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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 2  |  Issue : 1  |  Page : 28

Scleral resection in bilateral uveal effusion


Department of Retina, Caribbean Eye Clinic, Barranquilla, Colombia

Date of Submission27-Feb-2020
Date of Acceptance10-Mar-2020
Date of Web Publication20-Oct-2020

Correspondence Address:
Dr. Fernando DÍaz Castillo
Dr. Fernando DÍaz Castillo, Caribbean Eye Clinic, Barranquilla
Colombia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/PAJO.PAJO_10_20

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  Abstract 


Effective therapeutic options are limited to the management of exudative retinal detachments (RDs). The authors describe the resolution of the case following partial-thickness scleral resection. This 31-year-old male presented with a bilateral exudative RD. There was no history of steroid use either locally or systemically. The fundus eye showed typical of chronic exudative RDs. The axial length was 16.08 mm in the right eye and 15.17 mm in the left eye. Thickening of the sclera was evident in ocular ultrasound. Considering an axial length (nanophthalmic eye), four partial-thickness scleral resections were performed. The exudative RD resolved at 4 months. Partial-thickness scleral resections may be considered as an option for treating chronic exudative RDs patients with nanophthalmic eye.

Keywords: Exudative retinal detachments, nanophthalmos, sclerectomy, uveal effusion syndrome


How to cite this article:
Castillo FD, RamÍrez IA, Londoño CV. Scleral resection in bilateral uveal effusion. Pan Am J Ophthalmol 2020;2:28

How to cite this URL:
Castillo FD, RamÍrez IA, Londoño CV. Scleral resection in bilateral uveal effusion. Pan Am J Ophthalmol [serial online] 2020 [cited 2020 Nov 27];2:28. Available from: https://www.thepajo.org/text.asp?2020/2/1/28/298633




  Introduction Top


Uveal effusion syndrome (UES) is an extremely rare condition characterized by the presence of exudative ciliochoroidal detachment with or without a serious nonrhegmatogenous retinal detachment (RD).

The term was first coined by Gass and Jallow to describe spontaneous exudative detachment of the choroid, ciliary body, and retina.[1]

The pathophysiology of UES has been related to nanophthalmos with scleral abnormality and congestion of the choroidal venous system caused by compression of the vortex veins leading to subretinal fluid accumulation.[2]

Elagouz et al. reported that abnormalities of the sclera, including reduced scleral protein permeability, reduced scleral hydraulic conductivity, vortex vein compression, increased choroidal vessel permeability, chronic choroidal inflammation, and chronic hypotony, could cause UES.[3]

Gass and Jallow hypothesized that abnormal scleral composition may cause reduced scleral protein permeability, allowing for protein retention within the extravascular space, whereas vortex vein compression may cause increased intravascular pressure – two complementary processes that result in fluid accumulation within the suprachoroidal space, he has demonstrated the successful resolution of subretinal fluid in two such eyes within 3 months following sclerectomies and sclerostomies away from the vortex vein.[4]

There are multiple causes of uveal effusion, including inflammation, hypotony, and surgical complications; it may be mistaken, with several cases of circumferential uveal effusion enucleated for presumed ring melanoma.[5]

UES is thought to be idiopathic and is a diagnosis of exclusion that can only be made in the absence of secondary causes of choroidal effusion. Submacular fluid accumulation is common and is often the main cause of vision loss.[3],[6]

Patients with UES are divided into three groups on the basis of axial length, refractive error, and histopathologic finding of the sclera. Type I is a nanophthalmic eye and the axial length of the eyeball is <19.0 mm, with high-grade hyperopia in refraction and rigid and thick sclera at the time of surgery. Type II UES is a non-nanophthalmic eye with the abnormal sclera (normal axial length and no remarkable refractive errors), but with the rigid and thick sclera. Type III UES is a non-nanophthalmic eye with the normal sclera, normal size eyeball, and with normal scleral thickness.[6]

Histopathologic analysis of scleras excised from UES patients has supported this theory with findings of disorganized collagen fiber bundles and abnormal accumulation of glycosaminoglycan-like deposits.[2] Nanophthalmic eyes also have an abnormal, thickened scleral composition, thus accounting for their increased incidence of UES.[1]

Few reports have documented the medical treatment of UES. Andrijević Derk et al. used oral carbonic anhydrase inhibitors and topical prostaglandin analogs in three cases and demonstrated apparent resolution of chorio RD in two cases.[7]

Surgical management remains the definitive treatment with an 83% success rate after the first operation. The surgical management (choroidal drainage) includes vortex vein decompression, sclerotomy (scleral window), partial- or full-thickness sclerectomy, and ultrasound-guided sclerotomy or ExPRESS glaucoma implant (2–3 mm long and 0.4 mm diameter tube) shunt for choroidal fluid drainage, relieving scleral rigidity by relaxing scleral tension.[3],[8]

Uyama et al. treated 19 eyes of 16 patients with UES by making a two-third – thickness scleral flap and performing a scleral excision to expose the underlying choroid.[6]

However, the isolation of the vortex vein itself is very difficult and decompression is technically complicated to perform.[1] Significant complications such as vein rupture and considerable bleeding are also unavoidable. UES Type III has been reported to be the least common type and found not to respond to surgery.[6]

