Accepted Manuscript Phlegmasia Cerulea Dolens: A Rare Clinical Presentation Chisom U. Onuoha, MD PII:
S0002-9343(15)00350-2
DOI:
10.1016/j.amjmed.2015.04.009
Reference:
AJM 12955
To appear in:
The American Journal of Medicine
Received Date: 6 March 2015 Revised Date:
2 April 2015
Accepted Date: 3 April 2015
Please cite this article as: Onuoha CU, Phlegmasia Cerulea Dolens: A Rare Clinical Presentation, The American Journal of Medicine (2015), doi: 10.1016/j.amjmed.2015.04.009. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Title: Phlegmasia Cerulea Dolens: A Rare Clinical Presentation Author/ Academic affiliations: Chisom U. Onuoha, MD.
Saint Mary’s Hospital, Waterbury, CT
Contact Information for Author:
SC
Chisom U. Onuoha, MD
M AN U
Saint Mary’s Hospital, Department of Medicine,
RI PT
PGY 3, Department of Medicine,
56 Franklin Street, Waterbury, CT 06770 E-mail: [email protected] Mobile: 941-889-9069
Funding source: None
TE D
Fax: 203-709-3518
disclose.
EP
Conflict of interest: I, the author, hereby state that I do not have any conflicts of interest to
AC C
Author verification: I, the author had access to the data and prepared the manuscript. Article type: Clinical Communications to the Editor Key Words: Phlegmasia cerulea dolens Running head: Phlegmasia cerulea dolens Word count: 649
ACCEPTED MANUSCRIPT Dear Editor:
RI PT
Phlegmasia Cerulea Dolens: A Rare Clinical Presentation
Case Presentation:
A 72-year-old woman with recently-diagnosed squamous cell cancer of the neck presented with
SC
a three-day history of left leg pain and swelling. She had no prior episodes of such symptoms and her review of systems was otherwise negative. On physical examination, her left lower extremity
M AN U
appeared blue distally; there was marked edema and severe tenderness to palpation; dorsalis pedis pulse was present. Her right lower extremity and the rest of her systemic examination were unremarkable. Venous doppler of the left lower extremity revealed occlusive thromboses of the common femoral, superficial femoral, and popliteal veins. Arterial doppler was normal. Her
TE D
clinical presentation was consistent with a diagnosis of phlegmasia cerulea dolens. She was placed on a heparin drip with elevation of the affected extremtity. Twelve hours later, she had progressive pain and discoloration with a weaker dorsalis pedis pulse. A vascular
EP
surgery consultation was obtained and the patient underwent catheter-directed thrombolysis. Furthermore, mechanical thrombectomy was done the following day for significant residual clot
AC C
burden following thrombolysis. Seven days later, she had only minimal symptomatic improvement. A repeat venous doppler revealed a re-occlusion of the left common femoral and proximal superficial femoral vein. She then underwent a repeat mechanical thrombectomy and left common femoral balloon venoplasty. Subsequently, her left lower extremity pain subsided and she was discharged home.
ACCEPTED MANUSCRIPT Discussion: Phlegmasia cerulea dolens is a rare syndrome defined as a clinical triad of acute limb swelling, ischemic pain and cyanosis. The pathogenesis begins with massive thrombosis causing total or
arterial flow, and ultimately, varying degrees of ischemic damage.
