Wunderlich syndrome (WS) refers to a rare condition characterized by spontaneous and potentially life-threatening bleeding in the renal area. It is frequently linked to various underlying renal conditions, including neoplasms, infections, arteriovenous malformations, or coagulopathy. Renal angiomyolipoma (AML) take the lead as the main culprits behind the development of benign neoplasms. Prompt intervention relies on a high level of clinical suspicion and imaging as the cornerstone of diagnosis. While a significant portion of patients with this condition may require surgical intervention, cases with minor ruptures and limited internal bleeding can be managed effectively with conservative treatment. Wunderlich syndrome with tuberous sclerosis (TS) is a rarely documented condition in the literature. However, we are presenting a noteworthy case of Wunderlich syndrome in a patient with TS and bilateral AML who underwent therapeutic arterial embolization (TAE).

 

A 26-year-old woman with a history of epilepsy and under anti-epileptic drug treatment presented with left upper abdominal pain for 6 days. She had not experienced any trauma recently. Physical examinations showed pale conjunctiva, muscle guarding, and tenderness in the left abdomen. Additionally, angiofibroma was observed across her cheeks. The laboratory findings revealed leukocytosis, microcytic anemia and increased level of C-reactive protein (CRP), while creatinine, lipase and coagulation profile were within the normal range. Abdominal computed tomography (CT) revealed a well-defined subcutaneous soft tissue density mass with hyperdense component, an intraparenchymal renal hematoma in bilateral kidney. Additionally, active contrast extravasation and pseudoaneurysm formation were observed in the left kidney. (Figure 1) The patient received a diagnosis of tuberous sclerosis (TS) and bilateral angiomyolipoma (AML) with WS. As a result, she was transferred to our hospital for therapeutic arterial embolization (TAE) and was treated conservatively with fluid hydration, analgesic agents, antibiotics, and antifibrinolytic agents after TAE. Laboratory results showed a significant reduction in CRP levels and white blood cell count while creatinine levels remained within the normal range. The patient experienced a smooth recovery and was discharged from the hospital after ten days of hospitalization.

When encountered a patient suffering from acute flank pain along with unexplained haemodynamic instability and/or a decline in hemoglobin levels, clinicians should always consider the possibility of WS.