title: Association of localized intravascular coagulopathy with venous malformations.
abstract: OBJECTIVE
  To determine which venous malformations (VMs) are at risk for coagulopathy. Venous malformations are slow-flow vascular malformations present at birth, and localized intravascular coagulopathy (LIC) causes pain and thrombosis within a lesion and severe bleeding during surgical procedures.
  
  
  METHODS
  Prospective convenience sample accrued from 2 multidisciplinary sites in Brussels, Belgium, and Caen, France.
  
  
  METHODS
  The study population comprised 140 patients with clinical data and coagulation parameters. Magnetic resonance imaging was performed for 110 patients.
  
  
  METHODS
  Measurement of D-dimer levels.
  
  
  RESULTS
  Of the 140 participants, 59 (42%) showed high D-dimer levels, 36 (61%) of whom had levels higher than 1.0 microg/mL. Six of the participants had low fibrinogen levels. In univariate analysis, large surface, presence of palpable phleboliths, and truncal localization were associated with high D-dimer levels. In the multivariate analysis, only large surface area and presence of phleboliths remained independently associated with high D-dimer levels. Severe LIC, characterized by concomitant low fibrinogen level, was associated with extensive venous malformations of the extremities.
  
  
  CONCLUSIONS
  Localized intravascular coagulopathy is statistically significantly associated with large and/or deep venous malformations that affect any location, which can have a palpable phlebolith. These patients are at risk of local pain due to thrombosis. Lesions with elevated D-dimer levels associated with low fibrinogen levels (severe LIC) commonly affect an extremity and have a high risk of hemorrhage. Low-molecular-weight heparin can be used both to treat the pain caused by LIC and to prevent decompensation of severe LIC to disseminated intravascular coagulopathy.
authors.affiliation: Department of Dermatology, Université de Caen Basse Normandie, Centre Hospitalier Universitaire Caen, Caen, France.
authors.forename: Anne
authors.initials: A
authors.lastname: Dompmartin
authors.forename: Aurélie
authors.initials: A
authors.lastname: Acher
authors.forename: Pascal
authors.initials: P
authors.lastname: Thibon
authors.forename: Sébastien
authors.initials: S
authors.lastname: Tourbach
authors.forename: Cédric
authors.initials: C
authors.lastname: Hermans
authors.forename: Véronique
authors.initials: V
authors.lastname: Deneys
authors.forename: Ben
authors.initials: B
authors.lastname: Pocock
authors.forename: Agnès
authors.initials: A
authors.lastname: Lequerrec
authors.forename: Daniel
authors.initials: D
authors.lastname: Labbé
authors.forename: Marie-Thérèse
authors.initials: MT
authors.lastname: Barrellier
authors.forename: Romain
authors.initials: R
authors.lastname: Vanwijck
authors.forename: Miikka
authors.initials: M
authors.lastname: Vikkula
authors.forename: Laurence M
authors.initials: LM
authors.lastname: Boon
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001530:Belgium / Q000453:epidemiology; D001778:Blood Coagulation Disorders / Q000097:blood / Q000150:complications / Q000453:epidemiology* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000378:metabolism; D005602:France / Q000453:epidemiology; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010147:Pain Measurement; D011446:Prospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D012737:Sex Factors; D014463:Ultrasonography; D054079:Vascular Malformations / Q000097:blood / Q000150:complications / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000473:pathology
chemical_list: D005338:Fibrin Fibrinogen Degradation Products; C036309:fibrin fragment D