View source for Medicine:Phlegmasia cerulea dolens

{{Infobox medical condition (new)
| name            = Phlegmasia cerulea dolens
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| caption         = A person with a two hour history of phlegmasia cerulea dolens (left leg, right side of image)
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'''Phlegmasia cerulea dolens''' (PCD) (literally: 'painful blue inflammation'), not to be confused with preceding [[Medicine:Phlegmasia alba dolens|phlegmasia alba dolens]], is an uncommon severe form of lower extremity deep venous thrombosis (DVT) that obstructs blood outflow from a vein. Upper extremity PCD is less common, occurring in under 10% of all cases.<ref>{{Cite journal|last1=Greenberg|first1=Jacques|last2=Troutman|first2=Douglas A.|last3=Shubinets|first3=Valeriy|last4=Dougherty|first4=Matthew J.|last5=Calligaro|first5=Keith D.|date=February 2016|title=Phlegmasia Cerulea Dolens in the Upper Extremity: A Case Report and Systematic Review and Outcomes Analysis|url=http://journals.sagepub.com/doi/10.1177/1538574416631645|journal=Vascular and Endovascular Surgery|language=en|volume=50|issue=2|pages=98–101|doi=10.1177/1538574416631645|pmid=26912398|s2cid=29270577|issn=1538-5744}}</ref> PCD results from extensive [[Medicine:Thrombosis|thrombotic]] occlusion (blockage by a [[Medicine:Thrombus|thrombus]]) of extremity veins, most commonly an iliofemoral DVT, of the iliac vein and/or common femoral vein.<ref>{{Cite book|url=https://www.worldcat.org/oclc/1037557259|title=Rutherford's vascular surgery and endovascular therapy|date=2019|others=Anton N. Sidawy, Bruce A. Perler|isbn=978-0-323-58130-1|edition=9th|location=Philadelphia, PA|oclc=1037557259}}</ref><ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref> It is a medical emergency requiring immediate evaluation and treatment.

==Symptoms and signs==

=== Primary symptoms ===
It is characterized by progressive lower extremity edema distal to the thigh, tight shiny skin, [[Medicine:Cyanosis|cyanosis]] (inadequate blood oxygenation), petechiae or [[Medicine:Purpura|purpura]], and sudden severe pain of the affected limb in proportion to the level of venous blockage.  Patients often have difficulty walking. [[Medicine:Blister|Blister]]s, bullae, [[Unsolved:Paresthesia|paresthesia]]s, and motor weakness may develop in severe cases, along with gangrene in ~50% of cases.<ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref><ref>{{Cite book|url=https://www.worldcat.org/oclc/1037557259|title=Rutherford's vascular surgery and endovascular therapy|date=2019|others=Anton N. Sidawy, Bruce A. Perler|isbn=978-0-323-58130-1|edition=9th|location=Philadelphia, PA|oclc=1037557259}}</ref> Distal pulses are palpable early on but may diminish over time, and [[Medicine:Doppler ultrasonography|doppler]] signal can be usually heard throughout disease progression.<ref>{{Cite book|url=https://www.worldcat.org/oclc/1037557259|title=Rutherford's vascular surgery and endovascular therapy|date=2019|others=Anton N. Sidawy, Bruce A. Perler|isbn=978-0-323-58130-1|edition=9th|location=Philadelphia, PA|oclc=1037557259}}</ref> The left limb is more commonly affected due to its vascular anatomy (the right internal iliac artery directly overlies the left iliac vein).<ref>{{Cite journal|last1=Chinsakchai|first1=Khamin|last2=ten Duis|first2=Kaj|last3=Moll|first3=Frans L.|last4=de Borst|first4=Gert J.|date=January 2011|title=Trends in Management of Phlegmasia Cerulea Dolens|url=http://journals.sagepub.com/doi/10.1177/1538574410388309|journal=Vascular and Endovascular Surgery|language=en|volume=45|issue=1|pages=5–14|doi=10.1177/1538574410388309|pmid=21193462|s2cid=64951|issn=1538-5744}}</ref><ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref>

