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

Abstract

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.