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Localised intravascular coagulation complicating venous malformations in children: Associations and therapeutic

Kevin Y Zhuo1,2, Susan Russell2,3, Orli Wargon2,4

  • 1Department of Paediatric Surgery, Sydney Children's Hospital, Sydney, New South Wales, Australia.

Journal of Paediatrics and Child Health
|February 8, 2017
PubMed

Insights

Venous malformations can cause localized intravascular coagulation, a bleeding disorder that worsens with age. This review explores the link between malformation size and coagulation issues, and discusses treatment options.

Area of Science:

  • Vascular Surgery
  • Hematology
  • Pediatric Medicine

Background:

  • Venous malformations (VMs) are congenital vascular anomalies characterized by slow blood flow.
  • VMs can progressively enlarge in children, potentially leading to localized intravascular coagulation (LIC).
  • LIC is a consumptive coagulopathy presenting with elevated D-dimer and reduced fibrinogen levels.

Purpose of the Study:

  • To review the correlation between VM characteristics (number, size, planes) and LIC.
  • To highlight the concept of LIC progression alongside VM growth in children.
  • To discuss current and emerging therapeutic strategies for VMs with LIC.

Main Methods:

  • Literature review of studies investigating the relationship between VMs and LIC.
  • Analysis of existing data on the progression of LIC in pediatric patients.
  • Synthesis of information on diagnostic and therapeutic interventions.

Main Results:

  • Established correlations exist between VM burden and the severity of LIC.
  • LIC can progress over time as VMs enlarge, impacting patient health.
  • Various treatment modalities are available, including conservative and interventional approaches.

Conclusions:

  • Understanding the progression of LIC in relation to VM growth is crucial for timely intervention.
  • Multidisciplinary management involving vascular surgeons, hematologists, and pediatricians is recommended.
  • Further research into optimizing treatment protocols for VMs complicated by LIC is warranted.

Keywords:
aspirinbiomarker/bloodfibrin degradation productheparinrisk factorsirolimus

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