From the Department of Ophthalmology, University Hospitals Leuven, Leuven, Belgium.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Ingele Casteels, MD, PhD, Department of Ophthalmology, University Hospitals Leuven, Capucijnenvoer, 33, B-3000 Leuven, Belgium.
Exudative detachment of the choroid and ciliary body is a nonspecific response to a variety of stimuli and has been described in association with intraocular surgery, especially with cataract surgery. It may also occur spontaneously with no apparent cause in healthy individuals. This phenomenon is defined as the idiopathic uveal effusion syndrome.1
Mohney et al. described a 75-year-old phakic woman with visual loss due to cystoid macular edema following a second strabismus surgery 15 months after the first procedure.2 Merino et al. reported an infectious cause to be the underlying etiology of the uveal effusion syndrome in a 76-year-old patient after strabismus surgery under topical anesthesia.3 To our knowledge, uveal effusion syndrome has not been described in children following uncomplicated strabismus surgery.
A 12-year-old girl had undergone surgery at 3 years of age for a convergent squint with a V-pattern and had also had bilateral recession of the medial recti combined with anteropositioning of the inferior oblique muscles. The eyes remained straight until the age of 12 years and then turned divergent with a slight A-pattern. The patient was +8 diopters hypermetropic.
A bilateral recession of the lateral recti combined with a bilateral posterior tenotomy of the superior oblique muscles and a revision of the inferior oblique muscles previously operated on was performed. The surgical procedure was uneventful. She noticed a central scotoma in the right eye 1 day postoperatively and complained of loss of vision 2 days later. She was immediately referred to our department.
On examination, visual acuity was hand motions in the right eye and 0.8 in the left eye. She could not read Snellen 10 in the right eye but near vision was normal in the left eye. Biomicroscopic examination showed no cells or flare in the anterior segment or vitreous. Humphrey visual field examination showed a central scotoma on the right (Fig. 1) and a normal central visual field in the left eye. Funduscopy of the right eye showed macular and posterior pole edema that was confirmed by fluorescein angiography (Fig. 2). Funduscopy of the left eye was unremarkable. Optical coherence tomography showed a massive macular and posterior pole edema in the right eye (Fig. 3) and normal macular findings in the left eye. Axial length in both eyes was 19 mm (23 mm is the mean axial length at this age4).
Figure 1. Humphrey Visual Field Examination Showing a Central Scotoma in the Right Eye.
Figure 2. (A) Funduscopy Showing Posterior Pole Edema in the Right Eye that Was Later Confirmed by (B) Fluorescein Angiography.
Figure 3. Optical Coherence Tomography Showing Massive Subretinal Fluid in the Right Eye.
She was treated with parabulbar triamcinolone acetonide and topical steroids six times per day in the right eye. One day later, visual acuity in the right eye had recovered to 0.1. During the next 2 weeks, visual acuity recovered to 0.8 and Snellen 1 in the right eye with continuous treatment with topical steroids. Macular and choroidal edema disappeared (Fig. 4) and visual field examination normalized.
Figure 4. The Macular and Choroidal Edema Disappeared on (A) Funduscopy and (B) Optical Coherence Tomography.
In our patient, unilateral visual loss due to macular and posterior pole edema was an unexpected complication following an uneventful strabismus surgery. There was a bilateral hypermetropia of +8 diopters and small axial lengths; the eyes can be considered nanophthalmic. Nanophthalmos is a rare condition consequent on arrested development of the globe in all dimensions. Nanophthalmic eyes are typically deep set and are markedly hypermetropic with axial lengths less than 20 mm.5 Sclera and cornea appear too small compared to the intraocular content. Abnormal deposits of glycosaminoglycans and elevated levels of fibronectin (involved in cellular adhesion) contribute to the irregular arrangement of collagen bundles found in the sclera of nanophthalmic eyes.6,7 Accumulation of those abnormal products causes obstruction of the suprachoroidal drainage pathway with choroidal congestion.8
Gass hypothesized that the primary underlying cause of the idiopathic uveal effusion syndrome is a congenital anomaly of the sclera and in some cases of the vortex veins.1 Superimposed aging and hormonal changes in the sclera predispose the eye to vortex vein obstruction. The inability of the eye to transport extravascular protein across the abnormal sclera is probably the cause of prolonged exudative detachment of the uvea and retina in these patients. This accumulation of fluid has also been described as a complication of various intraocular surgeries (cataract, glaucoma, and retinal detachment surgery) where hypotony is combined with postoperative inflammation. Other nonsurgical conditions associated with uveal effusion are idiopathic (uveal effusion syndrome and nanophthalmos) and inflammatory diseases.
