Extensive venous/lymphatic malformations causing life-threatening haematological complications

Large venous/lymphatic slow-flow malformations (SFM) can be associated with a coagulopathy resulting in thrombosis and haemorrhage. Such potentially life-threatening complications of SFM have been reported only rarely.

To better define the clinical characteristics of haematological complications associated with SFM, to highlight the importance of recognition and to discuss the management of these difficult-to-treat patients.

A cohort of six children who presented with massive SFM associated with serious haematological complications was seen between January 1980 and June 2005 in the Department of Paediatric Dermatology, Great Ormond Street Hospital for Children, London, U.K. (tertiary referral centre for vascular anomalies). Clinical and haematological characteristics were recorded.

Patients were aged 1-20 years. All suffered with recurrent episodes of pain, localized skin necrosis and bleeding. All had intravascular coagulopathy and life-threatening complications. These included brain haemorrhage, massive bleeding from the uterus and colon, large and extensive thromboses of the deep vessels in the abdomen and pelvis and severe haemoptysis. One patient died suddenly at the age of 20 years from pulmonary thromboembolism and thrombosis within the deep vessels of the vascular malformation. The youngest patient underwent a leg amputation to remove the huge vascular malformation due to the major risk of complications and lack of limb function. Three of the patients underwent anticoagulation treatment and showed improvement in their coagulopathy.

It is essential that patients with extensive SFM have their coagulation screened regularly to detect intravascular coagulopathy. This may progress to disseminated vascular coagulopathy and a serious risk of thrombosis and haemorrhage. Such patients require early anticoagulation in an attempt to prevent these secondary complications.