Spontaneous perinephric hematoma (SPH), or Wunderlich syndrome, is a rare but potentially life-threatening emergency most often caused by the rupture of a renal angiomyolipoma (AML). A 60-year-old woman presented with sudden-onset right flank pain and anemia in the absence of trauma. Imaging revealed a large right perinephric hematoma secondary to a ruptured renal AML with giant aneurysms and pseudoaneurysms. Despite the extensive hemorrhage, she remained hemodynamically stable and underwent urgent selective transarterial embolization. Post-embolization imaging confirmed complete hemostasis with preservation of renal parenchyma. A compressive perinephric hematoma resulted in transient Page kidney physiology, which was managed conservatively with blood pressure control. This case illustrates several uncommon features: hemodynamic stability despite massive hemorrhage, concurrent giant aneurysms and pseudoaneurysms, and the development of Page kidney. It underscores the diagnostic utility of contrast-enhanced CT and the increasing role of selective endovascular therapy as a nephron-sparing alternative to nephrectomy. Emerging evidence suggests that vascular complexity within AMLs, particularly giant aneurysms and pseudoaneurysms, may be more predictive of rupture than tumor size alone. Prompt imaging, multidisciplinary coordination, and minimally invasive transarterial embolization can yield excellent outcomes in ruptured AML with complex vascular anatomy. Early angiographic evaluation should be considered in symptomatic or high-risk AMLs, with close monitoring for Page kidney to optimize long-term renal preservation.
Alataa et al. (Sat,) studied this question.