The spectrum of uveal effusion syndrome, medical therapy, and surgical treatment

Rodrigo Anguita; Juan Pablo Marquez; Janice Roth; Alejandro Salinas; René Moya

doi:10.4103/pajo.pajo_64_20

ORIGINAL ARTICLE

Year : 2021 | Volume : 3 | Issue : 1 | Page : 4

The spectrum of uveal effusion syndrome, medical therapy, and surgical treatment

Rodrigo Anguita¹, Juan Pablo Marquez², Janice Roth¹, Alejandro Salinas³, René Moya³
¹ Department of Ophthalmology, Moorfields Eye Hospital NHS Foundation Trust, London, UK
² Ophthalmology, Hospital de Puerto Montt, Santiago, Chile
³ Department of Ophthalmology, Hospital del Salvador, Universidad de Chile, Santiago, Chile

Date of Submission	15-Nov-2020
Date of Acceptance	26-Nov-2020
Date of Web Publication	27-Jan-2021

Correspondence Address:
Dr. Rodrigo Anguita
162 City Road, London EC1V 2PD
UK

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_64_20

Abstract

Aims: The aim of the study is to describe and evaluate the clinical features, multimodal imaging, differential diagnoses, treatment options, and visual outcomes in patients with diagnosis of uveal effusion syndrome (UES).
Materials and Methods: This was a retrospective, observational case series of patients with diagnosis of UES. Variables analyzed included age, gender, medical history, initial and final best-corrected visual acuity (BCVA), clinical features, type of treatment, and follow-up.
Results: We report three cases, consisting of 2 males and 1 female, aged 37, 58, and 72 years with the diagnosis of UES. One patient was treated with medical therapy (acetazolamide) and the other two were submited to surgical treatment including one case of two-thirds thickness sclerotomy and one case with subscleral sclerectomy. All cases had complete resolution of subretinal fluid (SRF). BCVA improved in two cases, while there was no improvement of BCVA in one case due to outer retina atrophy as a consequence of chronic SRF.
Conclusions: The diagnosis of UES still remains a challenge for a clinician. It is essential to know the clinical features differential diagnosis and be familiarized with the different therapeutic approaches to establish accurate diagnosis and treatment.

Keywords: Choroidal diseases, isolated posterior uveal effusion, medical therapy, surgical treatment, uveal effusion syndrome

How to cite this article:
Anguita R, Marquez JP, Roth J, Salinas A, Moya R. The spectrum of uveal effusion syndrome, medical therapy, and surgical treatment. Pan Am J Ophthalmol 2021;3:4

How to cite this URL:
Anguita R, Marquez JP, Roth J, Salinas A, Moya R. The spectrum of uveal effusion syndrome, medical therapy, and surgical treatment. Pan Am J Ophthalmol [serial online] 2021 [cited 2021 Mar 8];3:4. Available from: https://www.thepajo.org/text.asp?2021/3/1/4/308110

Introduction

The term “uveal effusion” was first used by Schepens and Brockhurst in 1963 to define a disease characterized by spontaneous and exudative detachment of the choroid and the ciliary body.^[1] In 1982, Gass and Jallow reported a disorder characterized by exudative detachment of the choroid, ciliary body, and retina, which was not associated with ocular inflammation or tumors coining the term ïdiopathic uveal effusion syndrome (UES).”^[2] UES is a rare condition and its incidence and prevalence remain unknown. Typically, this disease is characterized by spontaneous serous detachment of the peripheral choroid, ciliary body, and retina.^[1],[2] Here, we report an small series of UES which shows the spectrum of the disease and their alternatives of treatment.

Materials and Methods

This was a retrospective, observational case series of patients with diagnosis of UES. Variables analyzed included age, gender, medical history, initial and final best-corrected visual acuity (BCVA), clinical features, type of treatment, and follow-up. The research protocol fulfilled the Declaration of Helsinki.

