|Year : 2016 | Volume
| Issue : 8 | Page : 593-594
Diagnosis and a minimum effective management for nanophthalmic uveal effusion syndrome
Wei Jin1, Yaopeng Xu2, Wenjun Wang1, Anhuai Yang1
1 Eye Center, Renmin Hospital of Wuhan University, Wuhan, Hubei, P.R. China
2 Department of Urology, Wuhan General Hospital of Guangzhou Military Command, Wuhan, Hubei, P.R. China
|Date of Submission||25-Nov-2015|
|Date of Acceptance||28-Jul-2016|
|Date of Web Publication||30-Sep-2016|
Dr. Anhuai Yang
#238, Jiefang Road, Wuhan, Hubei
Source of Support: None, Conflict of Interest: None
Keywords: Full-thickness sclerectomy, lamina sclerectomy, minimum management, nanophthalmic uveal effusion syndrome
|How to cite this article:|
Jin W, Xu Y, Wang W, Yang A. Diagnosis and a minimum effective management for nanophthalmic uveal effusion syndrome. Indian J Ophthalmol 2016;64:593-4
A 38-year-old male presented with a 2-week history of sudden blurred vision (best-corrected visual acuity: 20/200) and pain in his right eye. Spectral domain optical coherence tomography (OCT) revealed focal thickening of the retinal pigment epithelium layer as leopard spots [Figure 1]a. Fundus examination demonstrated exudative retinal detachment and retinal vascular dilation [Figure 1]c whereas the contralateral eye remained unremarkable [Figure 1]b and d. B-scan ultrasonography showed a short axial length (17.3 mm). Fundus fluorescein angiography and indocyanine green angiography revealed leopard spots of granular hyperfluorescence in the posterior and inferior quadrants [Figure 1]e, which were increased with time and persisted until the late phase [Figure 1]f. On the basis of clinical findings, we diagnosed him and it revealed to be nanophthalmic uveal effusion syndrome (UES).  After 10 mg dexamethasone was applied intravenously, two-third thickness scleral flaps measuring 4 mm × 5 mm were performed at inferotemporal and inferonasal sites [Figure 2]a. Under them, the remaining sclerae were excised measuring 1 mm × 2 mm accordingly [Figure 2]b and c. Biopsy [Figure 2]d and pathologic evaluation revealed fibroblastic proliferation and hyaline degeneration of lamellar sclera [Figure 2]e and f. The effusion was not detected at least on 6-month follow-up [Figure 3], and the visual acuity was stabled at 20/100.
|Figure 1: A 38-year-old male with a 2-week history of sudden blurred vision and pain in his right eye. Preoperative fundus photography (c) revealed exudative retinal detachment and bilateral retinal vascular dilation and tortuosity. Spectral domain optical coherence tomography revealing focal thickening of the retinal pigment epithelium layer at the same locations as leopard spots (a) while fundus examination in the left eye was unremarkable (b and d). Fundus fluorescein angiography showed a leopard spot pattern of granular hyperfluorescence of the retinal pigment epithelium. Indocyanine green angiography was demonstrated diffusely granular-marked hyperfluorescence in the choroidal fluorescence in the 17-37 s after indocyanine green injection did not reveal any abnormality except retinal vein dilation in the left eye, but was seen in the posterior pole and inferior quadrants in the right eye (e) which increased with time and persisted until the late phase (25 min) as diffuse intense choroidal hyperfluorescence (f)|
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|Figure 2: Lamellar sclerotomy combined with full-thickness sclerectomy. At the equator of the two quadrants, we made a two-third thickness scleral flap measuring 4 mm × 5 mm to all the patients (a). Under the scleral flap, the remaining thickness of the sclera was excised in pieces measuring 1 mm × 2 mm at the lower sites according to the situation, and the choroid was exposed (b and c). Biopsy (d) and pathologic evaluation were performed which revealed fibroblastic proliferation and hyaline degeneration of lamellar sclera and episclera (e and f)|
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|Figure 3: The effusion was not detected at 6-month follow-up with spectral domain optical coherence tomography examination|
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| Discussion|| |
UES is an extremely rare disease that trends to follow a relapsing-remitting course. Nanophthalmic eyes characteristically have a relatively normal corneal diameter but a shallow anterior chamber.  At the time of the surgery, we noted the insertions of the rectus muscles, and the equator was located unusually anteriorly. The sclera was abnormally rigid and unusually thick. When sclerectomy was performed and the choroid was exposed, abundant serous suprachoroidal fluid was spontaneously released. After the surgery, ciliochoroidal detachment resolved, and the retinal detachment gradually resolved within 1 month. OCT displayed that subretinal fluid under macula was absorbed completely. Other treatments with high-dose systemic steroids or prostaglandins analogs have been described with rare success. , Thus, further studies are needed to explore the different causative mechanisms of UES, and to determine whether treatment can be tailored to a given patient.
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Conﬂicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]