Hypertensive retinopathy as a presenting sign in phaeochromocytoma associated with Von Hippel–Lindau disease in an adolescent: A case report and literature review

Abstract Hypertensive retinopathy is rare in adolescents, with pheochromocytoma being an uncommon cause of hypertension. We report a case of an adolescent who experienced gradual blurring of vision and headaches. Fundus examination revealed bilateral grade four hypertensive retinopathy. Further evaluation identified a right-sided pheochromocytoma and a pancreatic tumor. Genetic testing confirmed a Von Hippel–Lindau (VHL) gene mutation. The patient underwent right adrenalectomy and pancreatic nodulectomy, successfully controlling the blood pressure and improving visual acuity. This case underscores the importance of early diagnosis and prompt treatment of VHL-related pheochromocytoma in adolescents.