Chapter 105 Hemobilia and bilhemia
Overview
Hemorrhage into the biliary tract occurs when disease or trauma produces an abnormal communication between blood vessels and bile ducts, termed hemobilia (Sandblom, 1948), from the Greek haima (“blood”) and the Latin bilis (“bile”); the term bilhemia describes the rare condition of bile in the bloodstream. Causes of hemobilia include iatrogenic and accidental trauma, gallstones, inflammation, vascular disorders, and neoplasms. With the increasing use of invasive diagnostic and therapeutic procedures that involve the biliary tract, iatrogenic trauma has become the predominant etiology of hemobilia (see Chapters 28, 50D, 102, and 104).
Major, profuse hemobilia is a rare and sometimes life-threatening complication of liver or biliary tract disease or trauma. Minor hemobilia occurs more frequently but is rarely of long-lasting clinical significance. Although often difficult to recognize, hemobilia should be in the differential diagnosis of upper gastrointestinal (UGI) bleeding, especially in patients with a recent or remote history of liver trauma or biliary tree instrumentation (see Chapters 19, 28, 50D, and 102).
With the advent of interventional radiologic approaches, treatment of symptomatic hemobilia has shifted from surgical therapy to selective transcatheter hepatic arterial embolization (see Chapter 104). Surgical therapy via partial hepatectomy, hepatic artery ligation, or a combination of both procedures is rarely necessary and is reserved for hepatic arterial branches that cannot be safely or adequately embolized, for anatomic or technical reasons, or in patients who have large intrahepatic hematomas or sepsis.
History
Because of its obscure appearance and apparent rarity, hemorrhage into the biliary tract, although first described in the seventeenth century, was not called hemobilia until more recently. In the first detailed description of hepatic anatomy from 1654, Francis Glisson (Fig. 105.1) discussed the possibility of hemorrhage into the biliary tract when describing the case of a man who died from presumed GI hemorrhage following a stab wound to the liver suffered in a duel: “I believe that if the liver is injured by a contusion, it may lead to blood leaving the body by way of vomit or the stool for there is no doubt that the biliary tract takes unto itself (to the great good of the patient) some of the blood issuing into the liver and leads it down to the intestines. From there it is either impelled upwards through reverse peristalsis or downwards the normal way” (Glisson, 1654).
Over a century elapsed before the subject of hemorrhage into the biliary tract was brought up again. In Morgagni’s Epistles (1765), the founder of clinical pathology noted, in the section on the causes of dilation of the biliary tract, that abscesses in the liver and the voiding of sharp gallstones could lead to bleeding through the biliary ducts (Morgagni, 1765). In 1777, Antoine Portal presented a case in which he made the diagnosis before the death of the patient and confirmed it at autopsy. In this early treatise, Portal drew attention to the difficulty of finding the source of hemorrhage in the biliary tract, “when they are slight in quantity and occur but seldom,” and to the risk of mistakenly tracing them to a healthy organ, a mistake that has been made repeatedly in the history of hemobilia (Portal, 1813).
The first published case in North America was by a Boston surgeon who reported on the clinical and pathologic consequences of an “aneurysm of the hepatic artery bursting into the hepatic duct,” the first direct observation of an abnormal communication between the blood vessels and biliary ducts (Jackson, 1834; see Chapter 104). In 1871, the German surgeon Quincke further characterized biliary tract hemorrhage by establishing three cardinal symptoms—GI hemorrhage, biliary colic, and jaundice—commonly referred to as Quincke’s triad (Quincke, 1871). Hemobilia formerly was regarded as a medical curiosity, but it is now being diagnosed with increasing frequency, owing to more widespread knowledge of the syndrome and to improved diagnostic means (Sandblom, 1972).
Etiology
Common causes of abnormal vascular and biliary communication giving rise to hemobilia include trauma (accidental and iatrogenic), gallstones, neoplasms, inflammatory processes, and vascular disorders. Other less common causes include nematodes, blood coagulation defects, choledochal cysts, pancreatitis, and portal hypertension. Figure 105.2 illustrates the three largest reported case series of hemobilia and demonstrates a shift in etiology over the course of time from accidental to iatrogenic trauma. In one of the first publications devoted solely to hemobilia, Sandblom reported on 355 cases reported prior to 1972, with the largest number secondary to accidental trauma (Sandblom, 1972). Over the ensuing 30 years, iatrogenic trauma surpassed all other causes of hemobilia, and today it is responsible for the majority of cases (Green et al, 2001; Yoshida et al, 1987). The increase in hemobilia caused by iatrogenic trauma parallels the advent and increasing use of both diagnostic and therapeutic instrumentation of the biliary tract.
