Abstract
Abstract: :
Purpose: To describe optical coherence tomography (OCT) findings in a 17 year–old male with papillorenal syndrome and high–dose corticosteroid treatment after kidney transplantation. We report previously undescribed development of bilateral serous macular detachment and schisis on high–dose corticosteroids, with resolution following tapered doses. Methods: Clinical case report. Extensive review of 17 years of medical history. Examinations included best–corrected Snellen visual acuity, indirect ophthalmoscopy, contact and hand–held lens biomicroscopy, stereoscopic fundus photography, fluorescein angiography, and OCT. Results: Six months after kidney transplantation for a solitary dysplastic kidney, and while being treated with high–dose oral corticosteroids, visual acuity decreased to 20/60 OU due to serous macular detachment. Examination showed anomalous disc vessels emanating more peripherally than normal and an optic disc pit OS. The patient subsequently had two acute rejection episodes that were treated with high–dose intravenous (IV) corticosteroids followed by oral tapered doses. Visual acuity declined to 20/100 OD and 20/400 OS. Submacular fluid and macular schisis were present OU and documented with OCT. Fifteen months after the last treatment with high–dose IV corticosteroids, visual acuity improved to 20/20 OD and 20/25 OS due to resolution of the serous macular detachment and schisis OU, as documented by OCT. Conclusions: The papillorenal syndrome is an uncommon disease that consists of vascular dysgenesis in the eye, kidneys, and urinary tract. In addition to hypoplastic kidneys, patients show anomalous optic nerve vessels consistent with numerous cilioretinal shunt vessels. Decreased vision secondary to macular detachment and schisis developed in our patient while being treated with high–dose corticosteroids. Macular findings by OCT and visual acuity improved one year following tapered doses of corticosteroids. The development and resolution of these macular abnormalities suggest that high–dose systemic corticosteroids may be causal, possibly related to their effect on the retinal pigment epithelial pump mechanism. Since patients with this syndrome may need renal transplantation, managing physicians should be aware that these macular abnormalities may occur. Decreased systemic corticosteroids doses and months of observation can obviate the need for surgical intervention to treat macular detachment and schisis in the papillorenal syndrome. OCT is a useful examination method to diagnose and monitor these macular changes.
Keywords: imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) • corticosteroids • macula/fovea