The Chiari Malformation & What is Chiari and Chiari 0?

What is Chiari?

Chiari (kee-AR-ee) malformation (CM) is a serious neurological disorder where the bottom part of the brain, the cerebellum, descends out of the skull and crowds the spinal cord, putting pressure on both the brain and spine and causing many symptoms. There are three main types of Chiari: CM1 the simplest and most prevalent form, is generally considered to be a congenital malformation, although acquired cases are recognized. It is rarely apparent at birth. CM2 and CM3 are more severe congenital malformations that are apparent at birth and associated with complex defects of the brain and spinal cord.

CHIARI ), csfinfo.org

The Chiari 0 Malformation

The four traditional varieties of Chiari malformations represent varying degrees of involvement of rhombencephalic (hindbrain) derivatives. Three of these (Types I-III) have progressively more severe herniation of hindbrain structures outside of the posterior cranial fossa as a common feature. Many times, syringomyelia is found as a result of these pathologies. In 1998, a group led by Dr. Jerry Oakes1 identified five patients with syringomyelia and no evidence of tonsillar herniation (i.e. no Chiari I malformation). MR imaging of the entire neuraxis ruled out other causes of a syrinx (e.g. tumor, post-traumatic). Ultimately, abnormal CSF flow at the posterior fossa or foramen magnum was identified as the suspected cause. The label “Chiari 0” was used to categorize these patients by virtue of the fact that they behave as though the afflicted patients have fourth ventricular outlet obstruction – and at surgery, frequently do have physical barriers to CSF movement but do not have caudal displacement of the cerebellar tonsils beyond a point that could be considered pathological. All underwent a posterior fossa decompression and duraplasty identical to the technique used to treat patients with a Chiari I malformation. No attempt was made to directly treat the syrinx. Significant syrinx and symptom resolution was observed in all patients. Interestingly, two patients who did not respond to previous shunting of their syrinx experienced long-term improvement only after the posterior fossa decompression. Their response to surgery suggests “Chiari-like” pathophysiology is present in the absence of tonsillar herniation. This contention is supported by the identification of a crowded foramen magnum in two patients, multiple arachnoid adhesions in two others and a 4th ventricular arachnoid veil in one. Each of these findings can detrimentally alter CSF flow. In this study, cine-MRI failed to accurately identify patients most likely to benefit from surgery.

Morphological studies of the posterior fossa in the Chiari 0 group have found the sagittal anteroposterior diameter of the foramen magnum to be greater than controls and inferior descent of the obex (i.e. caudal displacement of the brainstem) was found to be three standard deviations below normal.2 These findings suggest caudal displacement of the medulla and compensatory enlargement of the foramen magnum may result from a small posterior fossa.

More recently, Kyoshima et al.3 have reported four patients with what they termed a “tight cistern magna” and syringomyelia. These patients did not have hindbrain hernias and each underwent posterior fossa decompression with improvement of the syrinx in three. We would consider these patients as examples of the Chiari 0 malformation.

It is emphasized that this subgroup represents a very small cohort found within the Chiari malformations. In addition, the original authors considered the diagnosis of Chiari 0 only in patients with syringomyelia of no apparent cause. Careful patient selection is critical when making the diagnosis of Chiari 0 malformation. Without an obvious Chiari I malformation, 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 with a posterior fossa decompression.

REFERENCES

Iskandar BI, Hedlund GL, Grabb PA, Oakes WJ. The resolution of syringomyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg 1998;89: 212–216.
Kyoshima K, Kuroyanagi T, Oya F, Kamijo Y, El-Noamany H, Kobayashi S. Syringomyelia without hindbrain herniation: tight cistern magna. Report of four cases and a review of the literature. J Neurosurg 2002;96:239-249.
Tubbs RS, Elton S, Grabb P, et al. Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery 2001;48:1050–1055.

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