Iatrogenic
Liver biopsy
Transhepatic cholangiography
Transhepatic ablative therapy
Transhepatic biliary drainage
Cholecystectomy
Bile duct surgery
Endoscopic retrograde cholangiopancreatography (ERCP) manipulation (stenting, sphincterotomy, biopsy, lithotripsy, stricture dilation, etc.)
Trauma
Penetrating injury to liver or bile duct
Blunt liver trauma
Neoplastic
Primary liver cancer
Gallbladder cancer
Bile duct cancer
Benign liver tumor
Metastatic cancer to liver/bile duct
Gallstones
Gallstone irritation
Gallbladder/bile duct
Inflammation
Vascular
Pseudoaneurysm from inflammatory condition
Arteritis
Arteriovenous malformation
Arterial aneurysm
Pancreatic
Pseudocyst
Cancer invasion
Infection
Parasite
Liver abscess
Table 16.2
Causes of hemosuccus pancreaticus
Pancreatitis | Pancreatic necrosis |
Pseudocyst | |
Splenic artery pseudoaneurysm | |
Pancreatic tumor | Pancreatic cancer |
Neuroendocrine tumor | |
Metastatic cancer to the pancreas | |
Serous cystadenoma | |
Vascular disease | Aneurysm of the celiac or splenic artery |
Segmental arterial mediolysis | |
Pancreatic arteriovenous malformation | |
Pancreatic trauma | Penetrating injury |
Blunt trauma | |
Iatrogenic pancreatic injury | Needle aspiration of pancreatic cyst |
Endoscopic necrosectomy | |
Pancreatic stenting | |
Ductal dilation | |
Pancreatic stone lithotripsy | |
Pancreatic infection | Brucellosis |
Tuberculosis |
Case 1
A 33-year-old man presented with right upper quadrant pain and jaundice. He underwent a cholecystectomy, common duct exploration and T-tube placement for acute cholecystitis and a large common bile duct stone. He remained deeply jaundice after surgery and had required six units of packed red blood cell transfusion over the next week. He then developed intermittent low grade fever. During this time, both the T-tube and percutaneous subhepatic drain had low outputs of blood tinged fluid.
What is the Differential Diagnosis?
An extensive biliary and gallbladder surgery, followed by cholangitis and persistent jaundice, is worrisome for a bile duct injury such as extrahepatic bile duct transection, retained stone or diffuse liver injury. The low T-tube output suggested a patent bile duct or T-tube malfunction. The latter possibility was supported by difficult bedside irrigation through this small-caliber tube. The blood tinged fluid via the percutaneous drain and T-tube pointed to bleeding within and outside of the biliary tract, raising suspicion for both bleeding and communication between these two drains. On the other hand, having some minor oozing after extensive manipulations of the bile duct was not definitive evidence of a bleeding complication. Of the four possible ways to investigate the integrity of the biliary tract, including radionuclide biliary scan, T-tube cholangiograms, magnetic resonance cholangiopancreatography (MRCP), and endoscopic retrograde cholangiopancreatography (ERCP). ERCP is perhaps most accurate and potentially therapeutic. Before doing so, an abdominal computed tomography (CT) should be done to exclude an abscess, hematoma, or biloma.
Case Continued
An abdominal CT showed no significant fluid collection or abscess cavity. On post-operation day number 12, an ERCP was performed. At the procedure, the papilla appeared normal. Initial contrast injection was difficult, as the entire bile duct was packed with some ill-defined filling defects. After a sphincterotomy, balloon sweeps retrieved a large amount of fresh blood and clots. Bile leak was discovered at the cystic duct stump and at the T-tube site (Fig. 16.1). After evacuating blood and debris from the bile duct, two 10 French plastic stent biliary stents were placed. This patient recovered uneventfully after the ERCP, without further jaundice, fever, or bleeding. The stents were removed 2 months later.
