A 55 year old male with a past medical history of colon cancer on chemotherapy presents with a chief complaint of right lower extremity pain/discoloration to his calf and thigh as well shortness of breath that has developed over the past 48 hours. A physical exam reveals dopplerable DP and PT pulses. The right calf and thigh is visualized as in the image below. What’s the diagnosis?
Answer: Phlegmasia Cerulea Dolens (PCD) – a near-total occlusion of the major deep venous system of an extremity as well as the majority of microvascular collateral veins of the extremity.
PCD occurs on a spectrum with phlegmasia albans dolens (PAD): thrombosis of the deep venous system with patency of the collateral veins and venous gangrene: when there is complete obstruction of venous outflow with irreversible capillary involvement and muscle infarction. It is differentiated from PAD by a pale/white limb versus a dusky/cyanotic limb in PCD.
Clinical Features: Triad of swelling, pain, cyanosis. Limb can develop firmness and there is a risk for arterial compromise and compartment syndrome. Thrombosis can extend into the IVC and it is often accompanied by pulmonary embolism with the incidence reported to vary from 12-40%.
Diagnosis: Clinical history/exam in conjunction with imaging. Gold standard diagnosis is contrast venography, however often due to difficulty and length of time to attain this ultrasound venography is often preferred. CT-venogram is useful for visualization of extension of thrombus in the IVC.
Management: Immediate elevation of affected extremity above the level of the heart to encourage return of circulation. Anticoagulation with unfractionated IV heparin bolus at 10-15 units/kg followed by an infusion titrated to an aPTT of 1.5-2 times the lab control value. Immediate vascular/interventional radiology consultation for possibly thrombectomy versus catheter-directed thrombolysis. If no service is available and transfer is unable to be arranged within 6 hours then consider systemic fibrinolytics if no contraindications are present.
Case Continued: Duplex ultrasound, CT-venogram, and CTA Chest on our patient revealed DVT of the major deep veins of the right lower extremity that extended into the IVC as well as bilateral pulmonary embolisms. He was started on heparin and had a mechanical thrombectomy of the right iliofemoral/IVC DVT. He was able to be discharged on apixaban several days later.
Resources:
Cline, D., Ma, O. J., Meckler, G. D., Stapczynski, J. S., Thomas, S. H., Tintinalli, J. E., Yealy, D. M., & Kline, J. A. (2020). Venous Thromboembolism Including Pulmonary Embolism. In Tintinalli’s emergency medicine: A comprehensive study guide (pp. 389–398). essay, McGraw-Hill Education.
Gardnella, L., & Falk, J. (n.d.). Phlegmasia Alba and cerulea Dolens – StatPearls – NCBI Bookshelf. Phlegmasia Alba and Cerulea Dolens. https://www.ncbi.nlm.nih.gov/books/NBK563137/
Perkins, J. M., Magee, T. R., & Galland, R. B. (1996). Phlegmasia caerulea dolens and venous gangrene. British Journal of Surgery, 83(1), 19–23. https://doi.org/10.1002/bjs.1800830106
