0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Surgical Technique |

Ex-PRESS Shunt for Choroidal Fluid Drainage in Uveal Effusion Syndrome Type 2 A Potentially Novel Technique FREE

Juan B. Yepez, MD1; J. Fernando Arevalo, MD2,3
[+] Author Affiliations
1Department of Vitreoretinal Surgery, Clinica de Ojos, Maracaibo, Venezuela
2Division of Retina, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland
3Division of Vitreoretinal, King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia
JAMA Ophthalmol. 2015;133(4):470-471. doi:10.1001/jamaophthalmol.2014.5913.
Text Size: A A A
Published online

Uveal effusion syndrome (UES) was reported by Schepens and Brockhurst in 1963,1 and later described as a nanophthalmic disorder with a scleral abnormality.2 Gass3 described idiopathic UES and hypothesized that the cause was a congenital anomaly of the sclera and vortex veins that was related to aging, hormonal changes, or impairment of the permeability of the sclera. Using histological methods, Trelstad et al4 reported the scleral abnormalities in UES. In addition, Forrester et al5 observed migration of retinal pigment epithelial cells into the subretinal space.

For the treatment of UES, Brockhurst6 described good surgical results with decompression of the vortex veins by scleral resection with a sclerotomy. Gass3 reported an effective surgical procedure with a sclerectomy and sclerostomy without decompression of the vortex veins owing to the difficulty of isolating the vortex veins. Uyama et al7 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.

The Ex-PRESS shunt (Alcon Laboratories) glaucoma implant is a small (2- to 3-mm–long and 0.4-mm–diameter tube) stainless steel nonvalved device that was designed to lower intraocular pressure by shunting aqueous humor from the anterior chamber into the subconjuntival space under a partial-thickness scleral flap space adjacent to the limbus.8

In the current study, we report the surgical outcomes of the use of an Ex-PRESS shunt device for choroidal drainage without vortex vein decompression for the management of 3 eyes with UES.

A retrospective medical record review was performed of 2 consecutive patients (3 eyes) with UES without nanophthalmos who underwent Ex-PRESS shunt implantation at the Clinica de Ojos de Maracaibo, Maracaibo, Venezuela, from March 2012 through March 2014 (Table). Institutional review board/ethics committee approval was obtained from Clinica de Ojos de Maracaibo and the patients signed a standard informed consent. This study adhered to the tenets of the Declaration of Helsinki for research involving human participants.

Table Graphic Jump LocationTable.  Demographic and Baseline Characteristics, Functional and Anatomic Results, and Complications in Eyes With Ex-PRESS Shunt for Choroidal Drainage in UES

After peribulbar anesthesia and standard asepsis and antisepsis in the operating room, a conjunctival incision was performed, bare sclera was exposed in the quadrant with more accumulation of fluid according to ultrasonography, and an oblique sclerotomy was performed with a 25-gauge needle 13 mm posterior to the limbus (to decrease the risk for infection), at which time, a small amount of suprachoroidal fluid was spontaneously drained. The Ex-PRESS shunt (model P-50) was then inserted obliquely in the sclerotomy to facilitate the continuous drainage of the suprachoroidal fluid (eFigure 1 in the Supplement). Balanced salt solution was injected into the anterior chamber with a 30-gauge needle to reconstitute intraocular pressure. The conjunctiva was closed with 7-0 polyglactin 910 suture and subconjunctival antibiotics were injected.

Patient 1

A woman in her 60s presented to the emergency department with a 15-day history of a “shadow” that affected the upper part of the visual field in her left eye. She had undergone cataract surgery in both eyes 3 years prior to presentation. Her best-corrected visual acuity (BCVA) without any refractive error was 20/20 OD and 20/200 OS. Fundus examination revealed choroidal detachment and shifting subretinal fluid underneath the exudative retinal detachment of the left eye. Ultrasonography was performed demonstrating thick sclera (1.5 mm), choroidal detachment, and exudative retinal detachment (eFigure 2 in the Supplement). The axial length was 22 mm in the right eye and 22.5 mm in the left eye. She was diagnosed as having UES in the left eye. Despite treatment with steroids (oral prednisolone, 50 mg/d), the retinal findings and BCVA did not improve. Ex-PRESS shunt drainage surgery was performed in the inferotemporal quadrant (eFigure 3 in the Supplement and Video) without vortex vein decompression without complications. Two days postoperatively, a flat retina was seen on fundus examination. The retina remained attached during the 24-month follow-up, and an improved BCVA of 20/30 was achieved. We did not observe subretinal fluid or choroidal effusion by ultrasonography after surgery at 24 months (eFigure 4 in the Supplement).

Video.

Ex-PRESS Shunt for Uveal Effusion Syndrome

Ex-PRESS shunt implantation was performed in 3 eyes of 2 patients with uveal effusion syndrome without nanophthalmos. In all of the eyes, exudative choroidal retinal detachment associated with uveal effusion syndrome resolved shortly after the device implantation. Ex-PRESS shunt implantation without vortex vein decompression or scleral windows is an effective surgical option for the management of significant uveal effusion syndrome.

