Phlegmasia cerulea dolens is a rare form of massive proximal e. Overview phlegmasia cerulea dolens all information alternative names phlegmasia alba dolens definition of phlegmasia cerulea dolens. Phlegmasia cerulea dolens radiology reference article. The extreme gravity of this disease can be deduced from the frequent development of shock, gangrene, and pulmonary.
If pe is undoubtfully the most dangerous complication of vte, contributing to about 15% of all hospital deaths, less frequent complications comprise phlegmasia alba dolens pad and phlegmasia cerulea dolens pcd. Endovascular treatment of phlegmasia cerulea dolens with impending venous gangrene. We describe the endoluminal management of a patient with recent splenic trauma presenting with pcd and threatened low. Although uncommon, phlegmasia cerulea dolens pcd is a lifethreatening manifestation of acute deep vein thrombosis requiring early recognition and aggressive intervention to preserve life and limb. Dec 01, 2018 in the milder version, phlegmasia alba dolens, collateral venous outflow of the limb remains despite the venous thrombosis. This study presents current management of these disorders, including diagnostic and interventional strategies. Venous thrombectomy is contraindicated for 1 patients with a short life.
Deep vein thrombosis dvt is a common disease that is diagnosed in approximately 1 in adults annually. It has been used in the medical literature in reference to extreme cases of lower extremity deep venous thrombosis dvt that cause critical limb. Trends in management of phlegmasia te d cerulea dolens. Phlegmasia cerulea dolens from radiationinduced venous stenosis. The patient had painful swelling and bluishred discoloration of the right leg at the time of presentation.
Phlegmasia cerulean dolens is a rare condition, whose prevalence is unknown, and it represents the intermediate state between venous thrombosis and phlegmasia alba dolens. Deep vein thrombosis is a common condition encounter by hospitalists and managed by either oral or intravenous anticoagulation. Postoperatively he developed painful diffuse swelling of his left lower extremity with bluish discoloration figure 581. A multimodal therapeutic approach to phlegmasia cerulea dolens in.
Pcd is characterized by marked swelling of the lower extremities. Phlegmasia cerulea dolens pcd is a rare entity that refers to a painful, edematous, and cyanotic limb due to a massive deep vein thrombosis dvt. Phlegmasia cerulea dolens postgraduate medical journal. Some patients may ignore perelated mild symptoms, or they may give priority to dvtrelated ones. Phlegmasia cerulea dolens pcd is a rare, fulminant, potentially lethal and. Historically, it was commonly seen during pregnancy and in mothers who have just given birth. Jan 15, 2020 phlegmasia cerulea dolens a person with a two hour history of phlegmasia cerulea dolens left leg, right side of image phlegmasia cerulea dolens literally. Rapid deterioration and death caused by bilateral phlegmasia. Outcomes of rheolytic thrombectomy in phlegmasia cerulea. Jul 27, 2020 oguzkurt l, ozkan u, demirturk os, gur s. Extensive dvt can lead to life or limbthreatening diagnoses such as phlegmasia cerulea dolens pcd, phlegmasia alba dolens, and venous gangrene. Blood test resulted in significantly raised creatinine and creatine kinase ck level.
Phlegmasia cerulea dolens in a patient with breast cancer. Phlegmasia cerulea dolens color atlas and synopsis of. What is the pathophysiology of phlegmasia alba dolens pad. Cureus phlegmasia alba dolens complicating rhabdomyolysis. Phlegmasia cerulea dolens associated with the lupus anticoagulant. Bilateral phlegmasia cerulea dolens after warfarin reversal. Ep must concentrate on subtle perelated sign and symptoms. The rheolytic thrombectomy device was used in all patients with a combination of catheterdirected thrombolysis as an adjunctive therapy to facilitate more rapid thrombus clearance except for one patient who had a. Understanding how to accurately and effectively diagnose acute venous thromboembolism vte remains a primary focus in hospitalized. We conclude that although vascular complications are fortunately rare after hip arthroplasty, they can have significant. The differences between pad and pcd are the amount of deoxyhemoglobin in the venous plexus of the skin and subcutaneous tissue and the degree of discomfort produced by the associated venous hypertension and elevated compartment pressures 2. For orthopaedic surgeons, it is important to know that phlegmasia cerulea dolens and phlegmasia alba dolens can present as and lead to compartment syndrome 12. Rare case of unilateral phlegmasia cerulea dolens with.
