Coagulation abnormalities associated with extensive venous malformations of the limbs: differentiation from Kasabach-Merritt syndrome

Abstract

Confusion in the nomenclature of vascular malformations has been a major obstacle to the understanding of these conditions, so that misdiagnosis and treatment inconsistencies are common. Coagulation abnormalities occurring in combination with venous malformations (VM) have been misdiagnosed as Kasabach-Merritt syndrome (KMS), despite marked differences in clinical features, pathology and treatment. A homogenous group of 24 patients with diffuse limb VM was entered into a retrospective chart review study. The VM affected an upper limb in 12 patients, a lower limb in 10 and both in two. Localized intravascular coagulation (LIC) was characterized by a decrease in fibrinogen (0.5-1 g/l), an increase in d-dimers (2-64 micro g/ml) and presence of soluble complex of fibrin (+ to +++). Platelet counts were normal or slightly decreased. Higher VM severity scores were associated with more severe LIC. A number of events such as sclerotherapy, surgery, bone fracture, prolonged immobilization and pregnancy or menstruation triggered conversion of the LIC to disseminated intravascular coagulation (DIC), with bleeding related to factor consumption and multiorgan failure related to disseminated microvascular thrombosis. Clinical symptoms associated with worsening of LIC were pain, thrombosis and bleeding at wound sites or during surgery. None of the patients had the large ecchymotic and inflammatory tumours seen in KMS. Graded permanent elastic compression with heparin therapy was the only effective treatment. In conclusion, VM-associated LIC is a distinctive lifelong coagulopathy that must be differentiated from KMS, which is characterized by platelet trapping within a vascular tumour of infancy. The treatment of the two conditions is very different.

Conservative Therapy for Surgically Untreatable Extensive Arteriovenous Malformation from the Lower Extremityto the Pelvis with Secondary Consumptive Coagulopathy

“…However, Kasabach-Merritt syndrome is a congenital hemangioma with disseminated intravascular clotting in children and is a different condition from vascular malformations with coagulopathy. 1) In the present case, the lesions developed very slowly as the patient grew, and she had no sign of right heart strain or mild symptoms of venous stasis, therefore, her disease was first considered being AVM with a small shunt volume. However, she had extensive venous enlargement which developed into serious consumptive coagulopathy due to a mechanism similar to that of venous-predominant vascular malformation.…”

“…Mazoyer et al reported that a vitamin K antagonist was ineffective and advocated that permanent elastic compression with heparin therapy was the only effective treatment for LIC. 1) However, the present patient's coagulopathy improved and has been well controlled by only warfarin therapy and compression. Nevertheless, careful follow-up of possible stasis complications and coagulation disorder will be required for some time in the future.…”

“…1,2) Not only the prognosis, but also the treatment methods for vascular malformations vary according to the particular lesions of each patient. 3) We report a woman with extended lesions of arteriovenous malformation (AVM) in the entire left lower extremity and the interior of the pelvis, associated with serious consumptive coagulopathy, who has been successfully followed up for 17 years with favorable control achieved by conservative methods, including compression and anticoagulation therapies.…”

Protocolo de actuación ante hemangiomas y/o malformaciones vasculares

“…Este trastorno, en función de la gravedad puede producir hemorragias y trombosis en situación basal y empeorar claramente con algunos tratamientos (inmovilización postquirúrgica, escleroterapia). Su estudio recomienda realizar un hemograma, donde el número de plaquetas suele estar en el límite bajo de la normalidad (distinto de la CID que se produce por atrapamiento local de plaquetas en el fenómeno de KasabachMerritt), un tiempo de protrombina, fibrinógeno (que suele estar disminuido), dímero-D (habitualmente elevado), y complejos solubles de fibrina (que suelen estar presentes) 11,12 .…”

Klippel Trenaunay Syndrome, Inverse Klippel Trenaunay Syndrome: Hypertrophy of Lower Limbs and Atrophy of the Upper Limbs and Facial Muscles: Case Report and Literature Review

“…Klippel Trenaunay Syndrome must be differentiated from the Parkes-Weber syndrome [6]. Both are characterized by overgrowth limbs.…”