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Case Report: A Neuro-Ophthalmological Assessment of Vision Loss in a Pediatric Case of McCune-Albright Syndrome

Patients diagnosed with McCune-Albright Syndrome (MAS) frequently manifest craniofacial fibrous dysplasia (FD). Craniofacial FD can impinge nerve fibers causing visual loss as well as craniofacial pain. Surgical decompression of affected nerves is performed, with variable efficacy, in an attempt to...

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Autores principales: Lemme, Jordan D., Tucker-Bartley, Anthony, Drubach, Laura A., Shah, Nehal, Romo, Laura, Cay, Mariesa, Voss, Stephan, Kwatra, Neha, Kaban, Leonard B., Hassan, Adam S., Boyce, Alison M., Upadhyay, Jaymin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8964938/
https://www.ncbi.nlm.nih.gov/pubmed/35372387
http://dx.doi.org/10.3389/fmed.2022.857079
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author Lemme, Jordan D.
Tucker-Bartley, Anthony
Drubach, Laura A.
Shah, Nehal
Romo, Laura
Cay, Mariesa
Voss, Stephan
Kwatra, Neha
Kaban, Leonard B.
Hassan, Adam S.
Boyce, Alison M.
Upadhyay, Jaymin
author_facet Lemme, Jordan D.
Tucker-Bartley, Anthony
Drubach, Laura A.
Shah, Nehal
Romo, Laura
Cay, Mariesa
Voss, Stephan
Kwatra, Neha
Kaban, Leonard B.
Hassan, Adam S.
Boyce, Alison M.
Upadhyay, Jaymin
author_sort Lemme, Jordan D.
collection PubMed
description Patients diagnosed with McCune-Albright Syndrome (MAS) frequently manifest craniofacial fibrous dysplasia (FD). Craniofacial FD can impinge nerve fibers causing visual loss as well as craniofacial pain. Surgical decompression of affected nerves is performed, with variable efficacy, in an attempt to restore function or alleviate symptoms. Here, we present a case of a 12-year-old MAS patient with visual deficits, particularly in the left eye (confirmed by enlarged blind spots on Goldmann visual field testing), and craniofacial pain. Decompression surgery of the left optic nerve mildly improved vision, while persistent visual deficits were noted at a 3-month follow-up assessment. An in-depth, imaging-based evaluation of the visual system, including the retinal nerve fiber layer, optic nerves, and central nervous system (CNS) visual pathways, revealed multiple abnormalities throughout the visual processing stream. In the current FD/MAS patient, a loss of white matter fiber density within the left optic radiation and functional changes involving the left primary visual cortex were observed. Aberrant structural and functional abnormalities embedded within central visual pathways may play a role in facilitating deficits in vision in FD/MAS and contribute to the variable outcome following peripheral nerve decompression surgery.
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spelling pubmed-89649382022-03-31 Case Report: A Neuro-Ophthalmological Assessment of Vision Loss in a Pediatric Case of McCune-Albright Syndrome Lemme, Jordan D. Tucker-Bartley, Anthony Drubach, Laura A. Shah, Nehal Romo, Laura Cay, Mariesa Voss, Stephan Kwatra, Neha Kaban, Leonard B. Hassan, Adam S. Boyce, Alison M. Upadhyay, Jaymin Front Med (Lausanne) Medicine Patients diagnosed with McCune-Albright Syndrome (MAS) frequently manifest craniofacial fibrous dysplasia (FD). Craniofacial FD can impinge nerve fibers causing visual loss as well as craniofacial pain. Surgical decompression of affected nerves is performed, with variable efficacy, in an attempt to restore function or alleviate symptoms. Here, we present a case of a 12-year-old MAS patient with visual deficits, particularly in the left eye (confirmed by enlarged blind spots on Goldmann visual field testing), and craniofacial pain. Decompression surgery of the left optic nerve mildly improved vision, while persistent visual deficits were noted at a 3-month follow-up assessment. An in-depth, imaging-based evaluation of the visual system, including the retinal nerve fiber layer, optic nerves, and central nervous system (CNS) visual pathways, revealed multiple abnormalities throughout the visual processing stream. In the current FD/MAS patient, a loss of white matter fiber density within the left optic radiation and functional changes involving the left primary visual cortex were observed. Aberrant structural and functional abnormalities embedded within central visual pathways may play a role in facilitating deficits in vision in FD/MAS and contribute to the variable outcome following peripheral nerve decompression surgery. Frontiers Media S.A. 2022-03-15 /pmc/articles/PMC8964938/ /pubmed/35372387 http://dx.doi.org/10.3389/fmed.2022.857079 Text en Copyright © 2022 Lemme, Tucker-Bartley, Drubach, Shah, Romo, Cay, Voss, Kwatra, Kaban, Hassan, Boyce and Upadhyay. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Medicine
Lemme, Jordan D.
Tucker-Bartley, Anthony
Drubach, Laura A.
Shah, Nehal
Romo, Laura
Cay, Mariesa
Voss, Stephan
Kwatra, Neha
Kaban, Leonard B.
Hassan, Adam S.
Boyce, Alison M.
Upadhyay, Jaymin
Case Report: A Neuro-Ophthalmological Assessment of Vision Loss in a Pediatric Case of McCune-Albright Syndrome
title Case Report: A Neuro-Ophthalmological Assessment of Vision Loss in a Pediatric Case of McCune-Albright Syndrome
title_full Case Report: A Neuro-Ophthalmological Assessment of Vision Loss in a Pediatric Case of McCune-Albright Syndrome
title_fullStr Case Report: A Neuro-Ophthalmological Assessment of Vision Loss in a Pediatric Case of McCune-Albright Syndrome
title_full_unstemmed Case Report: A Neuro-Ophthalmological Assessment of Vision Loss in a Pediatric Case of McCune-Albright Syndrome
title_short Case Report: A Neuro-Ophthalmological Assessment of Vision Loss in a Pediatric Case of McCune-Albright Syndrome
title_sort case report: a neuro-ophthalmological assessment of vision loss in a pediatric case of mccune-albright syndrome
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8964938/
https://www.ncbi.nlm.nih.gov/pubmed/35372387
http://dx.doi.org/10.3389/fmed.2022.857079
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