It is a cannot miss diagnosis with potentially deadly sequelae, and we highlight the importance of a thorough physical examination and initiation of empiric treatment to help prevent such. A 75yearold man, known for nephrotic syndrome currently under investigation, presented to the emergency department with a. It also shows how rare pathologies can combine to create a life and limbthreatening condition. Venous thromboembolism has also become a major health concern in subsaharian africa. On arrival, he underwent a venous duplex that demonstrated thrombosis of his tibial, popliteal, femoral, common femoral, and external. The imported definitions may be significantly out of date, and any more recent senses may be completely missing. Phlegmasia alba dolens complicating rhabdomyolysis. Management of bilateral phlegmasia cerulea dolens in a. The patient was seen 6 months after discharge and was free of any symptoms of chronic. Phlegmasia alba and cerulea dolens statpearls ncbi bookshelf. Phlegmasia cerulea dolens as the initial symptom of abdominal aortic aneurysm eggum r, lie b, stavis p tidsskr nor laegeforen, 11930. This condition is also known as milk leg, especially as it affects women in the third trimester of pregnancy or post partum. They are phlegmasia alba dolens, phlegmasia cerulean dolens pcd, and venous gangrene.
It is characterized by sudden severe pain, swelling, cyanosis and edema. Pdf phlegmasia cerulea dolens with compartment syndrome. Phlegmasia cerulea dolens pcd, painful blue leg is a rare manifestation of deep vein. All two are severe forms of vte resulting from acute massive thrombosis and obstruction of venous drainage of the extremity. May 20, 2016 they are phlegmasia alba dolens, phlegmasia cerulean dolens pcd, and venous gangrene. Phlegmasia alba dolens, resulting in a white appearance of the leg. Phlegmasia cerulea dolens in a patient treated with carboplatin. Phlegmasia cerulea dolens pcd is a rare condition of massive proximal venous thrombosis obstructing superficial and deep venous drainage in the lower extremities. Phlegmasia cerulea dolens a rare, life threatening condition. Phlegmasia cerulea dolens is an acute limbthreatening venous thrombosis which requires prompt and aggressive management. Phlegmasia alba dolens pad describes the patient with swollen and white leg because of early compromise of arterial flow secondary to extensive dvt. This case report shows how a hybrid approach might be successful.
Mar 31, 2021 this patient has phlegmasia cerulea dolens pcd. To our knowledge, this complication has not yet been described following revision total hip arthroplasty. On arrival, he underwent a venous duplex that demonstrated thrombosis of his tibial. A fatal case of upperextremity deep vein thrombosis porto. Pregnancy has often been associated with phlegmasia alba dolens, especially during the third trimester when the uterus is large enough to compress the left common iliac vein against the pelvic rim i. This case gives us the chance to focus on three rare pathologies. Successful venous thrombectomy significantly reduces early morbidity in patients with phlegmasia cerulea dolens and phlegmasia alba dolens. Oct 21, 2017 we present a unique case of phlegmasia cerulea dolens and compartment syndrome secondary to external iliac vein disruption after revision total hip arthroplasty. Although phlegmasia cerulea dolens, maythurner syndrome, and nutcracker syndrome are rare entities, knowledge of these syndromes associated with the deep veins is essential.
