Clinical profile of Indonesian children with anterior segment dysgenesis as a contributor for childhood blindness

Background: Anterior segment dysgenesis (ASD) is an ocular malformation that can lead to childhood blindness. Although ASD prevalence is rare, it is potentially devastating for children and caretakers. We aim to report the clinical features of ASD patients in a tertiary referral center in Indonesia....

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Main Authors: Dian Estu Yulia, Febianza Mawaddah Putri, Diajeng Ayesha Soeharto
Format: Article
Language:English
Published: Elsevier 2023-03-01
Series:Clinical Epidemiology and Global Health
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2213398423000453
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author Dian Estu Yulia
Febianza Mawaddah Putri
Diajeng Ayesha Soeharto
author_facet Dian Estu Yulia
Febianza Mawaddah Putri
Diajeng Ayesha Soeharto
author_sort Dian Estu Yulia
collection DOAJ
description Background: Anterior segment dysgenesis (ASD) is an ocular malformation that can lead to childhood blindness. Although ASD prevalence is rare, it is potentially devastating for children and caretakers. We aim to report the clinical features of ASD patients in a tertiary referral center in Indonesia. Methods: We conducted a retrospective review of medical records of ASD patients in the Pediatric Ophthalmology clinic in Cipto Mangunkusumo National Central Public Hospital, Jakarta, Indonesia between January 1, 2018, and December 31, 2021. Results: A total of 172 eyes of 107 children with ASD were included in this study. Most of the patients were male, aged 3.4 years ±5.0 SD at first examination, 67.3% patients came from outside Jakarta, and 75.6% of eyes had bilateral ASD. Most common clinical manifestations was congenital corneal opacities (CCO), microcornea, and congenital glaucoma. The most common visual acuity (VA) was fixation and following light/object. Intraocular pressure (IOP) increased in 41% eyes. The most common surgery performed was glaucoma surgery, which was performed in 6.4% of eyes in the study. Conclusion: ASD has a devastating impact on children's VA, along with its poor prognosis despite surgery thus contributes largely to childhood blindness. ASD detection and management is highly challenging in children, and increased awareness on ASD is pivotal in earlier referral and treatment of ASD.
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spelling doaj.art-03dcaffd8c7f4a2e9435368e1279cc832023-04-10T04:03:59ZengElsevierClinical Epidemiology and Global Health2213-39842023-03-0120101258Clinical profile of Indonesian children with anterior segment dysgenesis as a contributor for childhood blindnessDian Estu Yulia0Febianza Mawaddah Putri1Diajeng Ayesha Soeharto2Corresponding author. Department of Ophthalmology, Faculty of Medicine University of Indonesia, Cipto Mangukusumo National Hospital, Jl. Kimia, No. 8, Jakarta, Indonesia.; Department of Ophthalmology, Faculty of Medicine, Universitas Indonesia/Cipto Mangunkusumo National Hospital, Jakarta, IndonesiaDepartment of Ophthalmology, Faculty of Medicine, Universitas Indonesia/Cipto Mangunkusumo National Hospital, Jakarta, IndonesiaDepartment of Ophthalmology, Faculty of Medicine, Universitas Indonesia/Cipto Mangunkusumo National Hospital, Jakarta, IndonesiaBackground: Anterior segment dysgenesis (ASD) is an ocular malformation that can lead to childhood blindness. Although ASD prevalence is rare, it is potentially devastating for children and caretakers. We aim to report the clinical features of ASD patients in a tertiary referral center in Indonesia. Methods: We conducted a retrospective review of medical records of ASD patients in the Pediatric Ophthalmology clinic in Cipto Mangunkusumo National Central Public Hospital, Jakarta, Indonesia between January 1, 2018, and December 31, 2021. Results: A total of 172 eyes of 107 children with ASD were included in this study. Most of the patients were male, aged 3.4 years ±5.0 SD at first examination, 67.3% patients came from outside Jakarta, and 75.6% of eyes had bilateral ASD. Most common clinical manifestations was congenital corneal opacities (CCO), microcornea, and congenital glaucoma. The most common visual acuity (VA) was fixation and following light/object. Intraocular pressure (IOP) increased in 41% eyes. The most common surgery performed was glaucoma surgery, which was performed in 6.4% of eyes in the study. Conclusion: ASD has a devastating impact on children's VA, along with its poor prognosis despite surgery thus contributes largely to childhood blindness. ASD detection and management is highly challenging in children, and increased awareness on ASD is pivotal in earlier referral and treatment of ASD.http://www.sciencedirect.com/science/article/pii/S2213398423000453Anterior segment dysgenesisVisual outcomeCorneal opacitiesIntraocular pressureChildhood blindness
spellingShingle Dian Estu Yulia
Febianza Mawaddah Putri
Diajeng Ayesha Soeharto
Clinical profile of Indonesian children with anterior segment dysgenesis as a contributor for childhood blindness
Clinical Epidemiology and Global Health
Anterior segment dysgenesis
Visual outcome
Corneal opacities
Intraocular pressure
Childhood blindness
title Clinical profile of Indonesian children with anterior segment dysgenesis as a contributor for childhood blindness
title_full Clinical profile of Indonesian children with anterior segment dysgenesis as a contributor for childhood blindness
title_fullStr Clinical profile of Indonesian children with anterior segment dysgenesis as a contributor for childhood blindness
title_full_unstemmed Clinical profile of Indonesian children with anterior segment dysgenesis as a contributor for childhood blindness
title_short Clinical profile of Indonesian children with anterior segment dysgenesis as a contributor for childhood blindness
title_sort clinical profile of indonesian children with anterior segment dysgenesis as a contributor for childhood blindness
topic Anterior segment dysgenesis
Visual outcome
Corneal opacities
Intraocular pressure
Childhood blindness
url http://www.sciencedirect.com/science/article/pii/S2213398423000453
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