The ophthalmic diagnosis and management of four siblings with Werner syndrome.
INTRODUCTION
Werner syndrome is a rare autosomal recessive disorder caused by mutations in the Werner syndrome WRN gene, on chromosome 8. Those affected manifest early the features of ageing.


DISCUSSION
Cataract surgery is prone to post-operative complications in those with Werner syndrome. The development of cystoid macular oedema (CMO) is likely multifactorial. Patients with WS have diabetes mellitus type 2 which can contribute to macular oedema. There is a deposition of abnormal WRN proteins in the macula which also predisposes to macular oedema. The trauma of cataract surgery appears to be the main stimulus for the development of CMO. CMO may, as a result, be difficult to manage in Werner syndrome patients.


CONCLUSION
Further study is needed to elucidate the precise role of retinal WRN protein expression in the development of CMO in those with Werner syndrome. A tailored and more successful approach to the treatment of CMO in such patients may result.
['Lyons|C|C|', 'Gallagher|D|D|', 'McSwiney|T|T|', 'McElnea|E|E|', 'Kinsella|F|F|']
D000328:Adult D002387:Cataract Extraction D003941:Diagnostic Techniques, Ophthalmological D005260:Female D006801:Humans D008269:Macular Edema D008297:Male D035781:Siblings D016896:Treatment Outcome D014898:Werner Syndrome D000071657:Werner Syndrome Helicase
Werner syndrome Cataract Cystoid macular oedema