Phlegmasia cerulea dolens is a devastating sequelae of propagating deep vein thrombosis causing total venous outflow obstruction of an extremity. It is characterized by significant pain, edema, cyanosis, and critical limb ischemia and may progress toward venous gangrene. Morbidity and mortality rates associated with this phenomenon are high. Treatment options are limited and consist of early and aggressive therapeutic anticoagulation and fluid resuscitation, followed by thrombectomy or thrombolysis if the patient fails to respond clinically in 6-12 hours.

Phlegmasia cerulea dolens (PCD) is a rare yet severe complication of deep vein thrombosis (DVT), characterized by a high amputation rate and mortality. Early diagnosis and treatment are crucial in managing this condition. PCD predominantly affects the lower extremities rather than the upper extremities. We herein present a rare upper extremity PCD case accompanied with supra vena cava and pulmonary embolism in a cervical cancer patient, who presented to our institution with severe pain, edema and irreversible venous gangrene of right upper limb with no response to anticoagulation therapy. Emergency fasciotomy and amputation were performed due to the progressed venous gangrene, however, the patient developed severe infection and coagulation disorders, gastrointestinal bleeding and disseminated intravascular coagulation after the surgery. Despite medical interventions, her family chose to withdraw treatment and the patient died in ICU at the fourth day following emergency surgery.

During the COVID-19 pandemic, high rates of arterial and venous thromboembolic (VTE) events were noted in association with increased rates of major amputation. VTE appears to be a significant source of morbidity and mortality for this patient population and numerous methods have been described to achieve limb salvage. Nevertheless, best management remains unclear. We describe the case of a 60-year-old male with severe venous gangrene secondary to a non-occlusive mid-femoral and occlusive infrapopliteal deep venous thromboses associated with COVID-19 infection who ultimately underwent meticulous local wound care and transmetatarsal amputation, which allowed for maximal preservation of foot function and limb salvage.

Lower limb amputation following arterial cannulation for VA-ECMO has been described in the literature. Limb ischemia however following venous cannulation is very rare and not quite understood. We present a case of limb ischemia following venous cannulation. A combination of venous congestion, compartment syndrome and subsequent arterial insufficiency is the proposed pathophysiology. Shock and use of vasopressors are compounding factors. Limb ischemia can be transient and reversible if diagnosed immediately and treated by early removal of the cannula. Our patient was unstable and ECMO dependent, and removal of the cannula was not an option. This resulted in limb loss and eventual above knee amputation. Use of the smallest appropriate venous cannula and early fasciotomy, in addition to hemodynamic optimization are measures that could help in preventing major amputation.

Presented in this article is a clinical case report regarding treatment of a patient with deep vein thrombosis complicated by venous gangrene having developed 10 days after the onset of the disease. Conservative therapy (infusion of colloids and crystalloids, anticoagulants, agents improving microcirculation, venotonics, nonsteroidal anti-inflammatory drugs, elevated position of the limb) made it possible to stabilize the patient\'s condition, but not improving haemodynamics of the affected limb. A decision was made to use endovascular techniques. Treatment was carried out in three stages. The first stage during 48 hours consisted in regional catheter thrombolysis with urokinase, exerting a minimal clinical effect. The second stage was percutaneous mechanical thrombectomy after which the diameter of thrombosed veins became free by half, with the beginning of disease regression. The third stage consisted in venous stenting of residual stenosis of the iliac vein, resulting in normalization of the venous outflow from the affected limb. A conclusion was drawn on feasibility of combined use of regional thrombolysis, percutaneous mechanical thrombectomy, and venous stenting in treatment of venous gangrene.
В статье представлен клинический случай лечения пациента с тромбозом глубоких вен, осложненным венозной гангреной, развившейся спустя 10 суток от начала заболевания. Консервативная терапия (инфузия коллоидов и кристаллоидов, антикоагулянты, средства улучшающие микроциркуляцию, венотоники, нестероидные противовоспалительные препараты, возвышенное положение конечности) позволила стабилизировать состояние больного, но не улучшила гемодинамику пораженной конечности. Было решено применить эндоваскулярные технологии. Лечение проводилось в три этапа. На первом этапе в течение 48 часов выполнен региональный катетерный тромболизис урокиназой, который оказал минимальный клинический эффект. На втором этапе – чрескожная механическая тромбэктомия, после чего диаметр тромбированных вен освободился наполовину, начался регресс заболевания. На третьем этапе – венозное стентирование остаточного стеноза подвздошной вены, что привело к нормализации венозного оттока из пораженной конечности. Сделано заключение о целесообразности комбинированного применения регионарного тромболизиса, механической чрескожной тромбэктомии и венозного стентирования при лечении венозной гангрены.