Fig. 16.1
Hemobilia occurring after cholecystectomy. a Initial contrast injection was difficult, and cholangiogram showed extensive filling defects in the common and right hepatic ducts. b After sweeping clear some blood clots from the extrahepatic bile duct, contrast was noted leaking out of the cystic duct stump and the T-tube insertion site of the bile duct
Hemobilia
Iatrogenic injury of the bile duct or liver tissue is the most common cause of hemobilia , accounting for roughly two thirds of all such cases [2]. Transient bleeding via the papilla is often noted after a percutaneous liver biopsy and is thought to be due to the close proximity between the intrahepatic bile duct, hepatic artery, and portal vein [3]. Needle puncture can easily penetrate these structures to form arteriovenous fistula, arterial bile duct fistula, or venous bile duct fistula. Venous bile duct fistula bleeding is typically mild and self-limited, rarely requiring any therapeutic intervention. Transhepatic cholangiography and percutaneous biliary drainage , which causes hemorrhage in 2− 2.5 % of the procedures [4], may result in life-threatening hemobilia through injury to the hepatic artery or portal vein [5]. Even internal biliary stents , particularly metallic prosthesis, may result in direct vascular puncture or formation of pseudoaneurysm of the hepatic artery [6].
A strong clue to hemobilia is the presence of the triad of overt GI bleeding , jaundice, and right upper quadrant pain [7]. However, this is often the exception rather than the rule, as all three signs exist only in 22 % of all hemobilia cases [2]. More commonly, the endoscopist discovers blood either within the bile duct or oozing from the major papilla during an ERCP. Rarely, hemobilia is the cause of unexplained obscure GI bleeding . Depending on the location of bleeding, cholangitis, cholecystitis, pancreatitis have all been reported. Massive hemorrhage can mimic lower GI bleeding.
Evaluation for hemobilia depends on the clinical presentation and suspicion for hemobilia [1]. In patients with high suspicion for hemobilia , CT angiography is the test of choice not only to identify presence of bleeding, but also to identify the source and plan for potential therapeutic angiography. Otherwise, patients displaying signs and symptoms of GI bleeding should be evaluated with upper endoscopy . A side-viewing duodenoscope is necessary to visualize the papilla adequately. In patients presenting with cholangitis or biliary obstruction, ERCP is a reasonable initial diagnostic procedure with or without antecedent radiology imaging such as transabdominal ultrasound, CT or MRCP. On cholangiography, blood clots are poorly outlined and they do not retain a constant shape like gallstones do. These ghost-like filling defects may mimic those from neoplastic tissues. Interestingly, fresh blood and blood clots are best seen during the initial contrast filling of the bile duct. When the bile duct is more saturated with contrast, the filling defects may disappear. Therefore, it is always a good practice to observe the fluoroscopy or obtain radiographs at the beginning of contrast injection. The ultimate proof of hemobilia is visualization of blood coming out of the bile duct, usually occurring at the time of a balloon sweep. Depending on the duration of clot formation , some of this material may appear as soft hemorrhagic tumors . Indeed, blood in the bile duct often contains tumor cells and should be suctioned into a container for cytology evaluation.
A trivial amount of bleeding may be seen during ERCP following balloon stricture dilation, forceps biopsy, or stone extraction and is not out of the ordinary or a major issue. However, when encountering spontaneous and large quantity bleeding, it is important to think about the probable cause, extent of disease and backup plan before embarking on a more thorough investigation or manipulation of the bile duct. In particular, biliary bleeding in advanced portal hypertension [8], pseudoaneurysm, indwelling stent erosion into the periductal vasculature, and sphincterotomy bleeding related blood reflux into the bile duct should all be taken seriously. Fatal hemobilia has been reported in the literature [9]. Sudden exsanguination may occur in these settings, and a good anticipatory plan should be in place before proceeding further. In massive bleeding that does not stop spontaneously, one possible way to temporize the situation is to occlude the bile duct with a retrieval balloon. Among the ultimate treatment options are emergency angiographic embolization [10], balloon tamponade, fully-covered metal stenting, and a full range of endoscopic bleeding treatment modalities.
Not all patients with hemobilia require treatment as most iatrogenic bleeding after percutaneous liver biopsy or percutaneous biliary drainage stop spontaneously. For ongoing or recurrent bleeding, angiography with embolization is the treatment of choice with reports of 75–100 % success [1]. ERCP does not have a role in treating bleeding and is only indicated for establishing biliary drainage.
This case illustrates that multiple biliary complications , including bile leak, hemorrhage , biliary obstruction, and cholangitis , can take place simultaneously when an adverse event has occurred during gallbladder surgery. In spite of the potential devastation, these problems can be successfully treated with simple biliary stenting. While fully covered metal stents have been reported as effective [8, 11, 12], even plastic stents as were used here may be just as useful.