Results

Case 1

A 37-year-old man presented with low visual acuity in his right eye (RE) for 2 weeks. His BCVA in the RE was 6/60 (+13.75 sph) and in the left eye (LE) was 6/24 (+15 sph). Anterior-segment examination showed a narrow anterior chamber in both eyes (BE). Fundus examination revealed hyperemic optic disc, a shallow serous retinal detachment (RD) with a macular fold, and inferior peripheral choroidal detachment (CD) [Figure 1]a and [Figure 1]b in the RE and hyperemic optic disc, some yellowish lesions, minimal subretinal fluid (SRF), and pigmentary changes in the macula in the LE. Fluorescein angiography (FFA) revealed areas of hyperfluorescence and fluorescein leakage in BE and pooling of dye in the areas of serous RD in the RE [Figure 1]c. At that time, he was referred and reviewed in the retina department, his RE vision had decreased to hand movements, and a bullous RD was observed in the RE. B-scan showed a bullous RD [Figure 1]d, CD, and axial length (AL) of 11.8 mm in the RE and 12.2 mm in the LE. The diagnosis of UES was done and the RE was treated surgically. We made a two-third of thickness scleral flap measuring 4 mm × 5 mm, and we excised the remaining thickness of the sclera under the scleral flap in pieces measuring 3 mm × 4 mm and the choroid was exposed. The edges of the scleral wound were cauterized and the scleral flap was loosely sutured, as Uyama et al. described. One month later, without resolution of RD, two subscleral sclerectomies were performed but now at the equator in the upper quadrants. After 2 months of follow-up, visual acuity improved to 6/30 in the RE and 6/24 in the LE. There was a complete resolution of SRF in the RE, while LE remained stable with minimal SRF [Figure 2]a and [Figure 2]b. FFA revealed the typical leopard spots appearance of hyperfluorescence and hypofluorescence in the RE and some areas of fluorescein leakage in the LE [Figure 2]c and [Figure 2]d.

Figure 1: (a and b) Hyperemic optic nerve, shallow retinal detachment, macular fold, and inferior choroidal detachment in the right eye. (c) Fluorescein angiography of the right eye showing areas of hyperfluorescence, leakage, and pooling of dye in the areas of exudative detachment. (d) Ultrasound showing a bullous retinal detachment and choroidal thickening

Click here to view

Figure 2: (a) Optical coherence tomography after second surgery revealed retinal pigment epithelium thickening without evidence of subretinal fluid in the right eye; (b) minimum subretinal fluid was observed in th left eye. (c) Fluorescein angiography in the right eye showing the typical leopard-spot appearance and (d) the left eye showing some areas of staining which were observed in the periphery

Click here to view

Case 2

A 72-year-old woman presented with a 1-month history of slow and progressive decrease in visual acuity. Her BCVA in the RE was 6/9.5 (+2.25 sph) and the LE was 6/12 (+2.50 sph). Intraocular pressure was 12 and 14 mmHg in the RE and LE, respectively. Anterior-segment examination showed a narrow anterior chamber in BE. Fundus examination in BE demonstrated hyperemic optic nerve, temporal peripapillary crescents, SRF in the macula, choroidal folds, and retinal pigment epithelium (RPE) changes without CDs [Figure 3]a and [Figure 3]b. FFA revealed macular mottled hyperfluorescence in the arteriovenous phase, hyperfluorescence dots, and macular leakage at late stage in BE [Figure 3]c and [Figure 3]d. Enhanced depth imaging (EDI) optical coherence tomography (OCT) showed SRF, choroidal folds, and choroidal thickening [Figure 4]a and [Figure 4]b. B-scan revealed an AL of 22.14 mm in the RE and 22.11 mm in the LE [Figure 3]e and [Figure 3]f. Infectious origins and tumors were ruled out. The findings were in keeping with isolated posterior UES.

Figure 3: (a and b) Hyperamic optic nerve, choroidal folds and retinal pigment epithelium changes. (c and d) Fluorescein angiography revealed macular mottled hyperfluorescence in the arteriovenous phase. (e and f) Ultrasound showing choroidal thickening without evidence of choroidal detachment

Click here to view

Figure 4: (a and b) Optical coherence tomography at presentation showing subretinal fluid and choroidal folds. (c and d) Three months after treatment, mild subretinal fluid in the right eye and no subretinal fluid in the left eye with an increase of choroidal thickness and improvement of choroidal folds. (e and f) Twelve months follow-up showing complete resolution of subretinal fluid and choroidal folds

Click here to view

She was treated with oral acetazolamide 250 mg QID for 3 months and then a progressive tapering, resulting in a resolution of SRF and choroidal folds. There was an improvement of BCVA to 6/7.5 in BE, as a consequence of the resolution of SRF and choroidal folds [Figure 4]c,[Figure 4]d,[Figure 4]e,[Figure 4]f.

Case 3

A 58-year-old man presented with a bilateral and progressive visual deterioration over 2 years' time who had been diagnosed as chronic central serous chorioretinopathy (CSCR). His BCVA in the RE was count fingers (CF) (+8.00 sph) and LE was CF (+9.00 sph). Slit lamp examination showed a narrow anterior chamber in BE. Fundus examination demonstrated hyperemic OD, serous RD, CDs, and RPE changes [Figure 5]a,[Figure 5]b,[Figure 5]c,[Figure 5]d. Detailed OCT image showed SRF and intraretinal fluid because of chronic accumulation of the fluid [Figure 5]e and [Figure 5]f. B-scan revealed an AL of 20.49 mm in the RE and 20.51 mm in the LE with choroidal thickening. First, two subscleral sclerectomies were made at the equator in the lower quadrants. One month later, because of no response, subscleral sclerectomy was performed in the upper quadrant. There was no visual improvement or resolution of SRF. After a month, a third surgery was performed, removing scleral flap, and mitomycin C 2% was used. There was a complete resolution of SRF after 30 days; however, visual improvement was not observed due to outer retina atrophy despite the complete resolution of SRF [Figure 5]g and [Figure 5]h.

