A case of polypoidal choroidal vasculopathy. Periphery is equally important for such patients.

BACKGROUND: To report a case of peripheral polypoidal choroidal vasculopathy (PCV) which was treated successfully.
METHODS: Interventional case report. Best-corrected visual acuity measurements (BCVA), slit-lamp examination, fundus biomicroscopy, fluorescein angiography (FFA), and indocyanine green angiography (ICGA) were performed at baseline examination and during the follow-up period. The patient underwent ICGA-guided argon laser to treat the active polyps.
RESULTS: An 82-year-old Caucasian man presented complaining of sudden deterioration of peripheral vision in his left eye (LE). His previous ocular history was associated with advanced age-related macular degeneration (AMD) involving both eyes (BE). Fundus examination revealed macular scars in BE and a large hemorrhagic pigment epithelial detachment (PED) temporal to the macula in the LE. ICGA revealed active polyps at the margins of the PED. The patient underwent ICGA-guided argon laser to treat the active polyps. Six months post-laser, the patient regained his peripheral vision with resolution of the hemorrhagic PED and remains stable until now, one year after treatment.
CONCLUSIONS: Appropriate treatment and regular follow-up is important in patients with PCV and peripheral lesions even if central vision is lost.

Introduction

Idiopathic polypoidal choroidal vasculopathy (PCV) is an exudative disorder of the macula. Although its pathogenesis remains unclear, it is generally thought to be an inner choroidal vascular abnormality1 which usually emanates from the posterior pole1 or less commonly from the peripheral retina.2 We report a case of PCV in the peripheral fundus in a patient with bilateral end-stage age-related macular degeneration (AMD) treated successfully.

Case presentation

An 82-year-old Caucasian man presented in our department complaining of sudden deterioration of peripheral vision in his left eye (LE). His previous ocular history was associated with advanced AMD involving both eyes. Best-corrected visual acuity (BCVA) was counting fingers in both eyes. The anterior segments and intraocular pressures were within normal limits in both eyes. Fundus examination revealed scar formation involving the center of the fovea bilaterally. In addition, a large hemorrhagic pigment epithelial detachment (PED) was detected temporal to the macula in the LE

Indocyanine green angiography (ICGA) disclosed the presence of actively leaking polypoidal vessels at the margins of the PED. The diagnosis of peripheral idiopathic PCV in a patient with advance AMD was made. After informed consent was obtained, ICGA-guided argon laser photocoagulation was applied. Six months post-laser, the hemorrhagic PED was resolved. One year later, the patient remains stable and his peripheral vision has significantly improved.

Discussion

In 1990, idiopathic PCV was first described by Yannuzzi and colleagues1 as a vascular disorder originating from the choroidal circulation. This disorder has been recognized increasingly in recent years as a distinct clinical entity separate from AMD. The pathogenesis of PCV remains unclear. Recent genetic research has shown that PCV and AMD share common genetic factors3 which may suggest that although PCV and wet AMD have different clinical characteristics, they may share same similar pathophysiologic aspects. In support of the latter, Lafaut and colleagues4 reported the possible coexistence of AMD and PCV in the same patient histopathologically.

Patients with end-stage AMD, characterized from the development of fibrotic tissue in the fovea, experience a significant loss of central vision. Peripheral retina is rarely affected allowing the patient to maintain some quality in their peripheral or side vision. Modifying the patient’s environment and using available low vision devices and aids, AMD patients can continue to maintain their lifestyle and independence.

Choroidal vascular lesions of PCV are more preferable found in the posterior pole of the retina,4 although further evidence suggests that the lesion could also be found in the peripheral retina up to 63%.3,5

We report a case of peripheral PCV in a male patient with advanced bilateral AMD affecting severely both foveal areas. The initial complain of the patient was deterioration of peripheral vision in addition to the already affected central vision. Such a clinical situation may impair significantly the quality of life of the patient and needs to be promptly identified, diagnosed and treated in order to preserve, in the best possible way, the peripheral vision of the patient.

Currently there are many therapeutics options for PCV. Photodynamic therapy alone or combined with anti-vascular endothelial growth factor treatment showed promising results.6 However, use of a conventional argon laser achieved up to 78% clinical or angiographic resolution of maculopathy for extrafoveal polyps.7 When PCV lesions accompany PED, the lesions are usually located at the margins of PED.8 In our case, ICGA-guided argon laser photocoagulation was selected, since central vision was impaired due to foveal scars and the polyp lesions were extrafoveal and at the margins of the PED.

In our case, treatment with ICGA-guided argon laser photocoagulation was beneficial and resolved the hemorrhagic PED, preserving the peripheral vision of the patient. Appropriate treatment and regular follow-up is important in patients with PCV even if central vision is lost.