BILATERAL NANOPHTHALMIC UVEAL EFFUSION SYNDROME: CLINICAL PRESENTATION AND SURGICAL MANAGEMENT Robin G. Abell, MBBS,* Nathan M. Kerr, MBChB,† Brendan J. Vote, FRANZCO*†

Purpose: To describe the clinical presentation and surgical management of bilateral uveal effusion syndrome in a type 1 diabetic patient with nanophthalmos. Methods: A 38-year-old man presented with decreased visual acuity. Fundus examination revealed exudative retinal detachment and choroidal folding. Ultrasound examination confirmed thickened sclera, choroidal effusion, and nanophthalmos. Partial thickness scleral windows and subscleral sclerectomy were performed. The patient returned 2 years postoperatively with the same presentation in the contralateral eye. Results: After subscleral sclerectomy, intraocular pressure remained stable and the patient recovered premorbid vision in both eyes. At 4 years of follow-up, the patient’s vision had remained stable without the need for further treatment. Conclusion: The association of uveal effusion syndrome with diabetes and other autoimmune disorders is unclear, warranting further research. Uveal effusion syndrome can be managed effectively by partial thickness scleral windows and sclerectomy with improvement and maintenance of visual acuity. RETINAL CASES & BRIEF REPORTS 0:1–5, 2013

procedures involving scleral resection.7–10 The abnormally thick sclera is thought to impair normal transcleral outflow of intraocular protein from the suprachoroidal space and compress the vortex veins, leading to congestion of choroidal veins.3,11 Intraocular fluid then accumulates, leading to exudative ciliochoroidal detachment.4 In our study, we present the clinical presentation, surgical management, and long-term follow-up of a 38-year-old man with nanophthalmic UES treated with subscleral sclerectomy.

From the *Launceston Eye Institute, Tasmania, Australia; and †Tasmanian Eye Institute, Tasmania, Australia.

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veal effusion syndrome (UES) is a rare clinical entity characterized by exudative choroidal detachments.1 There are multiple causes of uveal effusion, including inflammatory, hypotony, and surgical causes;2 however, UES is thought to be idiopathic and is a diagnosis of exclusion.3–5 It may be mistaken for ring melanoma, with several cases of circumferential uveal effusion enucleated for presumed ring melanoma.5 The pathogenesis of UES is thought to be due to an underlying abnormality of the sclera.3 Thickened sclera in these patients has been demonstrated on ultrasound and magnetic resonance imaging.6 This was also confirmed histologically in early studies after surgical

Case Report A 38-year-old man presented with a 2-week history of gradual deterioration of vision in his left eye, associated with a left temporal field defect and mild discomfort. He had a medical history of type 1 diabetes mellitus diagnosed 13 years ago and was on a novorapid infusion pump. His last glycosylated haemoglobin (HbA1c) was 8.1. His relevant ocular history included hypermetropia (+8.50 diopters) corrected with spectacles and amblyopia in his left eye. There was no history of phacomorphic glaucoma. He had regular diabetic retinopathy

None of the authors have any financial/conflicting interests to disclose. Reprint requests: Brendan J. Vote, Launceston Eye Institute, 36 Thistle Street West, Launceston 7250, Australia; e-mail: eye.vote@ bigpond.net.au

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screening, and before presentation, his best-corrected visual acuity was 6/9 in the right eye and 6/18 in the left eye. On examination, his best-corrected visual acuity was 6/9 in the right eye and 6/60 in the left eye. Both anterior chamber angles were shallow, and gonioscopy revealed Shaffer grade 2 angles in all quadrants of both eyes. Intraocular pressure was 21 mmHg in the right eye and 22 mmHg in the left eye. Fundus examination revealed extensive choroidal detachment with macular still attached and choroidal folding (Figure 1). There were no retinal breaks. Ultrasound examination of the left eye revealed extensive choroidal detachment with thickened sclera. The axial length was 19.91 mm. Surgical treatment was subsequently performed on the left eye under general anesthesia. Partial thickness scleral flap with subscleral sclerectomy was performed in all quadrants, and scleral specimens were sent for histopathology.3 Macroscopically, the sections of sclera were significantly thickened, ranging from 2.39 mm to 2.46 mm. Light microscopy demonstrated unremarkable superficial sclera, with regular and parallel orientation of collagen fibers (Figure 2, A and C). Sections of the deep sclera were abnormal, however, showing a haphazard orientation of thickened collagen fibers with loss of normal parallel fiber orientation (Figure 2, B and D). Alcian blue staining revealed increased stromal mucin in comparison with both control and superficial scleral tissue (Figure 2, E and F). Transmission electron microscopy was remarkable for interfibrillary deposits of glycosaminoglycans associated with multiple bundles of degenerative collagen fibrils. Collagen fibrils were irregular in diameter in both the superficial and the deep sclera; however, these were more regularly arranged in the superficial sclera. Collagen fibrils were also increased in diameter when compared with superficial scleral tissue. Approximately 2.5 years later, the patient returned with choroidal effusions in the right eye. Visual acuity was 6/18 in the right eye and had recovered to 6/12 in the left eye. Ultrasound examination revealed extensive choroidal detachment with thickened sclera (Figure 3). Intraocular pressure was not elevated. The same procedure was performed in the right eye with partial thickness scleral flaps and subscleral sclerectomy. There were no intraoperative or postoperative complications.

