A case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane

Abstract Purpose To report the first case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane in Chinese patient. Observations A 12-year-old female presented with bilateral visual decline. Examination revealed a porcelain-white optic nerve coloboma an...

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Main Authors: Bin Meng, Min Cui, Ang Ji
Format: Article
Language:English
Published: BMC 2025-07-01
Series:BMC Ophthalmology
Subjects:
Online Access:https://doi.org/10.1186/s12886-025-04243-0
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author Bin Meng
Min Cui
Ang Ji
author_facet Bin Meng
Min Cui
Ang Ji
author_sort Bin Meng
collection DOAJ
description Abstract Purpose To report the first case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane in Chinese patient. Observations A 12-year-old female presented with bilateral visual decline. Examination revealed a porcelain-white optic nerve coloboma and epiretinal membrane in the right eye. OCT confirmed deepened optic disc excavation and a thin epiretinal membrane. Visual field testing showed an inferonasal arcuate scotoma. Systemic findings included odontogenic keratocyst and cutaneous basal cell carcinomas. Genetic testing identified a heterozygous PTCH1 mutation. Conclusions and importance Optic nerve coloboma and epiretinal membrane, though rare, can be initial signs of nevoid basal cell carcinoma syndrome. Ophthalmologists are critical in identifying these manifestations, enabling early diagnosis and comprehensive management.
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spelling doaj-art-b4cab0c084eb46b0be070aec00eb778b2025-08-20T03:42:35ZengBMCBMC Ophthalmology1471-24152025-07-012511810.1186/s12886-025-04243-0A case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membraneBin Meng0Min Cui1Ang Ji2Department of Ophthalmology, Beijing Haidian Hospital (Haidian Section of Peking University Third Hospital)Department of Ophthalmology, Beijing Haidian Hospital (Haidian Section of Peking University Third Hospital)Department of Ophthalmology, Beijing Haidian Hospital (Haidian Section of Peking University Third Hospital)Abstract Purpose To report the first case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane in Chinese patient. Observations A 12-year-old female presented with bilateral visual decline. Examination revealed a porcelain-white optic nerve coloboma and epiretinal membrane in the right eye. OCT confirmed deepened optic disc excavation and a thin epiretinal membrane. Visual field testing showed an inferonasal arcuate scotoma. Systemic findings included odontogenic keratocyst and cutaneous basal cell carcinomas. Genetic testing identified a heterozygous PTCH1 mutation. Conclusions and importance Optic nerve coloboma and epiretinal membrane, though rare, can be initial signs of nevoid basal cell carcinoma syndrome. Ophthalmologists are critical in identifying these manifestations, enabling early diagnosis and comprehensive management.https://doi.org/10.1186/s12886-025-04243-0Nevoid basal cell carcinoma syndromeOptic nerve colobomaEpiretinal membraneCase report
spellingShingle Bin Meng
Min Cui
Ang Ji
A case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane
BMC Ophthalmology
Nevoid basal cell carcinoma syndrome
Optic nerve coloboma
Epiretinal membrane
Case report
title A case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane
title_full A case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane
title_fullStr A case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane
title_full_unstemmed A case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane
title_short A case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane
title_sort case of nevoid basal cell carcinoma syndrome associated with optic nerve coloboma and epiretinal membrane
topic Nevoid basal cell carcinoma syndrome
Optic nerve coloboma
Epiretinal membrane
Case report
url https://doi.org/10.1186/s12886-025-04243-0
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