• Neonatal Medicine
• /
• v.17 no.1
• /
• pp.116-122
• /
• 2010

Kasabach-Merritt syndrome is a rare thrombocytopenic consumptive coagulopathy associated with a giant hemangioma. We experienced a case of unexplained ascites with thrombocytopenia in a 32 week premature infant. An exploratory laparotomy was performed to determine the cause of the refractory ascites and thrombocytopenia. An intestinal hemangioma was found, but, surgical removal was not performed due to the extensive involvement. Hemangioma was confirmed by SPECT (single-photon emission computed tomography) and the thrombocytopenia was treated with steroid therapy. It is recommended that hemangioma of the visceral organs should be suspected when unexplained thrombocytopenia and disseminated intravascular coagulopathy persist.

• Advances in pediatric surgery
• /
• v.18 no.1
• /
• pp.24-29
• /
• 2012

Although hemangiomas are common vascular tumors that can occur anywhere in the body, they seldom involve the gastrointestinal tract. Hemangiomas of the gastrointestinal tract in infants and children are rare benign vascular tumors that most commonly present with gastrointestinal bleeding. We describe here the case of 2-year-old boy with intestinal bleeding caused by a large jejunal cavernous hemangioma, which was treated by laparoscopy-assisted resection of the affected portion of the jejunum.

• Clinical and Experimental Pediatrics
• /
• v.45 no.7
• /
• pp.923-927
• /
• 2002

Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year-old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.