Rhegmatogenous retinal detachment in wyburn mason syndrome. Wyburn masons syndrome is a condition in which blood vessels do. Image of the week jul 5, 2015 view all images of the week rating 4 ratings appears in. Affected infants have arteriovenous malformations avms, which are developmental abnormalities affecting the blood vessels, specifically the arteries, veins and capillaries. Wyburnmason syndrome also known as bonnetdechaumeblanc syndrome is a rare, nonhereditary neurocutaneous disorder that typically presents with unilateral vascular malformations that primarily involve the brain, orbits and facial structures. Dilated fundus examination disclosed large, dilated, and. Wyburnmason syndrome wms, also known as the bonnetdechaumeblanc syndrome, is a rare phakomatosis characterized by congenital ipsilateral retinal, brain usually midbrain, and, less frequently, facial angiomas 1. Bonnet 1937 and wyburnmason 1943 reported the vascular. Zimmergaller, md johns hopkins medical institutions coauthors. Bonnetdechaumeblanc syndrome, this is a rare condition is characterized by arteriovenous malformations avms in the retina and central nervous system cns. She underwent a successful 23gauge posterior vitrectomy. Wyburnmason syndrome, also known as racemose angioma, is a rare nonhereditary disorder that presents with multiple arteriovenous malformations that predominantly affect the face and brain.
Wyburnmason syndrome, also known as racemose angioma, is a rare nonhereditary. The association between wyburnmason syndrome and cutaneous reactive angiomatosis is also a novel finding. A 6yearold boy presented with light perception vision and an afferent pupillary defect in the right eye. Article in japanese fujita h, nakano k, kumon y, inoue h, sakaki s. Rhegmatogenous retinal detachment in wyburnmason syndrome. Wyburnmason syndrome presenting with bilateral retinal racemose. In my case, it involves the optic disc, retina and the midbrain, which is important to the movements of the eye and visual processing. Wyburnmason syndrome wms, also known as bonnetdechaumeblanc syndrome or retinoencephalofacial angiomatosis, is a rare condition.
Wyburnmason syndrome is associated with unilateral retinal racemose hemangioma. The diagnosis of wyburn mason syndrome is based on brain and orbital magnetic resonance angiography mra findings and ophthalmoscopy. The prognosis of wyburn masons syndrome may include the duration of wyburn masons syndrome, chances of complications of wyburn masons syndrome, probable outcomes, prospects for recovery, recovery period for wyburn masons syndrome, survival rates, death rates, and other outcome possibilities in the overall prognosis of wyburn masons. Bonnetdechaumeblanc syndrome, also known as wyburnmason syndrome, is a rare congenital disorder characterized by arteriovenous malformations of the brain, retina or facial nevi. Wyburnmason syndrome is a very rare congenital neurocutaneous disorder comprising of vascular malformations of the retina, ipsilateral cerebral avms and occasionally lesions in the oronasopharyngeal area. An avm is a tangle of abnormal and poorly formed blood vessels. Wyburnmason syndrome, a retinal vascular abnormality, had been diagnosed in his left eye 25 years previously. Wyburnmason syndrome is an uncommon condition of brainretinofacial angiomatosis resulting in multiple arteriovenous malformations avms, usually on the ipsilateral side. A rare genetic condition mainly involving enlarged brain blood vessels and skin and eye abnormalities. A case of rhegmatogenous retinal detachment in a patient with wyburnmason syndrome is described.
Wyburnmason syndrome is a condition in which blood vessels do not form correctly in both the retina of one eye and a part of the brain. Wyburn mason syndrome is an extremely rare nonhereditary disorder that is present at birth congenital. The disorder is also referred to as the bonnetdechaumeblanc syndrome and is classified as one of the nonhereditary congenital phakomatoses. Wyburn masons syndrome symptoms, diagnosis, treatments. Wyburn mason syndrome is a nonhereditary condition that affects the vascular system, causing arteriovenous malformation avm of the eye and brain. A posterior pole hole was found during the procedure and airfluid exchange was performed with no use of. Vitreous and intraretinal hemorrhage and neovascular glaucoma have been previously described. Review of the literature, survey of ophthalmology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips.
