Spherophakia and Ectopia Lentis in a Sturge-Weber Patient: A Case Report.
| Autor: | Avalos-Lara SJ; Department of Microsurgery of the Anterior Segment, Fundación Hospital Nuestra Señora de la Luz IAP, Cuauhtemoc, Mexico., Antonio-Aguirre B; Department of Microsurgery of the Anterior Segment, Fundación Hospital Nuestra Señora de la Luz IAP, Cuauhtemoc, Mexico.; Department of Molecular Biology and Biochemistry, Universidad Panamericana Escuela de Medicina, Benito Juarez, Mexico., Perez-Ortiz AC; Department of Public Health, Universidad Panamericana Escuela de Medicina, Benito Juarez, Mexico.; Department of Surgery, Massachusetts General Hospital, Boston, Massachusetts, USA., Mendoza Velásquez C; Department of Microsurgery of the Anterior Segment, Fundación Hospital Nuestra Señora de la Luz IAP, Cuauhtemoc, Mexico., Camacho-Ordoñez A; Department of Microsurgery of the Anterior Segment, Fundación Hospital Nuestra Señora de la Luz IAP, Cuauhtemoc, Mexico., Palacio Pastrana C; Department of Microsurgery of the Anterior Segment, Fundación Hospital Nuestra Señora de la Luz IAP, Cuauhtemoc, Mexico. |
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| Jazyk: | angličtina |
| Zdroj: | Case reports in ophthalmology [Case Rep Ophthalmol] 2020 Jul 14; Vol. 11 (2), pp. 356-363. Date of Electronic Publication: 2020 Jul 14 (Print Publication: 2020). |
| DOI: | 10.1159/000508064 |
| Abstrakt: | 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. Competing Interests: The authors have no conflicts of interest to declare. (Copyright © 2020 by S. Karger AG, Basel.) |
| Databáze: | MEDLINE |
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