=== Associated diseases ===
PCD is associated with an underlying [[Medicine:Malignancy|malignancy]] in 20-40% of cases.<ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref> There is a high risk of massive [[Medicine:Pulmonary embolism|pulmonary embolism]], even under anticoagulation.<ref name="ACS">{{cite web|author=John T. Owings|date=December 2005|title=Management of Venous Thromboembolism|url=http://www.acssurgery.com/acs/chapters/ch0606.htm|url-status=dead|archive-url=https://web.archive.org/web/20120127090223/http://www.acssurgery.com/acs/chapters/ch0606.htm|archive-date=January 27, 2012|access-date=January 16, 2012|work=ACS Surgery|publisher=American College of Surgeons}}</ref>

== Etiology ==
Risk factors, present in around 50% of documented cases, include malignancy, hyper-coagulable states, [[Biology:Heart|cardiac]] disease, venous stasis, venous insufficiency, May-Thurner syndrome (right iliac artery compressing the left iliac vein that runs beneath it), surgery, trauma, [[Medicine:Pregnancy|pregnancy]], [[Medicine:Inferior vena cava filter|inferior vena cava (IVC) filter]], hormone therapy, oral contraceptives, prolonged immobilization, [[Medicine:Inflammatory bowel disease|inflammatory bowel disease]], [[Medicine:Heart failure|heart failure]], and central venous catheters. Etiology is unknown in ~10% of PCD cases.<ref>{{Cite journal|last1=Greenberg|first1=Jacques|last2=Troutman|first2=Douglas A.|last3=Shubinets|first3=Valeriy|last4=Dougherty|first4=Matthew J.|last5=Calligaro|first5=Keith D.|date=February 2016|title=Phlegmasia Cerulea Dolens in the Upper Extremity: A Case Report and Systematic Review and Outcomes Analysis|url=http://journals.sagepub.com/doi/10.1177/1538574416631645|journal=Vascular and Endovascular Surgery|language=en|volume=50|issue=2|pages=98–101|doi=10.1177/1538574416631645|pmid=26912398|s2cid=29270577|issn=1538-5744}}</ref><ref>{{Cite journal|last1=Chinsakchai|first1=Khamin|last2=ten Duis|first2=Kaj|last3=Moll|first3=Frans L.|last4=de Borst|first4=Gert J.|date=January 2011|title=Trends in Management of Phlegmasia Cerulea Dolens|url=http://journals.sagepub.com/doi/10.1177/1538574410388309|journal=Vascular and Endovascular Surgery|language=en|volume=45|issue=1|pages=5–14|doi=10.1177/1538574410388309|pmid=21193462|s2cid=64951|issn=1538-5744}}</ref><ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref>
[[File:BladderCADVTleftMark.png|thumb|Deep vein thrombosis of the left external iliac in a person with bladder cancer resulting in this condition. (Author James Heilman, MD, 2016)]]

== Pathophysiology ==
When a [[Medicine:Thrombus|thrombus]] occludes an extremity [[Medicine:Vein|vein]], pressure backs up in the venous system leading plasma fluid to leak out into the [[Biology:Interstitium|interstitium]] of the affected limb. This increases the pressure of that limb compartment, which can collapse the arteries and lead to acute [[Medicine:Ischemia|ischemia]], [[Medicine:Gangrene|gangrene]], [[Medicine:Hypovolemia|hypovolemia]], and hemodynamic instability.<ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref><ref>{{Cite journal|last1=Chang|first1=Grace|last2=Yeh|first2=James J.|date=July 2014|title=Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant: a case report and review of the literature|url=https://pubmed.ncbi.nlm.nih.gov/24553060|journal=Blood Coagulation & Fibrinolysis|volume=25|issue=5|pages=507–511|doi=10.1097/MBC.0000000000000057|issn=1473-5733|pmid=24553060|s2cid=10642849}}</ref>

== Diagnosis ==
PCD is best diagnosed with contrast venography, but [[Medicine:Doppler ultrasonography|venous duplex ultrasonography]] is used more commonly in clinical practice. Magnetic resonance and computed tomography venography can also be used.<ref>{{Cite journal|last1=Chinsakchai|first1=Khamin|last2=ten Duis|first2=Kaj|last3=Moll|first3=Frans L.|last4=de Borst|first4=Gert J.|date=January 2011|title=Trends in Management of Phlegmasia Cerulea Dolens|url=http://journals.sagepub.com/doi/10.1177/1538574410388309|journal=Vascular and Endovascular Surgery|language=en|volume=45|issue=1|pages=5–14|doi=10.1177/1538574410388309|pmid=21193462|s2cid=64951|issn=1538-5744}}</ref>

