Rare thromboses of cerebral, splanchnic and upper-extremity veins: A narrative review

Ida Martinelli, Valerio De Stefano

Research output: Contribution to journalArticle

40 Citations (Scopus)

Abstract

Venous thrombosis typically involves the lower extremity circulation. Rarely, it can occur in the cerebral or splanchnic veins and these are the most frightening manifestations because of their high mortality rate. A third site of rare venous thrombosis is the deep system of the upper extremities that, as for the lower extremity, can be complicated by pulmonary embolism and post-thrombotic syndrome. The authors conducted a narrative review focused on clinical manifestations, risk factors, and treatment of rare venous thromboses. Local risk factors such as infections or cancer are frequent in thrombosis of cerebral or portal veins. Upper extremity deep-vein thrombosis is mostly due to local risk factors (catheter- or effort-related). Common systemic risk factors for rare venous thromboses are inherited thrombophilia and oral contraceptive use; chronic myeloproliferative neoplasms are closely associated with splanchnic vein thrombosis. In the acute phase rare venous thromboses should be treated conventionally with low-molecular-weight heparin. Use of local or systemic fibrinolysis should be considered in the case of clinical deterioration in spite of adequate anticoagulation. Anticoagulation with vitamin K-antagonists is recommended for 3-6 months after a first episode of rare venous thrombosis. Indefinite anticoagulation is recommended for Budd-Chiari syndrome, recurrent thrombosis or unprovoked thrombosis and permanent risk factors. In conclusion, the progresses made in the last couple of decades in diagnostic imaging and the broadened knowledge of thrombophilic abnormalities improved the recognition of rare venous thromboses and the understanding of pathogenic mechanisms. However, the recommendations for treatment mainly derive from observational studies.

Original languageEnglish
Pages (from-to)1136-1144
Number of pages9
JournalThrombosis and Haemostasis
Volume103
Issue number6
DOIs
Publication statusPublished - 2010

Fingerprint

Intracranial Thrombosis
Viscera
Upper Extremity
Venous Thrombosis
Veins
Thrombosis
Lower Extremity
Upper Extremity Deep Vein Thrombosis
Cerebral Veins
Budd-Chiari Syndrome
Thrombophilia
Vitamin K
Low Molecular Weight Heparin
Fibrinolysis
Diagnostic Imaging
Oral Contraceptives
Portal Vein
Pulmonary Embolism
Observational Studies
Neoplasms

Keywords

  • Budd-Chiari syndrome
  • Cerebral vein thrombosis
  • Extrahepatic portal vein thrombosis
  • Mesenteric vein thrombosis
  • Upper-extremity deep venous thrombosis

ASJC Scopus subject areas

  • Hematology
  • Medicine(all)

Cite this

Rare thromboses of cerebral, splanchnic and upper-extremity veins : A narrative review. / Martinelli, Ida; De Stefano, Valerio.

In: Thrombosis and Haemostasis, Vol. 103, No. 6, 2010, p. 1136-1144.

Research output: Contribution to journalArticle

@article{48b3018cc6d64e99a1399a7d5d835958,
title = "Rare thromboses of cerebral, splanchnic and upper-extremity veins: A narrative review",
abstract = "Venous thrombosis typically involves the lower extremity circulation. Rarely, it can occur in the cerebral or splanchnic veins and these are the most frightening manifestations because of their high mortality rate. A third site of rare venous thrombosis is the deep system of the upper extremities that, as for the lower extremity, can be complicated by pulmonary embolism and post-thrombotic syndrome. The authors conducted a narrative review focused on clinical manifestations, risk factors, and treatment of rare venous thromboses. Local risk factors such as infections or cancer are frequent in thrombosis of cerebral or portal veins. Upper extremity deep-vein thrombosis is mostly due to local risk factors (catheter- or effort-related). Common systemic risk factors for rare venous thromboses are inherited thrombophilia and oral contraceptive use; chronic myeloproliferative neoplasms are closely associated with splanchnic vein thrombosis. In the acute phase rare venous thromboses should be treated conventionally with low-molecular-weight heparin. Use of local or systemic fibrinolysis should be considered in the case of clinical deterioration in spite of adequate anticoagulation. Anticoagulation with vitamin K-antagonists is recommended for 3-6 months after a first episode of rare venous thrombosis. Indefinite anticoagulation is recommended for Budd-Chiari syndrome, recurrent thrombosis or unprovoked thrombosis and permanent risk factors. In conclusion, the progresses made in the last couple of decades in diagnostic imaging and the broadened knowledge of thrombophilic abnormalities improved the recognition of rare venous thromboses and the understanding of pathogenic mechanisms. However, the recommendations for treatment mainly derive from observational studies.",
keywords = "Budd-Chiari syndrome, Cerebral vein thrombosis, Extrahepatic portal vein thrombosis, Mesenteric vein thrombosis, Upper-extremity deep venous thrombosis",
author = "Ida Martinelli and {De Stefano}, Valerio",
year = "2010",
doi = "10.1160/TH09-12-0873",
language = "English",
volume = "103",
pages = "1136--1144",
journal = "Thrombosis and Haemostasis",
issn = "0340-6245",
publisher = "Schattauer GmbH",
number = "6",