Surgical Technique A 360° conjunctival peritomy was performed at the limbus. The rectus muscles were isolated with muscle hooks and secured with sling sutures using 2-0 silk. Sclerectomy was performed at four sites, one in each quadrant. A partial thickness scleral flap

using a number 11 scalpel blade was made at the equator, equidistant from the insertions of the rectus muscles, measuring approximately 5 mm · 7 mm. Care was taken to avoid the vortex veins. Superior quadrant flaps are more easily dissected from the posterior margin toward the limbus. Conversely, inferior quadrant flaps are more easily dissected from anterior to posteriorly. Of note, the sclera is often very thick (1.5–2mm), and dissection needs to be much deeper than a standard trabeculectomy flap (Figure 4A). The scleral flap was reflected back, and full-thickness sclerectomy was performed within the scleral bed, measuring approximately 4 mm · 3 mm, exposing the underlying choroid (Figure 4A). This allowed an egress of sluggish and predominantly proteinaceous effusion fluid. Cautious collagen fiber dissection using 2.6-mm angled bevel-up crescent blade or hockey stick blade (Feather, Osaka, Japan) is needed as the choroid is approached and the deepest layer peels away easily from choroid (Figure 4B). Only after all 4 full-thickness sclerectomies were performed, were the scleral flaps excised and conjunctiva closed (Figure 4C).

Postoperative Clinical Course Uveal effusions had resolved by the 3-month postoperative clinical review in both eyes. Postoperatively, visual acuity was 6/180 in the right eye at 1 week, 6/60 at 4 weeks, and 6/24 at 3 months. At 1 year postoperatively (3.5 years after the surgery on his left eye), visual acuity was 6/12 in the right eye and 6/9 in the left eye. Anterior segment examination revealed early posterior subcapsular cataract in both eyes. Dilated fundus examination showed fully attached retinas in both eyes, but the presence of peripheral leopard spot pigmentary changes were worse in the left eye (Figure 1). There was a mild drusen, but no signs of diabetic retinopathy in either eye. Optical coherence tomography showed no thickening or choroidal folds.

Discussion The present study highlights the effective surgical management of UES in a nanophthalmic middle-aged man with type 1 diabetes. To date, there are no large clinical studies but only case series or individual cases. The pathogenesis of UES remains unclear.5 Although there are several possible mechanisms for

Fig. 1. Fundus photograph of the left eye at the time of presentation and after surgical management.

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Fig. 2. Light microscopy of superficial (A, C, and E) and deep sclera (B, D, and F). Sections (A) and (B) are stained with hematoxylin and eosin, (C) and (D) are under polarized light, and (E) and (F) are stained with Alcian blue.

C O L O R

the formation of UES, the main risk factors seem to be nanophthalmos and high hypermetropia.4 It also seems to have a middle-aged male preponderance and commonly occurs bilaterally.4,5,12–15 The association with diabetes may be a coincidence, although the coexistence of diabetes may be significant.3,5,16–17 There are no previous reports of diabetes in association with UES. One may hypothesize that the decrease in plasma protein levels, particularly albumin, because of the lack of endogenous insulin and renal loss of albumin in diabetic

nephropathy may alter the osmotic pressure gradients across the sclera and exacerbate the fluid retention within the suprachoroidal space.18–20 Uveal effusion syndrome has been described in association with renal failure, and the underlying mechanism may be similar to that of our hypothesis.21 However, our subject had normal plasma albumin levels and no signs of nephropathy. Alternative treatments, including medical management of UES, were not trialed in this case. Evidence shows that steroids are not effective.5 Surgical

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Fig. 3. Ultrasound examination of the right eye at the time of presentation. The detached membranes are thick, quite convex, and not attached at the optic disk. The sclera is also thickened. This differentiates choroidal from retinal detachment.

