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Acquired syndrome with idiopathic portal hypertension and splenic anemia.

Banti-Senator Disease; Senator Syndrome; Spleen-Liver Syndrome; Chronic Congestive Splenomegaly; Hepatolienal Fibrosis; Noncirrhotic idiopathic portal hypertension.

Syndrome characterized by increased splenic and portal pressures resulting in portal hypertension of unknown etiology. Possible causes of portal hypertension include toxins, infections (bacterial/malaria), and immunologic or genetic factors. The portal hypertension is accompanied by splenomegaly and esophageal varices. Liver failure is an uncommon finding. Anemia and thrombocytopenia accompany the hypersplenism.

Portal hypertension in the absence of any obvious etiology. Liver biopsy excludes hepatic causes. Extrahepatic portal vein obstruction must be excluded.

Idiopathic portal hypertension is most commonly found in Japan (female > male) and India (male > female). Patients usually present with bleeding from gastroesophageal varices in the absence of ascites, jaundice, or other signs of hepatic failure. Patients usually present for treatment of varices by sclerotherapy or portocaval anastomoses. Prognosis is usually good. Symptoms may include ascites, weakness, anemia, leukopenia, thrombocytopenia, and/or episodes of bleeding from the gastrointestinal tract.

Intravascular volume, hemoglobin and platelet count must be evaluated and, depending on the procedure, corrected if necessary. Although liver function is usually normal, hepatic function should be evaluated.

Caution with passage of nasogastric tubes. Significant blood loss should be expected for abdominal operations (portal hypertension, thrombocytopenia). Rapid sequence induction may be considered if recent bleeding has occurred.

Avoidance of halothane prevents its implication in the etiology of postoperative jaundice. Avoid drugs requiring hepatic metabolism in the presence of deranged liver function. Nonsteroidal antiinflammatory drugs affect platelet function and gastric mucosa and should be avoided or used with caution only.

Bosch J, D'Amico G, Garcia-Pagon JC: Portal hypertension, in Schiff ER, Sorrell MF, Maddrey WC (eds): Schiff's Disease of the Liver, 9th ed. Philadelphia, Lippincott Williams and Wilkins, 2003; p 429.
Ohnishi K, Saito M, Terabayashi H, et al: Portal hemodynamics in idiopathic portal hypertension (Banti's syndrome). Gastroenterology 92:751, 1987.  [PubMed: 3817395]
Stankovics J, Nagy A, Mehes K, et al: Umbilical venous catheterization and development of Banti syndrome: The possible role of the factor V Leiden mutation. Eur J Pediatr 157:696, 1998.  [PubMed: 9727860]

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