scholarly journals 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

2020 ◽  
Vol 1 (1) ◽  
pp. 13-16 ◽  
Neha Tarannum ◽  
H.V.V.S.S. Lakshman K. ◽  
Mohammed Sadiq Azam ◽  
Rajendra Kumar Premchand

May-Thurner syndrome/Cockets syndrome/ileocaval syndrome is a rarely diagnosed, anatomical and pathologically variable condition, causing venous flow obstruction in iliocaval territory. We report a case of 64 year old male patient who presented with unprovoked proximal deep venous thrombosis (DVT) of left lower limb. Evaluated for hypercoagulable state, which was negative, the patient underwent catheter guided thrombolysis and anticoagulation for 6 months. Two years later, he developed left lower limb swelling, and venous doppler revealed acute DVT; CT venogram was consistent with left common iliac vein compression by right common iliac artery suggestive of May-Thurner syndrome. Catheter-guided thrombolysis was done, and percutaneous transluminal peripheral angioplasty/stent was placed from inferior vena cava to left common iliac vein. This case highlights the importance of high index of suspicion in managing patients with DVT for possible ileocaval syndrome wherein anticoagulation alone is insufficient and needs more aggressive and definitive treatment to relieve the obstruction and to prevent recurrence.

2021 ◽  
pp. 17-19
B. Santhi ◽  
Manigandan Manigandan ◽  
Nishok Nishok

May-Thurner syndrome (MTS) is a venous outow obstruction disorder characterized by compression of the left common iliac vein by an overriding right common iliac artery. MTS primarily affects young to middle-aged women, although many patients remain entirely asymptomatic.We report a unique case of a 25 -year-old male who presented with MTS-related multiple varicosities in left lower limb.

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.

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.

2013 ◽  
Vol 29 (7) ◽  
pp. 480-483 ◽  
Xiaodong Wang ◽  
Zhengxin Chen ◽  
Qianrong Cai

Double inferior vena cava (DIVC) with deep venous thrombosis (DVT) is rare, and there is only one reported case of DIVC with DVT treated by catheter-directed thrombolysis. We report a case of a 32-year-old man with an extensive venous clot involving the infrarenal segment of a double IVC who received filter implantation and catheter-directed thrombolysis.

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 ◽  
Elisabeth Ekkel ◽  
Tara Chandran ◽  
Ryan Qasawa ◽  
Michael Trpkovski ◽  
Sachinder Hans

Abstract This case is of a young female with a large uterine leiomyoma causing phlegmasia cerulea dolens with thrombosis of the left common and left external iliac veins. She underwent mechanical thrombectomy to temporize the condition until she could be evaluated by gynecology-oncologist to remove the cause of venous obstruction. Prior to hysterectomy, suprarenal inferior vena cava filter was placed. Less than 12 hours post hysterectomy she developed recurrent thrombosis involving the left common and external iliac veins. She underwent repeat mechanical thrombectomy with wall stent placement in the left common iliac vein with resolution of her symptoms.

2004 ◽  
Vol 62 (2b) ◽  
pp. 547-549 ◽  
Mariana Spitz ◽  
Henrique Ballalai Ferraz ◽  
Orlando G. P. Barsottini ◽  
Alberto Alain Gabbai

Progressive encephalomyelitis with rigidity and myoclonus (PEWR) is a rare neurological disorder, characterised by muscular rigidity, painful spasms, myoclonus, and evidence of brain stem and spinal cord involvement. A 73-year-old white man was admitted with a 10-day history of painful muscle spasms and continuous muscle rigidity on his left lower limb. He had involuntary spasms on his legs and developed encephalopathy with cranial nerves signs and long tract spinal cord symptomatology. Brain CT scan and spinal MRI were normal. The CSF showed lymphocytic pleocytosis and no other abnormalities. EMG showed involuntary muscle activity with 2-6 seconds of duration, interval of 30-50 ms and a frequency of 2/second in the left lower limb. Anti-GAD antibodies were detected in the blood. We detected radiological signs of lung cancer during the follow-up, which proved to be an oat cell carcinoma. The patient died two weeks after the diagnosis of the cancer.

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