Phlegmasia Cerulea Dolens in the Upper Extremity

Abstract

Phlegmasia cerulea dolens (PCD) is a rare form of venous gangrene that arises secondary to occlusion of venous outflow from a limb. Classically described in the lower extremity, it is rare in the upper extremity. We present a case report of upper extremity PCD and a systematic review with data analysis of 37 cases of PCD published in PubMed. Our analysis showed that concurrent lower extremity PCD was a statistically significant marker for worse outcomes in this patient population.

Upper-Extremity Phlegmasia Cerulea Dolens With Compartment Syndrome in Coronavirus Disease 2019 Sepsis

“…Phlegmasia cerulea dolens causing acute compartment syndrome in the upper extremity has been rarely described, and if present, the recommendations are to perform urgent thrombolysis or thrombectomy. 2,3,11 The unique surgical situation that arose in this patient, however, was that given her tenuous cardiopulmonary status in the setting of COVID-19 sepsis, she was unable to be brought to the operating room or interventional radiology suite. The complete reversal of the blood flow up to the subclavian artery during diastole, detected using ultrasonography, displays the striking potential of compartment pressures in this pathology.…”

“…Despite the high rate of limb and tissue loss without formal vascular interventions in the setting of phlegmasia, these fasciotomies, along with a therapeutic heparin drip, provided limb salvage in this critically ill patient. 3…”

“…2 The current recommendations include urgent vascular intervention for thrombolysis or thrombectomy and fasciotomies if compartment syndrome is diagnosed. 3 However, given the rising reports on severe arterial and venous thrombosis in critically ill COVID-19 patients, who may be unstable, difficult therapeutic decisions may have to be made. 1,4,5 We present a case that not only highlights the marked prothrombotic potential of severe COVID-19 infections, particularly in the setting of an indwelling intravenous line, but also characterizes the complexities of medical and surgical decision making in the era of COVID-19.…”

Upper Limb Phlegmasia Cerulea Dolens Secondary to Heparin-induced Thrombocytopenia Leading to Gangrene

“…Heparin-induced thrombocytopenia type II classically presents five to 14 days after the initiation of heparin therapy but delayed-onset heparin-induced thrombocytopenia has been described in the literature. The time of onset for patients on hemodialysis is usually one to two months with only 20% of patients being diagnosed within five to 10 days of starting dialysis [ 7 ]. It should be noted that it is the relative drop in the platelet count as compared to that of the baseline (at least a 30%-50% drop) rather than absolute thrombocytopenia that helps make the diagnosis of heparin-induced thrombocytopenia.…”

Pacemaker-associated Phlegmasia Cerulea Dolens Treated with Catheter-directed Thrombolysis

“…This leads to fluid extravasation and increased interstitial pressure, which impairs capillary blood flow. 4 While overall uncommon, PCD of the lower extremity is well studied and characterized in the literature. PCD of the upper extremity is even more rare, with the current medical literature on the topic existing mostly in the form of individual case reports.…”