Iatrogenic Trauma
To an increasing extent, biliary hemorrhage is reported as a complication of diagnostic and therapeutic procedures on the liver or the bile ducts and is now responsible for the majority of reported cases of hemobilia (Fig. 105.3). The incidence of clinically significant hemobilia following percutaneous liver biopsy is between 0.01% and 0.2% (Piccinino et al, 1986; Seeff et al, 2010; see Chapter 20). Hemobilia arises secondary to unintentional laceration of a hepatic artery during the procedure and results in subsequent communication with the biliary tree (Fig. 105.4). Previous studies demonstrated that the risk of hemobilia following percutaneous liver biopsy may be greater in patients with chronic liver disease because of the presence of ascites, coagulopathy, and platelet dysfunction; the risk may also be higher following liver transplantation (Jabbour et al, 1995; Piccinino et al, 1986). In a recent study 2740 percutaneous liver biopsies were performed on patients with advanced chronic liver disease (cirrhosis), and it was determined that only an elevated international normalized ratio (INR) above 1.3 and a platelet count below 60,000 was associated with an increased risk of periprocedural hemobilia (Seeff et al, 2010).
Percutaneous transhepatic biliary drainage (PTBD) and percutaneous transhepatic cholangiography (PTC) are associated with a significantly higher rate of hemobilia than percutaneous liver biopsy because of the proximity of the hepatic vasculature to the biliary tree (Fig. 105.5; see Chapters 28 and 50D). In a series of 1397 patients undergoing PTBD or PTC, the incidence of clinically significant hemobilia was 1.9% (Fidelman et al, 2008). Patients undergoing PTBD had a 3.7-fold higher incidence of hemobilia compared with patients undergoing PTC, and they had a delayed presentation of hemobilia; presumably this was due to multiple catheter exchanges and a tamponade effect in PTBD patients. In this series, only large-bore needles were associated with hemobilia.
Other studies have demonstrated that use of a 21-gauge needle to access the biliary tree during PTC or PTBD, rather than an 18-gauge needle, is associated with a reduced incidence of hemobilia (Burke et al, 2003; Dousset et al, 1997; Harbin et al, 1980). In addition, correction of coagulopathy should reduce the development of clinically significant hemobilia (Hines et al, 1972). Another study concluded that neither a left-sided or right-sided hepatic approach to either PTBD or PTC is specifically associated with a higher incidence of hemobilia (Rivera-Sanfeliz et al, 2004). Other potential risk factors include cirrhosis, ascites, and absence of biliary dilation; extensive hepatic metastases do not appear to be predictive of hemobilia.
Surgery of the biliary tree may also lead to hemobilia as a result of injury of the hepatic artery by suture, dissection, diathermy, clip migration, erosion and subsequent arteriobiliary fistula, or pseudoaneurysm eroding into the extrahepatic bile duct (Fig. 105.6). The development of a hepatic artery pseudoaneurysm may precede the clinical manifestations of hemobilia by several weeks because of a delayed presentation; therefore, a high clinical suspicion and accurate history taking is often needed to accurately diagnose hemobilia (see Chapter 19). The advent of laparoscopic cholecystectomy for treatment of cholelithiasis and cholecystitis was originally feared to have produced an increased incidence of hemobilia secondary to vascular injury within the porta hepatis, specifically to the right hepatic artery (see Chapter 34). Stewart and colleagues (1995) reported five cases of hemobilia following laparoscopic cholecystectomy, and four of these were the result of traumatic aneurysm of the right hepatic artery; however, a large registry of 77,604 cases of patients undergoing laparoscopic cholecystectomy demonstrated that significant vascular injury was seen in only 0.16% (Deziel et al, 1993). It appears that fears of increased incidence of hemobilia following laparoscopic cholecystectomy were unfounded, overstated, and most likely secondary to the learning curve of laparoscopic surgery; however, the presentation of UGI bleeding from an unknown source weeks after laparoscopic cholecystectomy should heighten the suspicion of a potential injury to the right hepatic artery and resultant hemobilia.
