiari malformation type I: a pilot study based on the Chicago Chiari Outcome Scale

2020 ◽  
pp. 1-7
Author(s):  
Michael Lumintang Loe ◽  
Tito Vivas-Buitrago ◽  
Ricardo A. Domingo ◽  
Johan Heemskerk ◽  
Shashwat Tripathi ◽  
...  
Keyword(s):  
Pilot Study ◽  
Chiari Malformation ◽  
Prognostic Significance ◽  
Foramen Magnum ◽  
Bivariate Analysis ◽  
Type I ◽  
Foramen Magnum Decompression ◽  
Swallowing Function ◽  
Chiari Malformation Type I

OBJECTIVEThe authors assessed the prognostic significance of various clinical and radiographic characteristics, including C1–C2 facet malalignment, in terms of surgical outcomes after foramen magnum decompression of adult Chiari malformation type I.METHODSThe electronic medical records of 273 symptomatic patients with Chiari malformation type I who were treated with foramen magnum decompression, C1 laminectomy, and duraplasty at Mayo Clinic were retrospectively reviewed. Preoperative and postoperative Neurological Scoring System scores were compared using the Friedman test. Bivariate analysis was conducted to identify the preoperative variables that correlated with the patient Chicago Chiari Outcome Scale (CCOS) scores. Multiple linear regression analysis was subsequently performed using the variables with p < 0.05 on the bivariate analysis to check for independent associations with the outcome measures. Statistical software SPSS version 25.0 was used for the data analysis. Significance was defined as p < 0.05 for all analyses.RESULTSFifty-two adult patients with preoperative clinical and radiological data and a minimum follow-up of 12 months were included. Motor deficits, syrinx, and C1–C2 facet malalignment were found to have significant negative associations with the CCOS score at the 1- to 3-month follow-up (p < 0.05), while at the 9- to 12-month follow-up only swallowing function and C1–C2 facet malalignment were significantly associated with the CCOS score (p < 0.05). Multivariate analysis showed that syrinx presence and C1–C2 facet malalignment were independently associated with the CCOS score at the 1- to 3-month follow-up. Swallowing function and C1–C2 facet malalignment were found to be independently associated with the CCOS score at the 9- to 12-month follow-up.CONCLUSIONSThe observed results in this pilot study suggest a significant negative correlation between C1–C2 facet malalignment and clinical outcomes evaluated by the CCOS score at 1–3 months and 9–12 months postoperatively. Prospective studies are needed to further validate the prognostic value of C1–C2 facet malalignment and the potential role of atlantoaxial fixation as part of the treatment.

scholarly journals Standard and cardiac-gated phase-contrast magnetic resonance imaging in the clinical course of patients with Chiari malformation Type I

2011 ◽  
Vol 31 (3) ◽  
pp. E5 ◽  
Author(s):  
Uwe Max Mauer ◽  
Andreas Gottschalk ◽  
Carolin Mueller ◽  
Linda Weselek ◽  
Ulrich Kunz ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Chiari Malformation ◽  
Phase Contrast ◽  
Foramen Magnum ◽  
Central Canal ◽  
Type I ◽  
Csf Flow ◽  
Chiari Malformation Type I ◽  
Cerebrospinal Fluid Flow ◽  
Contrast Magnetic Resonance

Object The causal treatment of Chiari malformation Type I (CM-I) consists of removing the obstruction of CSF flow at the level of the foramen magnum. Cerebrospinal fluid flow can be visualized using dynamic phase-contrast MR imaging. Because there is only a paucity of studies evaluating CSF dynamics in the region of the spinal canal on the basis of preoperative and postoperative measurements, the authors investigated the clinical usefulness of cardiacgated phase-contrast MR imaging in patients with CM-I. Methods Ninety patients with CM-I underwent preoperative MR imaging of CSF pulsation. Syringomyelia was present in 59 patients and absent in 31 patients. Phase-contrast MR imaging of the entire CNS was used to investigate 22 patients with CM-I before surgery and after a mean postoperative period of 12 months (median 12 months, range 3–33 months). In addition to the dynamic studies, absolute flow velocities, the extension of the syrinx, and tonsillar descent were also measured. Results The changes in pulsation were highly significant in the region of the (enlarged) cistern (p = 0.0005). Maximum and minimum velocities (the pulsation amplitude) increased considerably in the region where the syrinx was largest in diameter. The changes of pulsation in these patients were significant in the subarachnoid space in all spinal segments but not in the syrinx itself and in the central canal. Conclusions The demonstration of CSF flow pulsation can contribute to assessments of surgical outcomes. The results presented here, however, raise doubts about current theories on the pathogenesis of syringomyelia.

Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis

2008 ◽  
Vol 2 (1) ◽  
pp. 42-49 ◽  
Author(s):  
Susan R. Durham ◽  
Kristina Fjeld-Olenec
Keyword(s):  
Cerebrospinal Fluid ◽  
Surgical Treatment ◽  
Clinical Improvement ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Meta Analysis ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Patient Age

Object Surgery for Chiari malformation Type I (CM-I) is one of the most common neurosurgical procedures performed in children, although there is clearly no consensus among practitioners about which surgical method is preferred. The objective of this meta-analysis was to compare the outcome of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression without duraplasty (PFD) for the treatment of CM-I in children. Methods The authors searched Medline–Ovid, The Cochrane Library, and the conference proceedings of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons (2000–2007) for studies meeting the following inclusion criteria: 1) surgical treatment of CM-I; 2) surgical techniques of PFD and PFDD being reported in a single cohort; and 3) patient age < 18 years. Results Five retrospective and 2 prospective cohort studies involving a total of 582 patients met the criteria for inclusion in the meta-analysis. Of the 582 patients, 316 were treated with PFDD and 266 were treated with PFD alone. Patient age ranged from 6 months to 18 years. Patients undergoing PFDD had a significantly lower reoperation rate (2.1 vs 12.6%, risk ratio [RR] 0.23, 95% confidence interval [CI] 0.08–0.69) and a higher rate of cerebrospinal fluid–related complications (18.5 vs 1.8%, RR 7.64, 95% CI 2.53–23.09) than those undergoing PFD. No significant differences in either clinical improvement (78.6 vs 64.6%, RR 1.23, 95% CI 0.95–1.59) or syringomyelia decrease (87.0 vs 56.3%, RR 1.43, 95% CI 0.91–2.25) were noted between PFDD and PFD. Conclusions Posterior fossa decompression with duraplasty is associated with a lower risk of reoperation than PFD but a greater risk for cerebrospinal fluid–related complications. There was no significant difference between the 2 operative techniques with respect to clinical improvement or decrease in syringomyelia.

scholarly journals A Case of Chiari Malformation Type I : Improvement of Syringomyelia and Scoliosis by Foramen Magnum Decompression Alone

1999 ◽  
Vol 8 (11) ◽  
pp. 727-731
Author(s):  
Tetsuya Kubota ◽  
Kenichi Nishiyama ◽  
Akira Tamura ◽  
Kouichi Kawasaki ◽  
Hiroshi Masuda ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Type I ◽  
Foramen Magnum Decompression ◽  
Chiari Malformation Type I

Trzyipółletni chłopiec z malformacją Chiariego typu I – opis przypadku

2017 ◽  
Vol 21 (3) ◽  
Author(s):  
Anna Worch ◽  
Małgorzata Wielopolska
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Common Type ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Chiari Type I Malformation ◽  
Large Head ◽  
Exacerbation Of Symptoms ◽  
Chiari Type I ◽  
Cerebellar Tonsils

Chiari malformation is the most common structural defect of the posterior fossa and cerebellum, consisting of displacements of hindbrain inclination through the foramen magnum, often associated with syringomyelia. In the most common type I of this disease, only the cerebellar tonsils are descended. Chiari type I malformation may remain asymptomatic in early childhood, however a number of unspecific symptoms, such as: neck pains and headaches, vertigos, balance and sight disorders, may strengthen with age. Because of unspecificity and various exacerbation of symptoms the disease is often misdiagnosed or undiagnosed. We describe a case of a 3.5-year old boy with whom his mother visited a pediatrician because of a chronic cough. During the examination mother of the patient informed that all infections start with vomiting and the patient is not able to stand up from horizontal position without turning first to the side and then to the abdomen. During the examination, a large head circumference was noticed (56 cm, > 97 percentile). MRI showed elongated cerebellar tonsils descended below the foramen magnum (left 14 mm, right 11 mm). Chiari malformation type I with associated syringomyelia was diagnosed. The patient was reffered to neurosurgery consultation.