=== Differential Diagnosis ===
DDx is as follows:
<ref>{{Citation|last=Gardella|first=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref>
* [[Medicine:Cellulitis|Cellulitis]]
* Venous insufficiency
* Superficial thrombophlebitis
* [[Medicine:Deep vein thrombosis|DVT]]
* Arterial embolism
* Lymphedema
* Ruptured Bakers cyst

== Treatment ==
Treatment for PCD includes immediate [[Medicine:Anticoagulant|anticoagulation]], fluid [[Medicine:Resuscitation|resuscitation]], bed rest, limb elevation above 60º, limb wrap to reduce pain and edema, and either catheter-based thrombolysis, percutaneous transluminal [[Medicine:Angioplasty|angioplasty]], or surgical venous thrombectomy +/- [[Medicine:Fasciotomy|fasciotomy]] to remove the [[Medicine:Thrombus|blood clot]].<ref>{{Cite journal|last1=Chinsakchai|first1=Khamin|last2=ten Duis|first2=Kaj|last3=Moll|first3=Frans L.|last4=de Borst|first4=Gert J.|date=January 2011|title=Trends in Management of Phlegmasia Cerulea Dolens|url=http://journals.sagepub.com/doi/10.1177/1538574410388309|journal=Vascular and Endovascular Surgery|language=en|volume=45|issue=1|pages=5–14|doi=10.1177/1538574410388309|pmid=21193462|s2cid=64951|issn=1538-5744}}</ref><ref>{{Cite book|url=https://www.worldcat.org/oclc/1037557259|title=Rutherford's vascular surgery and endovascular therapy|date=2019|others=Anton N. Sidawy, Bruce A. Perler|isbn=978-0-323-58130-1|edition=9th|location=Philadelphia, PA|oclc=1037557259}}</ref><ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref><ref>{{Cite journal|last1=Chang|first1=Grace|last2=Yeh|first2=James J.|date=July 2014|title=Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant: a case report and review of the literature|url=https://pubmed.ncbi.nlm.nih.gov/24553060|journal=Blood Coagulation & Fibrinolysis|volume=25|issue=5|pages=507–511|doi=10.1097/MBC.0000000000000057|issn=1473-5733|pmid=24553060|s2cid=10642849}}</ref> Some people also suggest an [[Medicine:Inferior vena cava filter|IVC filter]] before thrombolysis.<ref>{{Cite journal|last1=Chinsakchai|first1=Khamin|last2=ten Duis|first2=Kaj|last3=Moll|first3=Frans L.|last4=de Borst|first4=Gert J.|date=January 2011|title=Trends in Management of Phlegmasia Cerulea Dolens|url=http://journals.sagepub.com/doi/10.1177/1538574410388309|journal=Vascular and Endovascular Surgery|language=en|volume=45|issue=1|pages=5–14|doi=10.1177/1538574410388309|pmid=21193462|s2cid=64951|issn=1538-5744}}</ref>

== Prognosis ==
PCD is fully reversible if the causal [[Medicine:Venous thrombosis|venous thrombus]] is promptly removed.<ref>{{Cite book|url=https://www.worldcat.org/oclc/1037557259|title=Rutherford's vascular surgery and endovascular therapy|date=2019|others=Anton N. Sidawy, Bruce A. Perler|isbn=978-0-323-58130-1|edition=9th|location=Philadelphia, PA|oclc=1037557259}}</ref> In the 40-60% of people who go on to develop venous gangrene, there is a 20-50% risk of amputation and 20-40% mortality rate.<ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-16|last2=Faulk|first2=JimBob}}</ref><ref>{{Cite journal|last1=Chinsakchai|first1=Khamin|last2=ten Duis|first2=Kaj|last3=Moll|first3=Frans L.|last4=de Borst|first4=Gert J.|date=January 2011|title=Trends in Management of Phlegmasia Cerulea Dolens|url=http://journals.sagepub.com/doi/10.1177/1538574410388309|journal=Vascular and Endovascular Surgery|language=en|volume=45|issue=1|pages=5–14|doi=10.1177/1538574410388309|pmid=21193462|s2cid=64951|issn=1538-5744}}</ref> Following PCD resolution patients are more likely to develop venous insufficiency and [[Medicine:Post-thrombotic syndrome|post-thrombotic syndrome]]<ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref>

