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CC BY-NC-ND 4.0 · Asian J Neurosurg 2025; 20(01): 043-051
DOI: 10.1055/s-0044-1791710
Original Article

A Prospective Study of CSF Flow Dynamics Across Foramen Magnum in Adult Chiari Malformation/Syringomyelia Complex and its Clinical Correlation with Outcomes after Surgery

Manas Prakash
1   Department of Neurosurgery, All India Institute of Medical Sciences, Bhopal, Saket Nagar, Madhya Pradesh, India
,
Adesh Shrivastava
1   Department of Neurosurgery, All India Institute of Medical Sciences, Bhopal, Saket Nagar, Madhya Pradesh, India
,
Pradeep Chouksey 
1   Department of Neurosurgery, All India Institute of Medical Sciences, Bhopal, Saket Nagar, Madhya Pradesh, India
,
Sumit Raj
1   Department of Neurosurgery, All India Institute of Medical Sciences, Bhopal, Saket Nagar, Madhya Pradesh, India
,
Rakesh Mishra
2   Department of Neurosurgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India
,
Amit Agrawal 
1   Department of Neurosurgery, All India Institute of Medical Sciences, Bhopal, Saket Nagar, Madhya Pradesh, India
,
Suresh Nair
3   Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India
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Abstract

Introduction Chiari I malformation refers to cerebellar tonsillar descent below the foramen magnum and is frequently associated with syringomyelia. Prior cerebrospinal fluid (CSF) flow studies correlated the clinical severity of these lesions with general flow velocity or bulk flow at the foramen magnum; however, these techniques have not assessed the effect on surgical outcomes. The study aims to present clinical and radiological factors and CSF flow parameters (pre- and postoperative) that affect the surgical outcome.

Materials and Methods The institutional ethics committee approved the study. We collected the prospective clinical data, including pre- and postoperative symptoms. Functional grades were determined along with the change in clinical improvement based on clinical examination notes, the change in functional grade was calculated, and the radiologic data were analyzed according to the degree of clinical improvement. The surgical procedure included suboccipital bony decompression with duroplasty. Patients were followed up at 1 month, 3 months, and 1 year.

Results There were a total of 25 patients. The mean age of the patients was 45.52 ± 13.37 years, with 40% being males and 60% being females. After the surgery, there was a significant increase in the anterior and posterior CSF flows at the foramen magnum. Most had a resolution in the headache and sensory symptoms, while the lower cranial nerve and motor symptoms had a minor resolution at follow-up. Age, sex, and headache do not correlate with the outcome or syrinx improvement. Motor power in the lower limb (preintervention) and syrinx shape (preintervention) were significantly associated with the variable “anterior flow at the foramen magnum” (preintervention; p < 0.05). Average flow, cervicomedullary angle (postintervention), sensory symptom progression, and CSF flow change anterior to the foramen magnum were significantly associated with the variable “anterior flow at the foramen magnum” (postintervention; p < 0.05).

Conclusion Even after surgery, persistent foramen magnum CSF outflow obstruction has a robust negative correlation with the outcome. The Chiari outcome predictability index has shown a significant correlation with patient outcomes and can be used to inform patients about the expected outcome. The results of the present study will be helpful in stratifying patients according to their desired outcomes.

Keywords

Chiari malformation - syrinx - CSF flow dynamics - Craniovertebral junction (CVJ) anomalies - foramen magnum decompression. - syringomyelia

Authors' Contributions

M.P. was responsible for data collection, design, analysis, and manuscript writing. A.S. was responsible for hypothesis, supervision, study design, data analysis, presentation of results, and reviewing the manuscript. P.C. was involved in supervision, manuscript writing, and administrative support. S.R. was involved in administrative support, clinical details, surgical details, and reviewing the manuscript. R.M. was involved in manuscript writing, reviewing the manuscript, and design. A.A. was involved in administrative support, reviewing the manuscript, and supervision. S.N. was involved in supervision, data analysis, reviewing the manuscript, and administrative support.


