ABSTRACT
ABSTRACT Occult macular dystrophy is characterized by a slowly progressive bilateral reduction of visual acuity in patients with normal fundus and fluorescein angiography. We describe a case of a 36-year-old male patient diagnosed with this condition, after extensive investigation with multimodal imaging, electrophysiology tests, and systemic screening.
RESUMO A distrofia macular oculta é caracterizada por perda visual, lentamente progressiva, em pacientes com fundoscopia e angiografia fluoresceínica normais. Relatamos o caso de um paciente de 36 anos do sexo masculino diagnosticado com essa condição após extensa investigação com exames de imagem multimodais, eletrofisiológicos e rastreio de doenças sistêmicas.
ABSTRACT
BACKGROUND: To report a case of acute exudative polymorphous paraneoplastic vitelliform maculopathy in a patient with a history of choroidal melanoma, with metastases to the pancreas, liver, and central nervous system. CASE PRESENTATION: A 63-year-old patient, with a history of enucleation of the right eye due to choroidal melanoma, complained of progressive visual loss during a follow-up visit. Fundoscopic examination revealed multiple small areas of serous retinal detachment scattered throughout the posterior pole and ancillary tests confirmed the diagnosis of acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM). Screening for systemic metastases showed pancreatic, hepatic, and central nervous system involvement. CONCLUSIONS: We describe a rare case of acute exudative polymorphous paraneoplastic vitelliform maculopathy, which should be considered in patients with or without a history of melanoma, who have vitelliform retinal detachments. Nevertheless, no previous reviews of literature have shown a correlation between AEPPVM and pancreatic metastasis.
ABSTRACT
The authors present a novel surgical approach for the treatment of retinal capillary hemangiomas (RCHs) secondary to von Hippel-Lindau (VHL) disease. This is a case report of a 23-year-old male patient with VHL that presented with multiple large RCHs and a thick epiretinal membrane (ERM) in his left eye, with best-corrected visual acuity (BCVA) of 20/80. This condition was surgically addressed with 23-gauge pars plana vitrectomy, ERM and internal limiting membrane peeling, and panretinal photocoagulation. Three monthly intravitreal injections of bevacizumab were administered after surgery. In a 14-month follow-up period, hemangiomas have regressed after laser therapy, macular anatomy has improved, retina remained completely attached, and there has been no development of new tumors or proliferative vitreoretinopathy. The patient achieved a BCVA of 20/40 in the treated eye. Panretinal photocoagulation combined with pars plana vitrectomy may be useful to reduce development of new capillary hemangiomas and reduce overall occurrence of complications in patients with VHL disease. Postoperative intravitreal injections of bevacizumab may have a role in this positive outcome.