RI PT
near total venous occlusion.1 This leads to massive fluid sequestration, edema, obstruction of
Risk factors for progression of deep vein thrombosis to phlegmasia cerulea dolens include
SC
malignancy, other hypercoagulable states, inferior vena cava filter, previous deep vein
thrombosis, contraceptive agents, venous stasis and trauma. Of these, malignancy is the most
M AN U
common, with rates as high as 33% in one study; 2 it was the implicated risk factor in the patient above. The left leg is more frequently affected than the right. 3 In 40-60% of cases, phlegmasia cerulea dolens will progress to venous gangrene, which is associated with high mortality rates of 25-40%; 3 amputation rates as high as 12-25% in survivors.3
TE D
Based on severity, phlegmasia cerulea dolens is classified as non complicated, impending venous gangrene or, venous gangrene.2 Diagnosis is based on classic physical examination findings and
EP
duplex ultrasonography. 4
Presently, no therapeutic algorithms or guidelines exist. Goals of treatment include restoration of
AC C
venous outflow by reducing the established thrombus load, prevention of further thrombus formation, and preservation of collateral circulation, all of which culminate in limb salvage. 2 Initial therapies involve elevation of the affected extremity, unfractionated heparin anticoagulation and fluid resuscitation. 1 If no clinical improvement occurs in 6-12 hours, or there is massive thrombosis and severe symptomatic swelling and ischemia, catheter-directed thrombolysis is employed.1, 2 Thrombectomy is used when there are contraindications to thrombolysis, but may also be used alongside thrombolysis. 2 Percutaneous transluminal
ACCEPTED MANUSCRIPT angioplasty with or without stenting has also been used. 4 Surgical fasciotomy is indicated in patients with progressive gangrene or compartment syndrome; such patients may ultimately
RI PT
require amputation.
In conclusion, phlegmasia cerulea dolens is a rare complication of deep vein thrombosis that requires prompt recognition. Delay in appropriate treatment can result in gangrene, limb
SC
amputation and, in extreme cases, death.
M AN U
References:
1. Chang G,Yeh JJ. Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant: a case report and review of the literature. Blood Coagul Fibrinolysis. 2014 Jul;25(5):507-11.
2. Chinsakchai K, Ten Duis K, Moll FL, de Borst GJ. Trends in management of phlegmasia
TE D
cerulea dolens. Vasc Endovascular Surg. 2011 Jan;45(1):5-14. 3. Perkins JM, Magee TR, Galland RB. Phlegmasia caerulea dolens and venous gangrene. Br J Surg 1996;83(1):19-23.
EP
4. Klok FA, Huisman MV. Seeking optimal treatment for phlegmasia cerulea dolens. Thromb
AC C
Res. 2013 Apr;131(4):372-3.
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
S0002-9343(15)00350-2
DOI:
10.1016/j.amjmed.2015.04.009
Reference:
AJM 12955
To appear in:
The American Journal of Medicine
Received Date: 6 March 2015 Revised Date:
2 April 2015
Accepted Date: 3 April 2015
Please cite this article as: Onuoha CU, Phlegmasia Cerulea Dolens: A Rare Clinical Presentation, The American Journal of Medicine (2015), doi: 10.1016/j.amjmed.2015.04.009. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Title: Phlegmasia Cerulea Dolens: A Rare Clinical Presentation Author/ Academic affiliations: Chisom U. Onuoha, MD.
Saint Mary’s Hospital, Waterbury, CT
Contact Information for Author:
SC
Chisom U. Onuoha, MD
M AN U
Saint Mary’s Hospital, Department of Medicine,
RI PT
PGY 3, Department of Medicine,
56 Franklin Street, Waterbury, CT 06770 E-mail: [email protected] Mobile: 941-889-9069
Funding source: None
TE D
Fax: 203-709-3518
disclose.
EP
Conflict of interest: I, the author, hereby state that I do not have any conflicts of interest to
AC C
Author verification: I, the author had access to the data and prepared the manuscript. Article type: Clinical Communications to the Editor Key Words: Phlegmasia cerulea dolens Running head: Phlegmasia cerulea dolens Word count: 649
ACCEPTED MANUSCRIPT Dear Editor:
RI PT
Phlegmasia Cerulea Dolens: A Rare Clinical Presentation
Case Presentation:
A 72-year-old woman with recently-diagnosed squamous cell cancer of the neck presented with
SC
a three-day history of left leg pain and swelling. She had no prior episodes of such symptoms and her review of systems was otherwise negative. On physical examination, her left lower extremity
M AN U
appeared blue distally; there was marked edema and severe tenderness to palpation; dorsalis pedis pulse was present. Her right lower extremity and the rest of her systemic examination were unremarkable. Venous doppler of the left lower extremity revealed occlusive thromboses of the common femoral, superficial femoral, and popliteal veins. Arterial doppler was normal. Her
TE D
clinical presentation was consistent with a diagnosis of phlegmasia cerulea dolens. She was placed on a heparin drip with elevation of the affected extremtity. Twelve hours later, she had progressive pain and discoloration with a weaker dorsalis pedis pulse. A vascular
EP
surgery consultation was obtained and the patient underwent catheter-directed thrombolysis. Furthermore, mechanical thrombectomy was done the following day for significant residual clot
AC C
burden following thrombolysis. Seven days later, she had only minimal symptomatic improvement. A repeat venous doppler revealed a re-occlusion of the left common femoral and proximal superficial femoral vein. She then underwent a repeat mechanical thrombectomy and left common femoral balloon venoplasty. Subsequently, her left lower extremity pain subsided and she was discharged home.
