Submacular cystic lesion that turned out to be a sclerosing
The retinal capillary hemangioblastoma may arise as a part of von Hippel-Lindau syndrome however, they may occur as an isolated entity but submacular capillary hemangioblastoma has never been reported.

The present case has been reported American Journal of Ophthalmology Case Reports.

A fifty-six-year-old female presented with a recent diminution of vision in the right eye of 3-months duration. The clinical evaluation revealed normal anterior segment, intact ocular motility and the visual acuity was counting fingers 27.5 inches.

The fundus examination revealed a large round elevated submacular lesion with well-defined borders. The Optical coherence tomography (OCT) revealed a well-defined large subfoveal lesion elevating the overlying retina. The fluorescein angiography showed a hyperfluorescent lesion with a mild late leakage. The examination of the left eye was unremarkable.

An excisional biopsy was planned via a 23 gauge pars plana vitrectomy. Intraoperatively, a yellow round lesion was identified. It was well-defined with no firm adhesions to the optic nerve or the surrounding structures. It was possible to deliver the mass as one unit by enlarging the sclerotomy incision. It was a cystic soft yellowish-brown compressible lesion measuring 1 × 0.5 centimeters. The postoperative course was uneventful and the visual acuity of the patient improved to 0.1.

Histopathological examination demonstrated an angiomatous lesion formed of small vascular spaces lined by flat endothelial cells together with vacuolated stromal cells, however, there was no evidence of atypia, necrosis, specific infection or malignancy in all the examined sections. A diagnosis of submacular sclerosing capillary hemangioblastoma was confirmed.

The patient was referred to the medical oncology department for further systematic evaluation to exclude von-Hippel Lindau syndrome after pathology indicated the lesion to be a retinal capillary hemangioblastoma. Neuroimaging, urine analysis, renal ultrasonography were done and all results were normal.

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