Patient 2

A woman in her 50s was referred owing to decreased visual acuity in her left eye for 3 weeks’ duration. She had been wearing glasses for hyperopia since childhood. Her BCVA was 20/25 OD and 20/400 OS, and her spherical equivalents were +1 diopters (D) and +1.50 D in the left and right eyes, respectively. Fundus examination revealed uveal effusion involving the macula in the left eye. Axial length was 21 mm in the left eye and 22 mm in the right eye on A-scan ultrasonography. B-scan ultrasonography demonstrated thick sclera (1.6 mm), an exudative choroidal, and retinal detachment.

An Ex-PRESS shunt was implanted in the inferotemporal sclera quadrant in the left eye. One week postoperatively, a flat retina was seen on fundus examination and BCVA improved to 20/40. The retina remained attached during the 24 months of follow-up. Six months later, she presented with impaired visual acuity in her right eye. Her BCVA was 20/200 and the intraocular pressure was 16 mm Hg in the right eye and 18 mm Hg in the left eye. Fundus examination demonstrated supraciliary effusion and exudative retinal detachment. Ex-PRESS shunt implantation was performed in the inferotemporal quadrant. The subretinal fluid and the choroidal effusion disappeared 48 hours after surgery and BCVA improved to 20/30. The retina and choroid have remained attached during 12 months of follow-up.

Uveal effusion syndrome is difficult to manage and often follows a relapsing course. Our 3 cases (eyes) can be classified as type 2 according to the classification by Uyama and colleagues7 because they were not associated with nanophthalmos or hyperopia but had a thick sclera. Although Brockhurst6 described good surgical results with decompression of vortex veins, other investigators3 have questioned the usefulness of this technique because vortex veins are difficult to isolate. Uyama et al7 reported that sclerectomy with a small sclerostomy under the scleral flap could be effective in both type 1 and type 2 UES because the abnormal sclera and increased trans-scleral outflow of intraocular fluid are thought to be the main causes of these disorders. However, this technique was not effective in type 3 UES, which develops in non-nanophthalmic eyes with normal eyeball size and normal scleral thickness.

We believe that isolation of the vortex veins is complicated and that vortex vein decompression is technically difficult to perform without complications such as vein rupture. Therefore, we elected to perform the Ex-PRESS shunt technique instead of full-thickness sclerotomy or scleral windows because it seemed likely to have a sufficient effect for resolving UES. We suspect that our technique may work by facilitating a constant drainage of uveal exudation from the suprachoroidal space to the subconjuntival space.

The actual surgical technique and implantation of the device are very simple. The tip of the Ex-PRESS shunt is not sharp and it is inserted obliquely so the chance to cause retinal damage is unlikely. In addition, considering the reported persistent and recurrence rates (23%-50%) for type 2 UES,7,9,10 it seems that the cost may be worthwhile. Compared with other surgical procedures, our Ex-PRESS shunt technique could simplify the operative procedure, is less invasive, and reduces surgical time.

Limitations of our report include that we cannot know the long-term benefits and risks from 3 eyes and limited follow-up. At this point, we know that the procedure is feasible and safe for a limited time.

Submitted for Publication: August 17, 2014; final revision received November 2, 2014; accepted November 12, 2014.

Corresponding Author: J. Fernando Arevalo, MD, Vitreoretinal Division, The King Khaled Eye Specialist Hospital, Al-Oruba St, PO Box 7191, Riyadh 11462, Kingdom of Saudi Arabia (arevalojf@jhmi.edu).

Published Online: February 12, 2015. doi:10.1001/jamaophthalmol.2014.5913.

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Dr Arevalo has received personal fees from Iridex, Optos Inc, Novartis Pharmaceuticals Corp, and Alimera Sciences Inc for lectures; from Second Sight LLC and Alcon Laboratories for serving as a consultant and lecturer; and from Springer SBM LLC related to a patent. No other disclosures were reported.