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Publication History

Article published online:
22 October 2024

© 2024. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Arnautovic A, Splavski B, Boop FA, Arnautovic KI. Pediatric and adult Chiari malformation type I surgical series 1965-2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr 2015; 15 (02) 161-177
    CrossrefPubMedSearch in Google Scholar
  • 2 Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry 1965; 28 (03) 247-259
    CrossrefPubMedSearch in Google Scholar
  • 3 Oldfield EH, Muraszko K, Shawker TH, Patronas NJ. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 1994; 80 (01) 3-15
    CrossrefPubMedSearch in Google Scholar
  • 4 Milhorat TH, Chou MW, Trinidad EM. et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 1999; 44 (05) 1005-1017
    CrossrefPubMedSearch in Google Scholar
  • 5 Oldfield EH. Pathogenesis of Chiari I: pathophysiology of syringomyelia—implications for therapy: a summary of 3 decades of clinical research. Neurosurgery 2017; 64 (CN_suppl_1): 66-77
    CrossrefPubMedSearch in Google Scholar
  • 6 du Boulay G, Shah SH, Currie JC, Logue V. The mechanism of hydromyelia in Chiari type 1 malformations. Br J Radiol 1974; 47 (561) 579-587
    CrossrefPubMedSearch in Google Scholar
  • 7 Goel A. Is atlantoaxial instability the cause of Chiari malformation? Outcome analysis of 65 patients treated by atlantoaxial fixation. J Neurosurg Spine 2015; 22 (02) 116-127
    CrossrefPubMedSearch in Google Scholar
  • 8 Hida K, Iwasaki Y, Koyanagi I, Abe H. Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes. Surg Neurol 1999; 51 (04) 383-390 , discussion 390–391
    CrossrefPubMedSearch in Google Scholar
  • 9 Mueller DM, Oro' JJ. The Chiari symptom profile: development and validation of a Chiari-/syringomyelia-specific questionnaire. J Neurosci Nurs 2013; 45 (04) 205-210
    CrossrefPubMedSearch in Google Scholar
  • 10 Holubar K. Looking back ... Paul Langerhans, Theodor Langhans, and Carl Touton: contemporary histopathologists. Am J Dermatopathol 1990; 12 (05) 534-535
    CrossrefPubMedSearch in Google Scholar
  • 11 Mortazavi MM, Tubbs RS, Brockerhoff MA, Loukas M, Oakes WJ. The first description of Chiari I malformation with intuitive correlation between tonsillar ectopia and syringomyelia. J Neurosurg Pediatr 2011; 7 (03) 257-260
    CrossrefPubMedSearch in Google Scholar
  • 12 Blegvad C, Grotenhuis JA, Juhler M. Syringomyelia: a practical, clinical concept for classification. Acta Neurochir (Wien) 2014; 156 (11) 2127-2138
    CrossrefPubMedSearch in Google Scholar
  • 13 Demetriades AK. The first descriptions of syringomyelia. J R Soc Med 2012; 105 (07) 278
    CrossrefPubMedSearch in Google Scholar
  • 14 Noudel R, Gomis P, Sotoares G. et al. Posterior fossa volume increase after surgery for Chiari malformation type I: a quantitative assessment using magnetic resonance imaging and correlations with the treatment response. J Neurosurg 2011; 115 (03) 647-658
    CrossrefPubMedSearch in Google Scholar
  • 15 Furtado SV, Thakar S, Hegde AS. Correlation of functional outcome and natural history with clinicoradiological factors in surgically managed pediatric Chiari I malformation. Neurosurgery 2011; 68 (02) 319-327 , discussion 328
    CrossrefPubMedSearch in Google Scholar
  • 16 Schijman E, Steinbok P. International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst 2004; 20 (05) 341-348
    CrossrefPubMedSearch in Google Scholar
  • 17 Wetjen NM, Heiss JD, Oldfield EH. Time course of syringomyelia resolution following decompression of Chiari malformation type I. J Neurosurg Pediatr 2008; 1 (02) 118-123
