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
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
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
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