同一家族7名成员双侧后圆锥晶状体的诊断与处理
Diagnosis and management of bilateral posterior lenticonus in 7 members of the same family.
作者信息
Schipper Isaak, Senn Peter, Schmid Martin
机构信息
Eye Clinic, Cantonal Hospital, Lucerne, Switzerland.
出版信息
J Cataract Refract Surg. 2006 Feb;32(2):261-3. doi: 10.1016/j.jcrs.2005.07.046.
PURPOSE
To report on the occurrence of monosymptomatic bilateral posterior lenticonus in 7 members of the same family and discuss the diagnoses and methods of management.
SETTING
Eye Clinic, Cantonal Hospital, Lucerne, Switzerland.
METHODS
Two sisters were found to suffer from bilateral posterior lenticonus. The 3 sons of the elder sister and the 2 daughters of the younger sister all presented with bilateral posterior lenticonus. A cataract operation or a clear lens extraction with intraocular lens implantation was performed in 4 of the 7 patients.
RESULTS
The disorder was found to be inherited and to follow a pattern of autosomal dominance. Visual acuity improved to 20/20 in all operated eyes.
CONCLUSION
This may be the largest group in 1 family presenting with bilateral posterior lenticonus described to date. Treatment of posterior lenticonus, if necessary, can be successfully managed as an ordinary cataract.
目的
报告同一家族7名成员中出现的单症状双侧晶状体后圆锥，并讨论其诊断和处理方法。
地点
瑞士卢塞恩州立医院眼科诊所。
方法
发现两名姐妹患有双侧晶状体后圆锥。姐姐的3个儿子和妹妹的2个女儿均表现为双侧晶状体后圆锥。7名患者中有4名接受了白内障手术或透明晶状体摘除联合人工晶状体植入术。
结果
该疾病被发现具有遗传性，遵循常染色体显性遗传模式。所有手术眼的视力均提高到20/20。
结论
这可能是迄今为止报道的同一家族中出现双侧晶状体后圆锥的最大群体。如有必要，晶状体后圆锥的治疗可作为普通白内障成功进行。
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