ACCEPTED MANUSCRIPT Discussion: Phlegmasia cerulea dolens is a rare syndrome defined as a clinical triad of acute limb swelling, ischemic pain and cyanosis. The pathogenesis begins with massive thrombosis causing total or
arterial flow, and ultimately, varying degrees of ischemic damage.
RI PT
near total venous occlusion.1 This leads to massive fluid sequestration, edema, obstruction of
Risk factors for progression of deep vein thrombosis to phlegmasia cerulea dolens include
SC
malignancy, other hypercoagulable states, inferior vena cava filter, previous deep vein
thrombosis, contraceptive agents, venous stasis and trauma. Of these, malignancy is the most
M AN U
common, with rates as high as 33% in one study; 2 it was the implicated risk factor in the patient above. The left leg is more frequently affected than the right. 3 In 40-60% of cases, phlegmasia cerulea dolens will progress to venous gangrene, which is associated with high mortality rates of 25-40%; 3 amputation rates as high as 12-25% in survivors.3
TE D
Based on severity, phlegmasia cerulea dolens is classified as non complicated, impending venous gangrene or, venous gangrene.2 Diagnosis is based on classic physical examination findings and
EP
duplex ultrasonography. 4
Presently, no therapeutic algorithms or guidelines exist. Goals of treatment include restoration of
AC C
venous outflow by reducing the established thrombus load, prevention of further thrombus formation, and preservation of collateral circulation, all of which culminate in limb salvage. 2 Initial therapies involve elevation of the affected extremity, unfractionated heparin anticoagulation and fluid resuscitation. 1 If no clinical improvement occurs in 6-12 hours, or there is massive thrombosis and severe symptomatic swelling and ischemia, catheter-directed thrombolysis is employed.1, 2 Thrombectomy is used when there are contraindications to thrombolysis, but may also be used alongside thrombolysis. 2 Percutaneous transluminal
ACCEPTED MANUSCRIPT angioplasty with or without stenting has also been used. 4 Surgical fasciotomy is indicated in patients with progressive gangrene or compartment syndrome; such patients may ultimately
RI PT
require amputation.
In conclusion, phlegmasia cerulea dolens is a rare complication of deep vein thrombosis that requires prompt recognition. Delay in appropriate treatment can result in gangrene, limb
SC
amputation and, in extreme cases, death.
M AN U
References:
1. Chang G,Yeh JJ. Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant: a case report and review of the literature. Blood Coagul Fibrinolysis. 2014 Jul;25(5):507-11.
2. Chinsakchai K, Ten Duis K, Moll FL, de Borst GJ. Trends in management of phlegmasia
TE D
cerulea dolens. Vasc Endovascular Surg. 2011 Jan;45(1):5-14. 3. Perkins JM, Magee TR, Galland RB. Phlegmasia caerulea dolens and venous gangrene. Br J Surg 1996;83(1):19-23.
EP
4. Klok FA, Huisman MV. Seeking optimal treatment for phlegmasia cerulea dolens. Thromb
AC C
Res. 2013 Apr;131(4):372-3.
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
![[Phlegmasia cerulea dolens].](/assets/img/hold.png)
