Investigative Ophthalmology & Visual Science Cover Image for Volume 65, Issue 7
June 2024
Volume 65, Issue 7
Open Access
ARVO Annual Meeting Abstract  |   June 2024
Histopathological Description of Sclera in Idiopathic Uveal Effusion Syndrome (IUES)
Author Affiliations & Notes
  • Sohaib Fasih-Ahmad
    Ocular Pathology, University of Miami Health System Bascom Palmer Eye Institute, Miami, Florida, United States
  • Charissa H. Tan
    Ocular Pathology, University of Miami Health System Bascom Palmer Eye Institute, Miami, Florida, United States
  • Sander R. Dubovy
    Ocular Pathology, University of Miami Health System Bascom Palmer Eye Institute, Miami, Florida, United States
  • Footnotes
    Commercial Relationships   Sohaib Fasih-Ahmad None; Charissa Tan None; Sander Dubovy None
  • Footnotes
    Support  Florida Lions Eye Bank
Investigative Ophthalmology & Visual Science June 2024, Vol.65, 5018. doi:
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      Sohaib Fasih-Ahmad, Charissa H. Tan, Sander R. Dubovy; Histopathological Description of Sclera in Idiopathic Uveal Effusion Syndrome (IUES). Invest. Ophthalmol. Vis. Sci. 2024;65(7):5018.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : IUES is a rare condition presenting with serous choroidal effusions and is commonly treated with sclerectomy. Histologic studies of IUES have demonstrated irregular sclera with accumulation of sulfated, acidic glycosaminoglycans (GAGs). Prior studies utilize Alcian blue to identify GAGs with inconsistent results. Colloidal iron is a less frequently used, but more sensitive stain for sulfated, acidic GAGs. In this series, we describe clinical and histologic findings of IUES, and utilize both Alcian blue and colloidal iron to describe the prevalence of GAG accumulation.

Methods : The Florida Lions Ocular Pathology Laboratory database was searched for specimens at the Bascom Palmer Eye Institute. Patients with clinical history of “idiopathic uveal effusion” with sclera submitted to pathology between 1997 and 2023 were included (n=23). Patient demographics and clinical features were evaluated. Histologic features including scleral collagen bundle morphology and thickness were evaluated with trichrome staining. GAG accumulation was evaluated with Alcian blue and colloidal iron staining on a scale of 0-3.

Results : In this series of 23 patients, 19 were male and 4 female. The mean age was 58.25 (range: 10-84). 4 patients had bilateral IUES, 3 of which were managed with bilateral sclerectomies and 1 was nanophthalmic. Four patients underwent repeat sclerectomy. Presurgical clinical data was available for 16 patients. The choroid was diffusely thickened on ultrasonography for 13 patients, RPE changes were identified in 8 cases, subretinal fluid was present in 9 cases, and 12 cases had exudative retinal detachments (ERDs), two of which were combined rhegmatogenous detachments. Thickened, irregular collagen bundles are identified in 29/30 specimens with trichrome staining. Mean grade for Alcian blue is 1.50 with 16 cases grading 1 or lower. Mean grade for colloidal iron is 2.57 with 1 case grading 1 or lower.

Conclusions : IUES primarily occurs in males and is associated with choroidal thickening, ERDs, RPE changes, and subretinal fluid. Thickened and irregular collagen bundles are present in most cases and highlighted with trichrome staining. Increased GAG accumulations are present and may be identified more easily with colloidal iron than with Alcian blue.

This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.

 

A and B: H&E and trichrome demonstrate irregular, thickened collagen bundles. C: Alcian blue (grade: 1) and D: Colloidal iron (grade: 3) demonstrate GAG accumulation

A and B: H&E and trichrome demonstrate irregular, thickened collagen bundles. C: Alcian blue (grade: 1) and D: Colloidal iron (grade: 3) demonstrate GAG accumulation

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