Abstract

Purpose: Wunderlich syndrome (WS) with hypovolemic shock secondary to ruptured renal angiomyolipoma (rAML) represents an urgent condition. Hence, we reported our experience with transcatheter arterial embolization (TAE) using different embolic materials under this condition.Methods: This retrospective study consisted of 22 patients. Embolic materials including particles, microcoils, and liquid embolic agents were selectively used based on the decisions of interventional radiologists. Technical success was defined as the complete occlusion of bleeding vessels on the final renal angiogram. Clinical success was defined as the absence of re-hemorrhage needed for repeat endovascular or surgery treatment after TAE.Results: The articulated materials were used when WS presented without aneurysms; a combination of particulate materials and microcoils or Glubran 2 alone were used for WS with aneurysms. The technical success based on 24 episodes of TAEs in 22 patients was 100% (24 of 24). Repeat TAE was achieved in two patients with hemorrhages re-occurring two days after the initial embolization with microcoils alone. The clinical success was 90.9% (20 of 22). No nontarget embolization or embolization-related complications occurred during the TAE procedure. Of the patients, 27.3% (6 of 22) experienced minor complications of post-embolization syndrome (PES). During a median follow-up time of 34 months, no recurrent hemorrhage that required repeat endovascular or surgical treatment for hemostasis occurred.Conclusion: Urgent TAE with the selective use of different embolic materials is an effective alternative to control WS with hypovolemic shock secondary to ruptured rAML. The condition of presenting with or without aneurysms may determine the embolic materials employed.

Highlights

  • Renal angiomyolipoma is an uncommon hamartomatous neoplasm of the renal cortex

  • We present our experience with transcatheter arterial embolization (TAE) using different embolic materials, alone or in combination, to treat Wunderlich syndrome (WS) with hypovolemic shock secondary to ruptured Renal angiomyolipoma (rAML)

  • All patients presented with hypovolemic sock, accompanied with flank pain (81.8%) and hematuria (59.1%)

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Summary

Introduction

Renal angiomyolipoma (rAML) is an uncommon hamartomatous neoplasm of the renal cortex It consists of tri-phasic tissue with fat, smooth muscle, and abnormal blood vessels in varying proportions, and it usually occurs randomly or in adjunctive with tuberous sclerosis (TSC) [1, 2]. Despite being benign, it can possibly grow larger over time, potentially leading to various complications. Hemorrhage is a major complication of rAML that can be severe or life threatening. Ruptured rAML may be the most common cause (accounting for 35–40%) of benign neoplasm for Wunderlich syndrome (WS), which is a rare clinical condition classically characterized by the Lenk’s triad (flank pain, flank mass, and hypovolemic shock) [4, 5]

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