Wolfram Syndrome with Advanced Proliferative Diabetic Retinopathy: A Case Report and Review

Wolfram syndrome or DIDMOAD is a rare autosomal recessive neurological disorder characterized by clinical symptoms of diabetes insipidus, diabetes mellitus, hearing loss, and optic nerve atrophy. Due to the rarity of the disease, the rare prevalence of this disease, and the importance of identifying and patient recognition, this study aimed at introducing a patient with Wolfram syndrome, who was a candidate for vitrectomy with the diagnosis of retinal tachycardia.

A 19-year-old girl with Wolfram with symptoms of diabetes mellitus, optic nerve and hearing atrophy, advanced Proliferative Diabetic Retinopathy, and Tractional Retinal Detachment was referred to Imam Khomeini Hospital for Vitrectomy.

Since optic nerve atrophy in the reported patient appears earlier than other symptoms, the referral of young diabetic patients for ophthalmologic examinations to early diagnosis is suggested. According to the results of this study, children with parents who have a family relationship and a family history of diabetes are more likely to develop Wolfram syndrome. So, it is necessary to pay attention to this issue in premarital counseling. Also, it is recommended that authorities make appropriate arrangements for screening susceptible patients for this syndrome.