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Spherophakia and Ectopia Lentis in a Sturge-Weber Patient: A Case Report

Sturge-Weber syndrome (SWS) is a rare, sporadic neurocutaneous disorder, primarily characterized by port-wine stain (PWS) over the ophthalmic division of the trigeminal nerve (V1) territory (hallmark feature) and glaucoma (in 30–60% of cases). Other ocular manifestations include episcleral involveme...

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Autores principales: Avalos-Lara, Samuel Josue, Antonio-Aguirre, Bani, Perez-Ortiz, Andric C., Mendoza Velásquez, Cristina, Camacho-Ordoñez, Azyadeh, Palacio Pastrana, Claudia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7443686/
https://www.ncbi.nlm.nih.gov/pubmed/32884550
http://dx.doi.org/10.1159/000508064
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author Avalos-Lara, Samuel Josue
Antonio-Aguirre, Bani
Perez-Ortiz, Andric C.
Mendoza Velásquez, Cristina
Camacho-Ordoñez, Azyadeh
Palacio Pastrana, Claudia
author_facet Avalos-Lara, Samuel Josue
Antonio-Aguirre, Bani
Perez-Ortiz, Andric C.
Mendoza Velásquez, Cristina
Camacho-Ordoñez, Azyadeh
Palacio Pastrana, Claudia
author_sort Avalos-Lara, Samuel Josue
collection PubMed
description Sturge-Weber syndrome (SWS) is a rare, sporadic neurocutaneous disorder, primarily characterized by port-wine stain (PWS) over the ophthalmic division of the trigeminal nerve (V1) territory (hallmark feature) and glaucoma (in 30–60% of cases). Other ocular manifestations include episcleral involvement of the PWS, choroidal vascular malformations, and iris heterochromia. Two previous reports also associated ectopia lentis concomitantly among these cases. However, here we report spherophakia as a novel ophthalmological finding in SWS. A 56-year-old female previously diagnosed with SWS presented to the outpatient clinic complaining of right-sided decreased visual acuity and pain after a fall. Phenotypically, the patient had a PWS around V1 territory and involvement of both eyelids. Previous relevant ocular history included retinal detachment without macular involvement, ocular hypertension, and phacodonesis. The slit-lamp examination showed anterior lens luxation and elevated intraocular pressure (IOP) of 40 mm Hg by tonometry. Prior to the surgical approach, the patient received hypotensive treatment for elevated IOP. After intracapsular lens extraction, measurements were consistent with spherophakia. Postoperatively, the patient underwent optical coherence tomography (OCT). There was cystic macular edema (CME) by OCT and a detached posterior hyaloid membrane. The patient fully recovered with topical treatment of bromfenac for CME. To the best of our knowledge, this is the first report of concomitant anterior lens luxation and spherophakia (novel association) in a SWS patient. Our findings supplement the differential ocular diagnoses in SWS and should be considered in the routine ocular exam, specifically of the anterior segment. CME occurred similar to otherwise healthy eyes. However, in this case, topical anti-inflammatory medications had a good response and were well-tolerated.
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spelling pubmed-74436862020-09-02 Spherophakia and Ectopia Lentis in a Sturge-Weber Patient: A Case Report Avalos-Lara, Samuel Josue Antonio-Aguirre, Bani Perez-Ortiz, Andric C. Mendoza Velásquez, Cristina Camacho-Ordoñez, Azyadeh Palacio Pastrana, Claudia Case Rep Ophthalmol Case Report Sturge-Weber syndrome (SWS) is a rare, sporadic neurocutaneous disorder, primarily characterized by port-wine stain (PWS) over the ophthalmic division of the trigeminal nerve (V1) territory (hallmark feature) and glaucoma (in 30–60% of cases). Other ocular manifestations include episcleral involvement of the PWS, choroidal vascular malformations, and iris heterochromia. Two previous reports also associated ectopia lentis concomitantly among these cases. However, here we report spherophakia as a novel ophthalmological finding in SWS. A 56-year-old female previously diagnosed with SWS presented to the outpatient clinic complaining of right-sided decreased visual acuity and pain after a fall. Phenotypically, the patient had a PWS around V1 territory and involvement of both eyelids. Previous relevant ocular history included retinal detachment without macular involvement, ocular hypertension, and phacodonesis. The slit-lamp examination showed anterior lens luxation and elevated intraocular pressure (IOP) of 40 mm Hg by tonometry. Prior to the surgical approach, the patient received hypotensive treatment for elevated IOP. After intracapsular lens extraction, measurements were consistent with spherophakia. Postoperatively, the patient underwent optical coherence tomography (OCT). There was cystic macular edema (CME) by OCT and a detached posterior hyaloid membrane. The patient fully recovered with topical treatment of bromfenac for CME. To the best of our knowledge, this is the first report of concomitant anterior lens luxation and spherophakia (novel association) in a SWS patient. Our findings supplement the differential ocular diagnoses in SWS and should be considered in the routine ocular exam, specifically of the anterior segment. CME occurred similar to otherwise healthy eyes. However, in this case, topical anti-inflammatory medications had a good response and were well-tolerated. S. Karger AG 2020-07-14 /pmc/articles/PMC7443686/ /pubmed/32884550 http://dx.doi.org/10.1159/000508064 Text en Copyright © 2020 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Case Report
Avalos-Lara, Samuel Josue
Antonio-Aguirre, Bani
Perez-Ortiz, Andric C.
Mendoza Velásquez, Cristina
Camacho-Ordoñez, Azyadeh
Palacio Pastrana, Claudia
Spherophakia and Ectopia Lentis in a Sturge-Weber Patient: A Case Report
title Spherophakia and Ectopia Lentis in a Sturge-Weber Patient: A Case Report
title_full Spherophakia and Ectopia Lentis in a Sturge-Weber Patient: A Case Report
title_fullStr Spherophakia and Ectopia Lentis in a Sturge-Weber Patient: A Case Report
title_full_unstemmed Spherophakia and Ectopia Lentis in a Sturge-Weber Patient: A Case Report
title_short Spherophakia and Ectopia Lentis in a Sturge-Weber Patient: A Case Report
title_sort spherophakia and ectopia lentis in a sturge-weber patient: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7443686/
https://www.ncbi.nlm.nih.gov/pubmed/32884550
http://dx.doi.org/10.1159/000508064
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