Chiari II malformation with obstructive hydrocephalus and spinal dysraphism

Chiari II malformation with obstructive hydrocephalus and spinal dysraphism

Abnormal antenatal obstetric US with hydrocephalus underwent cesarean section. At birth, the patient presented with lower back swelling.

Evidence of supratentorial hydrocephalus with a markedly narrowed fourth ventricle. Patent aqueduct of Sylvius with flow void.

Small-sized posterior fossa with low insertion of the tentorium, low torcula, and abnormal course of the straight sinus.

Evidence of downward herniation of cerebellum and brain stembrainstem through the foramen magnum. A tectal peaking sign peaking sign of inferior colliculus is seen on the sagittal view as well as a cerebellar wrapping wrapping sign around the brain stem.

Thinning of the corpus callosum along with retrograde CSF permeation are noted.

An obstructive CSF flow curve (Zigzag(zigzag appearance) with very slow flow through the aqueduct of Sylvius.

MRI Spinal cord 6 days later

Sagittal and axial MRI spinal cord revealed:

Small-sized posterior fossa and downward herniation of cerebellar tonsils and brain stembrainstem through the foramen magnum.

Large spina bifida in the lumbar region with myelomeningocele and tethered cord (Spinal (spinal dysraphism).

Two levels of syringohydromyelia in cervical and lumbar regions.

Chiari II malformation with obstructive hydrocephalus