Rare Case of Unilateral Phlegmasia Cerulea Dolens With Bilateral Deep Vein Thrombosis at a Community Military Hospital Emergency Department

Abstract

Phlegmasia cerulea dolens (PCD) is a rare entity that refers to a painful, edematous, and cyanotic limb due to a massive deep vein thrombosis (DVT). Due to its rarity, the exact incidence is unknown; however, it is vital that the military health care provider recognize it as the condition can be limb and life threatening. Due to the recent increase in combat-related operations, DVT has had a steady increase in the past 10 years in the military population, and as such has become a condition of special interest and surveillance in the armed forces. PCD is part of a spectrum that consists of distal DVT, more proximal DVT, phlegmasia alba dolens (PAD), and finally PCD with venous gangrene. PAD is an early stage of PCD, in which although there is a massive DVT present, the collateral and superficial circulation are not yet involved; this in turn results in a painful, edematous, white leg. PCD is preceded by PAD in approximately 50% to 60% of the cases. PCD has an amputation rate of up to 50% and a mortality rate of up to 40%. The patient will present with a swollen, cyanotic, painful leg that may or may not show signs of venous gangrene. In PCD, the collateral circulation is not spared and this causes severe congestion and fluid sequestration in the limb leading to venous hypertension. This can lead to circulatory shock and arterial insufficiency as it progresses. We review a case report of a 66-year-old woman that presented to small community army hospital after a 26-hour bus drive with knee pain and leg swelling. The diagnosis of PCD was made after Doppler ultrasonography showed bilateral iliofemoral, common, femoral, and saphenous veins thrombosis. The patient's left lower extremity was discolored, tender, and swollen, although it had not progressed to venous gangrene or dermal necrosis. While the management of PCD is not standardized due to the rarity of the condition, several options are available. These options include anticoagulation, minimally invasive procedures such as catheter-directed thrombolysis, or more invasive procedures such as surgical thrombectomy. In the active duty military population, clot reduction techniques would be preferred to long-standing anticoagulation, as the morbidity is greater with anticoagulation alone as well as the probability of major hemorrhage. Besides pulmonary embolism, which is a complication in up to 30% of the patients with PCD, one must keep in mind the extent and duration of the thrombus when choosing a treatment method, as these factors are directly related to the morbidity associated with post-thrombotic syndrome. Functional impairment after a massive DVT or PCD is an important factor that must be kept in mind for troop readiness.