Chiari II malformation

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Upper thoracic swelling.

Patient Data

Age: 1 year
Gender: Male
mri

The MRI sequences demonstrate:

  • small posterior fossa with low lying cerebellar tonsils which are extended up to C7 level as well as descent of the medulla oblongata with effacement of the 4th ventricle, elongated corpus callosum and supratentorial hydrocephalus

  • midline cystic mass with no skin cover at the upper dorsal region (T1-T2 level), communicating with the spinal canal through a spinal dysraphism (spina bifida aperta). The adjacent segment of the spinal cord is angulated towards the cystic mass, indicating myelomeningocele

  • central cystic cavity of the spinal cord of low signal on T1, and high signal on T2 and STIR, extending from the T3-T4 level up to the conus medullaris in keeping with a syrinx.

Case Discussion

MRI features of Chiari II malformation with associated syrinx of the spinal cord.

MRI is the modality of choice for detecting and characterizing the full constellation of findings associated with Chiari II malformations.

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