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Semin Thromb Hemost 2014; 40(01): 081-087
DOI: 10.1055/s-0033-1363471
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Venous Thrombosis in Rare or Unusual Sites: A Diagnostic Challenge

Antonio Girolami
1   Department of Medicine, University of Padua Medical School, Padua, Italy
,
Martina Treleani
1   Department of Medicine, University of Padua Medical School, Padua, Italy
,
Emanuela Bonamigo
1   Department of Medicine, University of Padua Medical School, Padua, Italy
,
Valentina Tasinato
1   Department of Medicine, University of Padua Medical School, Padua, Italy
,
Bruno Girolami
2   Division of Medicine, Padua City Hospital, Padua, Italy
› Author Affiliations
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Publication History

Publication Date:
17 December 2013 (online)

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Abstract

Venous thrombosis usually involves the veins of the limbs, most frequently the leg veins. All other venous districts may sometimes be affected by the thrombotic process. Sometimes, the thrombotic occlusion of the veins of a given region show typical signs and symptoms. In other cases, the picture may not be clear and a high degree of clinical suspicion is needed for a correct approach to patient diagnosis and management. Thrombosis of retinal and jugular veins, right heart thrombosis including thrombosis of coronary sinus and thrombosis of the azygos system may be included in this group. In addition, thromboses of umbilical, renal, ovarian, spermatic, and iliac veins also require attention. Finally, the dorsal veins of the penis may also be affected by thrombotic events. The main clinical features of these thromboses are reviewed herein with suggestions for a correct diagnostic approach. The importance of sonography and of other imaging techniques is emphasized. A prompt diagnosis is of paramount importance as most of these thromboses in rare or unusual sites may still cause severe systemic complications (pulmonary embolism, sepsis, and heart failure).

Keywords

thrombosis - venous - rare - unusual site - diagnosis
 

  • References

  • 1 Moll S. Unusual bleeds, unusual clots. Hamostaseologie 2007; 27 (3) 191-199
    Article in Thieme ConnectPubMedGoogle Scholar
  • 2 Buehl W, Sacu S, Schmidt-Erfurth U. Retinal vein occlusions. Dev Ophthalmol 2010; 46: 54-72
    PubMedGoogle Scholar
  • 3 Ehlers JP, Fekrat S. Retinal vein occlusion: beyond the acute event. Surv Ophthalmol 2011; 56 (4) 281-299 Review
    CrossrefPubMedGoogle Scholar
  • 4 London NJ, Brown G. Update and review of central retinal vein occlusion. Curr Opin Ophthalmol 2011; 22 (3) 159-165 Review
    CrossrefPubMedGoogle Scholar
  • 5 Vergou T, Moustou AE, Maniateas A, Stratigos AJ, Katsambas A, Antoniou C. Central retinal vein occlusion following infliximab treatment for plaque-type psoriasis. Int J Dermatol 2010; 49 (10) 1215-1217
    CrossrefPubMedGoogle Scholar
  • 6 Viola MI, Meyer D, Kruger T. Association between clomiphene citrate and visual disturbances with special emphasis on central retinal vein occlusion: a review. Gynecol Obstet Invest 2011; 71 (2) 73-76
