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Revista Española de Enfermedades Digestivas

versión impresa ISSN 1130-0108

Rev. esp. enferm. dig. vol.101 no.9 Madrid sep. 2009




Spontaneous aneurysmal portohepatic fistula

Fístula aneurismática portohepática venosa espontánea



J. L. Domínguez Jiménez, J. J. Puente Gutiérrez, E. Bernal Blanco, M. A. Marín Moreno and V. Palomo Gallego1

Departments of Digestive Diseases and 1Radiology. Hospital Alto Guadalquivir. Andújar, Jaén. Spain



Case report

A 77-year-old asymptomatic male was referred by his family doctor with this liver test: total bilirubin (TB) 1.84 mg/dl, WBC 3,700/µL, platelets 109,000/mL, and GGT 71 U/L. Past medical history: no allergies to medication. Hypertension treated with captopril 25 mg/day. No abdominal surgery or trauma or liver biopsies. Drinker of 40 g alcohol daily. No known liver disease. No family history of interest. Physical examination showed no stigmata for chronic liver disease, and the abdomen was soft. CBC: leukocytes 2,990/µL, neutrophils 860/µL, lymphocytes 1,570/µL, platelets 112,000/µL, hemoglobin 138 g/L, hematocrit 0.396 L/L, MCV 90.4 fl, prothrombin activity 75.6%, cephalin time 33.6 seg, TB 1.73 mg/dl, GGT 72 U/L, with glucose, urea, creatinine, AST, ALT, ALP, Ca, serum ferritin, amylase, Na, K, TSH, AFP, and autoantibodies (ANA, AMA, SMA) within normal values. Hepatitis B and C were negative. Doppler ultrasounds and computed tomography (CT) + abdominal reconstruction with volume-rendering phase (Figs. 1 and 2) showed a dilated right suprahepatic vein, in its distal end an expansion in the two converging aneurysmatic segmental right portal branches. At this stage no portal flow is seen in the other branches. The main portal branch and the left branch had a normal caliber. The spleen was normal in size. The gallbladder and pancreas had no changes. No free fluid. An inferior vena cava to the left, with retroaortic iliac bifurcation and iliac veins being prominent. There is some increased density in the mesenteric fat due to diffuse, likely venous engorgement.



Spontaneous portohepatic venous fistula in the liver (SPVF) is rare. Fistulas are located mostly in the right liver lobe, and found in adults older than 50 years, although cases in children do exist (1). These have been classified into four types according to etiology and both clinical and etiopathogenic features (2). This is a type-II case. The cause of SPVF is unknown and controversial. In the absence of cirrhosis and portal hypertension a congenital origin is possible. In cases where there is associated cirrhosis, SPVF can present with hepatic encephalopathy. In these cases as well as in children at risk of congestive heart failure percutaneous embolization should be considered for treatment (3).



1. Kim IO, Cheon JE, Kim WS, Cheng JW, Yeon KM, Yoo SJ, et al. Congenital intrahepatic portohepatic venous shunt: treatment with coil embolisation. Pediatr Radiol 2000; 30: 336-8.        [ Links ]

2. Chevallier P, Oddo F, Souci J, Diaine B, Padovani B. Macroscopic intrahepatic portosystemic venous shunt: review of the literature and reclassification. J Radiol 2000; 81: 597-604.        [ Links ]

3. Mayayo E, Lidón MC, Fuentes J, Yagüe D, Soriano A, Uribarrena R. Spontaneous aneurysmal portohepatic fistula: Imaging diagnosis. Gastroenterol Hepatol 2004; 27(9): 525-8.        [ Links ]

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