Published in Retina

Uveal Effusion Syndrome: A Case Report from Caroline Awh, MD

Rishi P. Singh, MD, FASRS

Rishi P. Singh, MD, FASRS

Caroline Awh, MD

Caroline Awh, MD

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Sit down with Rishi P. Singh, MD, FASRS, and Caroline Awh, MD, as they discuss a case of uveal effusion syndrome (UES).

In this episode of Evidence Based Retina, Rishi P. Singh, MD, FASRS, sits down with Caroline Awh, MD, a clinical Fellow in Ophthalmology at Massachusetts Eye and Ear Infirmary, to discuss a clinical case of uveal effusion syndrome (UES) and provides a comprehensive review of its diagnosis, classification, and management.

Case presentation: Uveal effusion syndrome

  • Patient background: A 56-year-old male with a history of type 2 diabetes presented with a 3-year history of gradually decreasing vision in both eyes
  • Clinical findings at presentation:
    • OD: VA was 20/150 with 360° choroidal effusions, inferior subretinal fluid (SRF), and focal subretinal fluid in the macula with axial length 21.90mm
    • OS: VA was 20/70 with shallower, but still present, choroidal effusion with axial length 21.92mm
  • Differential diagnosis: In cases like this, it is crucial to rule out other conditions, such as inflammatory causes (posterior scleritis, vasculitis), malignancies (melanoma, lymphoma, metastasis), hypotony, chronic rhegmatogenous retinal detachment (RRD), and drug toxicities.
  • Surgical management and outcome: He underwent a four-quadrant scleral window procedure in the right eye. He experienced near-complete resolution of choroidal effusions and central subretinal fluid by post-operative day 1, with continued improvement noted at the 2-month follow-up. Surgery for the left eye is planned.

Imaging from case

Figures 1 and 2: Fundus photographs OD and OS at presentation, respectively.
OD Fundus Baseline
Figure 1: Courtesy of Caroline Awh, MD.
OS Fundus Baseline
Figure 2: Courtesy of Caroline Awh, MD.
Figures 3 and 4: Optical coherence tomography (OCT) OD and OS at presentation, respectively.
OD Fundus Baseline
Figure 3: Courtesy of Caroline Awh, MD.
OS Fundus Baseline
Figure 4: Courtesy of Caroline Awh, MD.
Figures 5 and 6: Fluorescein angiography (FA) OD and OS at presentation, respectively. Both eyes demonstrated peripheral leakage attributed to diabetes.
OD Fundus Baseline
Figure 5: Courtesy of Caroline Awh, MD.
OS Fundus Baseline
Figure 6: Courtesy of Caroline Awh, MD.
Figure 7: B-scan ultrasonography OD and OS at presentation which can aid in determining axial length.
B-Scan OD and OS
Figure 7: Courtesy of Caroline Awh, MD.
Figures 8 and 9: Fundus photography of post-op day 1 and post-op month 2, respectively.
Post-op Day 1
Figure 8: Courtesy of Caroline Awh, MD.
Post-op Month 2
Figure 9: Courtesy of Caroline Awh, MD.

Classification of uveal effusion syndrome

Uveal effusion occurs when fluid accumulates in the suprachoroidal space, leading to exudative detachments in the choroid, ciliary body, and retina.1 UES diagnosis is utilized when no identifiable cause can be found. It is linked to nanophthalmos and increased scleral thickness.1
There are three types:1,2
  • Type 1: Characterized by nanopatholmic eyes and abnormal sclera. The average axial length is 16mm.
  • Type 2: Non-nanophthalmic eyes with abnormal sclera. These eyes have a more normal axial length but possess an abnormal sclera, often noted during surgery or pathology, with thickened or disorganized collagen fiber bundles.
  • Type 3: Non-nanophthalmic eyes with normal sclera (idiopathic). These may be related to the pachychoroid spectrum.
The majority of cases are bilateral and typically affect middle-aged males.1,2 For types 1 and 2, decreased scleral permeability creates a hyperosmotic gradient, leading to fluid retention,2 and as noted above, type 3 may be associated with the pachychoroid spectrum.3
Multimodal imaging is essential for making the diagnosis. Testing and imaging include:1
  • B-scan: Evaluates axial length, scleral thickness, and rules out malignancy
  • Ultrasound biomicroscopy (UBM): Shows thickening of the anterior choroid and subtle anterior effusions
  • A-scan: Measures axial length to identify nanopthalmos
  • FA: Shows no leakage in classic UES, helping to exclude uveitis or central serous retinopathy (CSR)
  • Indocyanine green angiography (ICG): May demonstrate dilated choroidal vessels.
  • OCT: Shows increased choroidal thickness and subretinal fluid

Check out a recent episode on the role of widefield imaging in diabetic retinopathy!

