CHOROIDAL SYNPHLEBIA IN A PATIENT WITH CENTRAL SEROUS CHORIORETINOPATHY

The purpose of this study was to describe the choroidal vascular abnormality of conjoined choroidal vessels, synphlebia, in a patient with central serous chorioretinopathy.

A patient initially referred for pigmentary retinal dystrophy later developed central serous retinopathy and underwent a comprehensive examination with multimodal imaging.

A 30-year-old man had an undiagnosed pigmentary retinopathy, and nyctalopia was found to have a dehydrodolichyl diphosphate synthase mutation, confirming a retinitis pigmentosa diagnosis. A year later, he presented with bilateral central serous chorioretinopathy. During the venous phase of indocyanine green angiography, individual vessels could not be differentiated in the posterior pole. Swept source optical coherence tomography revealed an abnormally thick choroid with enormous vascular channels that did not appear to have separation into individual tubular structures. These abnormal vessels nearly occupied the entire thickness of the choroid in the macular region. There was little sign of any intermediate-sized choroidal vessels in the posterior pole. A review of other patients with dehydrodolichyl diphosphate synthase mutation showed no similar choroidal vascular changes.

Large, conjoined veins, which we termed synphlebia, were seen in an eye with central serous chorioretinopathy. Loading of the choriocapillaris from these enlarged vascular channels may have contributed to hyperpermeability and formation of serous fluid. Observation of this choroidal vascular pattern suggests that a central serous chorioretinopathy phenotype may develop through more than one pathway.