Abstract 19342: Bilateral Phlegmasia Cerulea Dolens: More Than Meets the IVC

Abstract

A 40 year-old woman presented with edema, discoloration and severe pain of the lower extremities. Four weeks prior, she noted chest pain with pain and swelling of the right arm and both legs. Imaging demonstrated sub-segmental pulmonary embolism (PE) with bilateral deep venous thrombi (DVT) of the legs and thrombus of the right brachial vein. She was treated with warfarin however despite anticoagulation, had progressive dyspnea. Repeat imaging demonstrated new PE and progression of bilateral DVT. Anticoagulation was transitioned to argatroban, and an IVC [[Unable to Display Character: fi]]lter was placed. Subsequently, she had worsened edema and discoloration of the lower extremities, weakness, and severe pain. On physical examination, the lower extremities were cold and deeply violaceous with 2/5 strength at the bilateral hips and 0/5 strength below. Pulses were absent. Ultrasound examination demonstrated complete thrombosis of the lower extremity venous system to the level of the IVC. The clinical triad of limb swelling, ischemic pain, and blue color change suggested bilateral phlegmasia cerulea dolens (PCD). She was referred emergently for catheter based thrombolysis, however despite restoration of venous patency, she had progressive lower limb ischemia. She underwent bilateral above-the-knee amputation on hospital day 4. Given her profound thrombotic state, she underwent evaluation for inherited and acquired thrombophilia. To evaluate for malignancy, whole-body PET/CT was performed demonstrating FDG avidity of the gallbladder, multiple liver lesions, and adenopathy at the porta hepatis concerning for metastatic gallbladder carcinoma. Fine-needle biopsy of a liver lesion demonstrated poorly differentiated adenocarcinoma. Given poor prognosis, the patient elected not to pursue further therapy and passed away at home. This patient presented with bilateral PCD as a [[Unable to Display Character: fi]]rst manifestation of metastatic gallbladder carcinoma. PCD is a rare cause of limb ischemia characterized by the clinical triad of limb swelling, ischemic pain, and blue color change. Early attention to restoring venous patency is critical to avoid life and limb loss, and thrombophilia evaluation including evaluation for malignancy should be included in the diagnostic evaluation.