Wunderlich Syndrome in a Patient with Tuberous Sclerosis Complex Presenting with Hypovolemic Shock.: Wunderlich Syndrome in Tuberous Sclerosis

Abstract

Background:  Wunderlich syndrome is an uncommon but potentially fatal clinical entity defined by spontaneous, non-traumatic renal or perirenal hemorrhage. It is most frequently associated with renal angiomyolipoma, particularly in patients with tuberous sclerosis complex (TSC), where lesions are often bilateral, large, and highly vascularized, predisposing them to rupture.

Case Presentation: We report the case of a 30-year-old female with a known history of TSC who presented to the emergency department with acute right flank pain and signs of hypovolemic shock. Contrast-enhanced computed tomography (CECT) revealed a large right-sided perirenal and retroperitoneal hematoma with active contrast extravasation, originating from a ruptured renal angiomyolipoma. Multiple bilateral renal angiomyolipomas were also identified. The prompt use of advanced imaging facilitated rapid diagnosis and guided urgent management.

Discussion: This case underscores the pivotal role of CECT as the diagnostic modality of choice in suspected Wunderlich syndrome, allowing precise localization of bleeding, assessment of lesion characteristics, and evaluation of hemorrhage extent. Patients with TSC require close radiological surveillance due to the elevated risk of angiomyolipoma rupture and life-threatening hemorrhagic complications.

Conclusion: Wunderlich syndrome represents a rare but critical urological emergency, particularly in patients with TSC. Early recognition through contrast-enhanced imaging is essential for timely intervention and improved clinical outcomes. Proactive monitoring of renal angiomyolipomas in high-risk populations is imperative to prevent catastrophic events.