Syringomesencephaly

Syringomesencephaly is a very rare entity and refers to a syrinx that extends into the midbrain 1.

Patients with this condition demonstrate a wide variety of neurological symptoms localized to the brainstem and spinal cord, depending on where exactly the syrinx is located 1.

When present, it is seen in continuity with a long syrinx that demonstrates syringomyeliasyringobulbia and syringopontia 1. As with syringopontia, the syrinx may or may not have a communication with the fourth ventricle 1. In rare cases when it is present without syringomyelia, the term 'keyhole aqueduct syndrome' has been employed by some authors 2.

There are numerous causes and associations, and these are discussed in more depth in the general article on syrinx

Syringomesencephaly has the same radiographic characteristics on all imaging modalities as any other syrinx 1. See syrinx for an in-depth discussion of these characteristics.

When symptomatic, neurosurgical intervention may be required 1.

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rID: 56660
Tag: cases
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