Abstract
Renal lymphangiomatosis is a rare congenital condition characterized by the abnormal development of lymphatic channels in the kidney, resulting in cystic dilatations. While more commonly observed in children, it can occur in adults but is extremely rare. Clinical manifestations range from asymptomatic cases to symptoms such as abdominal pain, hypertension, and renal dysfunction. In this case report, we present a rare case of bilateral renal lymphangiomatosis in an eight-year-old male with high blood pressure. Renal ultrasound revealed bilateral kidney enlargement and perinephric hypoechoic collections with septations consistent with lymphangiomatosis. The diagnosis was confirmed through CT imaging which shows bilateral non-enhancing perinephric collections. As a part of the patient's management plan, bilateral perinephric cystic lesions were successfully aspirated without complications. In conclusion, accurate diagnosis is crucial for appropriate management decisions, and treatment primarily focuses on conservative measures to manage associated hypertension, reduce lymphatic fluid accumulation, and alleviate pain, reserving invasive interventions for severe cases or complications.