Phlegmasia Cerulea Dolens

Abstract

GANGRENE and eyanosis of an extremity, due to thrombophlebitis with massive venous occlusions, is an uncommon condition and is often confused with primary arterial oeclusive disease. With massive venous occlusion and the associated arterial venospasm the blood flow may be so impeded to an extremity that cyanosis and eventually gangrene may occur. The recognition of this is important, since this gangrene is usually superficial or limited to the digits. Conservative treatment of this type of gangrene is indicated in contrast to the more radical treatment advocated for gangrene due to primary arterial insufficiency. This disease has been called "phlegmasia cerulea dolens," "blue phlebitis of Gregoire," "acute massive venous occlusion of the extremities," "pseudoembolic phlebitis, " and " gangrene of venous origin. " That gangrene of an extremity can be of venous origin was first recognized by Fabricius Hiddanus in 1593.1 Excellent review articles on phlegmasia cerulea dolens have been published by Haimovici2 auid DeBakey,3 making a detailed review of the literature unnecessary. Of the 102 cases in the literature in which information is available, 69 occurred in the left lower extremity, 27 in the right lower extremity, four in the left upper extremity, and two in the right upper extremity. The high incidence in the left lower extremity is probably because the right common iliac artery crosses over the left common iliac veini shortly after the bifurcation of the abdominal aorta. Spontaneous involvement of the upper extremity is rare, and it has been reported in only five previous cases. It is the purpose of this paper to report two cases, one with involvement of the upper extremity.