Journal of Neuro-Ophthalmology

Skip Navigation LinksHome > March 1994 - Volume 14 - Issue 1 > Therapy-Resistant Papilledema in Achondroplasia.
Journal of Neuro-Ophthalmology:
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Therapy-Resistant Papilledema in Achondroplasia.

Landau, Klara m.d.; Gloor, Balder P. m.d.

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A 20-year-old achondroplastic dwarf developed severe papilledema over a short period of time. Since the age of 3 he had a ventriculoperitoneal shunt for presumed hydrocephalus. Severe ventriculomegaly was never documented, neither in the past, nor at presentation. Intracranial pressure was high, and the shunt was obliterated. Despite prompt shunt revision and subsequent bilateral optic nerve sheath decompression the patient developed postpapilledema optic atrophy with bilateral blindness. The pathophysiology of increased intracranial pressure in achondroplasia is discussed.

(C) Williams & Wilkins 1994. All Rights Reserved.


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