Diastematomyelia

Case contributed by Townsville radiology training

Presentation

Complex cervical, thoracic, lumbar lipomeningocele. Arnold Chiari malformation, VP shunt. For pre-operative planning.

Patient Data

Age: 4 years
Gender: Female

T1 FLAIR demonstrating small posterior fossa with tonsillar descent of 3.5mm.

Narrow cerebral aqueduct with enlarged suprapineal recess of the 3rd ventricle, quadrigeminal cystern, suprasellar and pontine cysterns. T2w coronal demonstrate cavum septum pellucidum.

T2 weighted axial demonstrating diastematomyelia.

T2 weighted  sagital showing syringohydromyelia and tethering of the cord at L1/2.. Syrinx C4-T7.

Loss of normal cervical and lumbar lordosis, and focal lumbar kyphosis. No scoliosis.

Case Discussion

Presence of tethered spinal cord indicates that this is Arnold Chiari malformation type 2.

Septum and separate dural sacs around the split cord indicate that this is diastematomyelia type 1. Many of the associated features of this condition is also seen.

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