Glyxambi
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 
  • Users Online: 1891
  • Home
  • Print this page
  • Email this page


 
   Table of Contents      
PHOTO ESSAY
Year : 2016  |  Volume : 64  |  Issue : 8  |  Page : 593-594

Diagnosis and a minimum effective management for nanophthalmic uveal effusion syndrome


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 Submission25-Nov-2015
Date of Acceptance28-Jul-2016
Date of Web Publication30-Sep-2016

Correspondence Address:
Dr. Anhuai Yang
#238, Jiefang Road, Wuhan, Hubei
P.R. China
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0301-4738.191507

Rights and Permissions

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

How to cite this URL:
Jin W, Xu Y, Wang W, Yang A. Diagnosis and a minimum effective management for nanophthalmic uveal effusion syndrome. Indian J Ophthalmol [serial online] 2016 [cited 2019 Oct 15];64:593-4. Available from: http://www.ijo.in/text.asp?2016/64/8/593/191507

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). [1] 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)

Click here to view
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)

Click here to view
Figure 3: The effusion was not detected at 6-month follow-up with spectral domain optical coherence tomography examination

Click here to view



  Discussion Top


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. [2] 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. [3],[4] 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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Elagouz M, Stanescu-Segall D, Jackson TL. Uveal effusion syndrome. Surv Ophthalmol 2010;55:134-45.  Back to cited text no. 1
[PUBMED]    
2.
Jackson TL, Hussain A, Salisbury J, Sherwood R, Sullivan PM, Marshall J. Transscleral albumin diffusion and suprachoroidal albumin concentration in uveal effusion syndrome. Retina 2012;32:177-82.  Back to cited text no. 2
[PUBMED]    
3.
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.  Back to cited text no. 3
[PUBMED]    
4.
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  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Discussion
References
Article Figures

 Article Access Statistics
    Viewed835    
    Printed3    
    Emailed0    
    PDF Downloaded147    
    Comments [Add]    

Recommend this journal