Surgical procedures and exploratory instrumentation of the intrahepatic ducts may damage their walls, and the risk of hemorrhage is great. Minor bleeding constantly accompanies such procedures, but the bleeding generally ceases spontaneously, and the resulting clots promptly dissolve. Forceful instrumentation during exploration of the biliary tree and extraction of stones can result significant hemorrhage (Fig. 105.7). Bleeding from a T-tube occasionally can be caused by local erosion of the mucosa from tube, but if an arteriogram is done, it frequently exposes another source: a pseudoaneurysm that originates from an intraductal lesion, caused by instrumentation (see Fig. 105.7). As in other kinds of traumatic hemobilia, sometimes a considerable interval passes between the trauma and the bleeding episode.
Accidental Trauma
The liver is one of the organs most commonly injured by blunt trauma, with injuries detected in almost 25% of patients on routine computed tomography (CT) scan (Matthes et al, 2003; see Chapter 102). Nonsurgical management of blunt liver trauma in hemodynamically stable patients has become the standard treatment and is successful in 50% to 80% of patients (Carrillo et al, 1998; Forlee et al, 2004). The incidence of hemobilia following major liver injury has been reported to be as high as 3% in one series (Merrell & Schneider, 1991), but in other reports, the overall incidence is less than 0.2% (Parks et al, 1999). Other accidental traumas that result in hemobilia include injuries to the gallbladder, which is the organ injured in up to 2% of blunt abdominal trauma cases (Erb et al, 1994).
The clinical manifestations of blunt trauma to the liver are dependent on the anatomic site of damage. Tearing or injury to the liver capsule without deep parenchymal injury typically results in intraperitoneal hemorrhage that is most often managed with emergent operative exploration. Deep parenchymal injury or a central rupture may leave a large cavity, into which damaged bile ducts and blood vessels drain; the resulting biloma or hematoma may continue to expand, liver healing is impaired, and subsequent necrosis of the cavity wall may cause erosion of adjacent structures or formation of an intrahepatic artery pseudoaneurysm (Fig. 105.8).
If operation for blunt liver trauma is necessary, careful exploration of central injuries should be performed to ensure adequate hemostasis and ligation of biliary radicles before using packs to control bleeding. Indiscriminate use of deep parenchymal sutures should be avoided to prevent further injury to biliary radicles and vessels, potentially creating a central cavity that predisposes to the development of postoperative hemobilia. The conservative management of liver injuries has not resulted in an increase in the incidence of hemobilia following accidental trauma (Carrillo et al, 1998). In a series of 135 patients with severe liver injuries managed nonoperatively, only two developed hemobilia (Carrillo et al, 1999).
Vascular Disorders
Vascular disorders account for only about 10% of cases of clinically significant hemobilia (see Chapter 19; Green et al, 2001; Yoshida et al, 1987). Hepatic artery aneurysms are the most common primary vascular disorder to produce hemobilia. Other less common causes include angiodysplasia, arteriovenous malformations, and hemangiomas (Vishnevsky et al, 1991). The frequency of true aneurysms (Ryan et al, 2002) of the hepatic artery or its branches (Maralcan et al, 2003; Morioka et al, 2004) rupturing into the biliary tract is diminishing with the disappearance of mycotic aneurysms, leaving only aneurysms of atherosclerotic origin or those associated with polyarteritis nodosa (Dutta et al, 2004; Yazici et al, 1997), fibromuscular dysplasia (Shussman et al, 2008), or trauma. When an aneurysm only leaks, it might give rise to inconspicuous hemobilia; when it ruptures into the biliary tract, the symptoms are generally clinically significant, with exsanguinating hemorrhage and intense biliary colic. Sometimes, vascular lesions associated with arterial hypertension can result in hemobilia; the structure usually affected is the gallbladder, and the disorder is known as apoplexy of the gallbladder (Parekh & Corvera, 2010; Fig. 105.9).