Management strategies for UES

Non-surgical management of UES success is generally limited, but options include steroids (oral, periocular, or topical) and prostaglandin analogs. Steroids have shown significant success, specifically in type 3 UES.2
Surgical management of UES includes:2,3
  • Vortex vein decompression: An older technique no longer commonly performed due to high risks of bleeding and amputation
  • Partial-thickness sclerectomy (scleral window): The most common surgical approach, designed to facilitate fluid outflow from the choroid through the sclera
  • Express shunt: A newer technique involving insertion into a sclerotomy to facilitate continuous drainage
  • Vitrectomy: May be used in combination with scleral windows, particularly in type 3 cases that are less responsive to windows alone
Most cases show resolution of choroidal effusions and reattachment of the retina, regardless of surgical technique. Sclerectomy is more effective for type 1 and type 2, but not for type 3.2,3 Recurrence can occur.4

Interested in seeing the sugery? Go to 6:55 in the video to watch the four-quadrant scleral window procedure.

  1. Zhou N, Yang L, Xu X, Wei W. Uveal Effusion Syndrome: Clinical Characteristics, Outcome of Surgical Treatment, and Histopathological Examination of the Sclera. Front Med (Lausanne). 2022;9:785444. Published 2022 Jun 9. doi:10.3389/fmed.2022.785444
  2. Shields CL, Roelofs K, Di Nicola M, Sioufi K, Mashayekhi A, Shields JA. Uveal effusion syndrome in 104 eyes: Response to corticosteroids - The 2017 Axel C. Hansen lecture. Indian J Ophthalmol. 2017;65(11):1093-1104. doi:10.4103/ijo.IJO_752_17
  3. Terubayashi Y, Morishita S, Kohmoto R, et al. Type III uveal effusion syndrome suspected to be related to pachychoroid spectrum disease: A case report. Medicine (Baltimore). 2020;99(31):e21441. doi:10.1097/MD.0000000000021441
  4. Uyama M, Takahashi K, Kozaki J, et al. Uveal effusion syndrome: clinical features, surgical treatment, histologic examination of the sclera, and pathophysiology. Ophthalmology. 2000;107(3):441-449. doi:10.1016/s0161-6420(99)00141-4
Rishi P. Singh, MD, FASRS
About Rishi P. Singh, MD, FASRS

Rishi P. Singh, MD, FASRS, is the Chair of the Department of Ophthalmology at Mass General Brigham, overseeing ophthalmology across Massachusetts Eye and Ear, Massachusetts General Hospital, Brigham and Women’s Hospital, and affiliated sites. He is also a Professor of Ophthalmology at Harvard Medical School.

Previously, Dr. Singh served as Vice President and Chief Medical Officer at Cleveland Clinic Martin Health in Stuart, Florida, and as a staff surgeon at the Cleveland Clinic, where he was also Professor of Ophthalmology at the Cleveland Clinic Lerner College of Medicine in Cleveland, Ohio. He received both his undergraduate degree in medical science and his medical degree from Boston University, completing his internship at Tufts University. Dr. Singh went on to complete his ophthalmology residency at the Massachusetts Eye and Ear Infirmary/Harvard Medical School and a medical and surgical vitreoretinal fellowship at the Cole Eye Institute at the Cleveland Clinic.

Dr. Singh specializes in the management of complex retinal diseases, including diabetic retinopathy, retinal vein occlusions, retinal detachment, and age-related macular degeneration. He has authored over 300 peer-reviewed publications, books, and book chapters and serves as Principal Investigator for numerous national and international clinical trials aimed at improving outcomes for patients with retinal diseases.

He is the founder and past president of the Retina World Congress, chairs some of the largest continuing medical education meetings in retina, and serves on editorial boards and review panels for major ophthalmology journals. His leadership has extended into digital innovation, having helped lead enterprise-wide implementation of clinical technologies including Epic modules, digital informed consent, and patient-facing kiosks.

Dr. Singh has received multiple accolades for his contributions to ophthalmic research and innovation, including the Alpha Omega Alpha Research Award, the American Society of Retina Specialists Young Investigator Award, and the J. Donald Gass Beacon of Sight Award. He also leads The Center for Ophthalmic Bioinformatics, a research initiative focused on leveraging big data and artificial intelligence to advance understanding and treatment of retinal disease.

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Caroline Awh, MD
About Caroline Awh, MD

Caroline Awh, MD, is a vitreoretinal surgical fellow at Massachusetts Eye and Ear, Harvard School of Medicine in Boston, MA. She received a BA in Anthropology and Biology from the Washington University in St. Louis and her Medical Degree from the University of Tennessee Health Science Center College of Medicine. She subsequently pursued an ophthalmology residency at the Cleveland Clinic Cole Eye Institute.

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