    CrossrefPubMedGoogle Scholar
  • 7 Tilleul J, Glacet-Bernard A, Coscas G, Soubrane G, Souied EH. [Underlying conditions associated with the occurrence of retinal vein occlusion]. J Fr Ophtalmol 2011; 34 (5) 318-324
    CrossrefPubMedGoogle Scholar
  • 8 Lemierre A. On certain septicemias due to anaerobic organism. Lancet 1936; 227: 701-703
    CrossrefPubMedGoogle Scholar
  • 9 Arya R, Shehata HA, Patel RK , et al. Internal jugular vein thrombosis after assisted conception therapy. Br J Haematol 2001; 115 (1) 153-155
    CrossrefPubMedGoogle Scholar
  • 10 Castro-Marín F, Kendall JL. Diagnosis of Lemierre syndrome by bedside emergency department ultrasound. J Emerg Med 2010; 39 (4) 436-439
    CrossrefPubMedGoogle Scholar
  • 11 Courtin P, Toro A, Gazagnes M, Berrouba A, Gallardo M, Dembele A. [Lemierre's syndrome]. Ann Fr Anesth Reanim 2010; 29 (11) 799-802
    CrossrefPubMedGoogle Scholar
  • 12 Hoffman MA, Birge ND, Chatterjee A , et al. Lemierre-like syndrome: a series of unique cases. Clin Pediatr (Phila) 2010; 49 (9) 893-896
    CrossrefPubMedGoogle Scholar
  • 13 Lakshminarayana PH, Woodske ME. A unique case of lemierre syndrome associated with thrombophilia in an adult and the role of anticoagulation. Case Rep Med 2010; 2010: 1-4
    PubMedGoogle Scholar
  • 14 Thakker A, Karande S. Lemierre syndrome in the antibiotic era. Indian Pediatr 2010; 47 (3) 285
    PubMedGoogle Scholar
  • 15 Ulug U, Aksoy E, Erden H, Bayazit N, Bahçeci M. Bilateral internal jugular venous thrombosis following successful assisted conception in the absence of ovarian hyperstimulation syndrome. Eur J Obstet Gynecol Reprod Biol 2003; 109 (2) 231-233
    CrossrefPubMedGoogle Scholar
  • 16 Allana AM, Teo LL, Chuah BY, Liu TC, Cheah WK. Effort thrombosis in a young triathlete: an unusual presentation of painless neck swelling secondary to right brachiocephalic vein thrombosis. Singapore Med J 2011; 52 (3) e37-e39
    PubMedGoogle Scholar
  • 17 Ko SF, Huang CC, Ng SH , et al. Imaging of the brachiocephalic vein. AJR Am J Roentgenol 2008; 191 (3) 897-907
    CrossrefPubMedGoogle Scholar
  • 18 Thomas R, Christopher DJ, Roy A , et al. Chylothorax following innominate vein thrombosis—a rare complication of transvenous pacemaker implantation. Respiration 2007; 74 (3) 338-340
    CrossrefPubMedGoogle Scholar
  • 19 Ishikura H, Kimura S, Fukumura Y, Ohtani T. Resection of an azygos vein aneurysm with thrombosis. Gen Thorac Cardiovasc Surg 2010; 58 (4) 209-211
    CrossrefPubMedGoogle Scholar
  • 20 Moise MA, Hadro N, El-Arousy H, Alvarez-Tostado JA. The azygos system as a rare alternative for chronic indwelling catheters placement. J Vasc Surg 2009; 50 (3) 655-658
    CrossrefPubMedGoogle Scholar
  • 21 Mouton KT, Zehnder T, Wagner HE, Mouton WG. Follow-up after deep venous thrombosis in azygos continuation. Vasa 2005; 34 (4) 266-268
    CrossrefPubMedGoogle Scholar
  • 22 Nakamura Y, Nakano K, Nakatani H, Fukuda T, Honda K, Homma N. Surgical exclusion of a thrombosed azygos vein aneurysm causing pulmonary embolism. J Thorac Cardiovasc Surg 2007; 133 (3) 834-835
    CrossrefPubMedGoogle Scholar
