Renal coloboma syndrome associated with double- chambered right ventricle

Renal coloboma syndrome (RCS) (papillorenal syndrome) is an autosomal dominant entity characterized by hypodysplastic kidneys and optic nerve abnormalities ranging from optic pit to total optic disc coloboma. The double-chambered right ventricle (DCRV) is a rare mg/dL), urea (200 mg/dL), phosphorus (5.84 mg/dL), parathyroid hormone (1196 pg/mL) and decreased levels of Fe+ (26 µg/dL), red blood cell (3.83x106/pL) and hemoglobin (11.6 g/dL). Urinalysis revealed proteinuria. Abdominal ultrasonography and computed tomography showed bilateral renal hypoplasia (Fig. 3) and a diagnosis of end-stage renal failure was made. Renal transplantation was performed, followed by six-month hemodialysis treatment. Two months after the successful renal transplantation, serous submacular fluid reduced and macular anatomy recovered but BCVA remained at the same level. The patient received no ophthalmologic treatment for the optic pit.