A case of Granulomatosis with Polyangiitis presenting with significant ocular cicatricial scarring and symblepharon formation

Purpose: To report a case of Granulomatosis with Polyangiitis presenting with rare findings of cicatricial entropion progressing to significant symblepharon and fornix obliteration. Observations: An otherwise healthy patient with initial presentation findings of lower lid entropion with minimal cica...

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Main Authors: Kevin Dunne, Muhammad Khalid
Format: Article
Language:English
Published: Elsevier 2016-12-01
Series:American Journal of Ophthalmology Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S245199361630041X
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author Kevin Dunne
Muhammad Khalid
author_facet Kevin Dunne
Muhammad Khalid
author_sort Kevin Dunne
collection DOAJ
description Purpose: To report a case of Granulomatosis with Polyangiitis presenting with rare findings of cicatricial entropion progressing to significant symblepharon and fornix obliteration. Observations: An otherwise healthy patient with initial presentation findings of lower lid entropion with minimal cicatricial changes and a corneal ulcer. Management with an entropion Jones procedure repair produced good early postoperative results. 2 months post entropion repair, this patient represented with rapid progression to severe corneal ulceration, symblepharon formation and total obliteration of lower fornix and near complete obliteration of upper fornix of the left eye with concurrent acute kidney injury. He fulfilled diagnostic criteria for Granulomatosis with Polyangiitis (GPA). Systemic immunosuppressive treatment with prednisone and cyclophosphamide allowed acute kidney injury to recover however progressive cicatricial scarring ensued. Conclusions and importance: Cicatricial entropion is a very rare presentation of GPA which can take years to progress however with surgical intervention, rapid recurrence and severe tarsal-conjunctival disease progression can ensue despite systemic immunosuppressive therapy. To prevent such unexpected surgical complication, we recommend a thorough systemic evaluation prior to consideration of lid surgery in any cases of cicatrising conjunctivitis presentation.
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spelling doaj.art-c01e23f8d1ab4246b59ccda6acd5bc6e2022-12-22T01:17:36ZengElsevierAmerican Journal of Ophthalmology Case Reports2451-99362016-12-014C111310.1016/j.ajoc.2016.06.010A case of Granulomatosis with Polyangiitis presenting with significant ocular cicatricial scarring and symblepharon formationKevin DunneMuhammad KhalidPurpose: To report a case of Granulomatosis with Polyangiitis presenting with rare findings of cicatricial entropion progressing to significant symblepharon and fornix obliteration. Observations: An otherwise healthy patient with initial presentation findings of lower lid entropion with minimal cicatricial changes and a corneal ulcer. Management with an entropion Jones procedure repair produced good early postoperative results. 2 months post entropion repair, this patient represented with rapid progression to severe corneal ulceration, symblepharon formation and total obliteration of lower fornix and near complete obliteration of upper fornix of the left eye with concurrent acute kidney injury. He fulfilled diagnostic criteria for Granulomatosis with Polyangiitis (GPA). Systemic immunosuppressive treatment with prednisone and cyclophosphamide allowed acute kidney injury to recover however progressive cicatricial scarring ensued. Conclusions and importance: Cicatricial entropion is a very rare presentation of GPA which can take years to progress however with surgical intervention, rapid recurrence and severe tarsal-conjunctival disease progression can ensue despite systemic immunosuppressive therapy. To prevent such unexpected surgical complication, we recommend a thorough systemic evaluation prior to consideration of lid surgery in any cases of cicatrising conjunctivitis presentation.http://www.sciencedirect.com/science/article/pii/S245199361630041XGranulomatosisWegner’sCicatricial entropionSymblepharon
spellingShingle Kevin Dunne
Muhammad Khalid
A case of Granulomatosis with Polyangiitis presenting with significant ocular cicatricial scarring and symblepharon formation
American Journal of Ophthalmology Case Reports
Granulomatosis
Wegner’s
Cicatricial entropion
Symblepharon
title A case of Granulomatosis with Polyangiitis presenting with significant ocular cicatricial scarring and symblepharon formation
title_full A case of Granulomatosis with Polyangiitis presenting with significant ocular cicatricial scarring and symblepharon formation
title_fullStr A case of Granulomatosis with Polyangiitis presenting with significant ocular cicatricial scarring and symblepharon formation
title_full_unstemmed A case of Granulomatosis with Polyangiitis presenting with significant ocular cicatricial scarring and symblepharon formation
title_short A case of Granulomatosis with Polyangiitis presenting with significant ocular cicatricial scarring and symblepharon formation
title_sort case of granulomatosis with polyangiitis presenting with significant ocular cicatricial scarring and symblepharon formation
topic Granulomatosis
Wegner’s
Cicatricial entropion
Symblepharon
url http://www.sciencedirect.com/science/article/pii/S245199361630041X
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