Pulmonary manifestation of a condition resembling Kasabach–Merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report

نویسندگان

  • Danjouma Housmanou Cheufou
  • Thomas Hager
  • Stefan Welter
  • Dirk Theegarten
  • Georgios Stamatis
چکیده

INTRODUCTION Kasabach-Merritt syndrome is a benign condition characterized by hemangiomatosis, severely disseminated intravascular consumption coagulopathy, and thrombocytopenia. The mortality rate increases from 12% to 30% in hemorrhagic cases. In general, the symptoms primarily manifest in the gastrointestinal tract, the skin, and the subcutaneous tissue. There is no publication about pulmonary manifestation of angiomatosis in combination with vascular malformation and hemoptysis. This is the first description of a Kasabach-Merritt syndrome-like condition in the lung. CASE PRESENTATION We present the case of a 29-year-old German woman with angiomatosis and associated pulmonary vascular malformation in her lower left lobe with a Kasabach-Merritt syndrome like condition. It was detected after hemoptysis. We also present our case observations and management. CONCLUSION In a case of angiomatosis with an associated pulmonary lobar vascular malformation, lobectomy can be safely performed to prevent life-threatening bleeding.

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عنوان ژورنال:

دوره 9  شماره 

صفحات  -

تاریخ انتشار 2015