Cargando…
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...
Autores principales: | , , , , , , , , , , , |
---|---|
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 |
_version_ | 1784678321380917248 |
---|---|
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. |
format | Online Article Text |
id | pubmed-8964938 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT lemmejordand casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT tuckerbartleyanthony casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT drubachlauraa casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT shahnehal casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT romolaura casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT caymariesa casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT vossstephan casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT kwatraneha casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT kabanleonardb casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT hassanadams casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT boycealisonm casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome AT upadhyayjaymin casereportaneuroophthalmologicalassessmentofvisionlossinapediatriccaseofmccunealbrightsyndrome |