Phlegmasia Cerulea Dolens: A Life- and Limb-threatening Disease

Wan Nuraisyah Azzahrah Wan Zuki

Phlegmasia cerulea dolens (PCD) is a rare syndrome caused by venous thrombosis and characterised by a triad of limb oedema, cyanosis and pain. It requires early recognition as delay of treatment can cause gangrene, limb amputation and in extreme cases, death. A 67- year-old Chinese lady, with underlying hypertension, diabetes mellitus and dyslipidaemia presented to the emergency department with a 2 days history of pain, oedema and bluish discoloration over the entire left leg. She had a history of fall 6 months prior and since then she used a walking stick for mobilization. This patient underwent ultrasound doppler left lower limb , which showed features suggestive of long-segment left lower limb deep vein thrombosis. A diagnosis of PCD was made. Subsequently, she went for a CT angiogram and venography of the left lower limb which confirmed thrombosis of the left calf vein extending to the long segment of the left common iliac vein. She was commenced on intravenous heparin infusion and then underwent inferior vena cava filter insertion and catheter directed thrombolysis. Repeat venogram showed successful catheter directed thrombolysis of the left lower limb deep venous thrombosis (DVT). Treatment should be initiated as soon as the diagnosis of PCD is suspected. Currently, guidelines for treatment are lacking however 3 therapeutic options are advocated alone or in combination: anticoagulants, thrombolytic therapy, and venous thrombectomy. An early recognition of PCD and appropriate decision regarding the treatment is essential to preserve the limb.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S16

2019 ◽  
Vol 03 (03) ◽  
pp. 180-184 ◽  
Hiok Yang Chan ◽  
Edward Tieng Chek Choke ◽  
Tjun Yip Tang ◽  
Chandramohan Sivanathan ◽  
Chua Ming Er Jasmine ◽  

AbstractMay-Thurner syndrome (MTS) is rare condition thought to occur due to an anatomical variation. In MTS, there is left iliac vein compression by the right iliac artery and lumbar spine resulting in left lower limb deep venous thrombosis. The authors report a rare cause of MTS postarterial intervention. They describe the unusual case of an Asian man who presented with acute left lower limb deep venous thrombosis 5 days following left common iliac artery angioplasty and stent insertion. Computed tomographic (CT) venogram demonstrated the left iliac vein compression by the left iliac artery stent. Successful recanalization of the thrombosed iliac vein was achieved with a combination of pulse spray thrombolysis and rheolytic mechanical thrombectomy, followed by angioplasty and stent placement in the iliac vein. There was rapid and complete resolution of symptoms. This report illustrates the use of complementary imaging modalities and a multidisciplinary team approach to achieve a successful technical and clinical outcome following an unusual case of MTS postarterial intervention and the subsequent successful endovascular management.

2011 ◽  
Vol 93 (4) ◽  
pp. e15-e16 ◽  
Jagwinder Dhaliwal ◽  
Ajanta Jayatunga

Phlegmasia cerulea dolens (PCD) is a limb-threatening venous disorder involving massive proximal venous thrombosis. The clinical manifestations are of oedema, cyanosis and pain of lower extremity. Patients presenting with PCD have an underlying pathological condition that predisposes to the thrombotic process. We report a diabetic patient who presented with PCD and septicaemia due to a spontaneous psoas abscess. Anticoagulation with heparin and treatment of the underlying psoas abscess led to complete resolution of symptoms. An understanding of the underlying pathological process responsible is vital to early recognition and successful outcome in this rare limb- and life-threatening venous disorder.

