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Congenital aqueduct stenosis producing the "sunset eye" sign

Case contributed by: Dr Chris O'Donnell

Presentation:

PH Spina bifida. Large head with frontal bossing and downturned eyes

Patient Data:

Age: 19 months
Gender: Male
Race: Caucasian

Case Discussion:

Typical aqueduct stenosis characterized by a septum or diaphragm obstructing the lumen with gross lateral and 3rd ventricular dilatation.  Enlarged head with frontal bossing (i.e. enlarged supratentorium and small posterior fossa) and downturning of the globes characterized by the lenses pointing down rather than forward.

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