Solitary hepatic hemangioma in a newborn infant complicated by cardiac failure,consumption coagulopathy,microangiopathic hemolytic anemia,and obstructive jaundice

A newborn infant with a large hepatic hemangioma developed congestive heart failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. The patient was mildly heparinized (250 units per kg and day) and underwent successful resection of the tumor without lobectomy at the age of 3 days. Blood volume increased from 93.9 ml/kg at the age of 5 h to 124.2 ml/kg prior to surgery. Red-cell mass simultaneously decreased from 53.8 to 39.4 ml/kg. The increase of blood volume is explained by congestive heart failure, the decrease of red-cell mass by intravascular coagulation within the tumor resulting in formation of thrombi and microangiopathic hemolytic anemia. A review of the literature on infants with symptoms caused by an intrahepatic hemangioma during the first month of life confirms that surgical intervention is the treatment of choice for infants with giant solitary hemangioma of the liver.Supported by Deutsche Forschungsgemeinschaft (SFB 147)     

Blue rubber-bled naevus syndrome: Report of a case with consumption coagulopathy complicated by manifest thrombosis

Blue rubber-bleb naevus (BRBN) syndrome is a rare disorder characterized by subcutaneous and gastrointestinal haemangiomas. The latter may lead to bleeding complications. A case is reported in which a process of chronic intravascular coagulation resulted in serious thrombotic complications. In the presence of a chronic consumption coagulopathy, it remains uncertain whether antiplatelet drugs are of prophylactic antithrombotic value.     

Splenoptosis complicated by a large splenic cyst: case report and discussion of combined management

Splenoptosis is a rare condition in children. Its association with a cyst is rare. A case of splenoptosis complicated by a large cyst is reported. The child presented with features of intermittent abdominal pain, constipation and clinical examination revealed a mass apparently rising out of the pelvis. Ultrasound confirmed the mass as spleen with a 15 cm cyst in the lower pole. At surgery the cyst was marsupialised and splenopexy undertaken placing the cyst in a retro-peritoneal pouch. This is only the second report of such a case in the English literature.     

Liver transplant in an infant with bilateral cystic neuroblastoma complicated by hepatic metastases and life-threatening consumption coagulopathy

Abstract:  Here we report a patient with stage-4 bilateral cystic adrenal neuroblastomas with disseminated liver metastases and consumption coagulopathy who underwent liver transplant. Our patient was initially diagnosed with infantile hepatic hemangioendothelioma and bleeding into the adrenal glands secondary to consumption coagulopathy (Kasabach-Merritt syndrome). Liver transplant was performed as a life-saving procedure under this diagnosis. We discuss this unique patient because of the diagnostic pitfalls of this rare disease and the successful clinical outcome after LT and subsequent chemotherapy for neuroblastoma.