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Abordagem terapêutica do hemangioma coroideu difuso : a propósito de um caso clínico
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Hemangioma coroideu difuso (DCH) é um tumor vascular benigno, que é caracteristicamente associado à síndrome de Sturge-Weber (SWS). O diagnóstico é estabelecido recorrendo a métodos complementares, com destaque para as novas modalidades de tomografia de coerência óptica (modos swept-source e spectral-domain), que permitem uma visualização mais detalhada da coroideia e facilitam o diagnóstico precoce. O DCH é geralmente assintomático nos primeiros anos de vida, podendo causar mais tarde descolamento exsudativo da retina (ERD), com perda de visão associada. O tratamento é difícil devido ao mau prognóstico visual, sendo apenas realizado em casos sintomáticos. O principal objetivo é a involução do tumor, com reabsorção do líquido subretiniano e resolução do ERD. A modalidade terapêutica mais utilizada é a radioterapia, sob forma de feixe externo, feixe de protões e braquiterapia de placa. Uma modalidade alternativa recente é a terapia fotodinâmica (PDT), cada vez mais usada no tratamento do hemangioma coroideu difuso, por ser menos invasiva e apresentar uma menor taxa de complicações. A abordagem terapêutica destas lesões implica vigilância ativa e tratamento adequado, para resolução das lesões exsudativas, preservando a acuidade visual dos doentes.
Descreve-se um caso de um doente de 23 anos, sexo masculino, com diagnóstico de SWS e DCH no olho direito, manifestando-se por diminuição da acuidade visual associada a ERD superior. Realizou PDT, com boa eficácia na resolução do ERD referido. Posteriormente ocorreu o desenvolvimento de um ERD inferior, com progressão para ERD total e foi por isso submetido a cirurgia de drenagem de líquido subretiniano e vitrectomia, retomando as sessões de PDT após a cirurgia. Na última reavaliação, a retina encontrava-se aplicada, observando-se ainda uma pequena área de descolamento com líquido subretiniano.
Diffuse choroidal hemangioma (DCH) is a benign vascular tumor that is characteristically associated with the Sturge–Weber syndrome (SWS). Diagnosis is established using swept-source and spectral-domain optical coherence tomography that enables high-resolution visualization of the choroid and early detection of the condition. Although DCH may remain asymptomatic during childhood, it can present with exudative retinal detachment (ERD) later in life, resulting in visual loss. Treatment is difficult and generally unwarranted unless there is visual impairment caused by exudative complications. The main goal of the treatment is to shrink the tumor, while allowing the absorption of subretinal fluid and ERD resolution. The main treatment method is radiation therapy, with external beam radiation therapy, proton beam therapy and plaque brachytherapy. One method of alternative management is with photodynamic therapy (PDT) that, although less invasive with a lower rate of complications, is not always effective in cases with extensive exudative retinal detachment. Management of these lesions requires an active and lifelong surveillance and proper treatment, to limit tumor proliferation and resolve exudative complications, helping to preserve and stabilize the patients’ visual acuity. We report a case of a 22 years-old, male, diagnosed with SWS and DCH of the right eye, complicated by superior ERD, resulting in visual acuity impairment. The patient was treated with PDT, which was effective in superior ERD resolution. Posteriorly, an inferior ERD started developing, later progressing to total ERD. The patient was then submitted to subretinal fluid drainage surgery and vitrectomy and resumed PDT sessions after surgery. In the last follow-up, the retina was attached, but a small area of retinal detachment was observed.
Diffuse choroidal hemangioma (DCH) is a benign vascular tumor that is characteristically associated with the Sturge–Weber syndrome (SWS). Diagnosis is established using swept-source and spectral-domain optical coherence tomography that enables high-resolution visualization of the choroid and early detection of the condition. Although DCH may remain asymptomatic during childhood, it can present with exudative retinal detachment (ERD) later in life, resulting in visual loss. Treatment is difficult and generally unwarranted unless there is visual impairment caused by exudative complications. The main goal of the treatment is to shrink the tumor, while allowing the absorption of subretinal fluid and ERD resolution. The main treatment method is radiation therapy, with external beam radiation therapy, proton beam therapy and plaque brachytherapy. One method of alternative management is with photodynamic therapy (PDT) that, although less invasive with a lower rate of complications, is not always effective in cases with extensive exudative retinal detachment. Management of these lesions requires an active and lifelong surveillance and proper treatment, to limit tumor proliferation and resolve exudative complications, helping to preserve and stabilize the patients’ visual acuity. We report a case of a 22 years-old, male, diagnosed with SWS and DCH of the right eye, complicated by superior ERD, resulting in visual acuity impairment. The patient was treated with PDT, which was effective in superior ERD resolution. Posteriorly, an inferior ERD started developing, later progressing to total ERD. The patient was then submitted to subretinal fluid drainage surgery and vitrectomy and resumed PDT sessions after surgery. In the last follow-up, the retina was attached, but a small area of retinal detachment was observed.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2021
Palavras-chave
Síndrome de Sturge-Weber Hemangioma coroideu difuso Terapia fotodinâmica Radioterapia Oftalmologia