Case 2
A 46-year-old female presented with jaundice and a suspected hilar mass. A transhepatic study failed to pass through her biliary stricture. ERCP showed a high grade obstruction of the proximal common hepatic duct, with dilated right and left hepatic ducts (Fig. 16.2). Catheter aspiration of the intrahepatic fluid showed bloody material with some pus. Further injection of contrast demonstrated ill-defined filling defects throughout the obstructed ducts, consistent with hemobilia or tumor infiltration. After stricture brushing and dilation, a 10 French plastic stent was placed, draining a large amount of bloody fluid. The brush cytology was positive for adenocarcinoma.
Fig. 16.2
Cholangiograms after a failed transhepatic drainage of common hepatic duct stricture. a High grade obstruction of the common hepatic duct. Note: Blood appears as linear serpiginous filling defects. b Further contrast injection shows different appearance of the intrahepatic duct filling defects, characteristic of blood clots in the bile duct
Another Iatrogenic Cause of Hemobilia
This case demonstrates that transhepatic needle punctures may lead to hemobilia , especially after an extended effort made to access the bile duct for drainage. The initial cholangiograms show linear, serpiginous, filling defects that might represent biliary ascaris or tumor infiltration and not hemobilia. Of course, the only way to confirm biliary hemorrhage is to visualize blood through a patent biliary stent or balloon sweeps. While bleeding from a failed puncture attempt is usually self-limited, hemorrhage from an indwelling transhepatic catheter may present recurrently from a pseudoaneurysm [13]. It has previously been reported that 50 % of biliary source of bleeding takes place in the intrahepatic bile ducts and the other half is from the extrahepatic system and the gallbladder [14]. With increasing transhepatic therapies, there are probably more intrahepatic bleeding cases in these days.
Case 3
A 44-year-old male presented to an outside facility with jaundice and right upper quadrant pain. ERCP showed a liver hilum mass and blood clots in the extrahepatic bile duct, with bile duct biopsy showing hepatocellular carcinoma. Multiple subsequent ERCPs and stenting failed to improve his liver function and he was referred to our institution for further evaluation . Upon removal of his internal stents, a large amount of blood passed through the papilla. Some materials that were swept out appeared to be soft tumor tissue or well-formed clots (Fig. 16.3). Cholangiograms showed extensive, irregularly shaped, filling defects. Despite multiple plastic stents , metal stent, and even a nasobiliary drain placement over the next few weeks, the patient remained jaundice with on-going blood transfusion requirements. He ultimately underwent a very difficult biliary surgical resection and lived for another 2 years.
Fig. 16.3
Hepatocellular carcinoma presenting with hemobilia. a Initial cholangiogram shows blood filling the entire bile duct, leaving narrow spaces around the bile duct to be filled with contrast, giving the appearance of double contrast outlining of the extrahepatic bile duct. b Blood clots mixed with hilar liver cancer, presenting as a large mass occupying the bile duct bifurcation. c A large blood clot being extracted from the bile duct. d This large soft mass appears to be a cross between a clot and a tumor, containing tissue positive for hepatocellular carcinoma
A Non-iatrogenic Cause of Hemobilia
Hemobilia is a common presentation of hepatocellular carcinomas that locate centrally and have invaded the bile duct . These tumors are highly vascular and bleeding can be massive or continuous as in this case. The diagnosis should be suspected in spontaneous intraductal bleeding in the proximal common hepatic duct or intrahepatic ducts. However, we have observed some hepatocellular carcinomas that extended down the entire bile duct and even infiltrated the papilla. Tissue acquisition for diagnosis is readily achieved in these cases, as either the blood clots or exophytic tissues are easily obtained to determine the nature of the cancer. Most of these lesions are unresectable and are difficult to manage. As opposed to stenting a tumor with minimal bleeding tendency, palliative stenting of a hepatoma that causes hemobilia is frequently ineffective, as continuous hemorrhage may lead to early stent failure and clogging. Even large caliber metallic biliary stents do not ensure adequate patency because of the large blood clots. Likewise, transhepatic or vascular interventions may be ineffective in stopping bleeding of these highly vascular lesions. Chemotherapy treatment of advanced hepatocellular carcinoma had been linked to fatal hemobilia and should be used with caution in patients with a prior history of hemobilia [15]. This case is a perfect example of such a problematic situation. Despite the risk and technical difficulty, surgical resection may be the only viable option in some cases. Rarely, blood clots and even sloughed tumors may act like gallstones and induce acute pancreatitis [16, 17], requiring sphincterotomy for relief. The finding of pancreatitis in these cases may mislead us to consider hemosuccus pancreaticus instead of hemobilia.