One patient with clinical evidence of cirrhosis and portal hypertension had episodes of hepatic encephalopathy and elevated blood levels of ammonia. Five patients with this disorder are presented, each of whom was diagnosed by color Doppler imaging, including waveform spectral analysis. It may be congenital, or secondary to portal hypertension. Intrahepatic portosystemic venous shunt is a rare clinical entity only 33 such cases have been reported. Kudo, M Tomita, S Tochio, H Minowa, K Todo, A Intrahepatic portosystemic venous shunt: diagnosis by color Doppler imaging. This potentially serious complication appears to be specific for TIPS in BCS. Review of the literature reveals only two previous cases, both occurring in patients with BCS and presenting after a similar time interval. We describe a patient with Budd-Chiari syndrome (BCS), who presented with a large inrahepatic hematoma 13 days after TIPS. Late intrahepatic hematoma is a rare complication of the transjugular intrahepatic portosystemic shunt (TIPS) procedure. Terreni, Natalia Vangeli, Marcello Raimondo, Maria Luisa Late Intrahepatic Hematoma Complicating Transjugular Intrahepatic Portosystemic Shunt for Budd-Chiari SyndromeĭOE Office of Scientific and Technical Information (OSTI.GOV) A case of a 38-year-old woman with abdominal pain and low grade encephalopathy, diagnosed with an IPSS and treated by right hepatectomy was reported. Literature review reports of surgical and radiological occlusion of the shunt, but due to its rarity, a standard therapeutic protocol has not been established. When recognized, early intervention to occlude the shunt will reverse the associated complications. It has been described in both children and adults and may be asymptomatic or be associated with a variety of neurophysiological and pulmonary complications. Intrahepatic portosystemic shunts (IPSS) are rare congenital anomalies arising from disordered portal vein embryogenesis. Papamichail, Michail Ali, Amir Quaglia, Alberto Karani, John Heaton, Nigel Liver resection for the treatment of a congenital intrahepatic portosystemic venous shunt. • Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases. What is new: • There is very few studies with only patients diagnosed in antenatal and it is a large series of cases. • The evolution, management, and complication are well known. What is known: • Multiples studies exist on congenital porto systemic shunt but when the diagnosis is done after birth. Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases. Another patient had radiological interventional closure of his shunt which was complicated by a venal portal thrombosis. One patient had surgical treatment which failed and he developed a focal nodular hyperplasia. Ten patients had simple outcome on conservative treatment, eight of them having a spontaneous closure of their portosystemic shunt within the first 2 years of life. All the patients presented intrahepatic CPSS, three of them had associated congenital cardiopathy, and one a Bannayan-Zonana syndrome. Prenatal diagnosis was done at a median 26.5 weeks of gestation (21-34). This multicentric retrospective study included all the patients with a prenatal diagnosis of congenital portosystemic shunt. We analyzed the characteristics of the population with congenital portosystemic shunt diagnosed during the antenatal period and the organization of their perinatal care. Outcome of intrahepatic portosystemic shunt diagnosed prenatally.įrancois, Bérengère Gottrand, Fréderic Lachaux, Alain Boyer, Corinne Benoit, Bernard De Smet, Stéphanie We report a case of an asymptomatic adult female with a presumed congenital intrahepatic portosystemic shunt and discuss the pertinent imaging findings and important key concepts related to this condition. Chronic shunting may result in encephalopathy, bleeding or hyperinsulinism. Most cases of portosystemic shunt occur after trauma, surgery, liver biopsy or as a result of chronic portal hypertension. Asymptomatic Congenital Intrahepatic Portosystemic Shunt.Ĭongenital (spontaneous) intrahepatic portosystemic shunt is rare in the English literature.
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