Phlegmasia cerulea dolens is a severe form of venous thrombosis presenting as an edematous, painful, purplishblue lower extremity. Jul 27, 2020 phlegmasia alba dolens pad describes the patient with swollen and white leg because of early compromise of arterial flow secondary to extensive dvt. Phlegmasia alba dolens is part of a spectrum of diseases related to deep vein thrombosis. Advanced testicular cancer presenting with phlegmasia cerulea. Phlegmasia alba dolens pad and phlegmasia cerulea dolens pcd are less frequently encountered complications along a spectrum of dvt with a high incidence of mortality and limb loss. As the disease progresses, cyanosis develops, and can lead to bleb and bullae formation and venous gangrene. Phlegmasia cerulea dolens is a rare but serious complication of deep venous. In phlegmasia cerulea dolens, complete occlusion of venous. Jan 24, 2011 in phlegmasia cerulea dolens, complete occlusion of venous outflow occurs. The patient was treated with heparin as well as rehydration using saline solution and bicarbonate.
Deep vein thrombosis dvt international emergency medicine. This extensive venous obstruction ultimately leads to ischemic necrosis. Pdf phlegmasia cerulea dolens a rare, lifethreatening. Perhaps this term has been depreciated in the literature. Bilateral phlegmasia cerulea dolens after warfarin. In patients delaying initial presentation by less than three months survival. In the spectrum of dvt, phlegmasia alba dolens, phlegmasia cerulea dolens and venous gangrene are vascular emergencies. Phlegmasia alba dolens and phlegmasia cerulea dolens predominantly affect the lower extremities, involving the upper extremities in less than 5% of the cases. The patients pain and edema quickly subside and the discoloration resolves. Phlegmasia cerulea dolens is a condition in which total or neartotal occlusion of the venous outflow tract produces massive edema of the extremity and a characteristic violaceous discoloration of the skin.
Bilateral phlegmasia dolens associated with trousseaus. Arterial systems were without any obliteration but with relatively reduced flow to distal part. We present a 55yearold hypertensive, who presented with paedal oedema and breathlessness at. Phlegmasia cerulea dolens presenting with acute compartment. In the milder phlegmasia alba dolens version, collateral venous flow out of the limb remains despite the venous thrombosis. The definition of benefit, however, may be masked by. Phlegmasia cerulea dolens is an uncommon, severe form of deep venous thrombosis blood clots in the vein, usually in the upper leg. Phlegmasia cerulea dolens pcd is a rare presenting condition that carries a high mortality and morbidity risk. Catheterdirected thrombolysis in the treatment of phlegmasia cerulea dolens. A critical analysis of 175 collected cases including 50 personal observations is given in this monograph, which describes a truly frightening, ignored, and poorly understood syndrome. Successful venous thrombectomy significantly reduces early morbidity in patients with phlegmasia cerulea dolens and phlegmasia alba. Diagnosis and treatment of phlegmasia alba dolens and. Phlegmasia cerulea dolens of the lower extremities secondary to thrombosis of.
Phlegmasia alba dolens is distinguished, clinically, from phlegmasia cerulea dolens in that. Mar 24, 2021 it is clinically classified as phlegmasia cerulea dolens pcd and phlegmasia alba dolens pad. Early fasciotomy is an effective way of limb salvage for these patients. Pdf phlegmasia cerulea dolens is a rare form of massive proximal venous thrombosis of the lower extremities associated with a high degree of. A 65yearold gentleman was referred with left lower extremity phlegmasia cerulea dolens pcd 1 day following an exploratory laparotomy. Phlegmasia cerulea dolens and phlegmasia alba dolens can present as compartment syndrome. Phlegmasia alba dolens also colloquially known as milk leg or white leg. Diagnosis of phlegmasia alba dolens with complication of rhabdomyolysis and acute kidney injury had been made initially. Text is available under the creative commons attributionsharealike. Only 40% of ambulatory ed patients with pe have concomitant dvt.
