Racemose hemangioma presenting as a vitreous hemorrhage: case report / Hemangioma racemoso apresentando-se como hemorragia vítrea: relato de caso

ABSTRACT This report describes a case of retinal racemose hemangioma that first presented as a vitreous hemorrhage. The authors present the case of a 47-year-old woman with a sudden 5-day painless visual loss in her left eye. At the first visit, the best-correct visual acuities were 20/20 in the right eye and hand motions in the left eyes. Ultrasonography showed an attached retina and a massive vitreous hemorrhage. Pars plana vitrectomy was performed and a dilatation of large vessels was detected bulging from the optic disc. The best-correct visual acuities on day 30 postoperatively was 20/25 in the left eye. Fundus angiography and spectral-domain optical coherence tomography angiography showed anomalous arteriovenous communications with no intervening capillaries. The diagnosis was racemose hemangioma, an arteriovenous malformation of group 2 retina based on the Archer classification.

RESUMO Este relato descreve um caso de hemangioma racemoso da retina que se apresentou inicialmente como hemorragia vítrea. Os autores apresentam o caso de uma mulher de 47 anos com perda visual súbita e indolor 5 dias antes no olho esquerdo. Na primeira visita, a melhor acuidade visual corrigida foi de 20/20 no olho direito e movimentos das mãos no olho esquerdo. A ultrassonografia mostrou uma retina aderida e uma hemorragia vítrea maciça. Foi realizada vitrectomia pars plana, sendo detectada proliferação de grandes vasos salientes do disco óptico. A acuidade visual no dia 30 de pós-operatório foi de 20/25 no olho esquerdo. A angiografia de retina e a angiotomografia de coerência óptica de domínio espectral mostraram comunicações arteriovenosas anômalas sem capilares intermediários. O diagnóstico foi hemangioma racemoso, uma malformação arteriovenosa da retina do grupo 2 com base na classificação de Archer.

Wyburn–Mason syndrome presenting with bilateral retinal racemose hemangioma with unilateral serous retinal detachment

Wyburn–Mason syndrome is associated with unilateral retinal racemose hemangioma. Rarely, it presents with bilateral and symmetrical grade of malformation. We describe a 37-year old male, who presented with Wyburn–Mason syndrome presenting with bilateral but asymmetrical retinal hemangioma. The eye with advanced grade of hemangioma was complicated with exudation, intraretinal fluid, neurosensory detachment, and reduced vision. He was treated with one intravitreal injection of bevacizumab, after which both the intraretinal fluid and neurosensory detachment resolved. His vision improved and was maintained till 1 year of follow-up.

A Case of Wyburn-Mason Syndrome with Facial Nevus Flammeus and the Associated Optical Coherence Tomography Findings

PURPOSE: To describe a case of Wyburn-Mason Syndrome, which is a rare phakomatosis, and its associated optical coherence tomography findings. CASE SUMMARY: A 5-year-old boy was referred to our clinic for decreased vision in his right eye. He had nevus flammeus on his right forehead, upper eyelid, and cheek. His best corrected visual acuity was hand motion in the right eye, in which a relative afferent pupillary defect was noted. Fundus examination of the right eye showed marked dilation and tortuosity of retinal vessels, which could not be discriminated between arteries and veins on the posterior pole. Optical coherence tomography showed multiple large intraretinal vessels without involvement of the internal limiting membrane. Cerebral angiography revealed arteriovenous malformations (AVMs) and feeder vessels from the internal carotid artery to the ophthalmic artery, extending to the ethmoidal artery in the nasal cavity. CONCLUSIONS: Wyburn-Mason syndrome is a rare phakomatoses and shows unique retinal AVM. Misdiagnosis or undertreatment of Wyburn-Mason syndrome can lead to serious complications. Patients with facial nevus flammeus should undergo thorough ocular examinations to rule out this disorder. Optical coherence tomography is a useful method for understanding structural features of retinal vascular malformations in Wyburn-Mason syndrome.

A Case Report of Bilateral Retinal Racemose Hemangioma Restricted to Peripapillary Area

PURPOSE: The authors of the present case report observed a bilateral retinal racemose hemangioma which was located within the peripapillary area. CASE SUMMARY: A 17-year-old man presented with floaters in both eyes. Fundus revealed tortuous and anastomosed retinal vasculature around the optic disc. In addition, fluorescein angiography showed a non-leaking retinal arteriovenous anastomosis. Seven years after the initial visit, vitreous hemorrhage occurred in the patient's left eye, and then 1 year later, subretinal hemorrhage was found in his left eye. CONCLUSIONS: Because retinal racemose hemangioma can accompany vitreous hemorrhage and subretinal hemorrhage regardless of size, a routine periodic ophthalmic examination is recommended.

Wyburn-Mason Syndrome: A Case Report

PURPOSE: To describe a case of Wyburn-Mason Syndrome, which is characterized by arteriovenous malformations in the central nervous system and the retina. CASE SUMMARY: A 13-year-old girl who underwent neurosurgical intervention for intraventricular hemorrhage was referred to our ophthalmic clinic because brain angiogram and MRI finding revealed arteriovenous malformations (AVMs) that extended from the orbit and optic chiasm along the optic pathway. Fundus and fluorescein angiography of the left eye showed marked dilation and tortuosity of the retinal vessels. Fluorescein angiographic findings showed rapid filling of all vessels and no dye leakage. CONCLUSIONS: Retinal racemose hemangioma should be considered for Wyburn-Mason syndrome, and the finding of retinal AVMs should warrant cerebral imaging studies including MRI and angiography.

A Case of Retinal Racemose Hemangioma

PURPOSE: Racemose hemangiomas of the retina are rare developmental vascular anomalies with abnormal arteriovenous anastomoses. We report our observations of a retinal racemose hemangioma with marked dilation and tortuosity of the retinal vessels in the entire left retina. METHODS: In a 9-year-old boy with loss of vision and mild esotropia who presented with hugely dilated and tortuous retinal vessels in left retina, we examined fundus, fluorescein angiography, cerebral angiography, brain CT and MRI. RESULTS: Fundus and fluorescein angiography of the left eye showed marked dilation and tortuosity of the retinal vessels. Angiographic finding showed rapid filling of all vessels and no dye leakage. MRI finding revealed small tortuous vessels around left optic nerve, optic chiasm and tract. Brain CT and cerebral angiography showed nothing abnormal.