    CrossrefPubMedSearch in Google Scholar
  • 18 Zhang ZQ, Chen YQ, Chen YA, Wu X, Wang YB, Li XG. Chiari I malformation associated with syringomyelia: a retrospective study of 316 surgically treated patients. Spinal Cord 2008; 46 (05) 358-363
    CrossrefPubMedSearch in Google Scholar
  • 19 Aitken LA, Lindan CE, Sidney S. et al. Chiari type I malformation in a pediatric population. Pediatr Neurol 2009; 40 (06) 449-454
    CrossrefPubMedSearch in Google Scholar
  • 20 Greenberg JK, Yarbrough CK, Radmanesh A. et al. The Chiari Severity Index: a preoperative grading system for Chiari malformation type 1. Neurosurgery 2015; 76 (03) 279-285 , discussion 285
    CrossrefPubMedSearch in Google Scholar
  • 21 Batzdorf U, McArthur DL, Bentson JR. Surgical treatment of Chiari malformation with and without syringomyelia: experience with 177 adult patients. J Neurosurg 2013; 118 (02) 232-242
    CrossrefPubMedSearch in Google Scholar
  • 22 Sakushima K, Hida K, Yabe I, Tsuboi S, Uehara R, Sasaki H. Different surgical treatment techniques used by neurosurgeons and orthopedists for syringomyelia caused by Chiari I malformation in Japan. J Neurosurg Spine 2013; 18 (06) 588-592
    CrossrefPubMedSearch in Google Scholar
  • 23 Tisell M, Wallskog J, Linde M. Long-term outcome after surgery for Chiari I malformation. Acta Neurol Scand 2009; 120 (05) 295-299
    CrossrefPubMedSearch in Google Scholar
  • 24 Fernández AA, Guerrero AI, Martínez MI. et al. Malformations of the craniocervical junction (Chiari type I and syringomyelia: classification, diagnosis and treatment). BMC Musculoskelet Disord 2009; 10 (suppl 1): S1
    CrossrefPubMedSearch in Google Scholar
  • 25 Kitamura T, Miyazaki S, Kadotani H. et al. Type I Chiari malformation presenting central sleep apnea. Auris Nasus Larynx 2014; 41 (02) 222-224
    CrossrefPubMedSearch in Google Scholar
  • 26 Losurdo A, Dittoni S, Testani E. et al. Sleep disordered breathing in children and adolescents with Chiari malformation type I. J Clin Sleep Med 2013; 9 (04) 371-377
    CrossrefPubMedSearch in Google Scholar
  • 27 Bindal AK, Dunsker SB, Tew Jr JM. Chiari I malformation: classification and management. Neurosurgery 1995; 37 (06) 1069-1074
    CrossrefPubMedSearch in Google Scholar
  • 28 Gilmer HS, Xi M, Young SH. Surgical decompression for Chiari malformation type I: an age-based outcomes study based on the Chicago Chiari Outcome Scale. World Neurosurg 2017; 107: 285-290
    CrossrefPubMedSearch in Google Scholar
  • 29 Ozisik PA, Hazer B, Ziyal IM, Ozcan OE. Spontaneous resolution of syringomyelia without Chiari malformation. Neurol Med Chir (Tokyo) 2006; 46 (10) 512-517
    CrossrefPubMedSearch in Google Scholar
  • 30 Perrini P. Spontaneous resolution of syringomyelia in an adult patient with tight cisterna magna. Neurol Sci 2012; 33 (06) 1463-1467
    CrossrefPubMedSearch in Google Scholar
  • 31 Vaquero J, Ferreira E, Parajón A. Spontaneous resolution of syrinx: report of two cases in adults with Chiari malformation. Neurol Sci 2012; 33 (02) 339-341
    CrossrefPubMedSearch in Google Scholar
  • 32 Boman K, Iivanainen M. Prognosis of syringomyelia. Acta Neurol Scand 1967; 43 (01) 61-68
    CrossrefPubMedSearch in Google Scholar
  • 33 Novegno F, Caldarelli M, Massa A. et al. The natural history of the Chiari type I anomaly. J Neurosurg Pediatr 2008; 2 (03) 179-187
    CrossrefPubMedSearch in Google Scholar
  • 34 Ataizi S, Canakçi Z, Baloğlu M, Cerezci A. Spontaneously resorbed idiopathic syringomyelia: a case report. Turk Neurosurg 2007; 17 (04) 247-250
    PubMedSearch in Google Scholar