Schepens  CL, Brockhurst  RJ.  Uveal effusion, 1: clinical picture. Arch Ophthalmol. 1963;70:189-201.
PubMed   |  Link to Article
Brockhurst  RJ.  Nanophthalmos with uveal effusion: a new clinical entity. Arch Ophthalmol. 1975;93(12):1989-1999.
PubMed   |  Link to Article
Gass  JD.  Uveal effusion syndrome: a new hypothesis concerning pathogenesis and technique of surgical treatment. Retina. 1983;3(3):159-163.
PubMed   |  Link to Article
Trelstad  RL, Silbermann  NN, Brockhurst  RJ.  Nanophthalmic sclera: ultrastructural, histochemical, and biochemical observations. Arch Ophthalmol. 1982;100(12):1935-1938.
PubMed   |  Link to Article
Forrester  JV, Lee  WR, Kerr  PR, Dua  HS.  The uveal effusion syndrome and trans-scleral flow. Eye (Lond). 1990;4(pt 2):354-365.
PubMed   |  Link to Article
Brockhurst  RJ.  Vortex vein decompression for nanophthalmic uveal effusion. Arch Ophthalmol. 1980;98(11):1987-1990.
PubMed   |  Link to Article
Uyama  M, Takahashi  K, Kozaki  J,  et al.  Uveal effusion syndrome: clinical features, surgical treatment, histologic examination of the sclera, and pathophysiology. Ophthalmology. 2000;107(3):441-449.
PubMed   |  Link to Article
Kanner  EM, Netland  PA, Sarkisian  SR  Jr, Du  H.  Ex-PRESS miniature glaucoma device implanted under a scleral flap alone or combined with phacoemulsification cataract surgery. J Glaucoma. 2009;18(6):488-491.
PubMed   |  Link to Article
Johnson  MW, Gass  JDM.  Surgical management of the idiopathic uveal effusion syndrome. Ophthalmology. 1990;97(6):778-785.
PubMed   |  Link to Article
Jackson  TL, Hussain  A, Morley  AM,  et al.  Scleral hydraulic conductivity and macromolecular diffusion in patients with uveal effusion syndrome. Invest Ophthalmol Vis Sci. 2008;49(11):5033-5040.
PubMed   |  Link to Article

Figures

Tables

Table Graphic Jump LocationTable.  Demographic and Baseline Characteristics, Functional and Anatomic Results, and Complications in Eyes With Ex-PRESS Shunt for Choroidal Drainage in UES

References

Schepens  CL, Brockhurst  RJ.  Uveal effusion, 1: clinical picture. Arch Ophthalmol. 1963;70:189-201.
PubMed   |  Link to Article
Brockhurst  RJ.  Nanophthalmos with uveal effusion: a new clinical entity. Arch Ophthalmol. 1975;93(12):1989-1999.
PubMed   |  Link to Article
Gass  JD.  Uveal effusion syndrome: a new hypothesis concerning pathogenesis and technique of surgical treatment. Retina. 1983;3(3):159-163.
PubMed   |  Link to Article
Trelstad  RL, Silbermann  NN, Brockhurst  RJ.  Nanophthalmic sclera: ultrastructural, histochemical, and biochemical observations. Arch Ophthalmol. 1982;100(12):1935-1938.
PubMed   |  Link to Article
Forrester  JV, Lee  WR, Kerr  PR, Dua  HS.  The uveal effusion syndrome and trans-scleral flow. Eye (Lond). 1990;4(pt 2):354-365.
PubMed   |  Link to Article
Brockhurst  RJ.  Vortex vein decompression for nanophthalmic uveal effusion. Arch Ophthalmol. 1980;98(11):1987-1990.
PubMed   |  Link to Article
Uyama  M, Takahashi  K, Kozaki  J,  et al.  Uveal effusion syndrome: clinical features, surgical treatment, histologic examination of the sclera, and pathophysiology. Ophthalmology. 2000;107(3):441-449.
PubMed   |  Link to Article
Kanner  EM, Netland  PA, Sarkisian  SR  Jr, Du  H.  Ex-PRESS miniature glaucoma device implanted under a scleral flap alone or combined with phacoemulsification cataract surgery. J Glaucoma. 2009;18(6):488-491.
PubMed   |  Link to Article
Johnson  MW, Gass  JDM.  Surgical management of the idiopathic uveal effusion syndrome. Ophthalmology. 1990;97(6):778-785.
PubMed   |  Link to Article
Jackson  TL, Hussain  A, Morley  AM,  et al.  Scleral hydraulic conductivity and macromolecular diffusion in patients with uveal effusion syndrome. Invest Ophthalmol Vis Sci. 2008;49(11):5033-5040.
PubMed   |  Link to Article

Correspondence

CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.

Multimedia

Video.

Ex-PRESS Shunt for Uveal Effusion Syndrome

Ex-PRESS shunt implantation was performed in 3 eyes of 2 patients with uveal effusion syndrome without nanophthalmos. In all of the eyes, exudative choroidal retinal detachment associated with uveal effusion syndrome resolved shortly after the device implantation. Ex-PRESS shunt implantation without vortex vein decompression or scleral windows is an effective surgical option for the management of significant uveal effusion syndrome.

Supplement.

eFigure 1. The Ex-PRESS Shunt Was Inserted in the Sclerotomy to Facilitate the Continuous Drainage of the Suprachoroidal Fluid

eFigure 2. Ultrasonography Was Performed Demonstrating Choroidal Detachment and Exudative Retinal Detachment

eFigure 3. Ex-PRESS Shunt Drainage Surgery Was Performed in the Inferotemporal Quadrant Without Vortex Vein Decompression Without Complications

eFigure 4. Postoperative Ultrasonography 24 Months After the Procedure Demonstrating Choroidal Detachment and Exudative Retinal Detachment Resolution

Supplemental Content

Some tools below are only available to our subscribers or users with an online account.

812 Views
0 Citations
×

Related Content

Customize your page view by dragging & repositioning the boxes below.

Related Multimedia
Articles Related By Topic
Related Collections
Jobs
brightcove.createExperiences();