ABSTRACT
O objetivo é relatar o caso de um paciente de sete anos, nascido a termo, sem intercorrências perinatais, encaminhado ao Setor de Retina/Vítreo para elucidação diagnóstica. Apresentava história de redução da acuidade visual à esquerda, de caráter insidioso/progressivo, há quatro anos. Ao exame, apresentava diminuição do diâmetro corneano e corectopia do olho direito (OD), sem alterações à biomicroscopia do olho esquerdo (OE). A fundoscopia do OD revelava descolamento de retina (DR) total e, do OE, inicialmente, mostrava alterações vasculares retinianas periféricas e exsudação retiniana, associado à tração vitreorretiniana no setor temporal. As tomografias e ressonâncias de crânio/órbitas não apresentavam anormalidades, com exceção de achados sugestivos de DR antigo no OD, confirmado pela ultrassonografia do globo ocular, que também demonstrou microftalmia. Diante disso, aventou-se a hipótese diagnóstica de vitreorretinopatia exsudativa familiar, doença rara de caráter autossômico dominante e relacionada com casamentos consanguíneos, inicialmente simulando doença de Coats. O paciente foi tratado com fotocoagulação a laser diodo na periferia temporal do OE, com melhora das áreas de tração vitreorretiniana.
We report the case of a seven year-old male patient, born at term without any perinatal complications, referred to the Retina/Vitreous Service for diagnostic elucidation. He had a history of progressive visual acuity loss on his left eye that started four years ago. On examination, he had decreased corneal diameter and corectopia of the right eye (OD), without any noteworthy findings on the biomicroscopy of the left eye (OS). The fundus of the OD revealed total retinal detachment, and the OS initially showed peripheral retinal vascular abnormalities and retinal exudation, associated with retinal vitreous traction on the temporal sector. The CT and MRI of the brain/orbits showed no abnormalities, except for findings suggestive of an old retinal detachment on the OD, confirmed by ultrasonography, which also showed microphthalmia of the OD. The diagnosis of familial exudative vitreoretinopathy, a rare disease of autosomal dominant inheritance and related to consanguineous marriages, that can initially simulate Coats disease, was proposed. The patient was treated with diode laser photocoagulation in the temporal periphery of the OS, with improvement in the areas of vitreoretinal traction.
Subject(s)
Child , Humans , Male , Osteoporosis/diagnosis , Retinal Telangiectasis/diagnosis , Vitreoretinopathy, Proliferative/diagnosis , Diagnosis, DifferentialABSTRACT
Familial exudative vitreoretinopathy (FEVR) is an inherited blinding condition characterized by abnormal development of the retinal vasculature. The authors describe a rare case of the disease associated with severe infantile osteoporosis in a young female patient. The patient was submitted to multiple vitreoretinal procedures in both eyes due to tractional macular detachments. The case was complicated by diffuse uveitis of difficult control in one eye, which stimulated proliferative vitreoretinopathy and retinal redetachment. The inflammatory potential of drugs used in the control of the osteoporosis, in contrast with the inherent inflammatory activity the disease, are discussed.
Relato de um caso incomum de vitreorretinopatia exsudativa familiar (FEVR) bilateral em criança do sexo feminino portadora de grave osteoporose infantil. A paciente foi submetida a vitrectomias sucessivas para correção de descolamentos tracionais maculares em ambos os olhos. O quadro foi complicado em um olho por uveíte difusa de difícil controle, que estimulava o aparecimento de proliferação vitreorretiniana de difícil abordagem, responsável por redescolamentos sucessivos da retina. Discute-se sobre o potencial pró-inflamatório de drogas usadas no controle da osteoporose, em contraste com a atividade inflamatória inerente à doença.
Subject(s)
Child , Female , Humans , Osteoporosis/complications , Retinal Detachment/etiology , Vitreoretinopathy, Proliferative/etiology , Recurrence , Retinal Detachment/surgery , Severity of Illness Index , Vitrectomy , Vitreoretinopathy, Proliferative/diagnosis , Vitreoretinopathy, Proliferative/surgeryABSTRACT
PURPOSE: To investigate the short-term effects of panretinal photocoagulation (PRP) combined with an intravitreal injection of Avastin(R) (bevacizumab) as an adjuvant to high-risk proliferative diabetic retinopathy (PDR). METHODS: The data was collected retrospectively from the eyes of high-risk PDR patients, which were divided into two groups. One eye was treated with only PRP (PRP only group) and the fellow eye of same patient was treated with both PRP and intravitreal bevacizumab injection (Adjuvant group). Best corrected visual acuity (BCVA), IOP (intraocular pressure), and new vessel (NV) size in fluorescein angiography were recorded immediately and at the six-week follow-up visit. Adverse events associated with intravitreal injection were investigated. RESULTS: Of 12 patients with high-risk PDR, five were male and seven were female. There were no statistically significant BCVA or IOP changes after treatment in either group (p=0.916, 0.888). The reduction of NV size was found in both groups, but NV size in the adjuvant group showed a greater decrease than that of the PRP only group (p=0.038). Three patients had adverse events after intravitreal injection. Two patients had mild anterior uveitis and one patient had a serious complication of branched retinal artery obstruction (BRAO). CONCLUSIONS: Intravitreal bevacizumab injection with PRP resulted in marked regression of neovascularization compared with PRP alone. One serious side effect, BRAO, was noted in this study. Further studies are needed to determine the effect of repeated intravitreal bevacizumab injections and the proper number of bevacizumab injections as an adjuvant.