  • 23 Smith T, Prasad A, Lane T, Franklin I. Azygous collateral thrombosis presenting as ureteric colic. Vasc Endovascular Surg 2011; 45 (6) 557-558
    CrossrefPubMedGoogle Scholar
  • 24 Yang JY, Kim DH, Lee JH, Suk EH. Evaluating a thrombosed azygous vein aneurysm combined with pulmonary arterial thromboembolism by ECG-gated multidetector CT: a case report. Korean J Radiol 2011; 12 (6) 754-756
    CrossrefPubMedGoogle Scholar
  • 25 Girolami A, Berti De Marinis G, Treleani M, Tasinato V, Girolami B. Congenital thrombophilia and intracardiac thrombosis probably an underdiagnosed event. Cardiol Res 2013; 4 (3) 105-115
    PubMedGoogle Scholar
  • 26 Deluca G, Correale M, Ieva R, Di Biase M. Voluminous mass adherent to the eustachian valve. Echocardiography 2008; 25 (1) 100-101
    PubMedGoogle Scholar
  • 27 Frogel JK, Weiss SJ, Kohl BA. Transesophageal echocardiography diagnosis of coronary sinus thrombosis. Anesth Analg 2009; 108 (2) 441-442
    CrossrefPubMedGoogle Scholar
  • 28 Huang HL, Yeh KH. Successful treatment of acute coronary sinus thrombosis utilizing a Judkins right catheter in a patient receiving cardiac resynchronization therapy. Europace 2009; 11 (12) 1674
    CrossrefPubMedGoogle Scholar
  • 29 Yeo KK, Davenport J, Raff G, Laird JR. Life-threatening coronary sinus thrombosis following catheter ablation: case report and review of literature. Cardiovasc Revasc Med 2010; 11 (4) e1-e5
    CrossrefPubMedGoogle Scholar
  • 30 Kitazawa S, Kitazawa R, Kondo T , et al. Fatal cardiac tamponade due to coronary sinus thrombosis in acute lymphoblastic leukaemia: a case report. Cases J 2009; 2: 9095
    CrossrefPubMedGoogle Scholar
  • 31 Berkovich GY, Ramchandani P, Preate Jr DL, Rovner ES, Shapiro MB, Banner MP. Renal vein thrombosis after martial arts trauma. J Trauma 2001; 50 (1) 144-145
    CrossrefPubMedGoogle Scholar
  • 32 Cinemre H, Bilir C, Akdemir N. Isolated renal vein thrombosis associated with MTHFR-1298 and PAI-1 4G gene mutations. Clin Appl Thromb Hemost 2010; 16 (6) 708-710
    CrossrefPubMedGoogle Scholar
  • 33 Decoster T, Schwagten V, Hendriks J, Beaucourt L. Renal colic as the first symptom of acute renal vein thrombosis, resulting in the diagnosis of nephrotic syndrome. Eur J Emerg Med 2009; 16 (4) 170-171
    CrossrefPubMedGoogle Scholar
  • 34 O'Brien AA, O'Donnell JP, Keogh JA. Renal vein thrombosis with recurrent pulmonary emboli in the nephrotic syndrome: use of the Greenfield filter. Postgrad Med J 1986; 62 (725) 223-225
    CrossrefPubMedGoogle Scholar
  • 35 Janda SP. Bilateral renal vein thrombosis and pulmonary embolism secondary to membranous glomerulonephritis treated with percutaneous catheter thrombectomy and localized thrombolytic therapy. Indian J Nephrol 2010; 20 (3) 152-155
    CrossrefPubMedGoogle Scholar
  • 36 Chaturvedi S, Brandao L, Geary D, Licht C. Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy. Pediatr Nephrol 2011; 26 (6) 979-985
    CrossrefPubMedGoogle Scholar
  • 37 Skalova S, Minxova L, Lukes A, Dedek P, Tousovska K, Podhola M. Renal vein thrombosis with pulmonary embolism: first manifestation of lupus nephritis. J Paediatr Child Health 2011; 47 (5) 315-316
    CrossrefPubMedGoogle Scholar