Fig. 4. Surgical management of effusion. A. Thickened sclera is noted during creation of scleral flap. B. Delicate peeling of the collagen fibers away from underlying choroid. C. Full-thickness sclerectomy beneath scleral flap showing underlying choroid.

management remains the definitive treatment with 83% success after the first operation and 96% after the second operation if it is required.5 The surgical management involving scleral windows and subscleral sclerectomy is proving to be effective in managing UES. Scleral thickening has been noted during surgery in case reports and case series and has been confirmed via histopathology of scleral biopsy.1,4,5,12 Scleral thickening has also been noted on magnetic resonance imaging.6 Although multiple studies suggest computed tomogrpahy and magnetic resonance imaging for accurate preoperative classification and to further evaluate the presence of scleral thickening, the authors believe that ultrasound alone can provide an accurate diagnosis of UES.15 Occasionally, despite surgery, the disease takes a relapsing and remitting course.5 Results in this case, however, have been good. To date, there is minimal evidence of diabetes mellitus or other medical disease contributing to the development or pathogenesis of UES. Early diagnosis, appropriate surgical management,

and careful follow-up are important in maintaining vision function in patients with UES. Key words: uveal effusion, sclerectomy, choroidal detachment. References 1. Harada T, Machida S, Fujiwara T, et al. Choroidal findings in idiopathic uveal effusion syndrome. Clin Ophthalmol 2011;5: 1599–1601. 2. Faulborn J. Sclerotomy in uveal effusion syndrome. Retina 1999;19:504–507. 3. Gass J. Uveal effusion syndrome: a new hypothesis concerning pathogenesis and technique of surgical treatment. Trans Am Ophthamol Soc 1983;81:246–260. 4. Uyama M, Takahashi K, Kozaki J, et al. Uveal effusion syndrome: clinical features, surgical treatment, histologic of the sclera, and pathophysiology. Ophthalmology 2000;107:441–449. 5. Elagouz M, Stanescu-Segall D, Jackson T. Uveal effusion syndrome. Surv Ophthalmol 2010;55:134–145. 6. Lam A, Sambursky R, Maguire J. Measurement of scleral thickness in uveal effusion syndrome. Am J Ophthalmol 2005;140:329–331.

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SCLERECTOMY FOR UVEAL EFFUSION SYNDROME 7. Ward R, Gragoudas E, Pon D, et al. Abnormal scleral findings in uveal effusion syndrome. Am J Ophthalmol 1988;106:139–146. 8. Vine A. Uveal effusion in Hunter’s syndrome: evidence that abnormal sclera is responsible for uveal effusion syndrome. Retina 1986;6:57–60. 9. Stewart D, Streeten B, Brockhurst R, et al. Abnormal scleral collagen in nanophthalmos: an ultrastructural study. Arch Ophthalmol 1991;109:1017–1025. 10. Forrester J, Lee W, Kerr P, et al. The uveal effusion syndrome and trans-scleral flow. Eye 1990;4:354–365. 11. Jackson T, Hussain A, Morley A, et al. Scleral hydraulic conductivity and macromolecular diffusion in patients with uveal effusion syndrome. Invest Ophthalmol Vis Sci 2008;49:5033–5040. 12. Wu C, Dong F, Zhang H, et al. Diagnosis and treatment of uveal effusion syndrome: a case series and literature review. Chin Med Sci J 2011;26:231–236. 13. Roesel M, Heinz C, Heiligenhaus A. H1N1 and uveal effusion syndrome. Ophthalmology 2010;117:1467.e1. 14. Fledelius H, Fuchs H, Jensen P, et al. Uveal effusion and ultrasonic imaging: a clinical series. Acta Ophthalmol Scand 2002;80:202–210.

5 15. Park S, Song S. Castleman’s disease presenting with uveal effusion syndrome. Korean J Ophthalmol 2010;24: 182–185. 16. Johnson M, Gass J. Surgical management of the idiopathic uveal effusion syndrome. Ophthalmology 1990;97:778–785. 17. Casswell A, Gregor Z, Bird A. The surgical management of uveal effusion syndrome. Eye 1987;1:115–119. 18. Charlton J, Nair K. Protein metabolism in insulin-dependent diabetes mellitus. J Nutr 1998;128:323S–327S. 19. Folsom A, Ma J, Eckfeldt J, et al. Low serum albumin. Association with diabetes mellitus and other cardiovascular risk factors but not with prevalent cardiovascular disease or carotid artery intima-media thickness. Ann Epidemiol 1995;5:186–191. 20. Bhonsle H, Korwar A, Kote S, et al. Low plasma albumin levels are associated with increased plasma protein glycation and HbA1c in diabetes. J Proteome Res 2012;11:1391–1396. 21. 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;245:755–757.

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Bilateral nanophthalmic uveal effusion syndrome: clinical presentation and surgical management.

To describe the clinical presentation and surgical management of bilateral uveal effusion syndrome in a type 1 diabetic patient with nanophthalmos...
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