general surgery procedures in patients with significant liver disease , cirrhosis and concomitant portal hypertension are associated with high mortality rates . recently , minimally invasive transjugular intrahepatic portosystemic shunt ( tips ) procedures have replaced surgical portosystemic shunts . this technique is capable of reducing portal hypertension - associated complications leading to reduced intraoperative bleeding and perioperative complications in patients undergoing major extrahepatic surgery , but experience remains limited . pancreatic resection , in particular pancreaticoduodenectomy ( pd ) or whipple resection , is associated with significant morbidity requiring detailed preoperative assessment and cautious patient selection in order to minimize complications . the use of prophylactic tips for resectional cancer surgery is uncommon , with only a single report of utilization to achieve a major pancreatic resection . subsequently , we report on a patient undergoing portal decompression via tips prior to a whipple resection , a combination referred to as the  glasgow tipple. a 49-year - old male with no alcohol history presented to glasgow royal infirmary with a 3-month history of right upper quadrant pain , jaundice , fever and 15 kg weight loss . abdominal examination revealed hepatosplenomegaly , confirmed by abdominal ultrasound along with intrahepatic biliary tree dilatation . computed tomography ( ct ) imaging demonstrated abrupt distal common bile duct ( cbd ) narrowing yet normal pancreatic duct . notably , significant portal hypertension was evident with large para - esophageal varices and splenomegaly ( fig . 1a ) . with no obvious risk factors for chronic liver disease apart from obesity , alcoholic steatohepatitis was suspected . while magnetic resonance cholangiopancreatography was planned , cholangitis developed , prompting an ercp , which revealed grade b esophageal varices , an edematous ampulla ( fig . 1b ) and a distal cbd filling defect , which was biopsied , with biliary drainage achieved using a plastic double pigtail stent . histopathological examination revealed high - grade dysplasia within the bile duct adenoma . with potential for malignant progression , surgical resection was considered ; however , comorbidity including obesity and sleep apnea compounded by portosystemic hypertension created a management dilemma . figure 1:(a ) preoperative ct scan revealed significant portal hypertension with large para - esophageal varices and splenomegaly . ( a ) preoperative ct scan revealed significant portal hypertension with large para - esophageal varices and splenomegaly . hepatic vein wedge pressure measured 30 mmhg compared to an arterial pressure of 12 mmhg confirmed portal hypertension ; however , a fibroscan revealed no evidence of cirrhosis . liver histology from a percutaneous biopsy suggested congenital hepatic fibrosis ( chf ) as a potential underlying cause of portal hypertension ( fig . following intense multidisciplinary discussion , it was decided that in the context of a relatively young , cardiovascularly fit patient with non - cirrhotic liver disease , a tips procedure was appropriate , with a view to reconsidering surgery . a literature search failed to identify evidence to support a decision to adopt a staged tips with pd for a high - grade dysplastic lesion . significant residual biliary dilatation remained , which prompted placement of a 6-cm partially covered metal wall stent with subsequent improved drainage . a viatorr stent graft ( 10 mm , gore ) was then placed between the middle hepatic vein and left portal vein . pre - tips pressures measured as 19 mmhg was reduced to 7 mmhg postprocedure ; unfortunately , this initial tips failed requiring a second , successful procedure ( fig . figure 2:(a ) successful tipps deployment following insertion of a partially covered metallic biliary stent enabled portal decompression and resection to be performed . ( b ) postoperative ct scan revealed failure of the tips ( arrow ) along with smv thrombus resulting in significant venous congestion of the small bowel . ( a ) successful tipps deployment following insertion of a partially covered metallic biliary stent enabled portal decompression and resection to be performed . ( b ) postoperative ct scan revealed failure of the tips ( arrow ) along with smv thrombus resulting in significant venous congestion of the small bowel . following further deliberation and no evidence of metastatic disease on imaging , the patient proceeded to resection . while fibroscan had suggested a normal liver , although few high - pressure veins were noted , the tips had achieved successful portal decompression enabling pd . the patient subsequently developed postoperative pancreatitis , complicated on postoperative day 4 by respiratory distress requiring intensive care unit admission . ct imaging revealed small fluid collections related to the pancreatic anastomosis , which were not amenable to drainage . strikingly , repeat ct scan on postoperative day 6 demonstrated tips failure with extension of thrombus into the superior mesenteric vein ( smv ) and significant small bowel venous congestion . despite the ominous appearance ( fig . 2b ) conservative management with broad - spectrum antibiotics , inotropic support and low molecular weight heparin resulted in gradual improvement with discharge home achieved on day 28 . final pathology unexpectedly revealed a 15-mm pt1pn0 distal cholangiocarcinoma with clear resection margins ( fig . in the case presented above , portal hypertension potentially related to chf was managed by preoperative tips insertion enabling curative pd for a cholangiocarcinoma . while the minimally invasive modality of a tips can reduce portal pressure in patients with severe liver cirrhosis , pd in patients with portal hypertension secondary to liver disease previous studies have reported improved outcomes in patients undergoing tips placement prior to major nonhepatic surgery with a decrease in portal pressure improving surgical outcome due to reduced perioperative bleeding and ascites . there were however no previous literature reports of this staged approach being utilized to facilitate a pancreatic resection . while complications following such a high - risk pd were not unexpected , postoperative tips failure with subsequent significant visceral venous congestion was not predicted and generated a challenging postoperative period for which a conservative strategy achieved a favorable outcome . of further interest is the diagnosis of chf , a portobiliary system developmental disorder characterized histologically by defective ductal plate remodeling , progressive portal tract fibrosis and macroscopic cystic intrahepatic bile duct dilatation . clinical features include hepatomegaly , well - preserved hepatocellular function , portal hypertension resulting in splenomegaly , hypersplenism and gastro - esophageal varices [ 6 , 7 ] . furthermore , chf is associated with ciliopathies ( primary cilia disorders ) that can result in renal disease . relative preservation of hepatocellular function despite hepatic fibrosis was a factor in the decision to undertake resection in the described case . there have been four reported cases of intrahepatic and a single extrahepatic cholangiocarcinoma occurring on the background of chf , this patient being the second [ 69 ] . a potential mechanism by which the chf intrahepatic biliary system undergoes malignant transformation may be the result of abnormal cholangiocyte cilia function . this case demonstrates that in carefully selected patients with preserved liver function there may be a role for minimally invasive portal decompression to facilitate pancreatic resection for malignancy . while not without risk , our multidisciplinary strategy enabled a potentially curative resection for a patient for whom abdominal surgery would normally be contraindicated . we believe that carefully selected patients with pancreatic disease may benefit from this two - step approach and therefore assessment in further patient collectives are warranted .
abdominal surgery performed in patients with significant liver disease and portal hypertension is associated with high mortality rates , with even poorer outcomes associated with complex pancreaticobiliary operations . 
 we report on a patient requiring portal decompression via transjugular intrahepatic portosystemic shunt ( tips ) prior to a pancreaticoduodenectomy . 
 the 49-year - old patient presented with pain , jaundice and weight loss . at ercp 
 an edematous ampulla was biopsied , revealing high - grade dysplasia within a distal bile duct adenoma . 
 liver biopsy was performed to investigate portal hypertension , confirming congenital hepatic fibrosis ( chf ) . 
 a tips was performed to enable a pancreaticoduodenectomy . 
 prophylactic tips can be performed for preoperative portal decompression for patients requiring pancreatic resection . 
 a potentially curative resection was performed when abdominal surgery was initially thought impossible . 
 notably , chf has been associated with the development of cholangiocarcinoma in only four previous instances , with this case being only the second reported distal bile duct cholangiocarcinoma .