Phlegmasia cerulea dolens (FCD) is a rare complication of deep vein thrombosis. Its cause is unknown. The main predisposing factors for the disease are neoformative processes, hypercoagulable states, congestive heart failure, pregnancy, prolonged immobilization, and surgeries on the affected limb. FCD is characterized by massive edema, severe pain, and cyanosis. The diagnosis is clinical. It is associated in most cases with pulmonary embolism and can lead to loss of the compromised limb if not treated in time. So far there is no consensus on its treatment. In clinical practice the use of anticoagulation with heparin, local thrombolysis, systemic fibrinolysis, surgical thrombectomy, fasciotomy, and inferior vena cava filter are described. In irreversible cases amputation is required. We present the case of a patient with FCD, the treatment performed and the evolution.
La flegmasia cerulea dolens es una complicación rara y poco frecuente de la trombosis venos a profunda. Los principales factores predisponentes son los procesos neoformativos, estados de hipercoagulabilidad, insuficiencia cardíaca congestiva, embarazo, inmovilización prolongada y cirugías. Se caracteriza por edema masivo, dolor intenso y cianosis. Sin tratamiento evoluciona con isquemia, necrosis y amputación del miembro comprometido. No existe consenso en su tratamiento, pero éste debe ser rápido, multidisciplinario y agresivo. La anticoagulación con heparina, la fibrinólisis sistémica, la trombectomía percutánea con fibrinólisis local, la trombectomía quirúrgica, la fasciotomía, la colocación de filtro de vena cava inferior y la amputación son algunos de los tratamientos propuestos.

BACKGROUND: Warfarin anticoagulation of oncologic patients with DVT may paradoxically progress to phlegmasia cerulea dolens and limb gangrene, due to cancer-associated disseminated intravascular coagulation. This case report, written in line with the SCARE criteria, endorses venous thrombectomy in selected patients to attempt limb salvage. A young woman\'s warfarin associated acute ileofemoral phlegmasia that developed over cervical cancer radiation therapy induced DVT, was successfully resolved by clot removal. Extracting venous thrombus at the onset, while still as unorganized masses, preserves ambulation and prevents post-thrombotic syndrome development, an improvement of quality of life especially significant for oncologic patients with limited life-expectancy.
METHODS: A 34 years old female, with history of stage 3 cervical cancer following radiation therapy, was admitted in regards to left lower limb painful pitting oedema with cramps. Doppler scan revealed a left ileofemoral DVT. She was set on LMWH, but on fourth day of warfarin co-administration, phlegmasia cerulea dolens developed. An emergency venous thrombectomy with fasciotomy was performed. Postoperatively, dry foot gangrene developed, which dictated transmetatarsal amputation. The patient was discharged after 2 months of inpatient treatment, preserving ambulation.
CONCLUSIONS: Venous thrombectomy (with fasciotomy) in oncologic phlegmasia, ±serial debridement, becomes an attractive opportunity for limb salvage when feasible at acute presentation, available in a limited resource setting.
CONCLUSIONS: Due to severe procoagulant/anticoagulant balance disturbances in cancer patients\' warfarin-bridged for DVT, phlegmasia onset should not defer surgical approach, unless a rapid response to conservative treatment.

BACKGROUND: Venous gangrene complicating deep vein thrombosis in the context of anticoagulation use in patients with gynecologic malignancy is rarely reported. We report an unusual presentation of venous gangrene of the lower limbs associated with warfarin therapy in a 53-year-old woman from the Cook Islands with an occult ovarian cancer.
METHODS: A 53-year-old woman of Cook Islands origin presented with exertional dyspnea, rapid atrial fibrillation, bilateral lower limb edema, and painful digital ischemia of her hallux. She was on warfarin therapy for atrial fibrillation and had a stable therapeutic international normalized ratio. Bilateral proximal lower limb deep vein thrombosis and digital gangrene subsequently developed in the setting of a supratherapeutic international normalized ratio and platelet count depletion. Her warfarin was reversed and heparin therapy was commenced with resulting correction of her thrombocytopenia.
CONCLUSIONS: We would like to attract the attention of the reader to the potential hazard of the use of warfarin in patients with malignancy. In our case, we also demonstrated a predictive value of supratherapeutic international normalized ratio and platelet count depletion that could herald massive thrombosis and gangrene in a patient who was previously stable on warfarin therapy. Early recognition and prompt reversal of warfarin in these circumstances is essential to correct the unbalanced prothrombotic process that leads to extensive thrombosis and gangrene. The outlook of such cases remains dismal and results in extensive morbidity and mortality.

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.

Bilateral venous thrombosis due to underlying malignancy is a rare entity. It is worthy to search for malignancy in patients of bilateral venous gangrene. Our patient presented with severe bilateral leg pain as a result of venous gangrene. There was associated left sided massive pleural effusion with scalp nodule. Fine needle aspiration cytology of scalp nodule revealed metastatic squamous cell carcinoma and fiber optic bronchoscopy guided biopsy from growth at left upper lobe bronchus confirmed the case as squamous cell carcinoma lung. It was rare for squamous cell carcinoma lung to present as bilateral venous gangrene with anticardiolipin antibody negative.