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Ruptured aortic aneurysm masquerading as phlegmasia cerulea.

Patrick O Myers1, Afksendiyos Kalangos, Sylvain Terraz

  • 1Division of Cardiovascular Surgery, Geneva University Hospital, Geneva, Switzerland. patrick.myers@hcuge.ch

The American Journal of Emergency Medicine
|December 19, 2008
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Summary

A ruptured abdominal aortic aneurysm can mimic phlegmasia cerulea dolens due to an aortocaval fistula. This rare condition, termed "blue fistula," presents with limb swelling and cyanosis, often leading to fatal hemorrhage.

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Area of Science:

  • Vascular Surgery
  • Cardiology
  • Emergency Medicine

Background:

  • Phlegmasia cerulea dolens is a severe venous thrombosis causing limb swelling, cyanosis, and pain.
  • Abdominal aortic aneurysms (AAAs) can rupture, posing a significant mortality risk.
  • Aortocaval fistulas are rare but life-threatening complications of ruptured AAAs.

Observation:

  • A 68-year-old male presented with symptoms mimicking phlegmasia cerulea dolens, including lower limb edema and cyanosis.
  • The patient also had asthenia, macrohematuria, and mild back pain.
  • Computed tomography revealed an 85-mm abdominal aortic aneurysm ruptured into the inferior vena cava, forming an aortocaval fistula.

Findings:

  • The patient's phlegmasia cerulea dolens-like symptoms were attributed to venous outflow compromise caused by the aortocaval fistula.
  • Aortocaval fistulas, occurring in 3-6% of ruptured AAAs, can present with abdominal/back pain, a pulsatile mass, and a murmur.
  • Diagnosis is often delayed until intraoperative hemorrhage, with high mortality rates.

Implications:

  • The study proposes a new term, "blue fistula," for the clinical syndrome of aortocaval fistula presenting with phlegmasia cerulea dolens-like symptoms.
  • Early recognition of aortocaval fistulas is crucial for improving survival rates in ruptured AAA cases.
  • This case highlights the importance of considering atypical presentations of ruptured AAAs in emergency settings.