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A Treatment Approach in Congenital Fibrosis of Extraocular Muscles

BACKGROUND: Congenital fibrosis of extraocular muscles ( CFEOM) is a group of genetically defined eye-moving disorders. The syndrome is clinically characterized by congenital non-progressive ophthalmoplegia caused by dysinervation of the cranial nerves with or without ptosis. As a main sign of a CFE...

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Autores principales: Mravicic, Ivana, Lukacevic, Selma, Biscevic, Alma, Pjano, Melisa Ahmedbegovic, Ziga, Nina, Tusek, Mateja
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Academy of Medical Sciences of Bosnia and Herzegovina 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10227850/
https://www.ncbi.nlm.nih.gov/pubmed/37260807
http://dx.doi.org/10.5455/medarh.2023.77.137-141
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author Mravicic, Ivana
Lukacevic, Selma
Biscevic, Alma
Pjano, Melisa Ahmedbegovic
Ziga, Nina
Tusek, Mateja
author_facet Mravicic, Ivana
Lukacevic, Selma
Biscevic, Alma
Pjano, Melisa Ahmedbegovic
Ziga, Nina
Tusek, Mateja
author_sort Mravicic, Ivana
collection PubMed
description BACKGROUND: Congenital fibrosis of extraocular muscles ( CFEOM) is a group of genetically defined eye-moving disorders. The syndrome is clinically characterized by congenital non-progressive ophthalmoplegia caused by dysinervation of the cranial nerves with or without ptosis. As a main sign of a CFEOM, extraocular muscles get shrunken and fibrotic, which makes surgery more technically demanding and the result more unpredictable, which makes the treatment challenging and highly customized. Our paper presents variations of the clinical picture and treatment cases of CFEOM1. OBJECTIVE: To outline the importance of the clinical examination with the exact measurement of deviations for the patients with ocular fibrosis and passive duction test under general anesthesia, establishing them as the main criteria for treatment. METHODS: We treated seven patients (14 eyes) with CFEOM1. The decision of the treatment was based on the measurement of the eye position in the primary position (PP), the severity of compensatory head position (CHP), restriction of motility, and passive motility test performed before surgery in general anesthesia. In 3 cases, patients were treated conservatively with the treatment of refractive error and amblyopia. However, in 4 patients, CHP and position of the eyes in PP were not acceptable, motility was severely impaired, and patients underwent surgery. The first surgery was performed on eye muscles: recession of inferior rectus muscle (IRM), anteposition, and resection of superior rectus muscle (SRM). As a second step procedure, ptosis surgery was performed. When the muscle was too tight, and it wasn’t possible to have a satisfying result with conventional surgery, we used a tissue expander to improve the position and motility of the affected eyes. RESULTS: In all operated cases, CHP has significantly improved and the position of the eyes in PP. CONCLUSION: Exact eye and head position measurements and a passive motility test during general anesthesia should guide the surgery. In the case when conventional surgery is not possible, implantation of a bovine pericard is a safe and effective method.
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spelling pubmed-102278502023-05-31 A Treatment Approach in Congenital Fibrosis of Extraocular Muscles Mravicic, Ivana Lukacevic, Selma Biscevic, Alma Pjano, Melisa Ahmedbegovic Ziga, Nina Tusek, Mateja Med Arch Case Study BACKGROUND: Congenital fibrosis of extraocular muscles ( CFEOM) is a group of genetically defined eye-moving disorders. The syndrome is clinically characterized by congenital non-progressive ophthalmoplegia caused by dysinervation of the cranial nerves with or without ptosis. As a main sign of a CFEOM, extraocular muscles get shrunken and fibrotic, which makes surgery more technically demanding and the result more unpredictable, which makes the treatment challenging and highly customized. Our paper presents variations of the clinical picture and treatment cases of CFEOM1. OBJECTIVE: To outline the importance of the clinical examination with the exact measurement of deviations for the patients with ocular fibrosis and passive duction test under general anesthesia, establishing them as the main criteria for treatment. METHODS: We treated seven patients (14 eyes) with CFEOM1. The decision of the treatment was based on the measurement of the eye position in the primary position (PP), the severity of compensatory head position (CHP), restriction of motility, and passive motility test performed before surgery in general anesthesia. In 3 cases, patients were treated conservatively with the treatment of refractive error and amblyopia. However, in 4 patients, CHP and position of the eyes in PP were not acceptable, motility was severely impaired, and patients underwent surgery. The first surgery was performed on eye muscles: recession of inferior rectus muscle (IRM), anteposition, and resection of superior rectus muscle (SRM). As a second step procedure, ptosis surgery was performed. When the muscle was too tight, and it wasn’t possible to have a satisfying result with conventional surgery, we used a tissue expander to improve the position and motility of the affected eyes. RESULTS: In all operated cases, CHP has significantly improved and the position of the eyes in PP. CONCLUSION: Exact eye and head position measurements and a passive motility test during general anesthesia should guide the surgery. In the case when conventional surgery is not possible, implantation of a bovine pericard is a safe and effective method. Academy of Medical Sciences of Bosnia and Herzegovina 2023-04 /pmc/articles/PMC10227850/ /pubmed/37260807 http://dx.doi.org/10.5455/medarh.2023.77.137-141 Text en © 2023 Ivana Mravicic, Selma Lukacevic, Alma Biscevic, Melisa Ahmedbegovic Pjano, Nina Ziga, Mateja Tusek https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) ) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Study
Mravicic, Ivana
Lukacevic, Selma
Biscevic, Alma
Pjano, Melisa Ahmedbegovic
Ziga, Nina
Tusek, Mateja
A Treatment Approach in Congenital Fibrosis of Extraocular Muscles
title A Treatment Approach in Congenital Fibrosis of Extraocular Muscles
title_full A Treatment Approach in Congenital Fibrosis of Extraocular Muscles
title_fullStr A Treatment Approach in Congenital Fibrosis of Extraocular Muscles
title_full_unstemmed A Treatment Approach in Congenital Fibrosis of Extraocular Muscles
title_short A Treatment Approach in Congenital Fibrosis of Extraocular Muscles
title_sort treatment approach in congenital fibrosis of extraocular muscles
topic Case Study
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10227850/
https://www.ncbi.nlm.nih.gov/pubmed/37260807
http://dx.doi.org/10.5455/medarh.2023.77.137-141
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