Compartment syndrome in patients with massive venous. Surgical management of phlegmasia cerulea dolens jama. Pdf phlegmasia cerulea dolens pcd is a fulminant condition of acute massive venous thrombosis. Pdf trends in management of phlegmasia cerulea dolens. Phlegmasia cerulea dolens pcd, a lifethreatening complication of deep venous thrombosis dvt, is characterized by massive iliofemoral thrombus that. Eventually, she had clinical improvement during hospitalization and been discharged with resolution of creatinine and ck level. Phlegmasia alba and cerulea dolens treatment, india. Studies addressing at this issue are rare in cameroon. Diagnosis, surgical treatment, postoperative care of patients. Although phlegmasia cerulea dolens, maythurner syndrome, and nutcracker. Inflammations vascular diseases circulatory disease stubs. Phlegmasia alba dolens an overview sciencedirect topics.
This article presents, to our knowledge, the first 2 cases of massive venous thrombosis 1 phlegmasia cerulea dolens, 1 phlegmasia alba dolens. Phlegmasia cerulea dolens in a patient with breast cancer and. In cases of pregnancy, it is most often seen during the third trimester, resulting from a. Phlegmasia cerulea dolens a rare, life threatening. It is the more severe form of phlegmasia alba dolens pad, and can lead to venous gangrene. Phlegmasia is a term that has been used to describe extreme cases of lower extremity dvt, which may progress to critical limb ischemia and potentially limb loss. Article information, pdf download for phlegmasia cerulea dolens in the upper. Thus, the case reported here presents singular characteristics. Phlegmasia cerulea dolens, aptly termed gangrene of venous origin, is a relatively infrequent condition, only 200 cases having been described up to the present time. The results of treating germ cell cancer continue to improve.
Finally, 10% of patients with phlegmasia have no apparent risk factors as in our case. No clear guidelines exist for the management of phlegmasia cerulea dolens. Pcd is the term used to describe lowerextremity dvt that causes limb ischemia, and is characterized by severe swelling and blue discoloration of the extremity. There is a high risk of massive pulmonary embolism. Phlegmasia cerulea dolens and external iliac vein disruption. High index of suspicion must be kept for the development of acute compartment syndrome in such patients.
The term pcd differentiates ischemiaassociated massive venous thrombosis from phlegmasia alba dolens, which describes fulminant venous thrombosis without ischemia. Clinically there is sudden severe pain in a part of an extremity which quickly spreads to involve the whole limb. Venous doppler of the left lower extremity revealed occlusive thromboses of the common femoral, superficial femoral, and popliteal veins. It has been used in the medical literature in reference to extreme cases of lowerextremity deep venous thrombosis dvt that cause critical limb.
Phlegmasia cerulea dolens is a rare form of massive proximal venous thrombosis of the lower extremities associated with a high degree of morbidity, presenting with sudden severe lower extremity. It is characterized by sudden severe pain, swelling, cyanosis and edema of the affected limb. We report a case of a 56yearold male medically free with a history of long travel two days prior to his presentation to another hospital complaining of. Use the first description of phlegmasia cerulea dolens is attributed to fabricius hildanus in the sixteenth century, and the term was first used in 1938 by grcgoire2 to distinguish this ischaemic form of venous thrombosis from the more common nonischaemic entity, then called phlegmasia alba dolens. Diagnosis and treatment of phlegmasia alba dolens and phlegmasia cerulea dolens. Phlegmasia cerulea dolens in the pediatric population. Its rapid recognition as venous thrombosis rather than arterial insufficiency is important in avoiding gangrene and other life threatening complications.
The extreme gravity of this disease can be deduced from the frequent development of shock, gangrene, and pulmonary embolism, often resulting in death. Fulminant phlegmasia cerulea dolens with concurrent cholangiocarcinoma and a lupus anticoagulant. Phlegmasia cerulea dolens, an uncommon severe form of deep venous thrombosis. Pdf download for syndromes associated with the deep veins. Advanced testicular cancer presenting with phlegmasia. Prompt fasciotomies were performed, but the underlying.
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