Sonographic diagnosis of fetal eye anomalies and their association with syndromal diseases: A retrospective multicenter analysis of 264 cases

Abstract Introduction This study aims to systematically describe eye malformations and correlate these with extraocular findings. Based on these findings, we propose a protocol for ultrasound evaluation of the fetal eye. Material and Methods In this multicentric retrospective cohort study, 264 fetus...

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Main Authors: Jorge Jiménez Cruz, Paul Böckenhoff, Laura Tascón Padrón, Norah Emrich, Philipp Kosian, Brigitte Strizek, Cristoph Berg, Eva Weber, Ulrich Gembruch, Annegret Geipel
Format: Article
Language:English
Published: Wiley 2025-05-01
Series:Acta Obstetricia et Gynecologica Scandinavica
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Online Access:https://doi.org/10.1111/aogs.15085
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author Jorge Jiménez Cruz
Paul Böckenhoff
Laura Tascón Padrón
Norah Emrich
Philipp Kosian
Brigitte Strizek
Cristoph Berg
Eva Weber
Ulrich Gembruch
Annegret Geipel
author_facet Jorge Jiménez Cruz
Paul Böckenhoff
Laura Tascón Padrón
Norah Emrich
Philipp Kosian
Brigitte Strizek
Cristoph Berg
Eva Weber
Ulrich Gembruch
Annegret Geipel
author_sort Jorge Jiménez Cruz
collection DOAJ
description Abstract Introduction This study aims to systematically describe eye malformations and correlate these with extraocular findings. Based on these findings, we propose a protocol for ultrasound evaluation of the fetal eye. Material and Methods In this multicentric retrospective cohort study, 264 fetuses with ocular malformations from two tertiary referral centers for prenatal medicine were analyzed. Anophthalmia, microphthalmia, exophthalmos, hyper‐ or hypotelorism, cataract, aphakia, cyclopia, and retinal detachment were assessed, and their association with extraocular findings and genetic changes was investigated. Results The majority of the cases (99.2%) were non‐isolated and presented further extraocular findings. Most commonly, the brain and central nervous system (65.9%), the limbs and the heart (46.6% each) and the cranial anatomy (41.2%) were affected. Significant associations were found between exophthalmos and anomalies of the fetal skeletal system (OR = 4.8, 95% CI 1.6–14) and cranial malformations (OR = 3.3, 95% CI 1.5–7.4). Hypotelorism showed an increased risk of cardiac anomalies (OR = 1.8, 95% CI 1.1–3.5) and brain malformations (OR = 2.16, 95% CI 1.2–4.1), with holoprosencephaly being the most common one. Fetuses with microphthalmia were more likely to have anomalies in the renal system (OR = 2.3, 95% CI 1.2–4.3). In 51.4% of the cases, a genetic aberration could be found, among them most frequently trisomy 13. Conclusions There is a significant association between specific fetal eye anomalies and certain extraocular anomalies, as well as genetic changes. Systematic evaluation of the eye using the proposed protocol is simple to learn and highly reproducible and could help to concentrate diagnosis on a certain group of malformations. Data from this study could help to develop targeted diagnostic molecular tools.