Figure 5: (a-d) Hyperemic optic nerves, subretinal fluid, and long-standing retinal pigment epithelium changes. (e and f) Optical coherence tomography showing subretinal fluid, intraretinal fluid, and schitic changes. (g and h) After third surgery, complete resolution of subretinal fluid and only a minimum intraretinal fluid in the left eye; however, bilateral outer retina atrophy was observed

Click here to view

Discussion

UES is an uncommon condition characterized by a relatively normal corneal diameter and narrow chambers, and some cases may be associated with the development of chronic angle-closure glaucoma. There are no inflammatory signs or hypotonia. Orange-brown color changes of the fundus and solid-looking serous retinal and ciliochoroidal detachments with secondary changes in the retinal pigment epithelium which have classically been described as leopard spots are common findings. These detachments usually start at the periphery, and often, ora serrata is visible without scleral depression.^[1],[2]

With respect to physiopathology of UES, it is widely known that, in normal sclera, collagen fibers are disposed parallel to the surface of the eyeball. In contrast, UES is characterized by a disorganization of sclera collagen fibers, abnormal deposition of proteoglycans in the scleral matrix, and scleral thickening. These abnormalities change the normal trans-scleral flux and compress vorticose veins producing the accumulation of fluid in the supra-choroidal space, producing exudative detachment of the uvea and retina in these patients.^[3],[4],[5]

Patients with UES have classically been divided into two groups: (1) hyperopic or nanophthalmic eye (AL <16 mm) and (2) idiopathic. However, a newer classification proposed by Uyama et al.^[4] classified UES patients in three subgroups according to their response to surgery: (a) Type 1 nanophthalmic eye (AL average 16 mm) and high hyperope; (b) Type 2 nonnanophthalmic eye (AL average 21 mm), small refractive error, and abnormal scleras; (c) Type 3 nonnanophthalmic eye (AL between 22.9 and 25 mm) and normal scleras. In 2014, Pautler and Browning reported a new spectrum of UES called isolated posterior uveal effusion. This entity was described as uveal effusion, which involves the posterior pole in the absence of peripheral cilioretinal effusion and peripheral serous RD with a good response to medical treatment. It is believed that, in this condition, the fluid passes through the disc margin to the intraretinal and subretinal space, and the presence of a temporal disc crescent may be an important predisposing factor.^[6] These features were presented in our Case 2 which had a complete resolution with the treatment.

The UES typically presents in the middle age. There are reports of patients from 20 to 80 years old. The idiopathic subtype has preponderance in males. This entity often presents bilaterally. However, unilateral cases could be observed in old males.^[7]

It is important to remember that the diagnosis of UES should be made after excluding other pathologies. Our efforts should focus on ruling out many of the differential diagnosis of this condition, such as hydrodynamic origin (ocular hypotonia, elevated uveal venous pressure), inflammatory origin (Vogt-Koyanagi-Harada, posterior scleritis, etc.), and neoplastic origin (choroidal melanoma, metastases, lymphoid infiltration of the choroid). In that sense, ophthalmic imaging results, including FFA, OCT, and B-scan, are essential for the diagnosis of UES. Ocular ultrasound allows us to measure the AL, visualize serous CD, estimate choroidal thickness, and rule out intraocular tumors, especially choroidal melanoma and choroidal metastasis. On the other hand, angiography is mostly useful in ruling out other pathologies. FFA reveals leopard skin fundus with dots of hypo- and hyperfluorescence. Indocyanine green angiography shows granular choroidal hyperfluorescence at early stages, indicating an increase permeability of choroidal vessels. This effect may persist in later stages and become more diffuse, revealing fluid accumulation in the choroid.^[4] The spectral-domain OCT is able to confirm serous RDs and focal thickening of RPE, which corresponds to leopard spots. In addition, EDI-OCT allows us to visualize the morphology of the choroid and the quantification of choroidal thickness which is typically increased in UES.^[8]

