Nikolaos Gkalapis, Marie Elisabeth Burghardt, Christin Busse, Arne Viestenz, Mohammad Javed Ali, Jens Heichel
{"title":"Chronic Dacryocystitis With Elevated IgG4-Plasma Cells in a Patient With Rubinstein-Taybi Syndrome: An IgG4-Related Disease?","authors":"Nikolaos Gkalapis, Marie Elisabeth Burghardt, Christin Busse, Arne Viestenz, Mohammad Javed Ali, Jens Heichel","doi":"10.1097/IOP.0000000000003071","DOIUrl":null,"url":null,"abstract":"<p><p>Rubinstein-Taybi syndrome (RTS) is a rare congenital neurodevelopmental disorder associated with several ocular and lacrimal anomalies. A 40-year-old female patient with RTS presented with persistent right-sided epiphora following an earlier unsuccessful endoscopic dacryocystorhinostomy performed elsewhere. The patient underwent revision external dacryocystorhinostomy with fistulectomy and intubation. Intraoperatively, a focal polypoidal lacrimal sac lesion was noted, and histological analysis revealed significant immunoglobulin G4 (IgG4)-plasma cell infiltration. However, the diagnostic criteria for IgG4-related ophthalmic disease were not fulfilled. The revision external surgery was anatomically and functionally successful. To the best of the authors' knowledge, this is the first report of IgG4-plasma cell infiltration in the lacrimal apparatus of a patient with RTS.</p>","PeriodicalId":19588,"journal":{"name":"Ophthalmic Plastic and Reconstructive Surgery","volume":" ","pages":""},"PeriodicalIF":1.3000,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Ophthalmic Plastic and Reconstructive Surgery","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1097/IOP.0000000000003071","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"OPHTHALMOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Rubinstein-Taybi syndrome (RTS) is a rare congenital neurodevelopmental disorder associated with several ocular and lacrimal anomalies. A 40-year-old female patient with RTS presented with persistent right-sided epiphora following an earlier unsuccessful endoscopic dacryocystorhinostomy performed elsewhere. The patient underwent revision external dacryocystorhinostomy with fistulectomy and intubation. Intraoperatively, a focal polypoidal lacrimal sac lesion was noted, and histological analysis revealed significant immunoglobulin G4 (IgG4)-plasma cell infiltration. However, the diagnostic criteria for IgG4-related ophthalmic disease were not fulfilled. The revision external surgery was anatomically and functionally successful. To the best of the authors' knowledge, this is the first report of IgG4-plasma cell infiltration in the lacrimal apparatus of a patient with RTS.
期刊介绍:
Ophthalmic Plastic and Reconstructive Surgery features original articles and reviews on topics such as ptosis, eyelid reconstruction, orbital diagnosis and surgery, lacrimal problems, and eyelid malposition. Update reports on diagnostic techniques, surgical equipment and instrumentation, and medical therapies are included, as well as detailed analyses of recent research findings and their clinical applications.