In our patient, the posterior pole edema can be considered a uveal effusion–like syndrome that developed after a second strabismus surgery with an interval of 9 years between both. Sener et al. described 5 of 15 nanophthalmic patients who underwent horizontal muscle surgery; none of them developed postoperative uveal effusion–like symptoms.9 We can wonder whether the strabismus surgery, although uneventful without scleral perforation, was the predisposing factor to develop this complication in our patient. The macular edema was more localized temporally (Fig. 3), where the recession of the lateral rectus muscle combined with a posterior tenotomy of the superior oblique muscle was performed. This could have compromised the vortex venous system with consequent choroidal edema and detachment. In the literature, two cases of macular edema and uveal effusion syndrome after strabismus surgery have been described in elderly patients.2,3 In the patient described by Merino et al., it is hypothesized that the uveal effusion syndrome was secondary to suture infection by Staphylococcus epidermidis.3
The rapid response to parabulbar and topical steroids in our patient could also suggest an underlying inflammatory factor. Sub-Tenon’s capsule or parabulbar injection of depot steroids is a currently established method for the treatment of various inflammatory eye diseases, with a good therapeutic response and ocular tolerance.10
To our knowledge, our patient is the first child described in the literature with a uveal effusion–like syndrome after strabismus surgery. The importance of a reoperation in the pathogenesis is not clear. The diagnosis was made efficiently due to quick referral and investigation. We are not aware of any other children with those postoperative complications. However, we do realize that they could remain unnoticed because referral and investigation is often delayed.
- Gass JD. Uveal effusion syndrome: a new hypothesis concerning pathogenesis and technique of surgical treatment. Trans Am Ophthalmol Soc. 1983;81:246–260.
- Mohney BG, Agarwal S. Cystoid macular edema following extraocular muscle surgery. J AAPOS. 2002;6:120–122. doi:10.1067/mpa.2002.121615 [CrossRef]
- Merino P, Gomez de Liano P, Martinez Yanez. Uveal effusion syndrome after strabismus surgery [article in Spanish]. Arch Soc Esp Oftalmol. 2006;81:409–412.
- Fledelius HC, Christensen AC. Reappraisal of the human ocular growth curve in fetal life, infancy, and early childhood. Br J Ophthalmol. 1996;80:918–921. doi:10.1136/bjo.80.10.918 [CrossRef]
- Serrano JC, Hodgkins PR, Taylor DS, Gole GA, Kriss A. The nanophthalmic macula. Br J Ophthalmol. 1998;82:276–279. doi:10.1136/bjo.82.3.276 [CrossRef]
- Kawamura M, Tajima S, Azuma N, Katsura H, Akiyama K. Biochemical studies of glycosaminoglycans in nanophthalmic sclera. Graefes Arch Clin Exp Ophthalmol. 1995;233:58–62. doi:10.1007/BF00241472 [CrossRef]
- Shiono T, Shoji A, Mutoh T, Tamai M. Abnormal sclerocytes in nanophthalmos. Graefes Arch Clin Exp Ophthalmol. 1992;230:348–351. doi:10.1007/BF00165943 [CrossRef]
- Yue BY, Duvall J, Goldberg MF, Puck A, Tso MO, Sugar J. Nanophthalmic sclera: morphologic and tissue culture studies. Ophthalmology. 1986;93:534–541.
- Sener EC, Mocan MC, Sarac ÖI, Gedik S, Sanac AS. Management of strabismus in nanophthalmic patients: a long-term follow-up report. Ophthalmology. 2003;110:1230–1236. doi:10.1016/S0161-6420(03)00267-7 [CrossRef]
- Lafranco Dafflon M, Tran VT, Guex-Crosier Y, Herbort CP. Posterior sub-Tenon’s steroid injections for the treatment of posterior ocular inflammation: indications, efficacy and side effects. Graefes Arch Clin Exp Ophthalmol. 1999;237:289–295. doi:10.1007/s004170050235 [CrossRef]