Spontaneous resolution of a Chiari malformation Type I and syrinx after supratentorial craniotomy for excision of a cavernous malformation

Case report

View More View Less
  • 1 Department of Neurosurgery, Tufts Medical Center;
  • 2 Tufts University School of Medicine, Boston; and
  • 3 Division of Neurosurgery, Baystate Medical Center, Springfield, Massachusetts
Restricted access

Purchase Now

USD  $45.00

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00
Print or Print + Online

The pathogenesis of Chiari malformation Type I (CM-I) and associated syringomyelia is incompletely understood. Patients often present in middle age with incidental or minimally symptomatic CM-I, whose management is controversial. One option is clinical and radiographic observation of asymptomatic and minimally symptomatic patients. The authors here present the case of a 36-year-old woman who had been monitored for 6 years for a minimally symptomatic CM-I and cervicothoracic syrinx. After 5 years of follow-up, she suffered spontaneous rupture of a cerebral cavernous malformation when she was 27 weeks pregnant. The ruptured cavernous malformation and hematoma were operatively managed via a right frontal craniotomy. Ten months after the craniotomy for resection of the ruptured cavernous malformation, follow-up MRI demonstrated resolution of the CM-I and syrinx. Few similar cases have been reported in adults. This case argues for the presence of dynamic factors in the development and maintenance of CM-I and supports the nonoperative treatment of asymptomatic and minimally symptomatic patients.

Abbreviations used in this paper:CM-I = Chiari malformation Type I; CTA = CT angiography; MRA = MR angiography.

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00

Contributor Notes

Address correspondence to: Carl B. Heilman, M.D., Department of Neurosurgery, Tufts Medical Center and Tufts University School of Medicine, 800 Washington Street, #178, Boston, Massachusetts 20111. email: cheilman@tuftsmedicalcenter.org.

Please include this information when citing this paper: published online February 10, 2012; DOI: 10.3171/2012.1.JNS11580.

  • 1

    Aboulker J: La syringomyélie et les liquides intra-rachidiens. Neurochirurgie 25:1 Suppl 1144, 1979

  • 2

    Albert GW, , Menezes AH, , Hansen DR, , Greenlee JDW, & Weinstein SL: Chiari malformation Type I in children younger than age 6 years: presentation and surgical outcome. Clinical article. J Neurosurg Pediatr 5:554561, 2010

    • Search Google Scholar
    • Export Citation
  • 3

    Attenello FJ, , McGirt MJ, , Gathinji M, , Datoo G, , Atiba A, & Weingart J, : Outcome of Chiari-associated syringomyelia after hindbrain decompression in children: analysis of 49 consecutive cases. Neurosurgery 62:13071313, 2008

    • Search Google Scholar
    • Export Citation
  • 4

    Avellino AM, , Kim DK, , Weinberger E, & Roberts TS: Resolution of spinal syringes and Chiari I malformation in a child. Case illustration. J Neurosurg 84:708, 1996

    • Search Google Scholar
    • Export Citation
  • 5

    Ball MJ, & Dayan AD: Pathogenesis of syringomyelia. Lancet 2:799801, 1972

  • 6

    Barkovich AJ, , Wippold FJ, , Sherman JL, & Citrin CM: Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol 7:795799, 1986

    • Search Google Scholar
    • Export Citation
  • 7

    Bhadelia RA, , Bogdan AR, , Wolpert SM, , Lev S, , Appignani BA, & Heilman CB: Cerebrospinal fluid flow waveforms: analysis in patients with Chiari I malformation by means of gated phase-contrast MR imaging velocity measurements. Radiology 196:195202, 1995

    • Search Google Scholar
    • Export Citation
  • 8

    Castillo M, & Wilson JD: Spontaneous resolution of a Chiari I malformation: MR demonstration. AJNR Am J Neuroradiol 16:11581160, 1995

