SLR - August 2015 - Kinjal Patel

Clinical Problem-Solving. Out of the Blue

Reference: Gibson CJ, Britton KA, Miller AL, Loscalzo J. Clinical Problem-solving. Out of the Blue. N Engl J Med. 2014 May 1; 370(18):1742-8
Scientific Literature Review
Reviewed By: Kinjal Patel, DPM
Residency Program: Wyckoff Heights Medical Center
Podiatric Relevance: Phlegmasia cerulea dolens is a severe form of deep venous thrombosis that results in congestion and cyanosis of a limb due to massive venous thrombosis. With prolonged ischemia, the viability of the limb is at risk. This condition is uncommon, however can be associated with a high degree of morbidity and mortality. The etiologies may include hypercoagulable syndrome, trauma, surgery; with malignancy being the most common cause. The patient will present with a triad of asymmetric edema, pain and cyanosis. The discoloration of the extremity usually presents distally and may extend proximally. In severe cases the patient may also present with gangrene.  For the less severe and non-gangrenous forms of phlegmasia cerulea dolens, anticoagulation and fluid restriction should be initiated and the leg should be elevated immediately. Surgical thrombectomy has been the procedure of choice for severe cases. This rare condition should be recognized immediately and proper treatment should be executed.

Case Report Summary: A 55 year-old male with a medical history of obesity, paroxysmal atrial fibrillation, hypertension, and heart failure presented to the Emergency Department. There was no history of arterial or venous thrombosis. Upon initial examination there was pain, swelling and dusky discoloration of the toes on his right leg and foot. Three days prior the patient noted sudden onset of swelling in the right lower extremity. The patient did report weight loss of 50 lbs over the past six months, which he believed was due to diet and exercise. This drastic weight loss was of concern and the authors believed that there was an underlying cancer, which could have resulted in the patient being in a hypercoagulable state.

On physical examination, the patient was afebrile, with a heart rate of 80 beats per minute and a blood pressure of 96/50 mm Hg. The abdomen was obese, soft, and nondistended, with normal bowel sounds and no hepatosplenomegaly or masses. The legs were markedly asymmetric, with 4+ pitting edema of the upper right thigh and 2+ pitting edema of the left calf. The right leg and foot were dusky and cold, and distal pulses were detectable only on doppler. There were discrete hemorrhagic bullae on a purpuric base with central, dusky discoloration to the right leg and foot. The entire right lower extremity was edematous but without eczematous change or scaling. Pulses in the left leg were easily palpable, and there was no edema. Muscle tone and strength and sensation of pinprick and light touch were intact and equal in both legs.

An ultrasonogram of the right leg showed noncompressibility of the deep veins, a finding consistent with thrombosis, extending from the common femoral vein to the popliteal vein. The patient had an INR of 4.3 which intravenous vitamin K was administered and the INR was decreased to 1.5. In addition to this, unfractionated heparin was also initiated. Leg venography revealed complete occlusion of the right common femoral vein, femoral vein and popliteal vein. Thrombolysis with tissue plasminogen activator was then performed.

The authors suggest that although the management of the limb ischemia must be the immediate focus, the presence of phlegmasia cerulea dolens suggests cancer as the underlying cause. This patient was then worked up for underlying cancer. Computed tomography of the abdomen and pelvis showed multiple hypodense liver lesions, up to 3 cm in diameter. The authors believe that this was a metastatic cancer that was causing a hepercoagulable state. Upper endoscopic biopsy of the mass was performed and revealed poorly differentiated, invasive adenocarcinoma of gastric origin. Unfortunately the patient died 10 days after admission due to progressive hypotension and renal failure.

Conclusion: Phlegmasia cerulea dolens is a medical emergency. Proper timely diagnosis and treatment is paramount in saving the limb and life. The triad of asymmetric edema, pain and cyanosis can help diagnose phlegmasia cerulea dolens. Phlegmasia cerulea dolens is a condition that podiatrists may encounter when evaluating patients with lower extremity manifestations. As podiatrists, it is important to be aware and to properly diagnosis and treat this rare limb and life threatening condition. 

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