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A Case of Esophageal Variceal Bleeding in a Child Secondary to Portal Hypertension Associated with Cavernous Transformation of the Portal Vein Suggesting Extrahepatic Portal Obstruction.

Journal of the Korean Pediatric Society 1990;33(10):1406-1412.
Published online October 31, 1990.
A Case of Esophageal Variceal Bleeding in a Child Secondary to Portal Hypertension Associated with Cavernous Transformation of the Portal Vein Suggesting Extrahepatic Portal Obstruction.
Sun Yang Hong1, Tae Won Oh1, Jeong Kook Lee1, Hahng Lee1, Keun Soo Lee1, Seok Chol Jeon2, Heung Suk Seo2
1Department of Pediatrics, Hanyang University, College of Medicine, Seoul, Korea
2Department of Diagnostic Radiology, Hanyang University, College of Medicine, Seoul, Korea
문정맥 폐쇄로 문맥 해면상 변형을 동반한 소아식도 정맥류 1례*
홍선양1, 오재원1, 이정국1, 이항1, 이근수1, 전석철2, 서흥석2
1한양대학교 의과대학 소아과학교실
2한양대학교 의과대학 진단방사선과학교실
Received: 5 June 1990   • Accepted: 24 July 1990
Abstract
Esophageal variceal bleeding is a rare manifestation in childhood and it usually occurs secondary to portal hypertension. We experienced a case of esophageal variceal bleeding secondary to portal hypertension associated with portal obstruction but with no evidence of liver cirrhosis in a 4-year- and-10-month-old boy who was hospitalized due to hematemesis. He was a product of fullterm normal spontaneous delivery at home unattended by midwife of medical staff, and had a history of abdominal distension requiring oral medication prescribed by a local clinic for one week after birth, but a definite history of omphalitis or sepsis is uncertain. He remained healthy till the sudden onset of bloody vomiting and melena one week prior to amission, when he was noted to have splenomegaly and moderate anemia with hemoglobin level of 6.8 mg/dl at a local clinic and he was transferred to the hospital after transfusion for further evaluation. He was found to have no abnormal physical finding except for an isolated splenomegaly and mild pallor. He was found to have normal liver function test results, normal coagulation profile, normal urinalysis, but mild anemia with hemoglobin level of 9.7 mg/dl and otherwise normal hemogram. Barium meal esophagogram showed tubular or ovoid worm-like filling defects compatible with esophageal varices (Fig. 3) And abdominal sonogram showed splenomegaly and coase echo-pattern of the liver (Fig. 1, 2). but 99mTc liver/spleen scan showed no liver abnormally but splenomegaly (Fig. 4). Arterial portography via superior mesenteric artery and splenic artery revealed moderate splenomegaly, esophageal varices with cavernous transformation of the portal vein, suggestive of preexisting extrahepatic portal obstruction, and revealed no evidence of cirrhotic pattern of the liver (Fig. 5). However liver biopsy was not performed. He was treated by conservative management and is presently followed on an outpatient basis with no further evidence of bleeding and is thriving well over the one year period of follow up. Portal systemic shunt operation is planned for a later date. We report a case of esophageal variceal bleeding secondary to portal hypertension from portal obstruction suggested by cavernous transformation of the portal vein on an arterial portography as the first such case in childhood reported in Korean literature and a brief review of related literatures is presented.
Key Words: Esoophageal Variceal Bleeding, Portal Hypertension, Extrahepatic Portal Obstruction, Cavernous Transformation.


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