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Posters

First results after photodynamic therapy for a symptomatic choroidal hemangioma previously masquerading as amblyopia and later mimicking Best Vitelliform dystrophy

Poster Details

First Author: A.Hajjar SPAIN

Co Author(s):    E. Ortega   E.M. Salinas   N. Mendieta   M.E. De Las Heras   S. Eguiza      0   0 0   0 0   0 0   0 0

Abstract Details



Purpose:

To describe the steps for diagnosing Circumscribed Choroidal Hemangioma mimicking other retinal pathologies. We report a case of a 44-year-old Caucasic woman presented with a history of gradual visual decline in her right eye. An ophthalmic antecedent was amblyopia in her right eye. Except from Fibrocystic Breast Disease that required biopsies, the patient was systemically well. Best-corrected visual acuity (VA) at presentation was 0,7/1 in the right eye and 1/1 in the left eye. The anterior segments and intraocular pressures were normal. Fundus examination revealed a peripapillar and macular discolourated orange-yellow area in the retina, only in the right eye.

Setting:

We report a case of a 44-year-old Caucasic woman followed-up in a Hospital that assists a population of 250.000 inhabitants.

Methods:

The diagnosis process was started providing retinal images using the Optical Coherence Tomography (OCT) that identified subretinal fluid and associated retinal pigment epithelium (RPE) changes in the right eye. Best Vitelliform Dystrophy (Best’s disease) at early stages was initially suspected due to the combination of similar fundus appearance, retinal changes in the OCT and chronicity of symptoms. It is inherited in an autosomal dominant pattern; the descendants were examined but the results were non pathological. The Electrooculogram (EOG) was normal with an Arden ratio of 2.16 in the right eye and 2.44 in the left eye. This ratio is less than 1.8 in Best’s disease, so it discarded it and subsequently directed the diagnosis to Adult Vitelliform Macular Dystrophy. Three years later the patient was assisted at the emergency department. VA was 0,3/1 in the right eye. Fundus examination revealed an oval reddish-orange mass with indistinct margins in the right eye. A new diagnosis process started using OCT that confirmed an increase of subretinal fluid. Fluorescein Angiography (FA) early phase showed marked hyperfluorescence, and Indocyanine Green Angiography (ICGA) late phase showed central washout of fluorescein with sustained marginal hyperfluorescence. Ultrasonograpy showed a dome-shaped lesion, with high homogeneous reflectivity.

Results:

Circumscribed Choroidal Hemangioma (CCH) is diagnosed. Combination of characteristic features on FA, ICGA and ultrasonography, finally helped to establish the correct diagnosis. Nevertheless, during the last diagnosis process, a metastatic survey was performed, and the results were non pathological, discarding choroidal metastasis the most common intraocular tumor. The chronic visual decline in the right eye and the uncertain former diagnosis caused a General Adaptation Syndrome (GAS) in the patient who is being followed-up by the Department of Psychiatry. The patient was treated with Photodynamic Therapy (PDT). Fourteen weeks after the first PDT treatment, the hemangioma shows slightly less exudation and VA is 0,4 in the right eye at this follow-up visit.

Conclusions:

Choroidal hemangioma is an uncommon vascular tumor of the choroid that can be circumscribed or diffuse. The Circumscribed Choroidal Hemangioma is unilateral and occurs sporadically, without any associated systemic anomaly. CCH is usually diagnosed between the second and fourth decade of life, when it causes visual symptoms related to the development of a progressive cystoid macular edema and exudative retinal detachment. Although CH is a benign tumor, the late diagnosis influences in a guarded prognosis related to the difficult improvement of VA. The differential diagnosis of CH can be complicated because of the similarities with other tumors or macular pathologies. Nevertheless, the combination of FA and ICGA is helping in the early diagnosis of this pathology. The PDT is an effective and minimally invasive treatment for CH but long-term follow-up is necessary to avoid recurrence after successful elimination of subretinal fluid.

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