Differentiation of Chiari malformation type 1 and spontaneous intracranial hypotension using objective measurements of midbrain sagging

View More View Less
  • 1 Department of Radiology, Division of Neuroradiology, Duke University Medical Center, Durham, North Carolina
Restricted access

Purchase Now

USD  $45.00

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

USD  $515.00

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

USD  $612.00
Print or Print + Online

OBJECTIVE

Chiari malformation type 1 (CM-1) and spontaneous intracranial hypotension (SIH) are causes of headache in which cerebellar tonsillar ectopia (TE) may be present. An accurate method for differentiating these conditions on imaging is needed to avoid diagnostic confusion. Here, the authors sought to determine whether objective measurements of midbrain morphology could distinguish CM-1 from SIH on brain MRI.

METHODS

This is a retrospective case-control series comparing neuroimaging in consecutive adult subjects with CM-1 and SIH. Measurements obtained from brain MRI included previously reported measures of brain sagging: TE, slope of the third ventricular floor (3VF), pontomesencephalic angle (PMA), mamillopontine distance, lateral ventricular angle, internal cerebral vein–vein of Galen angle, and displacement of iter (DOI). Clivus length (CL), an indicator of posterior fossa size, was also measured. Measurements for the CM-1 group were compared to those for the entire SIH population (SIHall) as well as a subgroup of SIH patients with > 5 mm of TE (SIHTE subgroup).

RESULTS

Highly significant differences were observed between SIHall and CM-1 groups in the following measures: TE (mean ± standard deviation, 3.1 ± 5.7 vs 9.3 ± 3.5 mm), 3VF (−16.8° ± 11.2° vs −2.1° ± 4.6°), PMA (44.8° ± 13.1° vs 62.7° ± 9.8°), DOI (0.2 ± 4.1 vs 3.8 ± 1.6 mm), and CL (38.3 ± 4.5 vs 44.0 ± 3.3 mm; all p < 0.0001). Eight (16%) of 50 SIH subjects had TE > 5 mm; in this subgroup (SIHTE), a cutoff value of < −15° for 3VF and < 45° for PMA perfectly discriminated SIH from CM-1 (sensitivity and specificity = 1.0). DOI showed perfect specificity (1.0) in detecting SIH among both groups. No subjects with SIH had isolated TE without other concurrent findings of midbrain sagging.

CONCLUSIONS

Measures of midbrain sagging, including cutoff values for 3VF and PMA, discriminate CM-1 from SIH and may help to prevent misdiagnosis and unnecessary surgery.

ABBREVIATIONS

3VF = slope of the third ventricular floor; CL = clivus length; CM-1 = Chiari malformation type 1; CVF = CSF-venous fistula; DOI = displacement of iter; LVA = lateral ventricular angle; MPD = mamillopontine distance; PMA = pontomesencephalic angle; SIH = spontaneous intracranial hypotension; TE = tonsillar ectopia; VHA = venous hinge angle.

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

USD  $515.00

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

USD  $612.00
  • 1

    McClugage SG, Oakes WJ. The Chiari I malformation. J Neurosurg Pediatr. 2019;24(3):217226.

  • 2

    Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA. 2006;295(19):22862296.

  • 3

    Spelle L, Boulin A, Tainturier C, Visot A, Graveleau P, Pierot L. Neuroimaging features of spontaneous intracranial hypotension. Neuroradiology. 2001;43(8):622627.

    • Search Google Scholar
    • Export Citation
  • 4

    Smith BW, Strahle J, Bapuraj JR, Muraszko KM, Garton HJ, Maher CO. Distribution of cerebellar tonsil position: implications for understanding Chiari malformation. J Neurosurg. 2013;119(3):812819.

    • Search Google Scholar
    • Export Citation
  • 5

    Raybaud C, Jallo GI. Chiari 1 deformity in children: etiopathogenesis and radiologic diagnosis. Handb Clin Neurol. 2018;155:2548.

  • 6

    Kranz PG, Tanpitukpongse TP, Choudhury KR, Amrhein TJ, Gray L. Imaging signs in spontaneous intracranial hypotension: prevalence and relationship to CSF pressure. AJNR Am J Neuroradiol. 2016;37(7):13741378.

    • Search Google Scholar
    • Export Citation
  • 7

    Evans RW, Burch RC, Frishberg BM, Marmura MJ, Mechtler LL, Silberstein SD, Turner DP. Neuroimaging for migraine: the American Headache Society systematic review and evidence-based guideline. Headache. 2020;60(2):318336.

    • Search Google Scholar
    • Export Citation
  • 8

    Hofkes SK, Iskandar BJ, Turski PA, Gentry LR, McCue JB, Haughton VM. Differentiation between symptomatic Chiari I malformation and asymptomatic tonsilar ectopia by using cerebrospinal fluid flow imaging: initial estimate of imaging accuracy. Radiology. 2007;245(2):532540.

    • Search Google Scholar
    • Export Citation
  • 9

    Headache Classification Committee of the International Headache Society. The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013;33(9):629808.

    • Search Google Scholar
    • Export Citation
  • 10

    Barkovich AJ, Wippold FJ, Sherman JL, Citrin CM. Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol. 1986;7(5):795799.

