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C.W. Vaughn, J.C. Affeldt; Neurotrophic Keratitis Associated with Goldenhar's Syndrome . Invest. Ophthalmol. Vis. Sci. 2004;45(13):104.
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Purpose:To describe for the first time the association of neurotrophic keratitis with Goldenhar's syndrome. Methods:Observational case report. Results:A 7–year–old Samoan female presented to the eye clinic for complaints of recurrent "pink eye" OD associated with corneal opacification. She carried the diagnosis of Goldenhar's syndrome as demonstrated by auricular appendices, vertebral abnormalities with hemifacial microsomia. Genetic workup revealed a normal chromosome study including 46, XX, and FISH. She additionally suffered from bilateral deafness. Ocular exam revealed a visual acuity of 20/60 OD and 20/20 OS, and an active Stage II neurotrophic ulcer of the right eye manifested by an inferocentral horizontally oval epithelial defect surrounded by heavy punctate keratopathy. Slit–lamp exam of the left eye was unremarkable. Corneal sensation was absent OD and normal OS. Basal tear secretion by Schirmer method was 3 mm OD and 11 mm OS. Treatment with a silicone plug to the right lower lid and copius lubrication resulted in ulcer resolution with residual 1+ punctate keratopathy. Conclusions:Goldenhar's syndrome can be associated with neurotrophic keratitis manifested by apparent chronic recurrent conjunctivitis, corneal scarring, and ultimately permanent visual loss. Once properly identified, however, the condition can be easily controlled with lubrication and punctal occlusive therapy.
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