  • 38 Freitas C, Fructuso M, Rocha MJ , et al. Late venous thrombosis of renal allograft: two cases with different treatment and outcome. Nefrologia 2011; 31 (1) 115-117
    PubMedGoogle Scholar
  • 39 Mabjeesh NJ, Bar-Yosef Y, Schreiber-Bramante L, Kaver I, Matzkin H. Spermatic vein tumor thrombus in renal cell carcinoma. ScientificWorldJournal 2004; 4 (Suppl. 01) 192-194
    CrossrefPubMedGoogle Scholar
  • 40 Shinsaka H, Fujimoto N, Matsumoto T. A rare case of right varicocele testis caused by a renal cell carcinoma thrombus in the spermatic vein. Int J Urol 2006; 13 (6) 844-845
    CrossrefPubMedGoogle Scholar
  • 41 Avagliano L, Marconi AM, Candiani M, Barbera A, Bulfamante G. Thrombosis of the umbilical vessels revisited. An observational study of 317 consecutive autopsies at a single institution. Hum Pathol 2010; 41 (7) 971-979
    CrossrefPubMedGoogle Scholar
  • 42 Rubabaza P, Persadie RJ. Two cases of umbilical vein thrombosis, one with associated portal vein thrombosis. J Obstet Gynaecol Can 2008; 30 (4) 338-343
    CrossrefPubMedGoogle Scholar
  • 43 Vanrykel K, Bruneel E, Van Hoestenberghe MR, Buekenhout L, Gyselaers W, Theyskens C. Neonatal disseminated intravascular coagulation after thrombosis of a fetal intra-abdominal umbilical vein varix. J Obstet Gynaecol 2010; 30 (3) 315
    CrossrefPubMedGoogle Scholar
  • 44 Williams S, Chan AK. Neonatal portal vein thrombosis: diagnosis and management. Semin Fetal Neonatal Med 2011; 16 (6) 329-339
    CrossrefPubMedGoogle Scholar
  • 45 Witters P, Reynaert N, Pattyn L , et al. Intrauterine thrombosis of the ductus venosus leading to neonatal demise. Arch Dis Child Fetal Neonatal Ed 2012; 97 (1) F17
    CrossrefPubMedGoogle Scholar
  • 46 Gharehbaghi MM, Nemati M, Hosseinpour SS, Taei R, Ghargharechi R. Umbilical vascular catheter associated portal vein thrombosis detected by ultrasound. Indian J Pediatr 2011; 78 (2) 161-164
    CrossrefPubMedGoogle Scholar
  • 47 Diana A, Gaze H, Laubscher B, De Meuron G, Tschantz P. A case of pediatric Henoch-Schönlein purpura and thrombosis of spermatic veins. J Pediatr Surg 2000; 35 (12) 1843
    CrossrefPubMedGoogle Scholar
  • 48 Gleeson MJ, McDermott M, McDonald G, McDermott TE. Spontaneous thrombosis of the left spermatic vein. Br J Urol 1992; 70 (5) 567
    CrossrefPubMedGoogle Scholar
  • 49 Hashimoto L, Vibeto B. Spontaneous thrombosis of the pampiniform plexus. Scand J Urol Nephrol 2006; 40 (3) 252-254 Review
    CrossrefPubMedGoogle Scholar
  • 50 Isenberg JS, Ozuner G, Worth MH, Ferzli G. Effort-induced spontaneous thrombosis of the left spermatic vein presenting clinically as a left inguinal hernia. J Urol 1990; 144 (1) 138
    CrossrefPubMedGoogle Scholar
  • 51 Maas C, Müller-Hansen I, Flechsig H, Poets CF. Acute scrotum in a neonate caused by renal vein thrombosis. Arch Dis Child Fetal Neonatal Ed 2011; 96 (2) F149-F150
    CrossrefPubMedGoogle Scholar
  • 52 Castillo OA, Diaz M, Vitagliano GJ, Metrebian E. Pulmonary thromboembolism secondary to left spermatic vein thrombosis: a case report. Urol Int 2008; 80 (2) 217-218
    CrossrefPubMedGoogle Scholar
