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Congenital primary aphakia.
Ernst, Julia; Medsinge, Anagha; Scanga, Hannah L; Hiasat, Jamila; Moore, William; Ali, Asim; Levin, Alex V; Stahl, Erin D; Nischal, Ken K.
Afiliación
  • Ernst J; Department of Ophthalmology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.
  • Medsinge A; Kittner Eye Center, University of North Carolina, Chapel Hill.
  • Scanga HL; Department of Ophthalmology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.
  • Hiasat J; Department of Ophthalmology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania.
  • Moore W; Pediatric Ophthalmology Services, Great Ormond Street Hospital for Children, London, United Kingdom.
  • Ali A; Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada.
  • Levin AV; Pediatric Ophthalmology and Ocular Genetics, Flaum Eye Institute, Pediatric Genetics, Golisano Children's Hospital, University of Rochester, Rochester, New York.
  • Stahl ED; Pediatric Ophthalmology, Children's Mercy Hospital, Kansas City, Missouri.
  • Nischal KK; Department of Ophthalmology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania. Electronic address: nischalkk@upmc.edu.
J AAPOS ; 26(1): 4.e1-4.e5, 2022 02.
Article en En | MEDLINE | ID: mdl-35051625
PURPOSE: To describe the natural history, management, and visual outcome in children with congenital primary aphakia (CPA). METHODS: This is a multicenter retrospective consecutive case series from five academic centers in England and North America. RESULTS: A total of 27 eyes of 14 patients were included (male:female, 1.7:1). Thirteen patients had bilateral CPA, and 1 patient had unilateral CPA. Mean age at diagnosis was 18 months (median, 21; range, 0.5-144). Of 11 patients who underwent genetic testing, 9 had FOXE3 pathogenic variants. In all patients, visual acuity at presentation was not better than fixing and following light. Typical findings included silvery appearance of the cornea with vascularization (96%), glaucoma (81%), iridocorneal adhesions (74%), optic nerve coloboma (55%), abnormal vitreous (33%), retinal detachment (30%), and aniridia with hypoplasia of ciliary body (19%). Surgical interventions in select patients included penetrating keratoplasty (PKP), glaucoma drainage device implantation, and cyclophotocoagulation (CPC). CONCLUSIONS: Eyes with corneal ectasia and a silvery appearance of the cornea with vascularization should alert the physician to the possibility of CPA. Glaucoma causes globe enlargement and may increase the risk of corneal perforation, but glaucoma is often refractory to medical treatment, and the threshold for surgical treatment should be low. PKP outcomes are very poor.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Afaquia / Presión Intraocular Tipo de estudio: Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Child / Female / Humans / Male Idioma: En Revista: J AAPOS Asunto de la revista: OFTALMOLOGIA / PEDIATRIA Año: 2022 Tipo del documento: Article Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Afaquia / Presión Intraocular Tipo de estudio: Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Child / Female / Humans / Male Idioma: En Revista: J AAPOS Asunto de la revista: OFTALMOLOGIA / PEDIATRIA Año: 2022 Tipo del documento: Article Pais de publicación: Estados Unidos