Article Text

PDF

Posterior uveal effusion syndrome after trabeculectomy in an eye with ocular venous congestion
  1. N Bhagat1,
  2. J I Lim2,
  3. D S Minckler2,
  4. R L Green2
  1. 1Department of Ophthalmology, DOC - 6th Floor, 90 Bergen Street, New Jersey Medical School, Newark, NJ 07103, USA
  2. 2Doheny Eye Institute, CA, USA
  1. Correspondence to: J I Lim Doheuy Eye Institute, 1450 San Pablo Street, Los Angeles, CA 90033, USA; bhagatneumdnj.edu

Statistics from Altmetric.com

Schepens and Brockhurst,1 in 1963, used the term uveal effusion syndrome to describe spontaneous bilateral serous detachments of the choroid and ciliary body with exudative retinal detachment, primarily occurring in middle aged healthy males. Many cases have been reported since then, especially in nanophthalmic eyes.1–3

We report an interesting case of a young, healthy female patient with mild ocular venous congestion who developed posterior serous retinal detachment and uveal effusions after trabeculectomy for open angle glaucoma.

Case report

A 32 year old white female was referred to the glaucoma service at Doheny Eye Institute for high intraocular pressure in the left eye. Visual acuity was 20/20 right eye and 20/25 left eye. Manifest refraction was +2.75+1.50×13 right eye and +0.50+2.00×160 left. An afferent pupillary defect was noted in the left eye. Applanation intraocular pressures were 17 mm Hg right eye and 36 mm Hg left.

Slit lamp examination of both eyes revealed dilated and tortuous conjunctival and episcleral vessels, although more obvious in the left eye than the right eye. The anterior chambers were deep and quiet. The cornea and lens were clear.

Gonioscopic evaluation of both eyes showed open, grade IV Shafer angles with no peripheral anterior synechiae. Blood was noted in Schlemm’s canal of both eyes. Cup to disc ratios were 0.80 right eye and 0.95 left. The remainder of the posterior segment was normal.

Past ocular history was remarkable for strabismus surgery on both eyes at the age of 2.

Because of clinical evidence of mild venous outflow congestion in both eyes, further tests were done to evaluate for a possible carotid-cavernous fistula. Magnetic resonance angiography revealed no evidence of a carotid-cavernous fistula. Ultrasonography revealed no evidence of any orbital mass. Orbital soft tissues were normal bilaterally. Superior ophthalmic veins were not dilated. Choroid and retinal layers were normal. However, bilateral diffuse moderate thickening of sclera was noted without a T sign in either eye. Axial lengths were 21.5 mm in the right eye 22.5 mm in the left.

No history of collagen vascular disease was elicited. Complete blood count (CBC) and erythrocyte sedimentation rate (ESR) were normal; antinuclear antibody (ANA) and rheumatoid (Rh) factors were negative. Since the patient continued to have high uncontrolled intraocular pressure on maximum medical therapy, trabeculectomy of the left eye was performed. Intraoperatively, after the scleral fistula was created, a sudden shallowing of the anterior chamber and anterior displacement of the lens-iris diaphragm occurred. An inferotemporal sclerotomy was used to drain 0.5 ml of clear serous suprachoroidal fluid, thereby relieving the posterior pressure.

On the first postoperative day, visual acuity was hand movements. A posterior retinal detachment was noted in the macular area, extending to the equator on the temporal side. The subretinal fluid was loculated in the posterior pole. Posterior retinal folds were noted, but no retinal holes were found in the macula or the periphery. Applanation intraocular pressure was 30 mm Hg. Ultrasonography revealed posteriorly thickened choroid, along with the diffusely thickened sclera. (Preoperatively, ultrasonographically, sclera was thickened bilaterally but choroid was normal.) A diagnosis of uveal effusion syndrome was made.

The shallow serous macular detachment seemed unchanged for the next 3 days. However, on postoperative day 4, the macular detachment resolved, but the choroid still looked boggy and macular choroidal folds were seen. The visual acuity improved from hand movements to 20/200 within a week, and to 20/60 over the next 3½ months.

Comment

Schepens and Brockhurst reported a series of 17 patients with uveal effusion syndrome in 1963,1 predominantly noted in middle aged males. Flat, annular choroidal detachments were seen in five (30%) of their patients. Rarely, glaucoma occurred secondary to anterior displacement of the ciliary body. Thickened sclera (about 2 mm) was found in 47% of their cases.

Since then, numerous cases have been reported.2,3 Gass2 described the features of idiopathic serous detachment of the choroid, ciliary body, and retina (uveal effusion syndrome) in nine patients. He noted dilated episcleral vessels, vitreous cells, leopard spot retinal pigment epithelium (RPE) changes, and characteristic ultrasonographic and angiographic findings. None of these patients had previous trauma, surgery, ocular or inflammatory conditions, or carotid cavernous fistula. All but one patient had refractive error within 1.50 dioptres of emmetropia. Ultrasonography revealed slightly smaller than normal globes, even in the presence of myopic refractive error in one patient. The two patients who had gonioscopy had partial or complete filling of Schlemm’s canal with blood. The author attributed the above clinical findings to partial obstruction of the vortex veins. Fluorescein abnormalities included slow perfusion of the choroid and prolonged choroidal fluorescence, suggesting choroidal vascular congestion.2

Uveal effusion syndrome is occasionally associated with a small but normal eye (nanophthalmos). This association was first reported by Brockhurst in 19744 in five nanophthalmic patients who underwent glaucoma surgery for either acute or chronic angle closure glaucoma. The thickened sclera seen in this anomaly may lead to partial obstruction of the vortex veins, causing the choroidal effusions.5

In our case, although the patient had slightly shortened globes (axial length of 21.5 mm right eye, 22.5 mm left), they were not nanophthalmic. The thickened sclera in each eye was consistent with slightly small hyperopic eyes. The corneal diameters were normal. The anterior chambers were deep.

The dilated episcleral vessels and blood in Schlemm’s canal suggested compromised venous outflow. Moderately thickened sclera, noted on ultrasonography, probably caused impaired venous drainage by mechanically compressing the vortex veins. Such an eye would be predisposed to uveal effusions. An abrupt lowering of intraocular pressure during sclerostomy may have triggered the formation of choroidal effusions. The peripheral serous choroidals were drained intraoperatively, leaving behind loculated, posterior suprachoroidal fluid, which resulted in an unusual serous macular detachment. In a normal eye, this small amount of suprachoroidal effusion and subretinal fluid is readily absorbed. In our patient, however, we presume that the thickened choroid impaired the retinal pigment epithelial pumping mechanism. Although the serous retinal detachment resolved within a week, the choroidal folds remained visible for 3 months.

Elective surgical intervention should be avoided in patients with compromised venous outflow. Potentially blinding complications can occur intraoperatively or postoperatively in these eyes. Patients with ocular venous congestion should be advised of the potential complications of uveal effusion syndrome with any intraocular surgery.

Recognition of the classic signs and symptoms of uveal effusion syndrome is very important. The natural course is variable. The effusions may resolve spontaneously. Medical treatment with steroids and antimetabolites is usually not helpful. Decompression of vortex veins has had limited success. Scleral windows to improve outflow of suprachoroidal fluid have been successful.2

References

View Abstract

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.