THERAPY-RESISTANT PAPILLEDEMA IN ACHONDROPLASIA

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