  • 35 Jinkins JR, Sener RN. Idiopathic localized hydromyelia: dilatation of the central canal of the spinal cord of probable congenital origin. J Comput Assist Tomogr 1999; 23 (03) 351-353
    CrossrefPubMedSearch in Google Scholar
  • 36 Kim J, Kim CH, Jahng TA, Chung CK. Clinical course of incidental syringomyelia without predisposing pathologies. J Clin Neurosci 2012; 19 (05) 665-668
    CrossrefPubMedSearch in Google Scholar
  • 37 Roy AK, Slimack NP, Ganju A. Idiopathic syringomyelia: retrospective case series, comprehensive review, and update on management. Neurosurg Focus 2011; 31 (06) E15
    CrossrefPubMedSearch in Google Scholar
  • 38 Levy WJ, Mason L, Hahn JF. Chiari malformation presenting in adults: a surgical experience in 127 cases. Neurosurgery 1983; 12 (04) 377-390
    CrossrefPubMedSearch in Google Scholar
  • 39 Pillay PK, Awad IA, Little JR, Hahn JF. Symptomatic Chiari malformation in adults: a new classification based on magnetic resonance imaging with clinical and prognostic significance. Neurosurgery 1991; 28 (05) 639-645
    CrossrefPubMedSearch in Google Scholar
  • 40 Hekman KE, Aliaga L, Straus D. et al. Positive and negative predictors for good outcome after decompressive surgery for Chiari malformation type 1 as scored on the Chicago Chiari Outcome Scale. Neurol Res 2012; 34 (07) 694-700
    CrossrefPubMedSearch in Google Scholar
  • 41 Goel A, Desai K. Surgery for syringomyelia: an analysis based on 163 surgical cases. Acta Neurochir (Wien) 2000; 142 (03) 293-301 , discussion 301–302
    CrossrefPubMedSearch in Google Scholar
  • 42 Klekamp J. The pathophysiology of syringomyelia: historical overview and current concept. Acta Neurochir (Wien) 2002; 144 (07) 649-664
    CrossrefPubMedSearch in Google Scholar
  • 43 Bogdanov EI, Heiss JD, Mendelevich EG, Mikhaylov IM, Haass A. Clinical and neuroimaging features of “idiopathic” syringomyelia. Neurology 2004; 62 (05) 791-794
    CrossrefPubMedSearch in Google Scholar
  • 44 Imae S. Clinical evaluation on etiology and surgical outcome in syringomyelia associated with Chiari type I malformation. No To Shinkei 1997; 49 (12) 1131-1138
    PubMedSearch in Google Scholar
  • 45 Kumar R, Kalra SK, Vaid VK, Mahapatra AK. Chiari I malformation: surgical experience over a decade of management. Br J Neurosurg 2008; 22 (03) 409-414
    CrossrefPubMedSearch in Google Scholar
  • 46 Nishizawa S, Yokoyama T, Yokota N, Tokuyama T, Ohta S. Incidentally identified syringomyelia associated with Chiari I malformations: is early interventional surgery necessary?. Neurosurgery 2001; 49 (03) 637-640 , discussion 640–641
    PubMedSearch in Google Scholar
  • 47 Nakamura M, Ishii K, Watanabe K. et al. Clinical significance and prognosis of idiopathic syringomyelia. J Spinal Disord Tech 2009; 22 (05) 372-375
    CrossrefPubMedSearch in Google Scholar
  • 48 McRae DL, Standen J. Roentgenologic findings in syringomyelia and hydromyelia. Am J Roentgenol Radium Ther Nucl Med 1966; 98 (03) 695-703
    CrossrefPubMedSearch in Google Scholar
  • 49 Roser F, Ebner FH, Sixt C, Hagen JM, Tatagiba MS. Defining the line between hydromyelia and syringomyelia. A differentiation is possible based on electrophysiological and magnetic resonance imaging studies. Acta Neurochir (Wien) 2010; 152 (02) 213-219 , discussion 219
    CrossrefPubMedSearch in Google Scholar
  • 50 Ellenbogen RG, Armonda RA, Shaw DW, Winn HR. Toward a rational treatment of Chiari I malformation and syringomyelia. Neurosurg Focus 2000; 8 (03) E6
    CrossrefPubMedSearch in Google Scholar
  • 51 Menezes AH. Chiari I malformations and hydromyelia: complications. Pediatr Neurosurg 1991-1992; 17 (03) 146-154
    CrossrefPubMed