Skip to main content

Main menu

  • Home
  • Content
    • Current Issue
    • Ahead of Print
    • Past Issues
    • Supplements
    • Article Type
  • Specialty
    • Articles by Specialty
  • CME/MOC
    • Articles
    • Calendar
  • Info For
    • Manuscript Submission
    • Authors & Reviewers
    • Subscriptions
    • About CCJM
    • Contact Us
    • Media Kit
  • Conversations with Leaders
  • Conference Coverage
    • Kidney Week 2024
    • CHEST 2024
    • ACR Convergence 2023
    • Kidney Week 2023
    • ObesityWeek 2023
    • IDWeek 2023
    • CHEST 2023
    • MDS 2023
    • IAS 2023
    • ACP 2023
    • AAN 2023
    • ACC / WCC 2023
    • AAAAI Meeting 2023
    • ACR Convergence 2022
    • Kidney Week 2022
    • AIDS 2022
  • Other Publications
    • www.clevelandclinic.org

User menu

  • Register
  • Log in

Search

  • Advanced search
Cleveland Clinic Journal of Medicine
  • Other Publications
    • www.clevelandclinic.org
  • Register
  • Log in
Cleveland Clinic Journal of Medicine

Advanced Search

  • Home
  • Content
    • Current Issue
    • Ahead of Print
    • Past Issues
    • Supplements
    • Article Type
  • Specialty
    • Articles by Specialty
  • CME/MOC
    • Articles
    • Calendar
  • Info For
    • Manuscript Submission
    • Authors & Reviewers
    • Subscriptions
    • About CCJM
    • Contact Us
    • Media Kit
  • Conversations with Leaders
  • Conference Coverage
    • Kidney Week 2024
    • CHEST 2024
    • ACR Convergence 2023
    • Kidney Week 2023
    • ObesityWeek 2023
    • IDWeek 2023
    • CHEST 2023
    • MDS 2023
    • IAS 2023
    • ACP 2023
    • AAN 2023
    • ACC / WCC 2023
    • AAAAI Meeting 2023
    • ACR Convergence 2022
    • Kidney Week 2022
    • AIDS 2022
The Clinical Picture

Phlegmasia cerulea dolens from radiation-induced venous stenosis

Suman Paul, MBBS, PhD and Mujeeb Sheikh, MD
Cleveland Clinic Journal of Medicine December 2016, 83 (12) 865-867; DOI: https://doi.org/10.3949/ccjm.83a.15166
Suman Paul
Department of Internal Medicine, University of Toledo, OH
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: [email protected]
Mujeeb Sheikh
Division of Cardiology, Department of Internal Medicine, University of Toledo, OH
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • PDF
Loading

A 77-year-old man presented with a 5-day history of painful swelling of his right leg. He reported no trauma, no recent surgery, no history of thrombophilic disorder, and no prolonged immobilization. However, he had a history of prostate cancer, treated 10 years earlier with pelvic radiation.

Examination revealed massive right leg swelling extending from the thigh to the ankle, along with bluish-red skin discoloration (Figure 1). Doppler ultrasonography demonstrated acute thrombosis involving the right iliofemoral veins. These findings were consistent with phlegmasia cerulea dolens.

Urgent percutaneous catheter-directed thrombolysis was performed. Venography revealed extensive thrombosis of the femoral vein (Figure 2A) extending into the right external iliac vein. This was treated with catheter-directed pharmacomechanical thrombectomy.

FIGURE 1
  • Download figure
  • Open in new tab
  • Download powerpoint
FIGURE 1

The patient had painful swelling and bluish-red discoloration of the right leg at the time of presentation.

FIGURE 2
  • Download figure
  • Open in new tab
  • Download powerpoint
FIGURE 2

Venography (A) showed obstruction of venous flow in the right femoral vein (black arrow). After catheter-directed pharmacomechanical thrombectomy (B), venous flow was restored. Stenosis of the right external iliac vein (C) caused indentation of the expanding balloon (white arrow) during angioplasty. Panel D shows the external iliac vein after balloon dilation.

Venography after this procedure showed significant improvement in venous blood flow (Figure 2B). However, stenosis of the right external iliac vein was also noted (Figure 2C) and was treated with balloon angioplasty (Figure 2D) followed by placement of a stent (14 × 40 mm).

