Adult-onset aqueductal stenosis: A case report

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Abstract

Purpose

The authors describe a case of an adult-onset aqueduct stenosis.

Methods

A 37-year-old man presented with a progressive blurring of vision in his left eye for a few days. He reported a fever episode two weeks before. Visual acuity was 10/10 P2 OD and 7/10 P2 OS. Pupils were equal without afferent defect. Neurologic examination was normal. Fundoscopic examination revealed a left optic disc oedema and a mild swelling of the right disc margins. Visual field examination demonstrated a bilateral enlargement of the blind spot associated with an infero-nasal defect on the left eye. Computed tomography showed enlargement of the third and lateral ventricles suggestive of aqueduct stenosis, which was confirmed by MRI. Ultimately we learned that the patient's mother presented an aqueduct stenosis too. Otherwise the febrile episode was linked to infectious mononucleosis attested by serology. Following endoscopic third ventriculostomy, there was an visual acuity improvement to 10/10 P2 OD and 9/10 P2 OS. Visual field was normal on the right eye, but infero-nasal defect remained on the left eye.

Results

Hydrocephalus secondary to aqueduct stenosis may present at any age, however it is more frequent in childhood. Stenosis can be attributed to different causes. In three quarters of cases, etiology remains unknown. Clinical course may vary according to the age of the patient. In our case age of onset is atypical, as symptomatology, consisting of isolated visual disturbances. The unilateral infero-nasal visual field defect turns out to be also uncommon.

Conclusion

In view of bilateral optic disc oedema, clinicians have to hint at the possibility of hydrocephalus secondary to aqueduct stenosis, even if clinical manifestations are not much suggestive.

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