  • 9

    Chantigian RC, , Koehn MA, , Ramin KD, & Warner MA: Chiari I malformation in parturients. J Clin Anesth 14:201205, 2002

  • 10

    Chiari H: Ueber Veränderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch Med Wochenschr 17:11721175, 1891

  • 11

    Chumas PD, , Armstrong DC, , Drake JM, , Kulkarni AV, , Hoffman HJ, & Humphreys RP, : Tonsillar herniation: the rule rather than the exception after lumboperitoneal shunting in the pediatric population. J Neurosurg 78:568573, 1993

    • Search Google Scholar
    • Export Citation
  • 12

    Coppa ND, , Kim HJ, & McGrail KM: Spontaneous resolution of syringomyelia and Chiari malformation Type I in a patient with cerebrospinal fluid otorrhea. Case report. J Neurosurg 105:769771, 2006

    • Search Google Scholar
    • Export Citation
  • 13

    Dyste GN, , Menezes AH, & VanGilder JC: Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 71:159168, 1989

    • Search Google Scholar
    • Export Citation
  • 14

    Elster AD, & Chen MYM: Chiari I malformations: clinical and radiologic reappraisal. Radiology 183:347353, 1992

  • 15

    Gardner WJ, , Abdullah AF, & McCormack LJ: The varying expressions of embryonal atresia of the fourth ventricle in adults: Arnold-Chiari malformation, Dandy-Walker syndrome, arachnoid cyst of the cerebellum, and syringomyelia. J Neurosurg 14:591605, 1957

    • Search Google Scholar
    • Export Citation
  • 16

    Gardner WJ, & Angel J: The cause of syringomyelia and its surgical treatment. Cleve Clin Q 25:48, 1958

  • 17

    Guillen A, & Costa JM: Spontaneous resolution of a Chiari I malformation associated syringomyelia in one child. Acta Neurochir (Wien) 146:187191, 2004

    • Search Google Scholar
    • Export Citation
  • 18

    Haroun RI, , Guarnieri M, , Meadow JJ, , Kraut M, & Carson BS: Current opinions for the treatment of syringomyelia and chiari malformations: survey of the Pediatric Section of the American Association of Neurological Surgeons. Pediatr Neurosurg 33:311317, 2000

    • Search Google Scholar
    • Export Citation
  • 19

    Jatavallabhula NS, , Armstrong J, , Sgouros S, & Whitehouse W: Spontaneous resolution of isolated Chiari I malformation. Childs Nerv Syst 22:201203, 2006

    • Search Google Scholar
    • Export Citation
  • 20

    Klekamp J, , Iaconetta G, & Samii M: Spontaneous resolution of Chiari I malformation and syringomyelia: case report and review of the literature. Neurosurgery 48:664667, 2001

    • Search Google Scholar
    • Export Citation
  • 21

    Koehler PJ: Chiari's description of cerebellar ectopy (1891). With a summary of Cleland's and Arnold's contributions and some early observations on neural-tube defects. J Neurosurg 75:823826, 1991

    • Search Google Scholar
    • Export Citation
  • 22

    Kyoshima K, & Bogdanov EI: Spontaneous resolution of syringomyelia: report of two cases and review of the literature. Neurosurgery 53:762769, 2003

    • Search Google Scholar
    • Export Citation
  • 23

    McGirt MJ, , Nimjee SM, , Fuchs HE, & George TM: Relationship of cine phase-contrast magnetic resonance imaging with outcome after decompression for Chiari I malformations. Neurosurgery 59:140146, 2006

    • Search Google Scholar
    • Export Citation
  • 24

    Meadows J, , Kraut M, , Guarnieri M, , Haroun RI, & Carson BS: Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging. J Neurosurg 92:920926, 2000

    • Search Google Scholar
    • Export Citation
  • 25

    Mikulis DJ, , Diaz O, , Egglin TK, & Sanchez R: Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology 183:725728, 1992

