title: Coagulation disorders in patients with venous malformation of the limbs and trunk: a case series of 118 patients.
abstract: OBJECTIVE
  To investigate the clinical characteristics of venous malformation of the limbs and trunk and known but poorly appraised associated coagulation disorders. Venous malformations are ubiquitous, slow-flow vascular anomalies known to be occasionally painful because of thrombotic episodes inside the lesion.
  
  
  METHODS
  Large case series, with screening of accepted standard coagulation tests.
  
  
  METHODS
  Ambulatory multidisciplinary clinics for vascular anomalies.
  
  
  METHODS
  This 2-year study (2003-2005) included 118 patients with clinical, radiological, and biological features informative for better defining venous malformation and associated coagulation abnormalities.
  
  
  METHODS
  The primary outcome was coagulation disorders associated with VM. Secondary measures include anatomic location, extent of lesion, localized pain, and impaired motion.
  
  
  RESULTS
  The mean age of patients was 27 years, and there was a female preponderance of 64%. The venous malformation involved the upper extremity, lower extremity, and trunk in 30%, 58%, and 36% of patients, respectively; it was plurifocal in 22%. Intralesional pain (in 92% of patients) had a higher frequency in female (63%) than in male (47%) patients. Tissular involvement concerned the skin (65%), muscle (73%), bone (13%), joints (12%), and viscera (9%). According to our severity scoring system, cases of less gravity had a score of 2 or 3 (52%), cases of intermediate severity had a score of 4 or 5 (32%), and cases of major severity had a score of 6 to 9 (10%). The most frequent blood coagulation abnormality was a high plasma D-dimer level (> 0.5 microg/mL) (58% of patients), which was correlated with muscle involvement and high severity score and was more frequent in women. The factor VIII-von Willebrand factor complex was documented in 84 patients, and plasma von Willebrand factor level was decreased (<60%) in 23 (27%) of them; 10 of the 84 patients (12%) had more notably decreased levels (<50%).
  
  
  CONCLUSIONS
  This study of a large case series of patients with pure venous malformation in the limbs and/or trunk highlights muscle involvement and frequency of pain. It validates that coagulation disorders, present in 58% of our patients, create thrombotic painful events. Under certain circumstances, these disorders entail a risk of hemorrhage because of the progression of localized intravascular coagulopathy to disseminated intravascular coagulopathy.
authors.affiliation: Consultation Multidisciplinaire des Angiomes, Hôpital Lariboisière, Assistance Publique-Hopitaux de Paris, Université Paris, Paris, France.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Mazoyer
authors.forename: Odile
authors.initials: O
authors.lastname: Enjolras
authors.forename: Annouk
authors.initials: A
authors.lastname: Bisdorff
authors.forename: Jérome
authors.initials: J
authors.lastname: Perdu
authors.forename: Michel
authors.initials: M
authors.lastname: Wassef
authors.forename: Ludovic
authors.initials: L
authors.lastname: Drouet
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001778:Blood Coagulation Disorders / Q000097:blood / Q000150:complications / Q000453:epidemiology* / Q000235:genetics / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005121:Extremities / Q000098:blood supply; D005260:Female; D005602:France / Q000453:epidemiology; D020022:Genetic Predisposition to Disease; D006801:Humans; D007596:Joints / Q000098:blood supply; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010147:Pain Measurement; D012720:Severity of Illness Index; D012737:Sex Factors; D054079:Vascular Malformations / Q000097:blood / Q000150:complications / Q000453:epidemiology* / Q000235:genetics / Q000473:pathology