Radiological and clinical predictors of scoliosis in patients with Chiari malformation type I and spinal cord syrinx from the Park-Reeves Syringomyelia Research Consortium

2019 ◽  
Vol 24 (5) ◽  
pp. 520-527 ◽  
Author(s):  
Jennifer M. Strahle ◽  
Rukayat Taiwo ◽  
Christine Averill ◽  
James Torner ◽  
Chevis N. Shannon ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Type I ◽  
Clinical Predictors ◽  
Chiari Malformation Type I ◽  
Inferior Aspect ◽  
Curve Magnitude ◽  
Multivariable Regression ◽  
Independent Association ◽  
Research Consortium

OBJECTIVEScoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors’ goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°).RESULTSBased on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB–C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude.CONCLUSIONSSyrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.

Extradural decompression versus duraplasty in Chiari malformation type I with syrinx: outcomes on scoliosis from the Park-Reeves Syringomyelia Research Consortium

2021 ◽  
pp. 1-9
Author(s):  
Brooke Sadler ◽  
Alex Skidmore ◽  
Jordan Gewirtz ◽  
Richard C. E. Anderson ◽  
Gabe Haller ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Curve Progression ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Curve Magnitude ◽  
Curve Stabilization ◽  
Research Consortium

OBJECTIVE Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

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.

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. 

A multicenter validation of the condylar–C2 sagittal vertical alignment in Chiari malformation type I: a study using the Park-Reeves Syringomyelia Research Consortium

2021 ◽  
pp. 1-7
Author(s):  
Vijay M. Ravindra ◽  
Rajiv R. Iyer ◽  
Alexander T. Yahanda ◽  
Robert J. Bollo ◽  
Huirong Zhu ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Intraclass Correlation ◽  
Misclassification Rate ◽  
Secondary Outcome ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Vertical Alignment ◽  
Disc Space ◽  
Chiari Malformation Type I ◽  
Research Consortium

OBJECTIVE The condylar–C2 sagittal vertical alignment (C-C2SVA) describes the relationship between the occipitoatlantal joint and C2 in patients with Chiari malformation type I (CM-I). It has been suggested that a C-C2SVA ≥ 5 mm is predictive of the need for occipitocervical fusion (OCF) or ventral brainstem decompression (VBD). The authors’ objective was to validate the predictive utility of the C-C2SVA by using a large, multicenter cohort of patients. METHODS This validation study used a cohort of patients derived from the Park-Reeves Syringomyelia Research Consortium; patients < 21 years old with CM-I and syringomyelia treated from June 2011 to May 2016 were identified. The primary outcome was the need for OCF and/or VBD. After patients who required OCF and/or VBD were identified, 10 age- and sex-matched controls served as comparisons for each OCF/VBD patient. The C-C2SVA (defined as the position of a plumb line from the midpoint of the O–C1 joint relative to the posterior aspect of the C2–3 disc space), pBC2 (a line perpendicular to a line from the basion to the posteroinferior aspect of the C2 body), and clival-axial angle (CXA) were measured on sagittal MRI. The secondary outcome was the need for ≥ 2 CM-related operations. RESULTS Of the 206 patients identified, 20 underwent OCF/VBD and 14 underwent repeat posterior fossa decompression. A C-C2SVA ≥ 5 mm was 100% sensitive and 86% specific for requiring OCF/VBD, with a 12.6% misclassification rate, whereas CXA < 125° was 55% sensitive and 99% specific, and pBC2 ≥ 9 was 20% sensitive and 88% specific. Kaplan-Meier analysis demonstrated that there was a significantly shorter time to second decompression in children with C-C2SVA ≥ 5 mm (p = 0.0039). The mean C-C2SVA was greater (6.13 ± 1.28 vs 3.13 ± 1.95 mm, p < 0.0001), CXA was lower (126° ± 15.4° vs 145° ± 10.7°, p < 0.05), and pBC2 was similar (7.65 ± 1.79 vs 7.02 ± 1.26 mm, p = 0.31) among those who underwent OCF/VBD versus decompression only. The intraclass correlation coefficient for the continuous measurement of C-C2SVA was 0.52; the kappa value was 0.47 for the binary categorization of C-C2SVA ≥ 5 mm. CONCLUSIONS These results validated the C-C2SVA using a large, multicenter, external cohort with 100% sensitivity, 86% specificity, and a 12.6% misclassification rate. A C-C2SVA ≥ 5 mm is highly predictive of the need for OCF/VBD in patients with CM-I. The authors recommend that this measurement be considered among the tools to identify the “high-risk” CM-I phenotype.

Incidence of basioccipital hypoplasia in Chiari malformation Type I: comparative morphometric study of the posterior cranial fossa

2009 ◽  
Vol 111 (5) ◽  
pp. 1046-1052 ◽  
Author(s):  
Rémy Noudel ◽  
Nicolas Jovenin ◽  
Cristophe Eap ◽  
Bernard Scherpereel ◽  
Laurent Pierot ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Posterior Cranial Fossa ◽  
Morphometric Study ◽  
Control Group ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Cranial Fossa ◽  
The Mean ◽  
Sphenooccipital Synchondrosis

Object The chronic tonsillar herniation defining Chiari malformation Type I (CMI) is thought to result from overcrowding of a normally developing hindbrain within a congenitally small posterior cranial fossa (PCF) due to occipital hypoplasia. The goals in the present study were to authenticate the cranioencephalic disproportion in a group of patients with CMI and to discuss new developmental aspects according to which part of the occipital bone was underdeveloped. Methods The authors retrospectively examined a group of 17 patients with CMI. Measurements of osteotentorial and neural structures of the PCF were made on MR images of the brain. The results were compared with findings in 30 healthy controls by using the Mann-Whitney U-test. Results Dimensions of the neural structures did not differ between the 2 groups of patients. The mean length of the basiocciput was significantly shorter in the CMI group (19.4 mm) compared with the control group (25.7 mm; p = 0.0003). The mean diameter of the foramen magnum was larger in the CMI group, but this difference was not statistically significant. The dimensions of the supraocciput and the mean angle of the cerebellar tentorium were identical in the 2 groups. Conclusions Data in this study support the idea that occipital hypoplasia is the main cause of overcrowding within the PCF. Basioccipital shortness is a cardinal feature of the resultant shallow PCF and could proceed from a congenital disorder of the cephalic mesoderm of the parachordal plate or occur later in the infancy because of premature stenosis of the sphenooccipital synchondrosis.

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