Recurrent Hematuria Unveiling Renal Lymphangioma: A Diagnostic Challenge in a Patient with von Willebrand Disease

Abstract

Renal lymphangioma is a rare benign lymphatic malformation that may present with hematuria and flank pain. We report the case of a 16-year-old female with type 2A von Willebrand disease who developed recurrent macroscopic hematuria and left lumbar pain. Imaging revealed a multiloculated intrarenal cystic lesion consistent with renal lymphangioma. After recurrence of symptoms and failure of conservative management, percutaneous intrarenal sclerotherapy with doxycycline was performed under factor replacement, resulting in complete symptom resolution and no recurrence on follow-up. This case highlights the diagnostic challenge in coagulopathic patients and supports sclerotherapy as an effective minimally invasive treatment.