C51-12 Phlegmasia Cerulea Dolens of the Upper Extremity: A Rare Perfect Storm of Thrombosis in Critical Illness

Introduction Phlegmasia cerulea dolens (PCD) is a rare manifestation of deep vein thrombosis, characterized by massive venous occlusion conferring limb ischemia, potential circulatory collapse, and mortality rates of 20-40%. Upper extremity involvement comprises <10% of PCD cases but portends worse outcomes. We present a fatal case of upper extremity PCD in refractory septic shock. Case Presentation An 89-year-old woman with advanced dementia, schizophrenia, severe malnutrition, recurrent extended-spectrum beta-lactamase (ESBL) Escherichia coli sepsis, suspected untreated bladder malignancy with severe bilateral hydronephrosis, and a large unstageable sacral ulcer with probable osteomyelitis presented unresponsive from a skilled nursing facility with acute respiratory failure and septic shock. She required emergent intubation and swift escalation to four concurrent vasopressors (norepinephrine, vasopressin, phenylephrine, epinephrine).Laboratory results revealed profound lactic acidosis, leukocytosis, anemia, hypernatremia, and acute kidney injury. Despite empiric broad-spectrum antimicrobial therapy and aggressive resuscitation, she developed progressive multiorgan dysfunction, including hepatic injury, and oliguric renal failure.A midline catheter was placed in her left arm for vascular access, after which she developed acute left upper extremity (LUE) swelling, violaceous discoloration, coolness, absent pulses, and delayed capillary refill. After removal of the midline, vascular duplex ultrasonography confirmed occlusive thrombi throughout the deep and superficial venous systems. Computed tomography angiography demonstrated patent arteries with diffuse soft tissue edema and fat stranding. The limb progressed to venous gangrene with hemorrhagic bullae and purulent drainage despite therapeutic anticoagulation with intravenous heparin. Surgical intervention was deferred given a poor overall prognosis. After 10 days of maximal support complicated by persistent shock, massive volume overload, ongoing infections (ESBL urinary tract infection, Clostridioides difficile colitis), and progressive LUE gangrene, goals of care were transitioned to comfort measures after discussion with the patient’s legal guardian, and she expired on hospital day 15. Discussion This case highlights an example of a very rare case of upper extremity PCD in a patient with critical illness precipitated by placement of a midline. The patient’s suspected malignancy, immobilization, sepsis, and malnutrition comprised a rare maelstrom of hypercoagulability, leading to thrombosis immediately after the placement of a midline catheter. Key lessons for ICU care can be learned. First, PCD should be suspected with any acute limb changes, even when pulses are present. Second, we should have a low threshold for performing imaging in non-communicative patients where pain cannot be assessed.