Landau K, Gloor B P
Department of Ophthalmology, University Hospital Zurich, Switzerland.
J Neuroophthalmol. 1994 Mar;14(1):24-8.
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.
一名20岁的软骨发育不全侏儒在短时间内出现了严重的视乳头水肿。自3岁起，他因疑似脑积水接受了脑室腹腔分流术。既往及就诊时均未记录到严重的脑室扩大。颅内压升高，分流管堵塞。尽管迅速进行了分流管修复及随后的双侧视神经鞘减压术，但患者仍出现了视乳头水肿后视神经萎缩并导致双眼失明。本文讨论了软骨发育不全患者颅内压升高的病理生理学。
