OPHTHALMOLOGIC FINDINGS IN FIFTEEN PATIENTS
WITH WOLFRAM SYNDROME
Dr. Maha Al-Till, (FRCS-Ophth.)
Jordan University Hospital
Dr. Nadim S. Jarrah, (MD)
Dr. Kamel M. Ajlouni, (MD)
National Center for Diabetes, Endocrinology and Genetics, Amman, Jordan
Purpose: To look for the ophthalmologic abnormalities in 15 patients with Wolfram syndrome, also known as DIDMOAD syndrome (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy & Deafness).
Method: Fifteen patients from four inbred families diagnosed to have Wolfram syndrome at the National Center for Diabetes, Endocrinology and Genetics, in Amman, Jordan were evaluated ophthalmologically. Their examination included best-corrected visual acuity, color vision testing, pupillary light reflexes, slitlamp biomicroscopy and fundus examination. Fundus fluorescein angiography was also performed to all patients.
Results: The prevalence of optic atrophy was (93.3%), color defect (92.9%) cataract (66.6%), pigmentary retinopathy (30%) and diabetic retinopathy (20%). Abnormal pupillary light reflexes and nystagmus were also reported.
Conclusion: Although our group of patients are genetically heterogeneous, the ophthalmic findings are consistent with those reported in other series except for cataract which was highly prevalent but mild & did not contribute significantly to decrease vision.
Keywords: Cataract, color defect, deafness, diabetes insipidus, diabetes mellitus, diabetic retinopathy, nystagmus, optic atrophy, pig retinopathy, Wolfram syndrome.