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Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre

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Fledelius, Hans Callø ; Danielsen, Patricia Louise ; Ullman, Susanne. / Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre. I: Eye. 2018 ; Bind 32, Nr. 11. s. 1688-1696.

Bibtex

@article{90983701d9e749e9b1a599ba83088d88,
title = "Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre",
abstract = "BACKGROUND: To evaluate ophthalmic involvement in a long-term series of patients with en coup de sabre (ECS) close to the eye based on the hypothesis that this is not commonly affected, or simply under-reported.METHODS: An observational study of ophthalmological findings in patients from Copenhagen University Dermatology Clinics. A standard eye examination further included exophthalmometry, axial length and keratometry (IOLMaster), and horizontal eye muscle thickness (B-scan ultrasonography).RESULTS: Thirty-one consecutive patients were included from 2014 to 2015 (25 females, 6 males; median age, 33 years; range, 11-71 years). Twenty-seven patients had undergone ophthalmic evaluation more than once (observation time, 1-31 years; median, 7 years). Most eyes were normal or had currently adapted to eventual adnexal lesions and to insidious changes in eye position and/or motility. However, significant ipsilateral complications had developed related to 8 eyes, where two patients had more than one disorder. The ophthalmic pathologies were: blind eye (n = 2) due to adult age keratopathy/perforation and to Coats-like retinal detachment in childhood; restricted eye motility and diplopia (n = 2); acquired corneal astigmatism (n = 2); and dense cataract with light sense only (n = 1). Two patients had optic neuritis-like presentations, and lacrimal sac pathology occurred in one.CONCLUSIONS: The main ophthalmic focus possibly explained the high proportion of significant lesions in this patient series (in 8 of 31). In addition to the established feature of enophthalmos, the oculometric evidence suggested smaller eye and rectus muscle involvement, interpreted as a secondary (late) negative trophic effect of the overlying skin disorder on eye structures.",
author = "Fledelius, {Hans Call{\o}} and Danielsen, {Patricia Louise} and Susanne Ullman",
year = "2018",
month = "11",
doi = "10.1038/s41433-018-0137-9",
language = "English",
volume = "32",
pages = "1688--1696",
journal = "Eye",
issn = "0950-222X",
publisher = "Nature Publishing Group",
number = "11",

}

RIS

TY - JOUR

T1 - Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre

AU - Fledelius, Hans Callø

AU - Danielsen, Patricia Louise

AU - Ullman, Susanne

PY - 2018/11

Y1 - 2018/11

N2 - BACKGROUND: To evaluate ophthalmic involvement in a long-term series of patients with en coup de sabre (ECS) close to the eye based on the hypothesis that this is not commonly affected, or simply under-reported.METHODS: An observational study of ophthalmological findings in patients from Copenhagen University Dermatology Clinics. A standard eye examination further included exophthalmometry, axial length and keratometry (IOLMaster), and horizontal eye muscle thickness (B-scan ultrasonography).RESULTS: Thirty-one consecutive patients were included from 2014 to 2015 (25 females, 6 males; median age, 33 years; range, 11-71 years). Twenty-seven patients had undergone ophthalmic evaluation more than once (observation time, 1-31 years; median, 7 years). Most eyes were normal or had currently adapted to eventual adnexal lesions and to insidious changes in eye position and/or motility. However, significant ipsilateral complications had developed related to 8 eyes, where two patients had more than one disorder. The ophthalmic pathologies were: blind eye (n = 2) due to adult age keratopathy/perforation and to Coats-like retinal detachment in childhood; restricted eye motility and diplopia (n = 2); acquired corneal astigmatism (n = 2); and dense cataract with light sense only (n = 1). Two patients had optic neuritis-like presentations, and lacrimal sac pathology occurred in one.CONCLUSIONS: The main ophthalmic focus possibly explained the high proportion of significant lesions in this patient series (in 8 of 31). In addition to the established feature of enophthalmos, the oculometric evidence suggested smaller eye and rectus muscle involvement, interpreted as a secondary (late) negative trophic effect of the overlying skin disorder on eye structures.

AB - BACKGROUND: To evaluate ophthalmic involvement in a long-term series of patients with en coup de sabre (ECS) close to the eye based on the hypothesis that this is not commonly affected, or simply under-reported.METHODS: An observational study of ophthalmological findings in patients from Copenhagen University Dermatology Clinics. A standard eye examination further included exophthalmometry, axial length and keratometry (IOLMaster), and horizontal eye muscle thickness (B-scan ultrasonography).RESULTS: Thirty-one consecutive patients were included from 2014 to 2015 (25 females, 6 males; median age, 33 years; range, 11-71 years). Twenty-seven patients had undergone ophthalmic evaluation more than once (observation time, 1-31 years; median, 7 years). Most eyes were normal or had currently adapted to eventual adnexal lesions and to insidious changes in eye position and/or motility. However, significant ipsilateral complications had developed related to 8 eyes, where two patients had more than one disorder. The ophthalmic pathologies were: blind eye (n = 2) due to adult age keratopathy/perforation and to Coats-like retinal detachment in childhood; restricted eye motility and diplopia (n = 2); acquired corneal astigmatism (n = 2); and dense cataract with light sense only (n = 1). Two patients had optic neuritis-like presentations, and lacrimal sac pathology occurred in one.CONCLUSIONS: The main ophthalmic focus possibly explained the high proportion of significant lesions in this patient series (in 8 of 31). In addition to the established feature of enophthalmos, the oculometric evidence suggested smaller eye and rectus muscle involvement, interpreted as a secondary (late) negative trophic effect of the overlying skin disorder on eye structures.

U2 - 10.1038/s41433-018-0137-9

DO - 10.1038/s41433-018-0137-9

M3 - Journal article

VL - 32

SP - 1688

EP - 1696

JO - Eye

JF - Eye

SN - 0950-222X

IS - 11

ER -

ID: 56291968