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Abstract #53582 Published in IGR 15-2
Secondary glaucoma with copper deposition in trabecular meshwork in Wilson disease
Li X; Ye T; Li Y; Zhang XClinical and Experimental Ophthalmology 2014; 42: 204-205
An 18-year-old boy experienced fatigue and developed jaundice of unknown aetiology five years ago, and he was diagnosed with and treated for haemolytic anaemia. In December 2011, he visited an ophthalmologist for blurred vision in the right eye. Visual acuity (VA) of the right and left eyes was 6/12 and 6/6 respectively, and the intraocular pressure (IOP) of each was 53 and 17 mmHg respectively. Prominent Kayser-Fleischer (KF) rings were detected in both eyes. Laboratory tests revealed normal serum copper (362.4 μg/L), low serum ceruloplasmin (56 mg/L), and very high 24-hour urinary copper excretion (1765.6 μg). Magnetic resonance imaging of the brain showed high signal intensity changes in the pons and the left thalamus. The diagnosis of Wilson disease was confirmed via a positive genetic test result for mutation in the ATP7B gene. The patient was placed on a low copper diet and administered D-penicillamine, hepatoprotective drugs, and antiglaucomatous medications. However, he complained that his vision was substantially reduced in his right eye. When the patient visited us, the VA of the right eye was hand motion and that of the left was 6/6 with correction. The IOP was 44 mmHg in the right eye and 15 mmHg in the left eye. His corneas were transparent, and KF rings were clearly evident in both. Both eyes exhibited a golden-brown ring located in the peripheral cornea and the anterior lens surfaces (see Figure 1 A, B). The optic nerve head was pale and the cup-to-disc (CD) ratio was almost 0.8 in the right eye. The optic nerve head was normal and the CD ratio was 0.4 in the left eye. Gonioscopy revealed that the anterior chamber angles of both eyes were wide and open; however, a thick layer of yellow-grey material covered the trabecular meshwork in the right eye (Figure 1 C), which was not evident in the left eye.
Zhongshan Ophthalmic Center, State Key Laboratory of Ophthalmology, Sun Yat-sen University, Guangzhou, 510060, PR China.
Full articleClassification:
9.4.15 Glaucoma in relation to systemic disease (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
2.5.1 Trabecular meshwork (Part of: 2 Anatomical structures in glaucoma > 2.5 Meshwork)
3.7 Biochemistry (Part of: 3 Laboratory methods)