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KMID : 1813520210100020071
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Journal of the Korean Glaucoma Society
2021 Volume.10 No. 2 p.71 ~ p.77
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Clinically Diagnosed Wolfram Syndrome with Neovascular Glaucoma
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Chey Ki-Hyoung
Lee Chang-Kyu
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Abstract
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Purpose : We report a case of clinically diagnosed Wolfram syndrome with neovascular glaucoma.
Case summary : A 37-year-old female with a history of type 1 diabetic mellitus was referred to the hospital complaining of decreased vision in both eyes. Fundus examinations showed bilateral optic atrophy and diabetic retinopathy, and the patient was clinically diagnosed with Wolfram syndrome considering the presence of hearing loss and short stature. Two months later, neovascular glaucoma developed in the right eye, and the patient underwent trabeculectomy after intracameral and intravitreal injection of bevacizumab. After 6 months, she underwent Ahmed valve implantation due to poor intraocular pressure (IOP) control. Two years later, tractional retinal detachment occurred after vitreous hemorrhage in the right eye, and her visual acuity decreased to light perception. Neovascular glaucoma also developed in her left eye, and IOP was stabilized after Ahmed valve implantation, with her best-corrected visual acuity remained at 20/60.
Conclusions : Wolfram syndrome is a rare progressive multisystem spectrum disorder with autosomal recessive inheritance. Therefore, it is imperative to suspect Wolfram syndrome if a young patient with type 1 diabetes mellitus and optic nerve atrophy is accompanied by hearing loss or diabetes insipidus. Severe ocular complications such as neovascular glaucoma are less common in Wolfram syndrome but may occur, as in this case.
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KEYWORD
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Neovascular glaucoma, Wolfram syndrome
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