First Author: M.Mohebbi IRAN
Co Author(s): H. Hashemi H. Behestnejad
Purpose:
To present causative factors in the pathogenesis of cornea/lesions in Cockayne syndrome (CS).
Setting:
Eye Research Center, Farabi Eye Hospital, Tehran University of Medical Sciences.
Methods:
We describe patient complaints and biomicroscopic findings of a 10 - year -old girl that presented to ophthalmic clinic for prolonged period of red eye & photophobia of both eyes.
Results:
The appearance was that of a short- statured girl with deeply sunken eyes, dry skin with hyperpigmentation over sun- exposed areas and subcutaneous fat atrophy. Weakened Bell's phenomenon, decreased rate of blinking and defective eyelid closure was also noticed. Schirmer's test-l revealed decreased tear secretion. The patient satisfied the clinical criteria for classic CS, an autosomal recessive DNA repair deficiency disorder, with growth and neuro-developmental abnormalities and progressive impairment of vision and hearing. Our patient was treated with frequent lubricating agents. After six-weeks despite intensive medications, corneal lesions did not heal completely; thus lateral tarsorrhaphy was performed
Conclusions:
The main factor responsible for involvement of corneal lesion is exposure, secondary to lack of Bell's phenomenon and blinking. Attention to and accurate management of any corneal changes in CS is imperative. FINANCIAL DISCLOSURE?: No
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