BACKGROUND
A 51-year-old Black woman with a past medical history of morbid obesity, schizophrenia, hypertension, myocardial infarction status post permanent pacemaker in the distant past, asthma, and obstructive sleep apnea presents with 3 days of progressive left leg swelling, pain, unsteady gait, wheezing, and shortness of breath. At baseline, the patient ambulates with a rollator. The patient denies periods of immobility, chest pain, palpitations, fever, chills, or cough. Social and family histories are noncontributory.
PHYSICAL EXAMINATION
The physical examination showed a large body habitus, decreased breath sounds, extensive cyanosis and edema of the left lower extremity, induration, tenderness to palpation, and livedo reticularis, which was most notable at the proximal portion of the extremity (see Figure 1). The right lower extremity was noted for trace edema only.
TESTING
The patient's chest x-ray showed clear lungs; no focal opacity, pleural effusion, or pneumothorax; and normal heart, mediastinum, and hila. A dual-lead pacemaker was in place. The chest computed tomography (CT) with intravenous contrast was a severely limited examination secondary to suboptimal contrast, and respiratory motion artifact showed pulmonary emboli at least within a right lower lobe extending to subsegmental branches. Additional emboli were questioned to be within the right upper lobe and left lower lobe. The left lower extremity venous ultrasound showed an occlusive thrombus within the left external iliac vein, common femoral vein, and deep femoral vein and proximal to the mid-femoral vein. CT venography of the left lower extremity and CT angiography of the abdomen and pelvis showed a sizable deep vein thrombosis (DVT) occupying the infrarenal inferior vena cava at the iliac bifurcation. It extended toward the left external iliac vein through the superficial femoral vein with reconstitution of the popliteal and tibioperoneal trunk. The deep femoral vein was not well visualized and thought to also be occluded (see Figure 2).
Because of the above findings, the patient was diagnosed with phlegmasia cerulea dolens (PCD) and pulmonary embolism. The patient received one dose of apixaban and was then switched to intravenous heparin. The patient was ultimately treated by percutaneous transluminal angioplasty and thrombectomy of the femoral vein. The left external and common iliac veins were treated by catheter-directed thrombolysis by the EKOS (EkoSonic Endovascular System, Boston Scientific, Marlborough, MA), thrombectomy, percutaneous transluminal angioplasty, and VICI Venous Stent (Boston Scientific, Marlborough, MA) placement. Afterward, the patient was discharged to a rehabilitation facility and continued on apixaban for anticoagulation.
DISCUSSION
PCD is an infrequent and rare presentation of a DVT, a precursor to venous gangrene and a medical emergency. The condition more frequently occurs in the left lower extremity versus the right lower extremity, and in general, it is more common in the lower extremities than the upper extremities (Chaar, 2020). The left lower extremity is thought to be affected more frequently because of compression of the left iliac vein by the right iliac artery (Perkins et al., 1996). It affects men more than women (Sajjan et al., 2015) at a ratio of 1.5:1 (Chaar, 2020). PCD can be idiopathic and can also result from risk factors such as malignancy, trauma, pregnancy, surgery, hypercoagulable states, Inferior vena cava filter insertion, and miscellaneous disease states (pneumonia, ulcerative colitis, aortic aneurysm, heart failure, May-Thurner syndrome; Chaar, 2020; Eggum et al., 1999; Haimovici, 1965; Lip & Hull, 2020). The inciting factor could not be identified in our patient.
Being able to promptly recognize its most prominent symptoms can lead to lifesaving treatment. Symptoms can slowly progress or be fulminant (Lip & Hull, 2020). They include extremely prominent edema of the extremity, cyanosis, and intense pain, all evidenced in the patient in this case. Temperature of the skin can vary from cool to normal to warm (Haimovici, 1965; Stallworth et al., 1965). If the condition is left to progress, paresthesias and weakness can occur (Chaar, 2020; Haimovici, 1965; Stallworth et al., 1965). Bullae may form with increasing fluid shifts (Chaar, 2020; Lip & Hull, 2020), and continued ischemia can lead to gangrene, limb loss, shock, and death. Mortality ranges from 20% to 41%, and amputation rates range from 12% to 50% (Yang & Yun, 2016).
It is important to exclude other illnesses such as cellulitis, arterial obstruction, and lymphatic obstruction. This can be distinguished from PCD through clinical appearance and testing such as blood cultures, complete blood counts, CT, CT angiography, and ultrasound. Duplex ultrasonography is used to diagnose the massive DVT that leads to PCD (Chaar, 2020).
Treatment of PCD is mainly focused on anticoagulation and surgical interventions. Medically, the patient is initially treated with intravenous heparin with eventual transition to low-molecular-weight heparins or Factor Xa inhibitors such as apixaban and rivaroxaban (Chaar, 2020). The patient should be maintained on anticoagulation for a minimum of 6 months (Chaar, 2020). Surgical interventions include catheter-directed thrombolysis or thrombectomy. Occluded vessels can have stents implanted after the thrombus has been dissolved. If the patient does not advance to limb ischemia, long-term use of compression stockings is necessary to help control chronic edema (Chaar, 2020).
Outpatient monitoring after this massive DVT includes repeat compression lower extremity ultrasounds and monitoring for postphlebitic syndrome, recurrent thrombosis, chronic thromboembolic pulmonary hypertension, thrombocytopenia, or bleeding-related adverse events (Lip & Hull, 2020).
This case highlights the need for medical providers to consider PCD when assessing a patient with livedo, cyanosis, pain, and edema of an extremity.
Acknowledgments
We thank Vivien Wong, a student at Edgemont High School, for assisting us in proofreading this article.
REFERENCES