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spelling doaj-art-14e4d34e7ef24dd18b3d91a8185e1fa92025-08-20T03:30:56ZengWileyActa Obstetricia et Gynecologica Scandinavica0001-63491600-04122025-05-01104585085910.1111/aogs.15085Sonographic diagnosis of fetal eye anomalies and their association with syndromal diseases: A retrospective multicenter analysis of 264 casesJorge Jiménez Cruz0Paul Böckenhoff1Laura Tascón Padrón2Norah Emrich3Philipp Kosian4Brigitte Strizek5Cristoph Berg6Eva Weber7Ulrich Gembruch8Annegret Geipel9Department of Obstetrics and Prenatal Medicine Bonn University Hospital Bonn GermanyDepartment of Obstetrics and Prenatal Medicine Bonn University Hospital Bonn GermanyDepartment of Obstetrics and Prenatal Medicine Bonn University Hospital Bonn GermanyDepartment of Obstetrics and Prenatal Medicine Bonn University Hospital Bonn GermanyDepartment of Obstetrics and Prenatal Medicine Bonn University Hospital Bonn GermanyDepartment of Obstetrics and Prenatal Medicine Bonn University Hospital Bonn GermanyDepartment of Obstetrics and Prenatal Medicine Bonn University Hospital Bonn GermanyDepartment of Obstetrics and Prenatal Medicine Cologne University Hospital Cologne GermanyDepartment of Obstetrics and Prenatal Medicine Bonn University Hospital Bonn GermanyDepartment of Obstetrics and Prenatal Medicine Bonn University Hospital Bonn GermanyAbstract Introduction This study aims to systematically describe eye malformations and correlate these with extraocular findings. Based on these findings, we propose a protocol for ultrasound evaluation of the fetal eye. Material and Methods In this multicentric retrospective cohort study, 264 fetuses with ocular malformations from two tertiary referral centers for prenatal medicine were analyzed. Anophthalmia, microphthalmia, exophthalmos, hyper‐ or hypotelorism, cataract, aphakia, cyclopia, and retinal detachment were assessed, and their association with extraocular findings and genetic changes was investigated. Results The majority of the cases (99.2%) were non‐isolated and presented further extraocular findings. Most commonly, the brain and central nervous system (65.9%), the limbs and the heart (46.6% each) and the cranial anatomy (41.2%) were affected. Significant associations were found between exophthalmos and anomalies of the fetal skeletal system (OR = 4.8, 95% CI 1.6–14) and cranial malformations (OR = 3.3, 95% CI 1.5–7.4). Hypotelorism showed an increased risk of cardiac anomalies (OR = 1.8, 95% CI 1.1–3.5) and brain malformations (OR = 2.16, 95% CI 1.2–4.1), with holoprosencephaly being the most common one. Fetuses with microphthalmia were more likely to have anomalies in the renal system (OR = 2.3, 95% CI 1.2–4.3). In 51.4% of the cases, a genetic aberration could be found, among them most frequently trisomy 13. Conclusions There is a significant association between specific fetal eye anomalies and certain extraocular anomalies, as well as genetic changes. Systematic evaluation of the eye using the proposed protocol is simple to learn and highly reproducible and could help to concentrate diagnosis on a certain group of malformations. Data from this study could help to develop targeted diagnostic molecular tools.https://doi.org/10.1111/aogs.15085fetal eyefetal syndromesfetal ultrasoundocular malformationsorbital malformations
spellingShingle Jorge Jiménez Cruz
Paul Böckenhoff
Laura Tascón Padrón
Norah Emrich
Philipp Kosian
Brigitte Strizek
Cristoph Berg
Eva Weber
Ulrich Gembruch
Annegret Geipel
Sonographic diagnosis of fetal eye anomalies and their association with syndromal diseases: A retrospective multicenter analysis of 264 cases
Acta Obstetricia et Gynecologica Scandinavica
fetal eye
fetal syndromes
fetal ultrasound
ocular malformations
orbital malformations
title Sonographic diagnosis of fetal eye anomalies and their association with syndromal diseases: A retrospective multicenter analysis of 264 cases
title_full Sonographic diagnosis of fetal eye anomalies and their association with syndromal diseases: A retrospective multicenter analysis of 264 cases
title_fullStr Sonographic diagnosis of fetal eye anomalies and their association with syndromal diseases: A retrospective multicenter analysis of 264 cases
title_full_unstemmed Sonographic diagnosis of fetal eye anomalies and their association with syndromal diseases: A retrospective multicenter analysis of 264 cases
title_short Sonographic diagnosis of fetal eye anomalies and their association with syndromal diseases: A retrospective multicenter analysis of 264 cases
title_sort sonographic diagnosis of fetal eye anomalies and their association with syndromal diseases a retrospective multicenter analysis of 264 cases
topic fetal eye
fetal syndromes
fetal ultrasound
ocular malformations
orbital malformations
url https://doi.org/10.1111/aogs.15085
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