Bilateral Pachychoroid disease with type 3 Uveal effusion syndrome in one eye and central serous Chorioretinopathy in contralateral eye: a case report

Abstract

Background We report a case of bilateral pachychoroid disease with type 3 uveal effusion syndrome (UES) in one eye and central serous chorioretinopathy (CSC) in the contralateral eye. Case presentation A 65-year-old man presented to our department because of decreased vision. Visual acuity was 16/20 in the right eye and 2/20 in the left eye, with normal axial lengths. The left eye was diagnosed with CSC and underwent photocoagulation 8 years ago. The right eye showed inferior non-rhegmatogenous retinal detachment and peripheral choroidal detachment. Macular optical coherence tomography showed submacular fluid in the right eye, pachychoroid in both eyes, and choroidal thickness of 565 μm in the right and 545 μm in the left eye. In both eyes, fluorescence angiography showed window defects and mild fluorescence leakage, and indocyanine green angiography showed dilated choroidal vessels, mild choroidal hyperpermeability, and mild dye leakage. The left eye was diagnosed with chronic CSC. Initially, chronic CSC was also suspected in the right eye. However, photodynamic therapy failed, with worsened retinal detachment and visual acuity. Pachychoroid in the peripheral fundus (choroidal thickness 820 μm) was observed only in the right eye. Based on these findings, UES was diagnosed in the right eye. Sclerectomies were performed. The absence of scleral thickening and glycosaminoglycan deposition led to a final diagnosis of type 3 UES. The procedure was not effective, due to connective tissue regeneration at the sclerectomy sites. In the revision surgery, mitomycin-C was used with sclerectomies. One month after surgery, retinal and choroidal detachment disappeared, visual acuity recovered to 8/20, pachychoroid in the macula and peripheral fundus decreased, and choroidal thickness decreased to 352 μm in the macula and 554 μm in inferior peripheral fundus. Conclusions Pachychoroid in the posterior pole was the common finding in type 3 UES and CSC, although extensive pachychoroid in the peripheral fundus may have caused retinal and choroidal detachment in the eye with type 3 UES. Full-thickness sclerectomies with mitomycin-C improved pachychoroid in the peripheral fundus and resolved retinal and choroidal detachment, clearly indicating that the sclera was the main cause of type 3 UES.

Biomechanical properties measured with dynamic Scheimpflug analyzer in central serous chorioretinopathy

“…Increased resistance to transscleral outflow is a principal hypothesized mechanism in uveal effusion syndrome [24], which is characterized by ciliochoroidal effusion and serous retinal detachment. This syndrome, where a histologically altered sclera has been documented and the effectiveness of sclerotomy for particular subtypes is evident [25,26], shares clinical characteristics with CSC and may have a common pathogenic mechanism involving scleral abnormalities [27,28].…”

Medical Therapy and Scleral Windows for Uveal Effusion Syndrome: A Case Series and Literature Review

“…Enhanced-depth OCT may provide better detection of increased choroidal thickness compared to normal eyes, as well as the presence of hypo-reflective areas corresponding to engorged choroidal veins or expansion of the suprachoroidal space [ 27 ]. Focal thickening of the RPE through leopard spots can also be seen using OCT. Interestingly, pachychoroid features have been identified in cases of type 3 UES on OCT [ 28 – 30 ]. The exact relationship with type 3 UES and pachychoroid spectrum disease remains unknown.…”