Case Report

Ruptured aortic aneurysm masquerading as Phlegmasia cerulea

Abstract

phlegmasia cerulea dolens, characterized by the triad of limb swelling, cyanosis, and acute ischemic pain, usually arises because of acute massive thrombosis of major deep, collateral, and superficial veins of an extremity. We report a patient with an atypically presenting ruptured aortic aneurysm masquerading as phlegmasia cerulea dolens. A 68-year-old man with a history of hypertension, inter- mittent claudication, and smoking presented with asthenia, macrohematuria, and mild back pain, as well as edema and blue mottling of the lower limbs and abdomen for 24 hours. The abdomen was nontender without a Pulsatile mass or murmur. Computed tomography showed an 85-mm Abdominal aortic aneurysm ruptured into the inferior vena cava. Phlegmasia cerulea dolens-like symp- toms were explained by compromised venous outflow from the lower limbs because of an aortocaval fistula. The patient died of uncontrollable hemorrhage during emergent Surgical repair. Three percent to 6% of patients with abdominal aortic aneurysm rupture present an aortocaval fistula, which can be suspected by the triad of abdominal or lower back pain, pulsatile Abdominal mass, and machinery- type abdominal murmur. Left untreated, this condition rapidly leads to death from congestive heart failure. Only one third of patients are diagnosed before surgery because a fistula is often not suspected until unexplained massive hemorrhage occurs during the operation. Operative mortality is extremely high (34%). We propose that the clinical syndrome presented by our patient, a “blue fistula” frequently associated with aortocaval fistulae, be called phlegmasia cerulea non dolens, by analogy to Gregoire’s “blue phlebitis.”

Phlegmasia cerulea dolens was first described in 1938 by Raymond Gregoire for a particular case of “blue phlebitis,” which he characterized by the triad of swelling, cyanosis, and acute ischemic pain of an extremity, to differentiate it from phlegmasia alba dolens [1]. It usually arises because of acute massive thrombosis of major deep veins as well as collateral and superficial veins of an extremity. It is often precipitated by a Hypercoagulable state, such as Trousseau

syndrome and primary abnormalities of blood coagulation, or after surgery, trauma, or giving birth [2]. It often progresses to venous gangrene, and amputation is necessary in 20% to 50% of patients. Mortality rate is reported as 20% to 40% [2]. Phlegmasia alba dolens is dominated by swelling and pallor of an extremity without ischemia and is due to massive deep venous thrombosis that spares collateral veins. An unusual cause of phlegmasia cerulea dolens-like symptoms occurs from impaired venous return from the lower limbs after rupture of an aortic aneurysm into the Inferior vena cava .

A 68-year-old man with a history of hypertension, intermittent claudication, and active smoking presented with asthenia, chills without fever, macrohematuria, and mild back pain, as well as edema and blue mottling of the lower limbs and abdomen for 24 hours. The patient was afebrile and had a heart rate of 150 beats per minute, with blood pressure of 80/54 mm Hg. Abdominal examination was normal, with no evidence of pain, pulsatile mass, or murmur. On vascular examination, the lower limbs were warm, severely cyanotic, and swollen with distended superficial veins. The arterial pulses were present, and there was no paresis or paresthesia. Hemodynamics did not improve after initial fluid resuscitation. Blood tests showed an increased white blood cell count (13.3 x 10⁹/L), C- reactive protein (95 mg/L), creatinine (135 umol/L), and lactate (3 mmol/L). Computed tomography showed abnor- mal enhancement of the IVC during arterial pHase (Fig. 1A) and an 85-mm-wide Abdominal aortic aneurysm . Reconstructed images revealed a direct communication between the AAA and the IVC (Fig. 1B and C). Phlegmasia cerulea dolens-like symptoms were thus explained by compromised venous outflow from the lower limbs because of an aortocaval fistula. Emergent surgical repair was attempted; however, the patient died of incontrollable hemorrhage.

Abdominal aortic aneurysm has a prevalence estimated at 1.0% to 8.3% and represents the 13th leading cause of death in the United States, from Spontaneous rupture. The annual rupture risk for AAA greater than 8 cm is 30% to 50%, and mortality rate after AAA rupture is 65% to 85%. Three percent to 6% of patients with AAA rupture present an aortocaval fistula [3], which is suspected by the triad of abdominal or lower back pain, pulsatile abdominal mass, and machinery-type abdominal murmur [4], although signs

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1067.e2 P.O. Myers et al.

typical of arteriovenous fistulae can be noted, such as venous congestion, high output congestive heart failure, widened Pulse pressures, and decreased distal pulses [4,5], Left untreated, this condition rapidly leads to death from congestive heart failure. Only one third of patients are diagnosed before surgical aneurysm resection and IVC repair because a fistula is often not suspected until unexplained massive hemorrhage occurs during the operation. Operative mortality is extremely high (34%). In our patient, an aortocaval fistula caused obstruction of venous outflow from the lower abdomen and legs, which led to edema, cyanotic mottling, and distended superficial veins in the characteristic pattern of phlegmasia cerulea dolens, without inducing ischemia and pain. Renal vein hypertension led to acute renal failure and hematuria. Despite normal arterial pulses and absence of pain, elevated lactate suggested impaired tissue perfusion. We propose that this clinical syndrome, a “blue fistula” frequently associated with aortocaval fistulae [3,5], be called phlegmasia cerulea non dolens, by analogy to Gregoire’s “blue phlebitis.”

Patrick O. Myers MD Afksendiyos Kalangos MD, PhD Division of Cardiovascular Surgery Geneva University Hospital Geneva, Switzerland

E-mail address: [email protected]

Sylvain Terraz MD Department of Radiology Geneva University Hospital Geneva, Switzerland

doi:10.1016/j.ajem.2008.03.019

References

1. Gregoire R. La phlebite bleue (phlegmatia caerulae dolens). Presse Med

1938;46(71):1313-5.

1. Perkins JMT, Magee TR, Galland RB. Phlegmasia cerulea dolens and venous gangrene. Br J Surg 1996;83:19-23.
2. Sakalihasan N, Limet R, Defawe O. Abdominal aortic aneurysm. Lancet

2005;365(9470):1577-89.

1. Viar WN, Lombardo TA. Abdominal aortic aneurysm with rupture into the inferior vena cava. Circulation 1952;5(2):287-9.
2. Davis P, Gloviczki P, Cherry K, et al. Aorto-caval and ilio-iliac arteriovenous fistulae. Am J Surg 1998;176(2):115-8.

Fig. 1 Abdominal CT during Arterial phase. Abnormal enhance- ment of the IVC (arrow) while the renal veins are not opacified (A). Both kidneys are normal. Reconstructed sagittal image (B) and reconstructed 3D image (C) showing a direct communication (arrow) between the AAA and the IVC, which is compressed at the level of the aneurysm and dilated elsewhere.