Wünderlich ist nicht so Wunderbar: Bilateral Angiomyolipomas and Wünderlich Syndrome Requiring Emergency Embolization in a Patient without Tuberous Sclerosis

Ann Vasc Surg. 2016 Jul:34:270.e7-270.e11. doi: 10.1016/j.avsg.2015.12.014. Epub 2016 May 11.

Abstract

Bilateral renal angiomyolipomata are rare and usually associated with tuberous sclerosis. Renal angiomyolipomata can rupture spontaneously giving rise to (potentially catastrophic) retroperitoneal hemorrhage (Wünderlich syndrome). We present a very rare case of bilateral renal angiomyolipomata in an individual without tuberous sclerosis, presenting with life-threatening hemorrhage. The patient had emergency embolization of the bleeding angiomyolipoma and received elective embolization of a contralateral lesion. A follow-up brain magnetic resonance imaging showed no tubers but revealed a pituitary adenoma of uncertain significance.

Publication types

  • Case Reports

MeSH terms

  • Angiomyolipoma / complications*
  • Angiomyolipoma / diagnostic imaging
  • Embolization, Therapeutic*
  • Emergencies
  • Hemorrhage / diagnostic imaging
  • Hemorrhage / etiology
  • Hemorrhage / therapy*
  • Humans
  • Kidney Neoplasms / complications*
  • Kidney Neoplasms / diagnostic imaging
  • Male
  • Middle Aged
  • Retroperitoneal Space
  • Rupture, Spontaneous
  • Tomography, X-Ray Computed
  • Treatment Outcome