A grading system has been established {{bywhom?|date=July 2022}}:<ref>{{Cite journal|last1=Chinsakchai|first1=Khamin|last2=ten Duis|first2=Kaj|last3=Moll|first3=Frans L.|last4=de Borst|first4=Gert J.|date=January 2011|title=Trends in Management of Phlegmasia Cerulea Dolens|url=http://journals.sagepub.com/doi/10.1177/1538574410388309|journal=Vascular and Endovascular Surgery|language=en|volume=45|issue=1|pages=5–14|doi=10.1177/1538574410388309|pmid=21193462|s2cid=64951|issn=1538-5744}}</ref><ref>{{Cite journal|last1=Chang|first1=Grace|last2=Yeh|first2=James J.|date=July 2014|title=Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant: a case report and review of the literature|url=https://pubmed.ncbi.nlm.nih.gov/24553060|journal=Blood Coagulation & Fibrinolysis|volume=25|issue=5|pages=507–511|doi=10.1097/MBC.0000000000000057|issn=1473-5733|pmid=24553060|s2cid=10642849}}</ref>
* Grade I = non-complicated (no [[Medicine:Blister|blister]]ing, strong sensory-motor function and strong distal pulses)
* Grade II = impending venous [[Medicine:Gangrene|gangrene]] (blistering, weak sensory-motor function and weak distal pulses)
* Grade III = venous gangrene

== Epidemiology ==
PCD is most likely to occur in people in their 50s and 60s, but can occur as early as 6 months old. There is slight male predominance of around 1.5:1.<ref>{{Cite journal|last1=Chinsakchai|first1=Khamin|last2=ten Duis|first2=Kaj|last3=Moll|first3=Frans L.|last4=de Borst|first4=Gert J.|date=January 2011|title=Trends in Management of Phlegmasia Cerulea Dolens|url=http://journals.sagepub.com/doi/10.1177/1538574410388309|journal=Vascular and Endovascular Surgery|language=en|volume=45|issue=1|pages=5–14|doi=10.1177/1538574410388309|pmid=21193462|s2cid=64951|issn=1538-5744}}</ref><ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref>

== History ==
This phenomenon was first discovered by Fabricus Hildanius in the [[History:16th century|16th century]], and was officially termed "phlegmasia cerulea dolens" by Gregoire in 1938.<ref>{{Citation|last1=Gardella|first1=Layne|title=Phlegmasia Alba And Cerulea Dolens|date=2020|url=http://www.ncbi.nlm.nih.gov/books/NBK563137/|work=StatPearls|place=Treasure Island (FL)|publisher=StatPearls Publishing|pmid=33085284|access-date=2021-02-11|last2=Faulk|first2=JimBob}}</ref><ref>{{Cite journal|last1=Chinsakchai|first1=Khamin|last2=ten Duis|first2=Kaj|last3=Moll|first3=Frans L.|last4=de Borst|first4=Gert J.|date=January 2011|title=Trends in Management of Phlegmasia Cerulea Dolens|url=http://journals.sagepub.com/doi/10.1177/1538574410388309|journal=Vascular and Endovascular Surgery|language=en|volume=45|issue=1|pages=5–14|doi=10.1177/1538574410388309|pmid=21193462|s2cid=64951|issn=1538-5744}}</ref> ''Phlegmasia'' originates from the [[Social:Greek language|Greek]] root ''phlegma'' ([[Medicine:Inflammation|inflammation]]), ''cerulea'' originates from [[Social:Latin|Latin]] root ''caeruleus'' (dark blue), and ''dolens'' originates from Latin word ''dolens'' (suffering).<ref>{{Cite book|url=https://www.worldcat.org/oclc/1037557259|title=Rutherford's vascular surgery and endovascular therapy|date=2019|others=Anton N. Sidawy, Bruce A. Perler|isbn=978-0-323-58130-1|edition=9th|location=Philadelphia, PA|oclc=1037557259}}</ref>

== References ==
{{reflist}}

== External links ==
{{Medical resources
|  DiseasesDB     = 32484
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|  MedlinePlus    = 000200
|  eMedicineSubj  = med
|  eMedicineTopic = 2767
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}}

{{DEFAULTSORT:Phlegmasia Cerulea Dolens}}
[[Category:Inflammations]]
[[Category:Vascular diseases]]

{{Sourceattribution|Phlegmasia cerulea dolens}}