First Author: T.Painhas PORTUGAL
Co Author(s): F. Esteves I. Almeida R. Soares J. Chibante-Pedro 0 0 0 0 0 0 0 0 0
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To evaluate the efficacy of intravitreal injection of ranibizumab in the treatment of choroidal neovascularization(CNV), in two cases of pseudoxanthoma elasticum (PXE) with angioid streaks and choroidal neovascularization. PXE is a rare, multisystem and hereditary disease that causes fragmentation and mineralization of elastic fibers in some tissues including the skin, eyes, cardiovascular and gastrointestinal systems. In the retina it is associated with EA and, eventually, NVC.
Ophthalmology Department of Centro Hospitalar entre o Douro e Vouga, Santa Maria da Feira, Portugal.
Case 1: 45 year old woman, without pathological history , presented with complaints of blurred vision and metamorphopsia in her left eye for 3 months. The best corrected visual acuity (BCVA ) was 20/20 in the right eye (OD) and 20/40 in the left eye (OS). The fundus examination revealed bilateral angioid streaks radiating from the optic disc and retina pigment with “ peau d’orange” appearance. Cutaneous examination revealed coalescent yellow papules with a 'plucked-chicken skin' appearance in the axillae and on the neck. The histopathological study of the skin lesions confirmed the diagnosis of pseudoxanthoma elasticum. Fluorescein angiography (FA) confirmed the presence of bilateral angioid streaks and CNV in the OS. The optical coherence tomography (OCT) also documented intraretinal fluid in the OS. Case 2: 49 year old woman with previously diagnosis of pseudoxanthoma elasticum, presented with complaints of decreased visual acuity in both eyes for the last months. BCVA was 20/200 in OD and 20/80 OS. Fundus examination revealed bilateral angioid streaks, and subretinal neovascularization in both eyes. Angiography and OCT confirmed the presence of bilateral angioid streaks and CNV and intraretinal fluid and in OD there was signs of adjacent fibrosis.
The response to treatment was assessed in terms of changes in lesion characteristics as measured by FA and OCT and change in visual acuity. In case 1 treatment was started with three intravitreal injections of ranibizumab (0.5 mg/0.05 ml) OE, monthly schedule. Two month after the end of the treatment there was functional improvement, with BCVA in OS of 20/20 as well as anatomical improvement as showed in OCT with the absence of subretinal fluid and AF showed no evidence of leakage . In case 2 the patient underwent three intravitreal injection of ranibizumab (0.5 mg/0.05 ml) in monthly scheme with improved vision in OE but no results OD. 4 months after the tratmente her visual acuity in the left eye was 20/60 with no fluid evidence in the OCT.
Angioid streaks represent linear breaks in Bruch's membrane secondary to a change in the elastic layer, with the potential for ingrowth of CNV. CNV is the major cause of severe visual loss in patients with angioid streaks. They are often associated with PXE. Various therapeutic approaches such as photodynamic therapy or laser photocoagulation have been proposed, with variable and sometimes limited results but intravitreal ranibizumab injections currently remain the best treatment. Based on the effectiveness of ranibizumab in other secondary CNVs, we use it in our patients, observing the functional and anatomical improvement.