Chiari I malformation
Citation, DOI & article data
Chiari I malformation is the most common variant of the Chiari malformations and is characterized by a caudal descent of the cerebellar tonsils (and brainstem in its subtype, Chiari 1.5) through the foramen magnum. Symptoms are proportional to the degree of descent. MRI is the imaging modality of choice. Treatment with posterior decompression is usually reserved for symptomatic patients or those with a syrinx.
Chiari I malformations are more frequently encountered in females 1.
Although Chiari I malformations are often isolated abnormalities, the following findings may be seen in association:
- cervical cord syrinx in ~35% (range 20-56%): more common in symptomatic patients
hydrocephalus in up to 30% 2,3 of cases
- thought to result from abnormal CSF flow dynamics through the central canal of the cord and around the medulla
- skeletal anomalies in ~35% (range 23-45%) 2,3:
Unlike Chiari II, III, and IV malformations, Chiari I malformations often remain asymptomatic until adulthood if it ever becomes symptomatic.
The likelihood of becoming symptomatic is proportional to the degree of descent of the tonsils. In one study, all patients with greater than 12 mm of descent were symptomatic, whereas approximately 30% of those whose descent measured between 5 and 10 mm remained asymptomatic 2. (see MRI section for measurement technique)
The Chiari I malformation is characterized by an inferior position of the cerebellar tonsils relative to the foramen magnum. This is believed to be due to a mismatch between the size and content of the posterior fossa.
Four groups of Chiari I patients can be distinguished, according to different pathogeneses 9:
- abnormal skull base (e.g. short clivus)
- cervical segmentation anomalies (e.g. Klippel-Feil syndrome)
- small cranial vault and/or posterior fossa and consequent overcrowding
- excessive brain tissue (sometimes referred to as "acquired Chiari malformation")
Chiari I malformations need to be distinguished from low-lying tonsils (benign tonsillar ectopia) which is an asymptomatic and incidental finding in normal individuals, whereby the tonsils protrude through the foramen magnum by no more than 3-5 mm 1,2.
The terminology of caudally displaced tonsils is discussed in the article on cerebellar tonsillar ectopia.
Although historically visible on myelography, cross-sectional imaging (especially MRI) is needed to diagnose accurately and assess for Chiari I malformations. In either case, the diagnosis is made by measuring the cerebellar tonsillar position (TP).
With modern volumetric scanning and high-quality sagittal reformats relatively good views of the foramen magnum and tonsils can be achieved although the intrinsic lack of contrast (compared to MRI) makes accurate assessment difficult. More frequently the diagnosis is suspected on axial images where the medulla is embraced by the tonsils and little if any CSF is present. This is referred to as a crowded foramen magnum.
MRI is the imaging modality of choice. On sagittal imaging, the best plane for assessing for the presence of Chiari I malformations, the tonsils are pointed, rather than rounded and referred to as peg-like. The sulci are vertically oriented, forming so-called sergeant stripes. Axial images through the foramen show crowding of the medulla by the tonsils.
The degree of tonsillar descent is determined by first drawing a line from the basion to the opisthion (anterior margin to the posterior margin of the foramen magnum); this defines the plane of the foramen magnum.
A measurement is then drawn perpendicular to this plan between it and the tip of the cerebellar tonsils, either in the midsagittal image or an adjacent parasagittal image, wherever the tonsils are most low lying 10,11.
See cerebellar tonsillar position article for more details.
Spinal cord MRI should be recommended as a syrinx may be seen if progressively enlarging or associated with symptoms may be an indication for surgical intervention 5.
CSF flow studies may also be useful to assess the flow surrounding the cervicomedullary junction.
Importantly, features of intracranial hypertension and craniospinal hypotension should be sought to ensure that cerebellar tonsillar ectopia is not secondary to abnormal intracranial pressure (and therefore not a true Chiari I malformation) 7,8.
Treatment and prognosis
Chiari I malformations can be divided into three stages (although not frequently used in day-to-day practice):
- I: asymptomatic
- II: brainstem compression
- III: syrinx
Treatment is usually reserved only for symptomatic patients or those with a syrinx. It consists of decompressing the posterior fossa, by removing part of the occipital bone, and posterior arch of C1 as well as performing a duroplasty.
History and etymology
It was first described in 1891 by Hans Chiari (1851-1916), an Austrian pathologist, based on the autopsies of children.
Imaging differential considerations include:
- incidental tonsillar ectopia: <5 mm
- Chiari 1.5 malformation (sometimes considered a variant of Chiari I malformation 4)
- Chiari II malformation
- acquired tonsillar ectopia
Distinguishing Chiari I malformation for pseudotumor cerebri is particularly important as treatment with a posterior fossa decompression can result in poor outcomes 5. Examination for features of intracranial hypertension is therefore crucial.
- 1. Ketonen L, Hiwatashi A, Sidhu R. Pediatric brain and spine, an atlas of MRI and spectroscopy. Springer Verlag. (2005) ISBN:3540213406. Read it at Google Books - Find it at Amazon
- 2. Elster AD, Chen MY. Chiari I malformations: clinical and radiologic reappraisal. Radiology. 1992;183 (2): 347-53. Radiology (abstract) - Pubmed citation
- 3. Kornienko VN, Pronin IN. Diagnostic Neuroradiology. Springer Verlag. (2008) ISBN:3540756523. Read it at Google Books - Find it at Amazon
- 4. Chiapparini L, Saletti V, Solero CL et-al. Neuroradiological diagnosis of Chiari malformations. Neurol. Sci. 2011;32 Suppl 3 (S3): S283-6. doi:10.1007/s10072-011-0695-0 - Pubmed citation
- 5. Jennifer Strahle, Karin M. Muraszko, Joseph Kapurch, J. Rajiv Bapuraj, Hugh J. L. Garton, Cormac O. Maher. Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging: Clinical article. (2011) Journal of Neurosurgery: Pediatrics. 8 (2): 205. doi:10.3171/2011.5.PEDS1121
- 6. Alnemari A, Mansour TR, Gregory S, et al. Chiari I malformation with underlying pseudotumor cerebri: Poor symptom relief following posterior decompression surgery. (2017) International journal of surgery case reports. 38: 136-141. doi:10.1016/j.ijscr.2017.07.039 - Pubmed
- 7. Aiken AH, Hoots JA, Saindane AM et-al. Incidence of cerebellar tonsillar ectopia in idiopathic intracranial hypertension: a mimic of the Chiari I malformation. AJNR Am J Neuroradiol. 2012;33 (10): 1901-6. doi:10.3174/ajnr.A3068 - Pubmed citation
- 8. Barkovich AJ, Wippold FJ, Sherman JL et-al. Significance of cerebellar tonsillar position on MR. (1986) American Journal of Neuroradiology. 7 (5): 795. Pubmed
- 9. D’Arco, Felice, Ganau, Mario. Which neuroimaging techniques are really needed in Chiari I? A short guide for radiologists and clinicians. (2019) Child's Nervous System. 35 (10): 1801. doi:10.1007/s00381-019-04210-3
- 10. Meadows J, Kraut M, Guarnieri M, Haroun R, Carson B. Asymptomatic Chiari Type I Malformations Identified on Magnetic Resonance Imaging. J Neurosurg. 2000;92(6):920-6. doi:10.3171/jns.2000.92.6.0920 - Pubmed
- 11. Smith B, Strahle J, Bapuraj J, Muraszko K, Garton H, Maher C. Distribution of Cerebellar Tonsil Position: Implications for Understanding Chiari Malformation. JNS. 2013;119(3):812-9. doi:10.3171/2013.5.jns121825 - Pubmed