}

TY - JOUR

T1 - Rare thromboses of cerebral, splanchnic and upper-extremity veins

T2 - A narrative review

AU - Martinelli, Ida

AU - De Stefano, Valerio

PY - 2010

Y1 - 2010

N2 - Venous thrombosis typically involves the lower extremity circulation. Rarely, it can occur in the cerebral or splanchnic veins and these are the most frightening manifestations because of their high mortality rate. A third site of rare venous thrombosis is the deep system of the upper extremities that, as for the lower extremity, can be complicated by pulmonary embolism and post-thrombotic syndrome. The authors conducted a narrative review focused on clinical manifestations, risk factors, and treatment of rare venous thromboses. Local risk factors such as infections or cancer are frequent in thrombosis of cerebral or portal veins. Upper extremity deep-vein thrombosis is mostly due to local risk factors (catheter- or effort-related). Common systemic risk factors for rare venous thromboses are inherited thrombophilia and oral contraceptive use; chronic myeloproliferative neoplasms are closely associated with splanchnic vein thrombosis. In the acute phase rare venous thromboses should be treated conventionally with low-molecular-weight heparin. Use of local or systemic fibrinolysis should be considered in the case of clinical deterioration in spite of adequate anticoagulation. Anticoagulation with vitamin K-antagonists is recommended for 3-6 months after a first episode of rare venous thrombosis. Indefinite anticoagulation is recommended for Budd-Chiari syndrome, recurrent thrombosis or unprovoked thrombosis and permanent risk factors. In conclusion, the progresses made in the last couple of decades in diagnostic imaging and the broadened knowledge of thrombophilic abnormalities improved the recognition of rare venous thromboses and the understanding of pathogenic mechanisms. However, the recommendations for treatment mainly derive from observational studies.

AB - Venous thrombosis typically involves the lower extremity circulation. Rarely, it can occur in the cerebral or splanchnic veins and these are the most frightening manifestations because of their high mortality rate. A third site of rare venous thrombosis is the deep system of the upper extremities that, as for the lower extremity, can be complicated by pulmonary embolism and post-thrombotic syndrome. The authors conducted a narrative review focused on clinical manifestations, risk factors, and treatment of rare venous thromboses. Local risk factors such as infections or cancer are frequent in thrombosis of cerebral or portal veins. Upper extremity deep-vein thrombosis is mostly due to local risk factors (catheter- or effort-related). Common systemic risk factors for rare venous thromboses are inherited thrombophilia and oral contraceptive use; chronic myeloproliferative neoplasms are closely associated with splanchnic vein thrombosis. In the acute phase rare venous thromboses should be treated conventionally with low-molecular-weight heparin. Use of local or systemic fibrinolysis should be considered in the case of clinical deterioration in spite of adequate anticoagulation. Anticoagulation with vitamin K-antagonists is recommended for 3-6 months after a first episode of rare venous thrombosis. Indefinite anticoagulation is recommended for Budd-Chiari syndrome, recurrent thrombosis or unprovoked thrombosis and permanent risk factors. In conclusion, the progresses made in the last couple of decades in diagnostic imaging and the broadened knowledge of thrombophilic abnormalities improved the recognition of rare venous thromboses and the understanding of pathogenic mechanisms. However, the recommendations for treatment mainly derive from observational studies.

KW - Budd-Chiari syndrome

KW - Cerebral vein thrombosis

KW - Extrahepatic portal vein thrombosis

KW - Mesenteric vein thrombosis

KW - Upper-extremity deep venous thrombosis

UR - http://www.scopus.com/inward/record.url?scp=77953149536&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77953149536&partnerID=8YFLogxK

U2 - 10.1160/TH09-12-0873

DO - 10.1160/TH09-12-0873

M3 - Article

C2 - 20352171

AN - SCOPUS:77953149536

VL - 103

SP - 1136

EP - 1144

JO - Thrombosis and Haemostasis

JF - Thrombosis and Haemostasis

SN - 0340-6245

IS - 6

ER -