Hydrancephaly within the vertebrobasilar territory

Case contributed by Mohammad A. ElBeialy
Diagnosis certain

Presentation

8 years old boy with delayed developmental milestones.

Patient Data

Age: 8 years.
Gender: Male
  • Almost total destruction and liquefaction of both cerebellar hemispheres (relatively sparing the superior cerebellar vermis) as well as the middle cerebellar peduncles and relative atrophy of the posterior portion of the pons and midbrain.
  • Partial agenesis of the posterior body and splenium of the corpus callosum with thinned its anterior portion.
  • Associated colpocephalic configuration of the lateral ventricles with mild dilatation of the occipital horns,trigones and posterior bodies.
  • No mass lesion or definite supratentorial parenchymal area of abnormal signal intensity.
  • Widened extra-axial CSF spaces with a small left temporal arachnoid cyst.
  • Normal sellar region.
  • Right otomastoiditis with T2 prolongation.

Case Discussion

The above described findings are those of hydrancephaly of the vertebro-basilar territory with almost total destruction and liquefaction of the cerebellar hemispheres mostly 2ry to in-utero vascular insult. Partial agenesis of the corpus callosum with colpocephaly of the lateral ventricles.

 

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