SpontaneouS life-threatening hemobilia during aCute liver failure SuCCeSSfully treated with tranSarterial embolization

syndrome resulting from a massive and rapid impairment of all crucial liver functions in patients with previ­ ously unknown liver disease. The syndrome of acute liver failure in­ cludes signs directly related to the liver cell damage, altered liver func­ tion and the consequences on other organs. These manifestations essen­ tially include jaundice, elevated serum transaminase levels, encepha­ lopathy, coagulopathy, severe infec­ tions, and renal, cardiovascular, pulmonary, metabolic disorders. The prognosis depends on the extent of the liver cell damage, the liver’s re­ generative capacity, associated co­ morbidities and possible therapeutic interventions as liver transplanta­ tion. The first objective in the management of patients with acute liver failure is to maintain an optimal condition for liver regeneration together with taking the decision with appropriate timing for emer­ gency transplantation. Here, we present and discuss the case of an acute liver failure in a young patient who developed a rupture of the oesophagus and spontaneous massive hemobilia. Both these conditions were life­ threatening but treated successfully, allowing a consecutive successful liver transplantation.

Acute liver failure is defined as a syndrome resulting from a massive and rapid impairment of all crucial liver functions in patients with previ ously unknown liver disease. The syndrome of acute liver failure in cludes signs directly related to the liver cell damage, altered liver func tion and the consequences on other organs. These manifestations essen tially include jaundice, elevated serum transaminase levels, encepha lopathy, coagulopathy, severe infec tions, and renal, cardiovascular, pulmonary, metabolic disorders. The prognosis depends on the extent of the liver cell damage, the liver's re generative capacity, associated co morbidities and possible therapeutic interventions as liver transplanta tion. The first objective in the management of patients with acute liver failure is to maintain an optimal condition for liver regeneration together with taking the decision with appropriate timing for emer gency transplantation.
Here, we present and discuss the case of an acute liver failure in a young patient who developed a rupture of the oesophagus and spontaneous massive hemobilia. Both these conditions were life threatening but treated successfully, allowing a consecutive successful liver transplantation.

Case presentation
A 28yearold patient was referred to our transplantation centre because of asymptomatic jaundice, regurgita tions, increasing dyspepsia and 2,456 IU/L, normal < 38 IU/L), direct hyperbilirubinaemia (26.08 mg/dl, normal range < 1 mg/dl) and coagu lopathy (INR 2.3). Biochemical screening for viral and immunologi cal causes of acute liver failure was negative; levels of ceruloplasmin, ferritin and α1antitrypsin were nor mal. Ultrasound of the abdomen re vealed a relatively small liver and mild splenomegaly without signs of biliary obstruction. Transjugular liver biopsy via right hepatic vein was performed uncomplicated and was suggestive for toxic hepatitis with more than 50% necrotic liver paren chyma. In accordance with the King's College criteria for acute liver failure (1), he was listed for urgent liver transplantation.
Within 12 hours after listing and following a vomiting episode, his clin ical condition suddenly deteriorated, vomiting since 2 weeks in combina tion with deteriorating coagulopa thy. His previous medical history was uneventful. There was no family history of liver disease, no recent travelling or contact with toxic agents. He did not take any medica tion or illicit drugs but there was occasional excessive alcohol intake during weekends. On admission, physical examination revealed jaundice, without other signs of liver failure or longstanding liver dys function.

SpontaneouS life-threatening hemobilia during aCute liver failure SuCCeSSfully treated with tranSarterial embolization
of the right hepatic artery and the right bile duct with contrast extrava sation in the gallbladder, through the common bile duct and into the duo denum ( Fig. 2AC). This arteriobiliary fistula was treated successfully with arterial embolization, using a mixture of enbucrylate (Histoacryl ® , B. Braun, Melsungen, Germany) and lipiodol (ethiodized oil, Laboratoires Guerbet, AulnaysousBois, France). Two days (normal range 1216 g/dl) and INR 10. He was resuscitated aggressively with colloids, blood and plasma transfusion, and administration of recombinant factor VIIa. An urgent gastroduodenoscopy revealed a continuous blood flow from the Vater's ampulla, stating the diagno sis of massive hemobilia. Consecu tive angiography revealed an arterio biliary fistula between a distal branch developing severe epigastric pain with signs of localised peritonitis ac companied by melaena. CT of the abdomen revealed free air around the oesophagus, consistent with a transmural tear (Boerhaave's syn drome) which was treated conserva tively ( Fig. 1). Within 24 hours, the patient was found unconsciously and in hypovolemic shock. Labora tory analysis showed Hb 2.9 g/dL  lar biopsy was preferred here over a percutaneous approach because of the profound coagulopathy which is considered a contraindication for percutaneous liver biopsy. In the presented case, however, it is unlike ly that the massive hemobilia was directly related to the transjugular biopsy, since the hemobilia present ed several days after the transjugular biopsy. Moreover, during arteriogra phy there were several small bleed ing points (distal branches of right hepatic artery) which were not located within the area of biopsy. However a facilitating effect of the transjugular biopsy can never be excluded. It is most likely that the fistula eroded slowly through the massive hepatocellular necrosis (as documented by liver biopsy) and ongoing inflammation into small segmental branches of the right hepatic artery resulting in an arterio biliary fistula.
In this particular case, we opted for glue embolization instead of coil ing. Injection of glue is fast and a few droplets can immediately occlude all bleeding points. Glueembolization of the hepatic artery has also been used for other indications, like treat ment of unresectable neuroendo crine liver metastases without clear damage to the residual hepatocytes or biliary tract (5). In general, trans arterial embolization is regarded an effective treatment for hemobilia and arteriobiliary fistulas (69). In case of failed transarterial haemostasis, surgical ligation of the feeding artery and/or liver resection (e.g. segmen tectomy) may be the preferred method of salvage (10).

Conclusion
Aggressive treatment of potential ly reversible lifethreatening compli cations, even when occurring during acute liver failure whilst awaiting ur gent liver transplantation, is essen tial. In this particular case, intensive medical treatment and intervention al radiological techniques, per formed in emergency setting, were crucial for the patient's survival until after the hypovolemic shock episode, the patient underwent an orthotopic liver transplantation. His recovery was uneventful apart from an anas tomotic biliary stricture which was treated endoscopically. He is currently doing well, more than 72 months after the transplantation.

discussion
Management and stabilization of patients with acute liver failure is challenging but crucial to maintain a condition as optimal as possible for liver regeneration and/or urgent transplantation. Once liver failure is diagnosed, it is mandatory that pa tients are referred to a centre with a liver transplantation program and experienced interventional radiolo gy. As discussed here, this patient developed -whilst awaiting an ur gent transplantation -two major life threatening conditions which were treated quickly and adequately en abling a livesaving liver transplanta tion.
This patient first suffered from a Boerhaave's syndrome. This rela tively rare disorder is caused by a barogenic rupture of the oesopha gus and is associated with a high mortality (2040%). In normal condi tions, conservative treatment and/or endoscopical intervention are indi cated in the absence of systemic effects whereas urgent surgical repair is mandatory in case of e.g. sepsis (2). The patient was treated conservatively since at that time his clinical condition remained stable in the next hours after onset of the symptoms, hereby also taking into account a high mortality riskrelated surgery during acute liver failure.
A second complication was a massive hemobilia secondary to an arteriobiliary fistula. Arteriobiliary fistulas have been described previ ously as rare but potentially fatal complications, mainly after trauma or liver biopsy (3). The risk of an arte riobiliary fistula is higher after a trans jugular liver biopsy (1.2%) com pared to the percutaneous approach (0.006%) (4). Obviously, a transjugu