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Retinal Racemose Hemangioma With Optic Neuropathy in a Child

The aim of this study is to report an unusual case of retinal racemose haemangioma (RRH) in a child resulting in optic neuropathy and its optical coherence tomography angiography (OCT-A) findings. This is a retrospective case report. For almost a year, a 13-year-old girl experienced gradual, painles...

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Autores principales: Ahmad Tarmizi, Nur Nadia, Bastion, Mae-Lynn Catherine, Abdul Aziz, Roslin Azni, Md Din, Norshamsiah, Mohd Khialdin, Safinaz
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8483998/
https://www.ncbi.nlm.nih.gov/pubmed/34646671
http://dx.doi.org/10.7759/cureus.17620
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author Ahmad Tarmizi, Nur Nadia
Bastion, Mae-Lynn Catherine
Abdul Aziz, Roslin Azni
Md Din, Norshamsiah
Mohd Khialdin, Safinaz
author_facet Ahmad Tarmizi, Nur Nadia
Bastion, Mae-Lynn Catherine
Abdul Aziz, Roslin Azni
Md Din, Norshamsiah
Mohd Khialdin, Safinaz
author_sort Ahmad Tarmizi, Nur Nadia
collection PubMed
description The aim of this study is to report an unusual case of retinal racemose haemangioma (RRH) in a child resulting in optic neuropathy and its optical coherence tomography angiography (OCT-A) findings. This is a retrospective case report. For almost a year, a 13-year-old girl experienced gradual, painless, generalized blurred vision in her right eye. Visual acuity was 6/60 with a positive relative afferent pupillary defect (RAPD) in her right eye. The right-eye fundus showed enlarged and tortuous retinal vessels extending from the optic disc to all four quadrants, including the juxta foveal region. OCT analysis revealed distortion in the region of enlarged vessels with minimal retinal fluid while OCT-A of the macula area demonstrated dilated and tortuous vessels in the superficial layers of the retina. Right intra-orbital vascular channels surrounding the optic nerve with optic nerve atrophy and gliosis were detected on magnetic resonance imaging angiography/venography (MRA/MRV). The cerebral angiogram reported an abnormal tangle of small vessels within the right orbit that received supply from a dilated right ophthalmic artery indicating the presence of retro-orbital arteriovenous malformation (AVM). She was then referred to the neurosurgeon and a decision was made not to embolize or resect the dilated vessel as this might lead to occlusion of the ophthalmic artery and thus worsen her vision. RRH may present in the paediatric age group, and optic nerve atrophy is one of the disease manifestations. OCT-A is a less invasive diagnostic option compared to fundus fluorescein angiography (FFA) for diagnosis and monitoring of disease progression.
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spelling pubmed-84839982021-10-12 Retinal Racemose Hemangioma With Optic Neuropathy in a Child Ahmad Tarmizi, Nur Nadia Bastion, Mae-Lynn Catherine Abdul Aziz, Roslin Azni Md Din, Norshamsiah Mohd Khialdin, Safinaz Cureus Ophthalmology The aim of this study is to report an unusual case of retinal racemose haemangioma (RRH) in a child resulting in optic neuropathy and its optical coherence tomography angiography (OCT-A) findings. This is a retrospective case report. For almost a year, a 13-year-old girl experienced gradual, painless, generalized blurred vision in her right eye. Visual acuity was 6/60 with a positive relative afferent pupillary defect (RAPD) in her right eye. The right-eye fundus showed enlarged and tortuous retinal vessels extending from the optic disc to all four quadrants, including the juxta foveal region. OCT analysis revealed distortion in the region of enlarged vessels with minimal retinal fluid while OCT-A of the macula area demonstrated dilated and tortuous vessels in the superficial layers of the retina. Right intra-orbital vascular channels surrounding the optic nerve with optic nerve atrophy and gliosis were detected on magnetic resonance imaging angiography/venography (MRA/MRV). The cerebral angiogram reported an abnormal tangle of small vessels within the right orbit that received supply from a dilated right ophthalmic artery indicating the presence of retro-orbital arteriovenous malformation (AVM). She was then referred to the neurosurgeon and a decision was made not to embolize or resect the dilated vessel as this might lead to occlusion of the ophthalmic artery and thus worsen her vision. RRH may present in the paediatric age group, and optic nerve atrophy is one of the disease manifestations. OCT-A is a less invasive diagnostic option compared to fundus fluorescein angiography (FFA) for diagnosis and monitoring of disease progression. Cureus 2021-08-31 /pmc/articles/PMC8483998/ /pubmed/34646671 http://dx.doi.org/10.7759/cureus.17620 Text en Copyright © 2021, Ahmad Tarmizi et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Ophthalmology
Ahmad Tarmizi, Nur Nadia
Bastion, Mae-Lynn Catherine
Abdul Aziz, Roslin Azni
Md Din, Norshamsiah
Mohd Khialdin, Safinaz
Retinal Racemose Hemangioma With Optic Neuropathy in a Child
title Retinal Racemose Hemangioma With Optic Neuropathy in a Child
title_full Retinal Racemose Hemangioma With Optic Neuropathy in a Child
title_fullStr Retinal Racemose Hemangioma With Optic Neuropathy in a Child
title_full_unstemmed Retinal Racemose Hemangioma With Optic Neuropathy in a Child
title_short Retinal Racemose Hemangioma With Optic Neuropathy in a Child
title_sort retinal racemose hemangioma with optic neuropathy in a child
topic Ophthalmology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8483998/
https://www.ncbi.nlm.nih.gov/pubmed/34646671
http://dx.doi.org/10.7759/cureus.17620
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