Slit ventricle syndrome

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Known for neurofibromatosis type 1, presented with a history of chronic intermittent headaches with vomiting. Had a VP shunt insertion 3 months prior for obstructive hydrocephalus.

Patient Data

Age: 8 years
Gender: Male
mri

Abnormal hyperintensities on FLAIR and T2 involving mainly the cerebellar hemispheres, left cerebral peduncle, globi pallidi and left thalamus with no contrast enhancement in keeping with FASI as well as hypothalamic mass, most likely glioma (known patient for neurofibromatosis type 1).

Right-sided VP shunt with the tip in the projection of the trigone of the lateral ventricle.

Collapsed overdrained right lateral ventricle with dilated underdrainage of the contralateral ventricle in keeping with Slit ventricle syndrome.

Case Discussion

MRI features of a slit ventricle syndrome in a child known for NF1 and who had a VP shunt for an obstructive hydrocephalus due to a hypothalamic glioma.

One of the aetiopathogenic explanation of the slit ventricle syndrome is an intermittent ventricular isolation, in which one overdrained ventricle collapses and occludes the shunt catheter resulting in under-drainage of the contralateral ventricle (as in this case).

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