  • 53 Barrellier MT, Lezin B, Monsallier JM. [Isolated iliac deep venous thrombosis. Study of 48 cases seen in 7 years among 18,297 echo-Doppler evaluations of the lower limbs]. J Mal Vasc 2001; 26 (5) 290-298
    PubMedGoogle Scholar
  • 54 Goodwin TM, Gazit G, Gordon EM. Heterozygous protein C deficiency presenting as severe protein C deficiency and peripartum thrombosis: successful treatment with protein C concentrate. Obstet Gynecol 1995; 86 (4 Pt 2) 662-664
    CrossrefPubMedGoogle Scholar
  • 55 Karaosmanoglu D, Karcaaltincaba M, Karcaaltincaba D, Akata D, Ozmen M. MDCT of the ovarian vein: normal anatomy and pathology. AJR Am J Roentgenol 2009; 192 (1) 295-299
    CrossrefPubMedGoogle Scholar
  • 56 Klima DA, Snyder TE. Postpartum ovarian vein thrombosis. Obstet Gynecol 2008; 111 (2 Pt 1) 431-435
    CrossrefPubMedGoogle Scholar
  • 57 Fraser DG, Moody AR, Martel A, Morgan PS. Re-evaluation of iliac compression syndrome using magnetic resonance imaging in patients with acute deep venous thromboses. J Vasc Surg 2004; 40 (4) 604-611
    CrossrefPubMedGoogle Scholar
  • 58 Peters M, Syed RK, Katz M , et al. May-Thurner syndrome: a not so uncommon cause of a common condition. Proc (Bayl Univ Med Cent) 2012; 25 (3) 231-233 (Bayl Univ Med Cent)
    PubMedGoogle Scholar
  • 59 Wolpert LM, Rahmani O, Stein B, Gallagher JJ, Drezner AD. Magnetic resonance venography in the diagnosis and management of May-Thurner syndrome. Vasc Endovascular Surg 2002; 36 (1) 51-57
    CrossrefPubMedGoogle Scholar
  • 60 Brodmann M, Gary T, Hafner F, Tiesenhausen K, Deutschmann H, Pilger E. Massive pulmonary embolism caused by internal iliac vein thrombosis with free-floating thrombus formation in the inferior vena cava. Ann Vasc Surg 2012; 26 (3) e5-e7
    CrossrefPubMedGoogle Scholar
  • 61 Girolami A, Prandoni P, Zanardi A, Girolami B, Zanon E. Isolated iliac vein thrombosis durino oral contraceptive therapy in three “normal” women. Clin Appl Thromb Hemost 1997; 3: 284-287
    CrossrefPubMedGoogle Scholar
  • 62 Merhi Z, Awonuga A. Acute abdominal pain as the presenting symptom of isolated iliac vein thrombosis in pregnancy. Obstet Gynecol 2006; 107 (2 Pt 2) 468-470
    CrossrefPubMedGoogle Scholar
  • 63 de Perrot T, Righini M, Bounameaux H, Poletti PA. Contrast-enhanced sonographic diagnosis of unsuspected internal iliac vein thrombosis. J Clin Ultrasound 2011; 39 (9) 553-555
    CrossrefPubMedGoogle Scholar
  • 64 Arango O, Lorente JA, Nohales G, Rijo E, Bielsa O. Superficial dorsal penile vein thrombosis: a little-known complication of subinguinal varicocelectomy. BJU Int 2011; 107 (1) 95-98
    CrossrefPubMedGoogle Scholar
  • 65 Han HY, Chung DJ, Kim KW, Hwang CM. Pulsed and color Doppler sonographic findings of penile Mondor's disease. Korean J Radiol 2008; 9 (2) 179-181
    CrossrefPubMedGoogle Scholar
  • 66 Kolbenstvedt A, Jenssen G, Hedlund H. Priapism of the glans and corpus spongiosum. Report of two cases with angiography. Acta Radiol 2003; 44 (4) 456-459
    CrossrefPubMedGoogle Scholar
  • 67 Molina Escudero R, Cabello Benavente R, Monzó Gardiner JI , et al. [Mondor's syndrome. Case review and bibliographic review]. Arch Esp Urol 2009; 62 (4) 317-319
    PubMedGoogle Scholar