Hemangioma racemoso de retina (síndrome de Wyburn-Mason): acompanhamento de um paciente por dez anos: relato de caso

Guerra, RL Leitão; Guerra, CL Leitão; Guerra, Marilyn Linares; Neto, AS Guerra; Guerra, AA Leitão

doi:10.1590/s0004-27492009000400022

Cited by 3 publications

(4 citation statements)

References 3 publications

(4 reference statements)

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“…Patel (40) reported a case of Wyburn–Mason syndrome with vascular abnormalities in the face, orbit, and brain (but not in the retina) of a newborn. RRH was once thought to be non-progressive, and patients can often continue to have good vision [39,40]. The longest follow-up period was 27 years without any progression in the retinal or cephalic condition [40].…”

Section: Case Presentationmentioning

confidence: 99%

Retinal vein occlusion in retinal racemose hemangioma: a case report and literature review of ocular complications in this rare retinal vascular disorder

2014

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BackgroudRetinal racemose hemangioma (RRH) is a rare congenital disorder that often co-occurs with other ocular complications. In this study, we present a case of RRH complicated with retinal vein obstruction in three branches and provide a review of ocular complications and associations with RRH.Case presentationOne case of RRH is presented. Fundus examination, fluorescein angiography (FFA) and optical coherence tomography (OCT) of the patient identified Group 3 RRH complicated with retinal vein occlusions in the superotemporal, inferotemporal, and inferonasal branches. Macular edema, which causes visual impairment, was detected. A brief literature review was also presented. The PubMed database was searched for RRH or related keywords to find reports of ocular complications or associations published on or before Dec. 31, 2013. A total of 140 papers describing167 RRH cases were found. The mean age of diagnosis was 22.97 years. Ocular complications were mentioned in 32 (19.16%) cases. Retinal vein occlusion (46.88%) was the major ocular complication in RRH, followed by hemorrhage (34.38%). Eight (4.79%) cases were associated with other ocular diseases such as Sturge–Weber syndrome , Morning glory disc anomaly and macroaneurysm.ConclusionsAlthough RRH is a relatively non-progressive condition, its complications may lead to vision loss and should be treated in time.

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Section: Case Presentationmentioning

confidence: 99%

Retinal vein occlusion in retinal racemose hemangioma: a case report and literature review of ocular complications in this rare retinal vascular disorder

2014

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“…To our knowledge, this is the first published case documenting the natural history of retinal vascular changes from infancy to adulthood in MWS. There are two published cases of the evolution of these retinal lesions with a longer follow-up of 10 and 27 years, but these were reported in adults [17,18].…”

Section: Discussionmentioning

confidence: 99%

The Natural History of Retinal Vascular Changes from Infancy to Adulthood in Wyburn-Mason Syndrome

et al. 2020

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Wyburn-Mason syndrome is a rare, non-hereditary congenital neurocutaneous disorder leading to arteriovenous malformations. Malformations are characterized by an artery that is directly connected to veins without a capillary system and forms a fragile mass of abnormal vessels. It can be found in the midbrain, in the eyes, orbit, and rarely in cutaneous nevi. Neurological and ocular symptoms are the most common. Ocular signs and symptoms include abnormally dilatated vessels of conjunctiva, nystagmus, strabismus, vitreous hemorrhage, vein occlusions, retinal detachment, etc. Neurological symptoms may include headaches, paralysis, epistaxis, hydrocephalus, and hemiparesis. Imaging modalities such as MRI/CT angiography, optical coherence angiography, and fluorescein angiography are the most important for the identification of arteriovenous malformations. In our case report, we present an eight-month-old girl with an incidental finding of retinal angiomatosis on the left eye and was subsequently diagnosed with Wyburn-Mason syndrome. We compare the findings from the first visit to her clinical findings 20 years later.

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“…As such Type I and II AVMs have stable clinical course without evidence of progression. [ 3 ] Common complications associated with AVMs include intraretinal hemorrhage, retinal vein obstruction, vitreous hemorrhage, and neovascular glaucoma. [ 4 ] This has been attributed to hyperdynamic flow through small caliber vessels and steal phenomenon caused by increased venous pressure and decreased arterial pressure.…”

Section: Discussionmentioning

confidence: 99%

“…[ 2 ] RRA was once thought to be benign and follow a stable clinical course. [ 3 ] However, they can be complicated with vascular occlusions, retinal hemorrhages, and macular edema, leading to vision loss. [ 4 ] We present a rare case of RRA complicated with fleeting macroaneurysm (MA) and describe various optical coherence tomography angiography (OCTA) features.…”

mentioning

confidence: 99%

Optical coherence tomography angiography of fleeting macroaneurysm in retinal racemose angioma

Shanmugam

Seshadri

Konana

et al. 2018

Indian J Ophthalmol

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We present a rare case of retinal racemose angioma complicated with fleeting macroaneurysm (MA). A 50-year-old female presented with diminution of vision in her right eye for 6 years. Fundus examination showed a racemose angioma with hemorrhagic MA temporal to the fovea in the right eye. On subsequent follow-ups, spontaneous thrombosis of MA was noted with the development of new MA inferior to the fovea, with intraretinal hemorrhage extending into the fovea. Focal laser to MA resulted in resolution of MA with improvement in vision. We report optical coherence tomography angiographic features of the fleeting MA in a case of racemose angioma.

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Hemangioma racemoso de retina (síndrome de Wyburn-Mason): acompanhamento de um paciente por dez anos: relato de caso

Cited by 3 publications

References 3 publications

Retinal vein occlusion in retinal racemose hemangioma: a case report and literature review of ocular complications in this rare retinal vascular disorder

Retinal vein occlusion in retinal racemose hemangioma: a case report and literature review of ocular complications in this rare retinal vascular disorder

The Natural History of Retinal Vascular Changes from Infancy to Adulthood in Wyburn-Mason Syndrome

Optical coherence tomography angiography of fleeting macroaneurysm in retinal racemose angioma

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