1995
DOI: 10.1016/0029-7844(94)00349-i
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Kasabach-Merritt coagulopathy complicating Klippel-Trenaunay-Weber syndrome in pregnancy

Abstract: Klippel-Trenaunay-Weber syndrome in pregnancy is rare. The potential for a refractory coagulopathy presenting as Kasabach-Merritt syndrome should be considered in any patient who presents with extensive hemangiomas.

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Cited by 55 publications
(35 citation statements)
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“…This theory is supported by the observation that blood extracted directly from a venous malformation has greater depletion of coagulation factors, compared with blood drawn from a peripheral site of the same patient. 25 Although the mechanism leading to hypercoagulability has yet to be proven, stagnation of blood in abnormal vascular channels, followed by activation of the coagulation cascade, is presumed to be responsible 5 and was suggested by low-flow Doppler signals and spontaneous contrast echocardiographic results ("rouleaux" formation) for patient 1 of this report. Rarely, localized intravascular coagulation may progress to disseminated intravascular coagulation, especially under acute conditions (eg, sclerotherapy of the venous malformation, surgery, bone fracture, or menstruation).…”
Section: E598mentioning
confidence: 99%
“…This theory is supported by the observation that blood extracted directly from a venous malformation has greater depletion of coagulation factors, compared with blood drawn from a peripheral site of the same patient. 25 Although the mechanism leading to hypercoagulability has yet to be proven, stagnation of blood in abnormal vascular channels, followed by activation of the coagulation cascade, is presumed to be responsible 5 and was suggested by low-flow Doppler signals and spontaneous contrast echocardiographic results ("rouleaux" formation) for patient 1 of this report. Rarely, localized intravascular coagulation may progress to disseminated intravascular coagulation, especially under acute conditions (eg, sclerotherapy of the venous malformation, surgery, bone fracture, or menstruation).…”
Section: E598mentioning
confidence: 99%
“…This case and a few other cases, which were described in the subsequent years, showed Localized Intravascular Coagulation (LIC) due to KMS with consumptive coagulopathy after a normal pregnancy and a normal at term delivery [6,7]. In one patient with KMS, LIC occurred after a cesarean delivery, performed because of a large complex of varicosities extending along the vaginal wall and the labia majora which partially obstructed the introitus [8]. As is described in these cases the Kasabach-Merritt syndrome is exclusively associated with kaposiform hemangioendothelioma and tufted angioma.…”
Section: Case Reportmentioning
confidence: 89%
“…Our patient did not demonstrate any other systemic abnormality; therefore, she could be an even rarer presentation of angiomatosis outside Klipper-TrenaunayWeber syndrome. A large angiomatosis could carry a high risk of intravascular coagulation and Kasabach-Merritt syndrome (9). This syndrome, usually affecting infants and also known as hemangioma with thrombocytopenia, leads to decreased platelet counts and sometimes other bleeding problems that can be life threatening.…”
Section: Discussionmentioning
confidence: 99%