In the immediate postprocedural period, there was marked reduction in swelling and normalization of skin color (Figure 3). The patient did not experience significant bleeding during or after the procedure. Treatment with intravenous unfractionated heparin was continued during the hospital stay, and he was discharged on warfarin with a therapeutic international normalized ratio. At a follow-up visit 3 months later, he was asymptomatic.

FIGURE 3
  • Download figure
  • Open in new tab
  • Download powerpoint
FIGURE 3

After pharmacomechanical thrombectomy and placement of an iliac vein stent, the skin color returned to normal and the swelling resolved.

A RARE BUT SEVERE TYPE OF ACUTE DEEP VEIN THROMBOSIS

Phlegmasia cerulea dolens (painful cyanotic swollen leg) is a rare and severe form of acute deep vein thrombosis (DVT) characterized by marked limb pain, swelling, and blue discoloration.1 DVT is the most common cause of acute-onset unilateral leg pain, swelling, and skin discoloration.2

The differential diagnosis

The differential diagnosis includes infection (cellulitis, necrotizing fasciitis), compartment syndrome from limb injury, musculoskeletal conditions such as ruptured Baker cyst, venous stasis due to external compression (May- Thurner syndrome, iliac vein compression syndrome, pelvic tumor), acute limb ischemia from arterial obstruction, and complex regional pain syndrome (reflex sympathetic dystrophy).

Management recommendations

As in most cases of DVT, initial treatment of phlegmasia cerulea dolens involves systemic anticoagulation with heparin, elevation of the affected extremity, and fluid resuscitation if the patient is hypotensive. However, phlegmasia cerulea dolens is a major indication for catheter-directed thrombolysis,3,4 so an urgent vascular surgery or interventional cardiology consultation is also required. The American College of Chest Physicians recommends catheter-directed thrombolysis for acute DVT of the iliofemoral veins in patients with symptoms for less than 14 days, good functional capacity, and a life expectancy beyond 1 year.5 This intervention results in reduced incidence of postthrombotic syndrome and improved quality of life5,6 compared with anticoagulation therapy alone.

Who is at risk?

Risk factors for phlegmasia cerulea dolens include a history of malignancy, inherited or acquired thrombophilia, surgery, radiation therapy, trauma, placement of an inferior vena cava filter, and pregnancy. In our patient, the iliac vein stenosis most likely was the result of the radiation therapy he had undergone for prostate cancer.

Arterial stenosis is a well-known complication of radiation therapy and is associated with an increased risk of cardiovascular events.7,8 Radiation induces endothelial damage followed by proliferation of smooth muscle cells, resulting in luminal stenosis and thrombosis. At the cellular level, radiation leads to an acute increase in pro-inflammatory cytokines and endothelial adhesion molecules, causing the recruitment of inflammatory cells to radiation-exposed vessels and chronic activation of transcription factor NF-kappa B, leading to long-term inflammation and angiogenesis.9

Carotid, coronary, and iliac artery stenosis are known to occur around 10 years after radiation therapy to the head, neck, breast, and pelvis. Radiation-induced iliac vein stenosis is rare and can manifest as acute proximal DVT.

  • Copyright © 2016 The Cleveland Clinic Foundation. All Rights Reserved.

