Iris Manifestations in Inadequately Treated Chronic Recurrent Vogt-Koyanagi-Harada Disease

ABSTRACT

Purpose

To describe the iris changes in chronic recurrent Vogt-Koyanagi-Harada Disease (VKH)

Methods

Retrospective case series. Clinical features of 3 VKH patients who developed bilateral depigmentation of the iris are described.

Results

Patients had delayed diagnosis and inadequately treated chronic recurrent VKH. Patient 1 presented with bilateral multiple contiguous small granulomatous iris stromal lesions, severe diffuse iris swelling more severe near the iris root, producing peripheral iris undulations. Diffuse iris atrophy and peripheral depigmentation subsequently developed. Patient 2 presented with peripheral iris depigmentation, atrophy of the ciliary zone iris stroma and sparing of the sphincter pupillae, giving a ‘sunflower appearance.’ Patient 3 had extensive iris depigmentation, atrophy, and transillumination defects involving the entire iris.

Conclusion

Uncontrolled bilateral diffuse granulomatous stromal iris inflammation leads to subsequent depigmentation, transillumination defects and atrophy, beginning in the peripheral iris. Sphincter pupillae is initially spared but complete iris atrophy may develop.

KEYWORDS:

• Vogt-Koyanagi-Harada disease
• iris depigmentation
• iris transillumination defect
• iris atrophy

Acknowledgments

Personal Fees: income from honoraria, consulting, advising, lectures, speakers’ bureaus, and grant support.

The author Soon-Phaik Chee has financial relationship with

1. AbbVie Pte. Ltd, Singapore: Consultant/Advisor, Grant Support

2. Alcon Laboratories, Inc. Singapore and USA: Consultant/Advisor, Lecture Fees, Grant Support

3. Allergan, Singapore: Grant Support

4. Bausch & Lomb Surgical, Singapore: Consultant/Advisor, Grant Support

5. Carl Zeiss Inc, Singapore: Consultant/Advisor, Lecture Fees, Grant Support

6. Gilead Sciences, Inc., USA: Grant Support

7. HOYA Medical Singapore Pte. Ltd: Consultant/Advisor, Grant Support

8. Johnson & Johnson Vision, Singapore and USA: Consultant/Advisor, Lecture Fees, Grant Support

9. Leica Microsystems Inc. Singapore: Consultant/Advisor, Grant Support

10. Santen Pharmaceutical Asia Pte. Ltd, Singapore: Lecture Fees, Grant Support

11. Ziemer Ophthalmics AG, Switzerland: Consultant/Advisor, Grant Support

May Zun Aung Win has no financial interests to declare.

1. Other Acknowledgments such as Statisticians, Medical Writers

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.