Focal laser and intravitreal bevacizumab for choroidal neovascularization secondary to Grönblad–Strandberg syndrome

Abstract

Introduction: Pseudoxanthoma elasticum is a rare genetic disorder. It has several ocular features including angioid streaks and choroidal neovascularization which can significantly affect vision. Case Report: A 50-year-old man presented with nine months history of poor vision affecting both distant and near vision. His best corrected visual acuities were 6/18 right eye and 1/60 left eye. His anterior segments were normal. Funduscopy revealed linear hyperpigmented irregular branching peripapillary lesions; angioid streaks both eyes. He had bilateral speckled retina pigment epithelial hyperpigmented lesions interspaced with dot hemorrhages in the macula region suggestive of likely choroidal neovascularization. He had “peau d’orange” skin appearance around his neck. Fundus fluorescein angiography showed features of occult subfoveal choroidal neovascularization. He was referred to a physician and had a skin biopsy which revealed histologic features of Pseudoxanthoma elasticum. He was treated with bilateral focal argon laser. However he had right intravitreal Bevacizumab after a large ipsilateral subfoveal hemorrhage. His visual acuities remained stable afterward. Conclusion: Patients with Pseudoxanthoma elasticum have to be closely monitored for ocular manifestations. Choroidal neovascularization should be detected early and promptly treated. Antivascular endothelial growth factor injections have become the preferred treatment option for choroidal neovascularization in these patients.