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.

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.

Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium

2020 ◽  
Vol 26 (1) ◽  
pp. 53-59 ◽  
Author(s):  
Jennifer M. Strahle ◽  
Rukayat Taiwo ◽  
Christine Averill ◽  
James Torner ◽  
Jordan I. Gewirtz ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Craniocervical Junction ◽  
Curve Progression ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Younger Age ◽  
Curve Magnitude ◽  
The Mean

OBJECTIVEIn patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.RESULTSOf 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.CONCLUSIONSIn this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.

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.

scholarly journals Cerebrospinal fluid area and syringogenesis in Chiari malformation type I

2020 ◽  
pp. 1-6 ◽  
Author(s):  
Davis G. Taylor ◽  
Ajay Chatrath ◽  
Panagiotis Mastorakos ◽  
Gabriella Paisan ◽  
Ching-Jen Chen ◽  
...  
Keyword(s):  
Subarachnoid Space ◽  
Chiari Malformation ◽  
Foramen Magnum ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Intensity Measurements ◽  
Before And After ◽  
Subarachnoid Spaces ◽  
The University

OBJECTIVESyringogenesis in Chiari malformation type I (CM-I) is thought to occur secondary to impaction of the cerebellar tonsils within the foramen magnum (FM). However, the correlation between the CSF area and syringogenesis has yet to be elucidated. The authors sought to determine whether the diminution in subarachnoid space is associated with syringogenesis. Further, the authors sought to determine if syrinx resolution was associated with the degree of expansion of subarachnoid spaces after surgery.METHODSThe authors performed a retrospective review of all patients undergoing posterior fossa decompression for CM-I from 2004 to 2016 at the University of Virginia Health System. The subarachnoid spaces at the FM and at the level of the most severe stenosis were measured before and after surgery by manual delineation of the canal and neural tissue area on MRI and verified through automated CSF intensity measurements. Imaging and clinical outcomes were then compared.RESULTSOf 68 patients, 26 had a syrinx at presentation. Syrinx patients had significantly less subarachnoid space at the FM (13% vs 19%, p = 0.0070) compared to those without syrinx. Following matching based on degree of tonsillar herniation and age, the subarachnoid space was significantly smaller in patients with a syrinx (12% vs 19%, p = 0.0015). Syrinx resolution was associated with an increase in patients’ subarachnoid space after surgery compared with those patients without resolution (23% vs 10%, p = 0.0323).CONCLUSIONSSyrinx development in CM-I patients is correlated with the degree to which the subarachnoid CSF spaces are diminished at the cranial outlet. Successful syrinx reduction is associated with the degree to which the subarachnoid spaces are increased following surgery.

scholarly journals Chiari Malformation and Attention Deficit Hyperactivity Disorder

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Anaïs DuBow ◽  
Aurélie Mourot ◽  
Smadar Valérie Tourjman
Keyword(s):  
Attention Deficit Hyperactivity Disorder ◽  
Ct Scan ◽  
Attention Deficit ◽  
Chiari Malformation ◽  
Foramen Magnum ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Hyperactivity Disorder ◽  
Chiari Malformations

Attention deficit hyperactivity disorder (ADHD) is a neurodevelopmental condition characterized by inattention, hyperactivity, and impulsivity. Chiari malformations (CM), first described approximately a hundred years ago, refer to a spectrum of hindbrain malformations characterized by cerebellar herniation through the foramen magnum. We present the case of a 28-year-old woman with ADHD and concurrent Chiari malformation type I (CM-I) that was diagnosed by CT scan. There is growing evidence supporting the role of the cerebellum and its associated structures in the pathophysiology of ADHD. Thus, a cerebellar malformation such as CM may impact neurological circuitry in a manner favoring the development of a neuropsychiatric disorder such as ADHD. Our case highlights the need for further studies pertaining to the role of the cerebellum in the pathophysiology of ADHD and the importance of considering the presence of CM when evaluating a patient with ADHD.

Impact of body mass index on cerebellar tonsil position in healthy subjects and patients with Chiari malformation

2015 ◽  
Vol 123 (1) ◽  
pp. 226-231 ◽  
Author(s):  
Brandon W. Smith ◽  
Jennifer Strahle ◽  
Erick Kazarian ◽  
Karin M. Muraszko ◽  
Hugh J. L. Garton ◽  
...  
Keyword(s):  
Body Mass Index ◽  
Posterior Fossa ◽  
Body Mass ◽  
Chiari Malformation ◽  
Age Groups ◽  
Foramen Magnum ◽  
Cerebellar Tonsil ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I

OBJECT It is unclear if there is a relationship between Chiari malformation Type I (CM-I) and body mass index (BMI). The aim of this study was to identify the relationship between BMI and cerebellar tonsil position in a random sample of people. METHODS Cerebellar tonsil position in 2400 subjects from a cohort of patients undergoing MRI was measured. Three hundred patients were randomly selected from each of 8 age groups (from 0 to 80 years). A subject was then excluded if he or she had a posterior fossa mass or previous posterior fossa decompression or if height and weight information within 1 year of MRI was not recorded in the electronic medical record. RESULTS There were 1310 subjects (54.6%) with BMI records from within 1 year of the measured scan. Of these subjects, 534 (40.8%) were male and 776 (59.2%) were female. The average BMI of the group was 26.4 kg/m2, and the average tonsil position was 0.87 mm above the level of the foramen magnum. There were 46 subjects (3.5%) with a tonsil position ≥ 5 mm below the level of the foramen magnum. In the group as a whole, there was no correlation (R2 = 0.004) between BMI and cerebellar tonsil position. CONCLUSIONS In this examination of 1310 subjects undergoing MRI for any reason, there was no relationship between BMI and the level of the cerebellar tonsils or the diagnosis of CM-I on imaging.

