Wolfram syndrome with childhood glaucoma: A rare case report with review of literature

Abstract

Wolfram syndrome (WFS) is a rare neurodegenerative disorder characterized by young-onset diabetes mellitus, central diabetes insipidus, optic nerve atrophy, and hearing loss. The ophthalmic association of this order has been limited to optic nerve atrophy. We report here a case of WFS and its disease course with childhood glaucoma as a rare ophthalmic manifestation which has been reported just once earlier. The patient had young-onset diabetes mellitus (insulin dependent), diabetes insipidus, and optic-disc pallor suggestive of partial optic atrophy. However, hearing assessment was normal at the time of presentation and in due course of disease. Glaucoma is one of the rare possible ocular manifestations of WFS which should be highly considered while evaluating patient with this disorder.

Keywords

• diabetes insipidus
• diabetes mellitus
• didmoad syndrome
• glaucoma
• optic atrophy
• wolfram syndrome