Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature

Aim:  To present a case of linear scleroderma (LS) involving the extraocular muscles and periorbita with follow-up over an 18-year period. Method:  Initial presentation was of a female aged 9 years with progressive left unilateral ptosis which ultimately underwent surgical correction. Results:  The...

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Main Authors: Claire Taylor, Melanie Riley, Jeremy Bowyer
Format: Article
Language:English
Published: White Rose University Press 2010-08-01
Series:British and Irish Orthoptic Journal
Subjects:
Online Access:https://www.bioj-online.com/articles/31
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spelling doaj-81e86b7fd3254cc3acad188c684b590d2020-11-25T02:17:11ZengWhite Rose University PressBritish and Irish Orthoptic Journal2516-35902010-08-017727610.22599/bioj.3130Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literatureClaire Taylor0Melanie Riley1Jeremy Bowyer2Orthoptic Department, University Hospital of North Staffordshire, Stoke-on-TrentOrthoptic Department, University Hospital of North Staffordshire, Stoke-on-TrentOrthoptic Department, University Hospital of North Staffordshire, Stoke-on-TrentAim:  To present a case of linear scleroderma (LS) involving the extraocular muscles and periorbita with follow-up over an 18-year period. Method:  Initial presentation was of a female aged 9 years with progressive left unilateral ptosis which ultimately underwent surgical correction. Results:  The main features of ‘en coup de sabre’ subsequently manifested on the left side of the face and head. An unusual feature was the characteristic subcutaneous cleft which also appeared on the opposite side above the right eyebrow. The left eye went on to develop a corneal ulcer with resulting poor visual acuity. An ipsilateral oculomotility disorder was then noted in addition to poor eyelid closure. Conclusion:  This case highlights the need to exclude ‘en coup de sabre’ as a potential differential diagnosis in any case of progressive or recurrent ptosis, especially in childhood. As oculomotility may also be affected ultimately, it could lead to corneal compromise following ptosis correction.https://www.bioj-online.com/articles/31‘En coup de sabre’Oculomotility disorderPtosisScleroderma
collection DOAJ
language English
format Article
sources DOAJ
author Claire Taylor
Melanie Riley
Jeremy Bowyer
spellingShingle Claire Taylor
Melanie Riley
Jeremy Bowyer
Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature
British and Irish Orthoptic Journal
‘En coup de sabre’
Oculomotility disorder
Ptosis
Scleroderma
author_facet Claire Taylor
Melanie Riley
Jeremy Bowyer
author_sort Claire Taylor
title Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature
title_short Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature
title_full Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature
title_fullStr Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature
title_full_unstemmed Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature
title_sort linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature
publisher White Rose University Press
series British and Irish Orthoptic Journal
issn 2516-3590
publishDate 2010-08-01
description Aim:  To present a case of linear scleroderma (LS) involving the extraocular muscles and periorbita with follow-up over an 18-year period. Method:  Initial presentation was of a female aged 9 years with progressive left unilateral ptosis which ultimately underwent surgical correction. Results:  The main features of ‘en coup de sabre’ subsequently manifested on the left side of the face and head. An unusual feature was the characteristic subcutaneous cleft which also appeared on the opposite side above the right eyebrow. The left eye went on to develop a corneal ulcer with resulting poor visual acuity. An ipsilateral oculomotility disorder was then noted in addition to poor eyelid closure. Conclusion:  This case highlights the need to exclude ‘en coup de sabre’ as a potential differential diagnosis in any case of progressive or recurrent ptosis, especially in childhood. As oculomotility may also be affected ultimately, it could lead to corneal compromise following ptosis correction.
topic ‘En coup de sabre’
Oculomotility disorder
Ptosis
Scleroderma
url https://www.bioj-online.com/articles/31
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AT jeremybowyer linearsclerodermaencoupdesabrewithptosisandoculomotilitydisorderscasereportandreviewoftheliterature
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