In scleral resection, mitomycin C (MMC) is thought to reduce scarring of the sclerectomy bed and prevent recurrence of the exudative RD due to the regeneration of thick sclera with abnormally arranged collagen fibers.[9] Reported complications of MMC include hypotony secondary to thin cystic bleb following trabeculectomy, endophthalmitis, and corneal or scleral melting.[10]


  Case Report Top


A 31-year-old man complained of decreased visual acuity for 4 months in both eyes. His family medical history was RDs. Best-corrected visual acuity was 20/300 (+14.25 sphere) in the right eye and 20/400 (+12.75 sphere) in the left eye. Slit-lamp examination showed shallow anterior chamber and narrow angle in both eyes. The intraocular pressure was 16 mmHg in the right eye and 15 mmHg in the left eye. Dilated fundus examination of both eyes was exudative serous chorio RD of the superior, temporal, and inferior quadrants. The vitreous was clear, and the optic nerve demonstrated sharp margins, without edema or hemorrhage. The retinal vasculature and macula were, likewise, unremarkable [Figure 1]. Ocular ultrasound [Figure 2] and Optical coherence tomography (OCT) [Figure 3] examination showed a chorio RD and an axial length of 16.08 mm in the right eye and 15.17 mm in the left eye, and ultrabiomicroscopy demonstrated the presence of a ciliary body detachment in both eyes. After analyzing the complementary tests performed, a diagnosis of UES was made.
Figure 1: Postoperative eye fund images (3 months)

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Figure 2: Optical coherence tomography showing exudative serous chorioretinal detachments

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Figure 3: Ocular ultrasound examination showed a chorioretinal detachment and an axial length of 16.08 mm in the right eye and 15.17 mm in the left eye

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[Figure 4] shows surgical treatment. We performed two 4 5-mm scleral flaps, about two-thirds of the scleral thickness (to 2 mm of the equator) in four quadrants. A 1 mm sclerostomy was subsequently placed in the center of the remaining sclera to reach the subchoroidal space. The first postoperative evaluation of the patient was performed at 24 h after surgery, where the partial resolution of uveal detachment was observed, the retina was attached, and the macular region preserved. At 3 and 6 months, the patient presented a visual acuity in both eyes of 20/200 and the retina was attached [Figure 5] and [Figure 6].
Figure 4: Surgical treatment, two 4 5-mm scleral flaps, about two-thirds of the scleral thickness in four quadrants

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Figure 5: Preoperative eye fund images

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Figure 6: Postoperative eye fund images (6 months)

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  Discussion Top


Uyama et al. reported a favorable response to equatorial sclerectomy under a scleral flap (subscleral sclerectomy) for the UES in nanophthalmic and non-nanophthalmic eyes with the thick and rigid sclera. In contrast, eyes with a normal axial length, scleral thickness, and scleral histology did not respond well to surgery.[6]

Our case had nanophthalmic eyes and thick scleral on light microscopy and ocular ultrasound, and the technique we used was effective in achieving resolution of choroidal swelling and the associated subretinal fluid, which was seen at the first postoperative control at 24 h.


  Conclusion Top


Like previous publications, subscleral sclerectomy may be effective for UES, inducing postoperative reattachment of the choroid. Although our report is about only one case, a larger study with a control group is required to prove the efficacy of the intervention. The rarity of UES makes the possibility of a more definitive study unlikely.

Declaration of patient consent

The authors certify that they have obtained informed patient consent. In the form, the patient has given his consent for his image and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Gass JD, Jallow S. Idiopathic serous detachment of the choroid, ciliary body, and retina (uveal effusion syndrome). Ophthalmology 1982;89:1018-32.  Back to cited text no. 1
    
2.
Brockhurst RJ. Nanophthalmos with uveal effusion. A new clinical entity. Arch Ophthalmol 1975;93:1989-99.  Back to cited text no. 2
    
3.
Elagouz M, Stanescu-Segall D, Jackson TL. Uveal effusion syndrome. Surv Ophthalmol 2010;55:134-45.  Back to cited text no. 3
    
4.
Gass JD. Uveal effusion syndrome: A new hypothesis concerning pathogenesis and technique of surgical treatment. Trans Am Ophthalmol Soc 1983;81:246-60.  Back to cited text no. 4
    
5.
Krema H, Pavlin C, Simpson R, Fernandes B. Uveal effusion simulating uveal ring melanoma. Can J Ophthalmol 2009;44:604-5.  Back to cited text no. 5
    
6.
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. 6
    
7.
Andrijević Derk B, Benčić G, Corluka V, Zorić Geber M, Vatavuk Z. Medical therapy for uveal effusion syndrome. Eye (Lond) 2014;28:1028-31.  Back to cited text no. 7
    
8.
Ghazi NG, Richards CP, Abazari A. A modified ultrasound-guided surgical technique for the management of the uveal effusion syndrome in patients with normal axial length and scleral thickness. Retina 2013;33:1211-9.  Back to cited text no. 8
    
9.
Venkatesh P, Majumdar SS, Kakkar A, Singh S, Gogia V, Garg S. Resolution of serous retinal detachment following partial sclerectomy with mitomycin C in nanophthalmos. Ophthalmic Surg Lasers Imaging Retina 2013;44:287-9.  Back to cited text no. 9
    
10.
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. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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