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.