2020 ◽  
Aamer Ubaid ◽  
Farishta Waheed ◽  
Awais Naeem

Neurofibromatosis type 2 is a genetic autosomal dominant disorder caused by a spontaneous mutation in the gene located on chromosome 22 q11-13.1, which usually emerges in adolescence or early adulthood and is characterized by the development of bilateral vestibular schwannoma. We hereby report the classical case of Neurofibromatosis type 2 in a 25-year-old young male with multiple tumors associated with the disease. This patient presented to us with 3 years history of multiple painless nodules on his skin, facial weakness, left-sided progressive hearing loss, and 20 days history of weakness in the left lower limb. On Examination, he was vital with a GCS of 15/15. He was anemic with no jaundice. He had left inguinal lymphadenopathy along with multiple subcutaneous nodules on different areas, including the scalp, face, left mid-axillary line over the abdomen. He also had Right-sided facial palsy and horizontal nystagmus. CNS examination revealed an upgoing plantar on the left side, right facial nerve palsy, and bilateral vestibulocochlear nerve paralysis. Spine examination revealed spinal tenderness in the lower lumbar region. Superficial abdominal reflexes were absent. Upper limb and right lower limb power, tone, and reflexes were normal while the tone and power in the left lower limb were reduced power being ⅗. Reflexes were also exaggerated in the left lower limb. The right ankle showed swelling, most probably a plexiform neuroma. On investigations, he had normochromic normocytic anemia with mild leucocytosis. Platelets were normal. The rest of the biochemical investigations, including serum electrolytes, liver function tests, and renal function tests, were also normal.MRI brain and spine confirmed bilateral acoustic neuroma and multiple cranial and peripheral nerve tumors i.e., classical presentation of a rare disease neurofibromatosis. He was referred to the neurology unit for further assessment and treatment.

2021 ◽  
pp. 026835552097728
Kirtan D Patel ◽  
Alison YY Tang ◽  
Ashik DJ Zala ◽  
Rakesh Patel ◽  
Kishan R Parmar ◽  

Objectives Post thrombotic syndrome (PTS) is a serious complication of deep venous thromboses (DVTs). PTS occurs more frequently and severely following iliofemoral DVT compared to distal DVTs. Catheter directed thrombolysis (CDT) of iliofemoral DVTs may reduce PTS incidence and severity. We aimed to determine the rate of iliofemoral DVT within our institution, their subsequent management, and compliance with NICE guidelines. Methods Retrospective review of all DVTs diagnosed over a 3-year period was conducted. Cases of iliofemoral DVT were identified using ICD-10 codes from patient notes, and radiology reports of Duplex scans. Further details were retrieved, such as patient demographics and referrals to vascular services. NICE guidance was applied to determine if patients would have been suitable for CDT. A survey was sent to clinicians within medicine to identify awareness of CDT and local guidelines for iliofemoral DVT management. Results 225 patients with lower limb DVTs were identified. Of these, 96 were radiographically confirmed as iliofemoral DVTs. The median age was 77. 67.7% of iliofemoral DVTs affected the left leg. Right leg DVTs made up 30.2% and 2.1% were bilateral DVTs. Of the 96 iliofemoral DVTs, 21 were deemed eligible for CDT. Only 3 patients (14.3%) were referred to vascular services, and 3 received thrombolysis. From our survey, 95.5% of respondents suggested anticoagulation alone as management for iliofemoral DVT. Only one respondent recommended referral to vascular services. There was a knowledge deficiency regarding venous anatomy, including superficial versus deep veins. Conclusions CDT and other mechanochemical procedures have been shown to improve outcomes of patients post-iliofemoral DVT, however a lack of awareness regarding CDT as a management option results in under-referral to vascular services. We suggest closer relations between vascular services and their “tributary” DVT clinics, development of guidelines and robust care pathways in the management of iliofemoral DVT.

2021 ◽  
Vol 14 (9) ◽  
pp. e245353
Sri Hari Priya Vemulakonda ◽  
Naveen Kumar Gaur ◽  
Oseen Hajilal Shaikh ◽  
Uday Shamrao Kumbhar

Primary lymphoedema is a rare disorder. Often presents at a young age with asymptomatic limb oedema with gradual progression. We present a 16-year-old woman who presented with a history of swelling of the left lower limb for 6 years. There was the presence of isolated left lower limb oedema, which was a non-pitting type. The patient underwent imaging studies and was diagnosed to have primary lymphoedema. The patient was managed conservatively as the patient did not have any other problems other than the left lower limb oedema.

