Golnik K C, Kersten R C
Department of Ophthalmology, University of Cincinnati, Ohio, USA.
J Neuroophthalmol. 1998 Mar;18(1):60-5.
The Hadju-Cheney syndrome is one of the idiopathic acroosteolyses. Associated neurologic abnormalities are often a result of progressive basilar invagination. A 48-year-old man with the Hadju-Cheney syndrome developed progressive bilateral visual loss. On examination, he had hyperopia, choroidal folds, optic nerve head swelling, and mild optic neuropathy. Computed tomographic scans showed massive enlargement of both intraorbital optic nerve sheaths. Improvement occurred after optic nerve sheath fenestration. Visual loss due to optic nerve meningocele can occur in the Hadju-Cheney syndrome. Optic nerve sheath fenestration can result in visual improvement. It is unclear whether the occurrence of optic nerve meningocele is causally or fortuitously related to the Hadju-Cheney syndrome.
哈朱-切尼综合征是特发性肢端骨质溶解症之一。相关的神经异常往往是进行性基底凹陷的结果。一名患有哈朱-切尼综合征的48岁男性出现了进行性双侧视力丧失。检查发现，他有远视、脉络膜皱褶、视神经乳头肿胀和轻度视神经病变。计算机断层扫描显示双侧眶内视神经鞘大量增粗。视神经鞘开窗术后病情有所改善。哈朱-切尼综合征可因视神经脊膜膨出导致视力丧失。视神经鞘开窗术可使视力改善。目前尚不清楚视神经脊膜膨出的发生与哈朱-切尼综合征是因果关系还是偶然相关。