Surgical outcomes in adult patients with syringomyelia associated with Chiari malformation Type I: the relationship between scoliosis and neurological findings

2007 ◽  
Vol 6 (3) ◽  
pp. 216-221 ◽  
Author(s):  
Atsushi Ono ◽  
Futoshi Suetsuna ◽  
Kazumasa Ueyama ◽  
Toru Yokoyama ◽  
Shuichi Aburakawa ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Clinical Status ◽  
Japanese Orthopaedic Association ◽  
Foramen Magnum ◽  
Neurological Dysfunction ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Group A ◽  
The Mean ◽  
Group B

Object The clinical characteristics of pediatric scoliosis associated with syringomyelia have been reported in previous studies, but scoliosis associated with syringomyelia in adults is rarely treated, and there is a paucity of detailed studies. In the present study of adult syringomyelia associated with Chiari malformation Type I, the authors investigated the relationships among the syrinx, scoliosis, and neurological data. Methods The population was composed of 27 patients (≥ 20 years of age) who underwent foramen magnum decompression for the treatment of syringomyelia. The patients were divided into two groups: those with scoliosis of 10° or more (Group A) and those without scoliosis (Group B). The authors assessed the length of the syrinx, duration of morbidity, and clinical status before and after surgery based on the Japanese Orthopaedic Association (JOA) Scale. There were 15 cases in Group A and 12 in Group B. The mean length of the syrinx was 12.8 vertebral bodies (VBs) in Group A and 7.2 VBs in Group B. The mean duration of morbidity was 14.2 years in Group A and 6.8 years in Group B. The mean preoperative JOA score was 10.1 in Group A and 14.4 in Group B, whereas the mean postoperative JOA scores were 11.9 and 15.8, respectively. There were significant differences between Groups A and B in length of the syrinx, duration of morbidity, and pre- and postoperative JOA scores. Conclusions In patients with syringomyelia and scoliosis the syringes spanned a greater number of VBs, the duration of morbidity was greater, neurological dysfunction was more severe, and surgical results were poorer. Scoliosis could be a predicting factor of the prognosis in patients with syringomyelia and Chiari malformation Type I.

scholarly journals Improvement of Hydrocephalus in Patients with Chiari Malformation After Posterior Fossa Decompression

2018 ◽  
Vol 27 (1) ◽  
pp. 71-73
Author(s):  
Lindolfo Carlos Heringer ◽  
Matheus Fernandes De Oliveira ◽  
Ulysses Oliveira De Sousa ◽  
Wanderley Cerqueira De Lima ◽  
Ricardo Vieira Botelho
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Clinical Symptoms ◽  
Foramen Magnum ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Cisterna Magna ◽  
Long Time ◽  
Small Posterior Fossa

Introduction. The association between hydrocephalus and Chiari malformation (CM) has not been described frequently. Ventricular dilation affects 7% to 10% of patients with CM, but the ideal choice of surgical treatment is controversial. Wereport a case of a patient with Chiari malformation and hydrocephalus with improvement in clinical symptoms and ventricular dilatation. Case Description. A 19-year-old male complaining of frontal headache when coughing, laughing and during valsalva maneuvers, associated with dizziness for 2 months. Magnetic resonance (MR) showed hydrocephalus and small posterior fossa with overcrowding of contents, characterizing Chiari malformation type I, with cerebellar tonsils protruding through magnum foramen. Patient underwent surgery with posterior fossa decompression in a semi-sitting position and removal of the arc C1.After 3 months of follow-up, headache disappeared becoming asymptomatic. Control MR showed improvement of hydrocephalus with restoration of the cisterna magna and CSF flow. Discussion. Hydrocephalus has been related to CM for a long time. In our case, we performed treatment with intradural and intra-arachnoidal approach with bilateral tonsillectomy without placing ventricular shunt. The cisterna magna was “recreated”. There was improvement of hydrocephalus with decreased Evans ratio index and symptoms disappearance. Although there is no other studies addressing such matter, in this case, the improvement suggests that the CSF compression at the foramen magnum was the cause of associated hydrocephalus with Chiari malformation. 

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