REFERENCES

  1. ↵
    1. Mumoli N,
    2. Invernizzi C,
    3. Luschi R,
    4. Carmignani G,
    5. Camaiti A,
    6. Cei M
    . Phlegmasia cerúlea dolens. Circulation 2012; 125:1056–1057.
    OpenUrlFREE Full Text
  2. ↵
    1. Ely JW,
    2. Osheroff JA,
    3. Chambliss ML,
    4. Ebell MH
    . Approach to leg edema of unclear etiology. J Am Board Fam Med 2006; 19:148–160.
    OpenUrlAbstract/FREE Full Text
  3. ↵
    1. Casey ET,
    2. Murad MH,
    3. Zumaeta-Garcia M,
    4. et al
    . Treatment of acute iliofemoral deep vein thrombosis. J Vasc Surg. 2012; 55:1463–1473.
    OpenUrlCrossRefPubMed
  4. ↵
    1. Chinsakchai K,
    2. Ten Duis K,
    3. Moll FL,
    4. de Borst GJ
    . Trends in management of phlegmasia cerulea dolens. Vasc Endovascular Surg 2011; 45:5–14.
    OpenUrlCrossRefPubMed
  5. ↵
    1. Kearon C,
    2. Kahn SR,
    3. Agnelli G,
    4. Goldhaber S,
    5. Raskob GE,
    6. Comerota AJ,
    7. American College of Chest Physicians
    . Antithrombotic therapy for venous thromboembolic disease: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest 2008; 133(suppl 6):454S–545S.
    OpenUrlCrossRefPubMed
  6. ↵
    1. Enden T,
    2. Haig Y,
    3. Kløw NE,
    4. et al
    5. CaVenT Study Group
    . Long-term outcome after additional catheter-directed thrombolysis versus standard treatment for acute iliofemoral deep vein thrombosis (the CaVenT study): a randomised controlled trial. Lancet 2012; 379:31–38.
    OpenUrlCrossRefPubMed
  7. ↵
    1. Hooning MJ,
    2. Botma A,
    3. Aleman BM,
    4. et al
    . Long-term risk of cardiovascular disease in 10-year survivors of breast cancer. J Natl Cancer Inst 2007; 99:365–375.
    OpenUrlCrossRefPubMed
  8. ↵
    1. Weintraub NL,
    2. Jones WK,
    3. Manka D
    . Understanding radiation- induced vascular disease. J Am Coll Cardiol 2010; 55:1237–1239.
    OpenUrlFREE Full Text
  9. ↵
    1. Halle M,
    2. Gabrielsen A,
    3. Paulsson-Berne G,
    4. et al
    . Sustained inflammation due to nuclear factor-kappa B activation in irradiated human arteries. J Am Coll Cardiol 2010; 55:1227–1236.
    OpenUrlFREE Full Text
PreviousNext
Back to top

In this issue

Cleveland Clinic Journal of Medicine: 83 (12)
Cleveland Clinic Journal of Medicine
Vol. 83, Issue 12
1 Dec 2016
  • Table of Contents
  • Table of Contents (PDF)
  • Index by author
Print
Download PDF
Article Alerts
Sign In to Email Alerts with your Email Address
Email Article

Thank you for your interest in spreading the word on Cleveland Clinic Journal of Medicine.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Phlegmasia cerulea dolens from radiation-induced venous stenosis
(Your Name) has sent you a message from Cleveland Clinic Journal of Medicine
(Your Name) thought you would like to see the Cleveland Clinic Journal of Medicine web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Citation Tools
Phlegmasia cerulea dolens from radiation-induced venous stenosis
Suman Paul, Mujeeb Sheikh
Cleveland Clinic Journal of Medicine Dec 2016, 83 (12) 865-867; DOI: 10.3949/ccjm.83a.15166

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
Phlegmasia cerulea dolens from radiation-induced venous stenosis
Suman Paul, Mujeeb Sheikh
Cleveland Clinic Journal of Medicine Dec 2016, 83 (12) 865-867; DOI: 10.3949/ccjm.83a.15166
Twitter logo Facebook logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Linkedin Share Button

Jump to section

  • Article
    • A RARE BUT SEVERE TYPE OF ACUTE DEEP VEIN THROMBOSIS
    • REFERENCES
  • Figures & Data
  • Info & Metrics
  • PDF

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • No citing articles found.
  • Google Scholar

More in this TOC Section

  • Tinea incognito
  • Prolonged venous filling time and dependent rubor in a patient with peripheral artery disease
  • Sarcoidosis with diffuse purplish erythematous plaques on the hands
Show more The Clinical Picture

Similar Articles

Subjects

  • Emergency Medicine
  • Geriatrics
  • Imaging
  • Men's Health
  • Oncology
  • Vascular Medicine

Navigate

  • Current Issue
  • Past Issues
  • Supplements
  • Article Type
  • Specialty
  • CME/MOC Articles
  • CME/MOC Calendar
  • Media Kit

Authors & Reviewers

  • Manuscript Submission
  • Authors & Reviewers
  • Subscriptions
  • About CCJM
  • Contact Us
  • Cleveland Clinic Center for Continuing Education
  • Consult QD

Share your suggestions!

Copyright © 2025 The Cleveland Clinic Foundation. All rights reserved. The information provided is for educational purposes only. Use of this website is subject to the website terms of use and privacy policy. 

Powered by HighWire