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J AAPOS. 2017 Jun;21(3):251-254. doi: 10.1016/j.jaapos.2016.12.004. Epub 2017 May 18.

Craniofacial linear scleroderma associated with retinal telangiectasia and exudative retinal detachment.

Author information

1
Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom; Eye Hospital, University Medical Centre, Ljubljana, Slovenia.
2
Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester.
3
Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom.
4
Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom; Centre for Ophthalmology & Vision Sciences, Institute of Human Development, Faculty of Medical & Human Sciences, University of Manchester, Manchester. Electronic address: jane.ashworth@cmft.nhs.uk.

Abstract

Linear scleroderma is a characteristic form of scleroderma that typically affects children. Ocular manifestations may be present, especially when the frontoparietal area of the head is affected. We present the case of a 5-year-old boy with craniofacial linear scleroderma ("en coup de sabre") who developed exudative retinal detachment. Angiographic and neuroimaging findings are presented, and the importance of regular fundus examination is highlighted.

PMID:
28528991
DOI:
10.1016/j.jaapos.2016.12.004
[Indexed for MEDLINE]

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