2001 ◽  
Vol 86 (10) ◽  
pp. 985-990 ◽  
M. L. Nelzy ◽  
L. R. Salmi ◽  
S. Skopinski ◽  
J. C. Saby ◽  
P. Le Métayer ◽  

SummaryWe evaluated two clinical scores for the prediction of deep venous thrombosis (DVT) in hospitalized patients (Wells’ and Kahn’s). We included 273 patients referred to the vascular exploration unit for the suspicion of DVT. A clinical questionnaire was filled in by the practitioner and the scores were calculated from this form. 66 of the 273 patients had a DVT. When Wells’ score was 3, a DVT was found by duplex echography in 51% patients ; when the score was 0, a DVT was found in 9%. Kahn’s score was not adapted to this population. We then developed a new simple score (cancer, palsy or plaster immobilization, warmth, superficial venous dilation, unilateral pitting edema, other diagnosis). A DVT was found in 76% patients with a score of 3 and in 11% in those with a score of 0. We therefore propose a 6-item score whose main advantages are simplicity and usefulness in routine practice.

Blood ◽  
1991 ◽  
Vol 78 (9) ◽  
pp. 2194-2197
C Demers ◽  
JS Ginsberg ◽  
P Brill-Edwards ◽  
A Panju ◽  
TE Warkentin ◽  

In order to determine the efficacy and safety of ancrod, a rapid acting defibrinogenating drug, for patients with heparin-induced thrombocytopenia, 11 consecutive patients who required anticoagulant therapy because of venous thromboembolism and who developed acute heparin-induced thrombocytopenia or had a history of heparin-induced thrombocytopenia were treated with ancrod. Heparin therapy was discontinued (in patients receiving heparin) and ancrod started at a dose of 1 to 2 U/kg every 24 hours with subsequent daily doses adjusted to maintain fibrinogen levels between 0.5 and 1.0 g/L. Ancrod was continued until warfarin had become effective. The platelet count increased to more than 150 x 10(9)/L within 2 to 10 days in all thrombocytopenic patients. Two patients with a history of heparin- induced thrombocytopenia maintained normal platelet counts while receiving ancrod. Two patients had recurrent venous thrombosis while receiving warfarin, 10 days after ancrod was discontinued: one of these patients had metastatic pancreatic carcinoma and developed phlegmasia cerulea dolens and the other patient developed a venographically proven extension of her deep venous thrombosis. One patient suffered a bleeding episode into the thigh with a 16-g/L decrease in her hemoglobin level while receiving ancrod therapy. No other side effects were noted. Our experience indicates that ancrod therapy is a reasonable approach for patients with heparin-induced thrombocytopenia who require anticoagulant therapy.

2017 ◽  
Vol 86 ◽  
Blanka Mahne ◽  
Mladen Gasparini ◽  
Matija Kozak

Background: Heparin-induced thrombocytopenia (HIT) is an immune-mediated disorder following heparin therapy presenting with thrombocytopenia and associated arterial or/and venous thrombosis (heparin induced thrombocytopenia with thrombosis–HITT). Unrecognised HIT can lead to severe complications like limb amputation and death.Case report: We report a case of a patient who presented with HIT-associated recurrent lower limb arterial thrombotic occlusions and popliteal venous thrombosis 29 days after coronary bypass graf surgery. The patient underwent urgent thrombectomy of superfcial femoral, popliteal and posterior tibial artery. Because of recurrent thrombotic occlusions of lower limb arteries three surgical revisions were performed. Te platelet count decreased from 124 × 109/l to 53 × 109/l on the fifth day after the first intervention. After clinical suspicion of HIT, heparin was discontinued and fondaparinux was started. Arterial thrombosis did not recur and the patient recovered without consequences.Conclusions: HIT occurs in 1–3 % of patients after cardiac surgery. Strict following of international guidelines regarding the frequency of platelet count monitoring, assessing probability for HIT and laboratory testing is mandatory in order not to miss the diagnosis of HIT. HIT can manifest clinically several days after the first exposure to heparin. If a patient presents with acute thrombosis and thrombocytopenia, HITT should be suspected. Postoperative HIT is associated with higher morbidity and mortality. Early recognition is crucial to prevent severe complications and death.

Sign in / Sign up

Export Citation Format

Share Document