Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and histopathological correlation after successful clear lens extraction
Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and histopathological correlation after successful clear lens extraction
复制标题
阿尔波特综合征病例中的双侧前晶状体：成功透明晶状体摘除后的临床和组织病理学相关性
DOI：
10.1136/bcr-2013-202036
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发表时间：
2014
期刊：
影响因子：
0.9
通讯作者：
K. Mulay
中科院分区：
文献类型：
--
作者：
Shilpa Sonarkhan;Muralidhar Ramappa;S. Chaurasia;K. Mulay
一位29岁的女性，在过去的8-10年里，出现了双眼视力逐渐、进行性、无痛性下降的症状。经检查，未矫正视力右眼20/60，左眼20/126。晶状体前段检查基本正常，除了晶状体前囊瞳孔轴有锥形突出，晶状体下缘清晰。术中，使用小儿撕囊钳对中央突出的易碎晶状体囊进行精心处理，形成中心良好的连续曲线撕囊。组织病理学显示晶状体囊明显变薄，上皮正常。前晶状体囊电镜显示多个线状和不规则的破裂区。其中很少有纤维状、不规则的电子致密物质和液泡；邻近细胞不规则，提示Alport综合征。术后过程平稳，视力在无辅助的情况下提高到20/20。摘出透明晶状体是恢复视觉症状性Alport综合征前晶状体的有效手段。
A 29-year-old woman presented with symptoms of gradual, progressive, painless diminution of vision in both eyes for past 8–10 years. On examination, uncorrected visual acuity was 20/60 in the right eye and 20/126 in the left eye. Anterior segment examination was essentially normal except for a conical protrusion in the pupillary axis of the anterior lens capsule with a clear underlying lens. During surgery, central protruded fragile lens capsule was meticulously handled to create well-centered continuous curvilinear capsulorhexis by using paediatric rhexis forceps. Histopathology of excised capsule showed markedly thinned lens capsule with normal epithelium. Electron microscopy of the anterior lens capsule showed multiple linear and irregular zones of dehiscence. Few of these had fibrillar, irregular electron-dense material and vacuoles; and adjacent cells were irregular suggestive of Alport syndrome. Postoperative course was uneventful and visual acuity improved to 20/20 unaided. Clear lens extraction is an effective means of rehabilitating visually symptomatic anterior lenticonus of Alport syndrome.