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

Pangtey, Bhanu; Kohli, Piyush; Ramasamy, Kim

doi:10.4103/ijo.ijo_455_18

Cited by 11 publications

(4 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Patients of this syndrome are also likely to be diagnosed of the same during their infancy or childhood. 2 No history of skin lesions, neurological manifestations or any other characteristic ocular abnormalities such as proptosis, blepharoptosis or cranial nerve palsies. This patient has to be treated symptomatically.…”

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Optic disc vascular malformation with venous stasis and macular edema

Varman¹,

Gnanadurai²,

Karunanidhi³

2021

IJCEO

View full text Add to dashboard Cite

Arteriovenous malformations are abnormal tangled blood vessels with multiple irregular connections between arteries and veins. These malformations can occur anywhere with a preponderance to the spinal cord and brain. AV-malformations are usually congenital and patients might also have an associated intracranial aneurysm. Ophthalmic manifestations of AVM depend upon the location, varying from being asymptomatic to visual phenomena, field defects, diplopia, nystagmus etc. AV malformations in the cortical or occipital lobe produce visual symptoms which are transient. They can be associated with scotomas and migraine type headache. Signs and symptoms of brainstem AVMs are nonspecific and may include diplopia, nystagmus, dizziness, ocular motor nerve palsy, gaze palsy, anisocoria, or pupillary light-near dissociation. Findings are usually unilateral. There can be transient monocular vision loss due to the "steal phenomenon" in rare cases. Diagnosis of an AVM are based on angiographic findings which demonstrate an engorged arterial system, rapid filling of the malformation and outflow of the venous system.

show abstract

Section: Resultsmentioning

confidence: 99%

“…Markedly dilated and tortuous vessels, shunt blood flow from arteries to veins which usually do not leak fluid. Additionally might present with proptosis and nerve palsies 2. This is a congenital neurocutaneous disorder affecting leading to areterio venous malformations.…”

mentioning

confidence: 99%

Optic disc vascular malformation with venous stasis and macular edema

Varman¹,

Gnanadurai²,

Karunanidhi³

2021

IJCEO

View full text Add to dashboard Cite

show abstract

“… 11 Cases of macular edema due to a racemose hemangioma have been treated with intra-vitreous injection of anti-vascular endothelial growth factor. 9 , 17 , 18 Additionally, some cases that showed spontaneous regression of dilated vessels within a few months or years have also been reported. 19 , 20 …”

Section: Discussionmentioning

confidence: 99%

Racemose hemangioma complicated with macular macroaneurysm rupture

Yamauchi

Suzuki

Tanaka-Gonome

et al. 2021

American Journal of Ophthalmology Case Reports

View full text Add to dashboard Cite

Purpose To describe a rare case of racemose hemangioma which developed spontaneous macular macroaneurysm (MA) rupture and vitreaous hemorrhage. Observations A 29-year-old healthy asian female visited our hospital and a racemose hemangioma was found in the left eye. At presentation, the best corrected visual acuity (BCVA) was 30/20 in her left eye. At 9 years after the first visit, MA-like lesion was noted in the macular area. After that, vitreous and subretinal hemorrhage appeared in the left eye. The patient underwent simultaneous vitrectomy and cataract surgery, but vitreous re-hemorrhage occurred two days after the operation. To avoid re-hemorrhage, silicone oil (SO) tamponade was added in the second vitrectomy. Two years after the second operation, SO was removed and postoperative BCVA in the left eye was 20/200 without re-bleeding in the vitreous. Conclusions and Importance Although retinal hemorrhages have been reported in the patients with a racemose hemangioma, in our case the macular MA rupture occurred at 9 years after the first visit. Congenital retinal arteriovenous anastomosis can show a change in vascular shape in some cases, thus it is important to observe carefully.

show abstract

“…Malformations mostly occur in the retina and brain and rarely on the facial skin. Unlike other phacomatosis with retinal involvement, WMS rarely affects the skin [5,6]. It has three main types.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

View full text Add to dashboard Cite

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.

show abstract

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

Cited by 11 publications

References 9 publications

Optic disc vascular malformation with venous stasis and macular edema

Optic disc vascular malformation with venous stasis and macular edema

Racemose hemangioma complicated with macular macroaneurysm rupture

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

Contact Info

Product

Resources

About