Shunt malfunction causing acute neurological deterioration in 2 patients with previously asymptomatic Chiari malformation Type I

2009 ◽  
Vol 4 (2) ◽  
pp. 170-175 ◽  
Author(s):  
Robert Elliott ◽  
Stephen Kalhorn ◽  
Donato Pacione ◽  
Howard Weiner ◽  
Jeffrey Wisoff ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Neurological Deterioration ◽  
Shunt Malfunction ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Partial Recovery ◽  
Chiari Malformation Type I ◽  
Vp Shunt ◽  
Acute Neurological Deterioration

Patients with symptomatic Chiari malformation Type I (CM-I) typically exhibit a chronic, slowly progressive disease course with evolution of symptoms. However, some authors have reported acute neurological deterioration in the setting of CM-I and acquired Chiari malformations. Although brainstem dysfunction has been documented in patients with CM-II and hydrocephalus or shunt malfunction, to the authors' knowledge only 1 report describing ventriculoperitoneal (VP) shunt malfunction causing neurological deterioration in a patient with CM-I exists. The authors report on their experience with the treatment of previously asymptomatic CM-I in 2 children who experienced quite different manifestations of acute neurological deterioration secondary to VP shunt malfunction. Presumably, VP shunt malfunction created a positive rostral pressure gradient across a stenotic foramen magnum, resulting in tetraparesis from foramen magnum syndrome in 1 patient and acute ataxia and cranial nerve deficits from syringobulbia in the other. Although urgent shunt revisions yielded partial recovery of neurological function in both patients, marked improvement occurred only after posterior fossa decompression.

Pediatric Chiari malformation Type 0: a 12-year institutional experience

2011 ◽  
Vol 8 (1) ◽  
pp. 1-5 ◽  
Author(s):  
Joshua J. Chern ◽  
Amber J. Gordon ◽  
Martin M. Mortazavi ◽  
R. Shane Tubbs ◽  
W. Jerry Oakes
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Foramen Magnum ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Csf Flow ◽  
Chiari Malformation Type I ◽  
Institutional Experience ◽  
Physical Barriers ◽  
Small Cohort

Object In 1998 the authors identified 5 patients with syringomyelia and no evidence of Chiari malformation Type I (CM-I). Magnetic resonance imaging of the entire neuraxis ruled out other causes of a syrinx. Ultimately, abnormal CSF flow at the foramen magnum was the suspected cause. The label “Chiari 0” was used to categorize these unique cases with no tonsillar ectopia. All of the patients underwent posterior fossa decompression and duraplasty identical to the technique used to treat patients with CM-I. Significant syrinx and symptom resolution occurred in these patients. Herein, the authors report on a follow-up study of patients with CM-0 who were derived from over 400 operative cases of pediatric CM-I decompression. Methods The authors present their 12-year experience with this group of patients. Results Fifteen patients (3.7%) were identified. At surgery, many were found to have physical barriers to CSF flow near the foramen magnum. In most of them, the syringomyelia was greatly diminished postoperatively. Conclusions The authors stress that this subgroup represents a very small cohort among patients with Chiari malformations. They emphasize that careful patient selection is critical when diagnosing CM-0. Without an obvious CM-I, other etiologies of a spinal syrinx must be conclusively ruled out. Only then can one reasonably expect to ameliorate the clinical course of these patients via posterior fossa decompression.

scholarly journals Syringobulbia in Patients with Chiari Malformation Type I: A Systematic Review

2019 ◽  
Vol 2019 ◽  
pp. 1-8 ◽  
Author(s):  
Jian Shen ◽  
Jie Shen ◽  
Kaiyuan Huang ◽  
Yixin Wu ◽  
Jianwei Pan ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Clinical Features ◽  
Chiari Malformation ◽  
Initial Therapy ◽  
Foramen Magnum ◽  
Sudden Onset ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Limb Weakness ◽  
Chiari Malformation Type I

This study aimed to summarize the clinical features, diagnosis, and treatment of Chiari malformation type I- (CM-1-) associated syringobulbia. We performed a literature review of CM-1-associated syringobulbia in PubMed, Ovid MEDLINE, and Web of Science databases. Our concerns were the clinical features, radiologic presentations, treatment therapies, and prognoses of CM-1-associated syringobulbia. This review identified 23 articles with 53 cases. Symptoms included headache, neck pain, cranial nerve palsy, limb weakness/dysesthesia, Horner syndrome, ataxia, and respiratory disorders. The most frequently involved area was the medulla. Most of the patients also had syringomyelia. Surgical procedures performed included posterior fossa decompression, foramen magnum decompression, cervical laminectomy, duraplasty, and syringobulbic cavity shunt. Most patients experienced symptom alleviation or resolution postoperatively. A syringobulbic cavity shunt provided good results in refractory cases. Physicians should be aware of the possibility of syringobulbia in CM-1 patients, especially those with symptoms of sudden-onset brain-stem involvement. The diagnosis relies on the disorder’s specific symptomatology and magnetic resonance imaging. Our review suggests that the initial therapy should be posterior fossa decomposition with or without duraplasty. In refractory cases, additional syringobulbic cavity shunt is the preferred option.

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