    • Search Google Scholar
    • Export Citation
  • 11

    Young SJ, Quisling RG, Bidari S, Sanghvi TS. An objective study of anatomic shifts in intracranial hypotension using four anatomic planes. Radiol Res Pract. 2018;2018:6862739.

    • Search Google Scholar
    • Export Citation
  • 12

    Shah LM, McLean LA, Heilbrun ME, Salzman KL. Intracranial hypotension: improved MRI detection with diagnostic intracranial angles. AJR Am J Roentgenol. 2013;200(2):400407.

    • Search Google Scholar
    • Export Citation
  • 13

    Shankar JJ, Chakraborty S, Lum C. The venous hinge--an objective sign for the diagnosis and follow-up of treatment in patients with intracranial hypotension syndrome. Neuroradiology. 2009;51(7):453456.

    • Search Google Scholar
    • Export Citation
  • 14

    Bordes S, Jenkins S, Tubbs RS. Defining, diagnosing, clarifying, and classifying the Chiari I malformations. Childs Nerv Syst. 2019;35(10):17851792.

    • Search Google Scholar
    • Export Citation
  • 15

    Farb RI, Forghani R, Lee SK, Mikulis DJ, Agid R. The venous distension sign: a diagnostic sign of intracranial hypotension at MR imaging of the brain. AJNR Am J Neuroradiol. 2007;28(8):14891493.

    • Search Google Scholar
    • Export Citation
  • 16

    Aiken AH, Hoots JA, Saindane AM, Hudgins PA. Incidence of cerebellar tonsillar ectopia in idiopathic intracranial hypertension: a mimic of the Chiari I malformation. AJNR Am J Neuroradiol. 2012;33(10):19011906.

    • Search Google Scholar
    • Export Citation
  • 17

    Kranz PG, Gray L, Malinzak MD, Amrhein TJ. Spontaneous intracranial hypotension: pathogenesis, diagnosis, and treatment. Neuroimaging Clin N Am. 2019;29(4):581594.

    • Search Google Scholar
    • Export Citation
  • 18

    Schievink WI. Misdiagnosis of spontaneous intracranial hypotension. Arch Neurol. 2003;60(12):17131718.

  • 19

    Kranz PG, Viola RJ, Gray L. Resolution of syringohydromyelia with targeted CT-guided epidural blood patching. J Neurosurg. 2011;115(3):641644.

    • Search Google Scholar
    • Export Citation
  • 20

    Parker SR, Harris P, Cummings TJ, George T, Fuchs H, Grant G. Complications following decompression of Chiari malformation Type I in children: dural graft or sealant? J Neurosurg Pediatr. 2011;8(2):177183.

    • Search Google Scholar
    • Export Citation
  • 21

    Nguyen VN, Moore KA, Hersh DS, Boop FA. Complications of Chiari surgery. In: Tubbs RS, Turgut M, Oakes, WJ, eds. The Chiari Malformations.Springer;2020:493502.

    • Search Google Scholar
    • Export Citation
  • 22

    Mauser ED, Rosen NL. So many migraines, so few subspecialists: analysis of the geographic location of United Council for Neurologic Subspecialties (UCNS) certified headache subspecialists compared to United States headache demographics. Headache. 2014;54(8):13471357.

    • Search Google Scholar
    • Export Citation
  • 23

    Kranz PG, Gray L, Amrhein TJ. Spontaneous intracranial hypotension: 10 myths and misperceptions. Headache. 2018;58(7):948959.

  • 24

    Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, et al. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery. 1999;44(5):10051017.

    • Search Google Scholar
    • Export Citation
  • 25

    Kranz PG, Luetmer PH, Diehn FE, Amrhein TJ, Tanpitukpongse TP, Gray L. Myelographic techniques for the detection of spinal CSF leaks in spontaneous intracranial hypotension. AJR Am J Roentgenol. 2016;206(1):819.

    • Search Google Scholar
    • Export Citation
  • 26

    Sun PP, Harrop J, Sutton LN, Younkin D. Complete spontaneous resolution of childhood Chiari I malformation and associated syringomyelia. Pediatrics. 2001;107(1):182184.

    • Search Google Scholar
    • Export Citation
  • 27

    Klekamp J, Iaconetta G, Samii M. Spontaneous resolution of Chiari I malformation and syringomyelia: case report and review of the literature. Neurosurgery. 2001;48(3):664667.

    • Search Google Scholar
    • Export Citation
  • 28

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

    • Search Google Scholar
    • Export Citation
  • 29

    Avellino AM, Britz GW, McDowell JR, Shaw DW, Ellenbogen RG, Roberts TS. Spontaneous resolution of a cervicothoracic syrinx in a child. Case report and review of the literature. Pediatr Neurosurg. 1999;30(1):4346.

    • Search Google Scholar
    • Export Citation
  • 30

    Dlouhy BJ, Capuano AW, Madhavan K, Torner JC, Greenlee JD. Preoperative third ventricular bowing as a predictor of endoscopic third ventriculostomy success. J Neurosurg Pediatr. 2012;9(2):182190.

    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 2560 2560 713
Full Text Views 269 269 122
PDF Downloads 332 332 143
EPUB Downloads 0 0 0