The aim of this article is to report a case of chronic localized intravascular coagulation in widespread sporadic multifocal venous malformations (VMs) associated with phleboliths, presenting with pathological femur fracture with very high D-dimer level and deranged coagulation profile hampering the surgical management of the patient.

Disseminated intravascular coagulation (DIC) is a well-known cause of raised D-dimer. It leads to derangement of coagulation profile with very poor management outcome and needs intensive care. A very high level of D-dimer present in localized intravascular coagulopathy (LIC) can be misleading to diagnosis of DIC. Localized intravascular coagulopathy is seen in few VMs and they show abnormally high D-dimer levels. Venous malformations are the most frequent slow-flow vascular malformations referred to specialized centers. Most of the VMs are sporadic, and unifocal, while 1% are multifocal VMs. Multifocal sporadic VMs are rare and associated with phleboliths. High D-dimer is associated with various types of VMs like cutaneomucosal VMs and capillarovenous malformations. We reported the case of a 22-year-old female with congenital multifocal VMs presenting with fracture shaft of left femur on trivial trauma with deranged coagulation profile as prolonged prothrombin time, activated partial thromboplastin time, thrombocytopenia, and persistently high D-dimer level along with the presence of multiple phleboliths.

In the view of deranged coagulation profile with raised D-dimer level in presence of trauma, patient was initially managed as a case of DIC and her surgical procedure for fracture femur was delayed for the correction of her coagulation abnormality. She was transfused 16 units of fresh frozen plasma, 18 units of platelets, and 3 units of packed red blood cells, but her D-dimer level remained high. Persistently raised D-dimer level without any hemorrhagic manifestation along with the presence of multiple phleboliths in his X-rays clinched the diagnosis of LIC. Low molecular weight heparin was started and after stabilization of coagulation profile, the patient got shifted to orthopedics department for the management of fracture femur.

This case describes a unique presentation of sporadic multifocal VMs as coagulation abnormality mimicking DIC. It emphasizes prompt diagnosis and workup when multiple VMs are present to prevent morbidity during surgical excision secondary to intravascular coagulopathy.

Multifocal VMs have been associated with an increased risk of spontaneous thrombosis and thrombolysis because of stasis of blood, a condition termed LIC. Severe LIC has potential to progress to DIC during surgical excision so this mandates preoperative evaluation.

Chaurasia AK, Gupta P, Purwar N. Chronic Localized Intravascular Coagulation in a Case of Sporadic Multifocal Venous Malformations with Phleboliths. J Postgrad Med Edu Res 2017;51(3):144-149.