UES patients remain asymptomatic while the fluid does not involve macular region; hence, the majority of patients do not complain of low visual acuity. It is important to minimize the damage suffered by these patients due to chronic accumulation of fluid, as we can see in our Case 3 who was being treated for CSCR for several months. In that sense, treatment should be started immediately. With respect to the treatment, various options have been used from medical therapy to surgical treatments, having the latter better results. Among surgery procedures, we found: (1) decompression of vorticose veins, proposed by Brockhurst, which involves making sclerotomies around vorticose veins to evacuate the supra-choroidal fluid. This technique is difficult and is associated with bleeding complications;^[9] (2) partial sclerotomies in four quadrants, proposed by Johnson and Gass, which is the technique with the largest described series and with good results;^[10],[11] (3) subscleral sclerectomies in two quadrants, described by Uyama et al., who showed that the success of the surgery depends on the type of UES, being effective in Types 1 and 2, but not in Type 3 UES, which does not improve with this technique;^[4] (4) scleral windows and topical mitomycin-C have only been described in isolated reports.^[12],[13] We strongly believe that surgical treatment is a safe and effective treatment for UES, how was demonstrated in our Case 1 and 3. However, this should be started early, otherwise chronic damage of outer retina will be responsible for poor visual prognosis.

In terms of pharmacological therapy, there are only few cases reported using topical prostaglandin analogs, orally carbonic anhydrase inhibitors, or combination of both.^{[6],[7],[8],[9],[10],[11],[12],[13],[14]} Recently, Shields et al. reported a retrospective series of 104 eyes with UES treated with corticosteroids using various corticosteroid delivery routes, achieving control of UES in 95% of cases.^[15] Currently, the mechanism of corticosteroids for UES is unclear, and further investigations are needed to elucidate the role of steroids in the treatment of UES.

Conclusions

The diagnosis of UES should be done by exclusion of other similar conditions; therefore, it is absolutely essential to know the differential diagnosis to do an accurate and early treatment. Unfortunately, there are no clinical trials which allow us to know the best management. However, numerous small published series can be a guide to choose the best option to treat this uncommon disease.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Schepens CL, Brockhurst RJ. Uveal effusion. 1. Clinical picture. Arch Ophthalmol 1963;70:189-201.
2.	Gass JD, Jallow S. Idiopathic serous detachment of the choroid, ciliary body, and retina (uveal effusion syndrome). Ophthalmology 1982;89:1018-32.
3.	Trelstad RL, Silbermann NN, Brockhurst RJ. Nanophthalmic sclera. Ultrastructural, histochemical, and biochemical observations. Arch Ophthalmol 1982;100:1935-8.
4.	Uyama M, Takahashi K, Kozaki J, Tagami N, Takada Y, Ohkuma H, et al. Uveal effusion syndrome: Clinical features, surgical treatment, histologic examination of the sclera, and pathophysiology. Ophthalmology 2000;107:441-9.
5.	Gass JD. Uveal effusion syndrome: A new hypothesis concerning pathogenesis and technique of surgical treatment. Trans Am Ophthalmol Soc 1983;81:246-60.
6.	Pautler S, Browning D. Isolated posterior uveal effusion: expanding the spectrum of the uveal effusion syndrome. Clinical Ophthalmol 2015;9:43-9.
7.	Besirli CG, Johnson MW. Uveal effusion syndrome and hypotony maculopathy. In: Ryan SJ. Retina. Ch. 73. London: Elsevier; 2013. p. 1306-17.
8.	Harada T, Machida S, Fujiwara T, Nishida Y, Kurosaka D. Choroidal findings in idiopathic uveal effusion syndrome. Clin Ophthalmol 2011;5:1599-601.
9.	Brockhurst RJ. Vortex vein decompression for nanophthalmic uveal effusion. Arch Ophthalmol 1980;98:1987-90.
10.	Johnson MW, Gass JD. Surgical management of the idiopathic uveal effusion syndrome. Ophthalmology 1990;97:778-85.
11.	Jackson TL, Hussain A, Morley AM, Sullivan PM, Hodgetts A, El-Osta A, et al. Scleral hydraulic conductivity and macromolecular diffusion in patients with uveal effusion syndrome. Invest Ophthalmol Vis Sci 2008;49:5033-40.
12.	Akduman L, Adelberg DA, Del Priore LV. Nanophthalmic uveal effusion managed with scleral windows and topical mitomycin-C. Ophthalmic Surg Lasers 1997;28:325-7.
13.	Suzuki Y, Nishina S, Azuma N. Scleral window surgery and topical mitomycin C for nanophthalmic uveal effusion complicated by renal failure: Case report. Graefes Arch Clin Exp Ophthalmol 2007;245:755-7.
14.	Andrijević Derk B, Benčić G, Corluka V, Zorić Geber M, Vatavuk Z. Medical therapy for uveal effusion syndrome. Eye (Lond) 2014;28:1028-31.
15.	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;65:1093-104. [PUBMED] [Full text]