Wyburnmason syndrome represents a rare phakomatosis characterized by ipsilateral arteriovenous malformations of the brain, eyes and skin with port. Nguyen ha, md, wilmer eye institute, johns hopkins university uploaded on jan 28, 20. Full text pdf 3099k abstracts references39 the wyburnmason syndrome is a rare congenital anomaly, consisting of arteriovenous malformations involving the retina and midbrain, and occasionally subcutaneous facial structures. Bilateral wyburnmason syndrome presenting as acute. Wyburnmason syndrome nord national organization for. Read the congenital unilateral retinocephalic vascular malformation syndrome bonnetdechaumeblanc syndrome or wyburnmason syndrome. Case report a 31yearold woman with wyburnmason syndrome diagnosed at age 12 years with a known retinal avm involving the left optic disc presented with sudden loss of vision in the left eye. The syndrome has a number of possible symptoms and can, more rarely, affect the skin, bones, kidneys, muscles, and gastrointestinal tract. Wyburnmason syndrome american academy of ophthalmology.
Rarely, it presents with bilateral and symmetrical grade. A 5 yearold girl was seen to a ophthalmologist complaining of exophthalmus of her left eye after trauma on her face for the past. Pdf rhegmatogenous retinal detachment in wyburnmason. Ophthalmoscope image of the retina showing wyburnmason syndrome. Two patients with unilateral optic atrophy had both suprasellar and intraorbital arteriovenous malformations. Bonnetdechaumeblanc syndrome, also known as wyburnmason syndrome, is a rare. It is currently classified as a craniofacial arteriovenous metameric syndrome cams 34 pathology. Wyburnmason syndrome is a rare disease in which vascular dysgenesis affects the retina and brain. Wyburnmason syndrome associated with cutaneous reactive. The first had associated vascular lesions of the i.
Patients with large retinal arteriovenous malformations should therefore undergo brain imaging. The academy will be performing website maintenance tuesday, april 14 from 7 a. Fundus photograph showing racemose angioma of the retina in a patient with wyburnmason syndrome. Pdf retinal racemose hemangioma wyburnmason syndrome. Subarachnoid haemorrhage associated with wyburnmason syndrome has been described in only 5 patients in the literature since 1973. Wyburnmason syndrome is a nonhereditary condition that affects the vascular system, causing arteriovenous malformation avm of the eye and brain. Kikuchi k, kowada m, sakamoto t, tamakawa y, sakuragi s j comput tomogr, 122. Wyburnmason syndrome is a very rare congenital neurocutaneuos disorder comprising of vascular malformations of the retina, ipsilateral cerebral avms and occasionally lesions in the oronasopharyngeal area. The embryology and possible pathogenesis, as well as management options for wyburnmason syndrome, are discussed. To the best of the authors knowledge, this is the first report of an anti vascular endothelial growth factor agent employed as an effective treatment for macular edema in the setting of wyburnmason syndrome. Retinal racemose hemangioma wyburn mason syndrome a patient ten years followup.
Vitrectomy in this case present challenges related to intraoperatory bleeding risk, to a posterior pole tear among avms and the difficulty of obtaining free retina for photocoagulation. The accumulation of synonyms is carried out by averbis gmbh with healthcare natural language processing. Wyburnmason syndrome astakhov ophthalmology journal. Et, and some features may become unavailable intermittently. A 27 yearold woman previously diagnosed with wyburnmason syndrome, sought attendance with sudden low vision in right eye 3 months before. A extremely rare case of unilateral retinocephalic vascular malformation wyburnmason syndrome was reported.
A case report of sturgeweber and wyburnmason syndrome occurring in the same individual. All content of the icd10 cm search is based on the classifications and codes of the centers for medicare and medicaid services cms and the national center for health statistics nchs. This malformation is also called bonnetdechaumeblanc syndrome or faciomesodiencephalic angiomatosis. It is considered one of the nonhereditary congenital phakomatoses and is characterized by arteriovenous malformations avms that affect the retina, visual pathways, midbrain, and facial structures. Updated from an earlier publication in 2004 no one has yet proven or disproven professor roger wyburnmason s hypothesis, but certainly the treatments derived from his hypothesis stand out among all other therapies as being the earliest and most successful.
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