    • Search Google Scholar
    • Export Citation
  • 26

    Milhorat TH, , Capocelli AL Jr, , Anzil AP, , Kotzen RM, & Milhorat RH: Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases. J Neurosurg 82:802812, 1995

    • Search Google Scholar
    • Export Citation
  • 27

    Milhorat TH, , Chou MW, , Trinidad EM, , Kula RW, , Mandell M, & Wolpert C, : Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44:10051017, 1999

    • Search Google Scholar
    • Export Citation
  • 28

    Miller JH, , Limbrick DD Jr, , Callen M, & Smyth MD: Spontaneous resolution of Chiari malformation Type I in monozygotic twins. Report of 2 cases. J Neurosurg Pediatr 2:317319, 2008

    • Search Google Scholar
    • Export Citation
  • 29

    Mueller DM, & Oro' J: Chiari I malformation with or without syringomyelia and pregnancy: case studies and review of the literature. Am J Perinatol 22:6770, 2005

    • Search Google Scholar
    • Export Citation
  • 30

    Navarro R, , Olavarria G, , Seshadri R, , Gonzales-Portillo G, , McLone DG, & Tomita T: Surgical results of posterior fossa decompression for patients with Chiari I malformation. Childs Nerv Syst 20:349356, 2004

    • Search Google Scholar
    • Export Citation
  • 31

    Nishikawa M, , Sakamoto H, , Hakuba A, , Nakanishi N, & Inoue Y: Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg 86:4047, 1997

    • Search Google Scholar
    • Export Citation
  • 32

    Nishizawa S, , Yokoyama T, , Yokota N, , Tokuyama T, & Ohta S: Incidentally identified syringomyelia associated with Chiari I malformations: is early interventional surgery necessary?. Neurosurgery 49:637641, 2001

    • Search Google Scholar
    • Export Citation
  • 33

    Novegno F, , Caldarelli M, , Massa A, , Chieffo D, , Massimi L, & Pettorini B, : The natural history of the Chiari Type I anomaly. J Neurosurg Pediatr 2:179187, 2008

    • Search Google Scholar
    • Export Citation
  • 34

    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 80:315, 1994

    • Search Google Scholar
    • Export Citation
  • 35

    Olivero WC, & Dinh DH: Chiari I malformation with traumatic syringomyelia and spontaneous resolution: case report and literature review. Neurosurgery 30:758760, 1992

    • Search Google Scholar
    • Export Citation
  • 36

    Payner TD, , Prenger E, , Berger TS, & Crone KR: Acquired Chiari malformations: incidence, diagnosis, and management. Neurosurgery 34:429434, 1994

    • Search Google Scholar
    • Export Citation
  • 37

    Schijman E, & Steinbok P: International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst 20:341348, 2004

    • Search Google Scholar
    • Export Citation
  • 38

    Steinbok P: Clinical features of Chiari I malformations. Childs Nerv Syst 20:329331, 2004

  • 39

    Sun JC, , Steinbok P, & Cochrane DD: Spontaneous resolution and recurrence of a Chiari I malformation and associated syringomyelia. Case report. J Neurosurg 2 Suppl 92:207210, 2000

    • Search Google Scholar
    • Export Citation
  • 40

    Sun PP, , Harrop J, , Sutton LN, & Younkin D: Complete spontaneous resolution of childhood Chiari I malformation and associated syringomyelia. Pediatrics 107:182184, 2001

    • Search Google Scholar
    • Export Citation
  • 41

    Williams B: A critical appraisal of posterior fossa surgery for communicating syringomyelia. Brain 101:223250, 1978

  • 42

    Williams B: On the pathogenesis of syringomyelia: a review. J R Soc Med 73:798806, 1980

  • 43

    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 46:358363, 2008

    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 631 128 8
Full Text Views 164 10 1
PDF Downloads 238 4 0
EPUB Downloads 0 0 0