Haemobilie K83.8

The first account of a hemobilia was by Francis Glisson and was published in 1654. It describes a nobleman who suffered a blow to the right upper abdomen during a duel and ultimately died as a result (Zhornitskiy 2019).

The first ante-mortem description, from 1777, is by Antonie Portal (Zhornitskiy 2019).

Heinrich Irenäus Quincke described the clinical symptoms of hemobilia nearly a century later, referred to as the "Quincke Triad" (Zhornitskiy 2019).

In 1948, the term "hemobilia" appeared for the first time (Zhornitskiy 2019).

Hemobilia is the presence of extravascular blood (Berry 2019) within the bile ducts that is emptied into the small intestine via the papilla vateri (Herold 2022).

Hemobilia represents a rare disease. It is mainly caused iatrogenically, traumatically, or by neoplasia (Zhornitskiy 2019).

To date, 3 major case series have been published. The last of these case series, from 2001 by Green et al, showed iatrogenic trauma as the cause of hemobilia in 65% and accidental trauma in 6% (Zhornitskiy 2019). Thus, due to the increase in minimally invasive procedures (Navuluri 2016), the formerly most common cause, accidental trauma, has moved to second place (Staszak 2019).

Hemobilia can be iatrogenic, traumatogenic, neoplastic, inflammatory, infectious, and vascular in origin (Berry 2019) such as those caused by

- Trauma to the liver and/or bile ducts (Kasper 2015).

- iatrogenic injury

- lithiasis of the bile ducts

- tumors of the bile ducts

- Liver puncture (occurs in 3% of cases [Nuvuluri 2016])

- aneurysms

- Hemorrhage from a pancreatic pseudocyst (Herold 2022)

- rupture of a liver abscess

- hepatobiliary parasite infestation (Kasper 2015).

Extravascular hemorrhage in the bile ducts causes a phase separation of these two in the biliary tree due to the differences in density and biochemical properties of blood and bile. Once the bleeding stops, the blood that has entered the biliary tract begins to clot, impeding bile drainage (Berry 2019).

Most commonly, hemobilia manifests within 4 weeks of bile duct injury (Nuvuluri 2016).

The blood in hemobilia can be venous or arterial (Zhornitskiy 2019).

The typical Quincke's triad consists of:

- pain in the right upper abdomen (Zhornitskiy 2019)

- occult blood in the stool

- obstructive icterus (Kasper 2015).

However, this is found in only about 22 - 35% of patients. It has been shown that other symptoms, depending on the cause of hemobilia (Berry 2019), may occur such as:

- Melena

- hematemesis

- chloruria

- other laboratory changes (Zhornitskiy 2019).

V. a. hemobilia should be present in any patient with, for example, an unclear cause of gastrointestinal bleeding, a forceful upper abdominal procedure, after bile duct manipulation. The diagnosis itself can be challenging (Berry 2019).

A fecal blood test is positive for hemobilia. Other investigations include abdominal ultrasonography (Herold 2022), computed tomographic angiography (Zhornitskiy 2019), MRC, ERC for inspection of the papilla and therapeutic hemostasis if necessary (Herold 2022), EUS (Zhornitskiy 2019), and angiography (Herold 2022).

Abdominal sonography

Sonographically, blood clots in the bile ducts may present as gallstones due to similar echogenicity (Berry 2019).

Computed tomographic angiography (CTA).

CTA represents a noninvasive method for evaluation in cases of V. a. hemobilia (Zhornitskiy 2019) and has generally become the diagnostic modality of choice (Berry 2019). It can be used to evaluate the abdomen intra- and extraluminally (Zhornitskiy 2019).

ERC(P).

ERC can be used to visualize the biliary tree. Tubular, amorphous, or cylindrical filling defects with dilatation of the common bile ducts or perihilar duct indicate hemobilia. The examination method simultaneously offers possible therapeutic options (Zhornitskiy 2019). ERC can be used to diagnose approximately 60% of hemobilias (Berry 2019).

Endoscopic ultrasound (EUS).

If ERC findings are inconclusive, EUS can be used to evaluate vascular abnormalities and blood clots in the bile duct (Zhornitskiy 2019).

Angiography

Angiography remains the gold standard for both diagnosis and treatment of V. a. hemobilia. However, because it is an invasive examination, it is generally no longer used as the first method for diagnosis, but only when the bleeding vessel cannot be visualized with noninvasive methods (Zhornitskiy 2019).

MRC

Magnetic resonance cholangiography is a noninvasive method for ERC, but it lacks therapeutic options (Zhornitskiy 2019).

Typical signs of hemobilia are:

- Leukocytosis

- anemia

- abnormal serum tests of liver enzymes (Zhornitskiy 2019)

- increase in serum bilirubin

- Increase in alkaline phosphatase (Berry 2019).

- obstructive jaundice

- bile duct obstruction

- acute cholangitis

- acute cholecystitis

- pancreatitis (Berry 2019)

Minor episodes of hemobilia may resolve without surgical intervention (Kasper 2015). In this case, correction of the coagulopathy should be performed in addition to intravenous fluid administration (Berry 2019).

In most cases, however, ligation of the bleeding vessel becomes necessary (Kasper 2015). This is typically done endoscopically, radiologically, and rarely surgically (Berry 2019).

However, treatment should not focus on hemostasis alone, but should additionally maintain bile flow (Zhornitskiy 2019).

If symptoms of cholangitis with or without sepsis also exist, additional broad-spectrum antibiotics should be administered immediately (Berry 2019)

Transarterial embolization (TEA).

If there is evidence that it is an arterial hemorrhage, angiography with TEA is the 1st choice therapy. Success rates with this range from 80-100% (Zhornitskiy 2019).

Stent insertion

Another therapeutic option is the insertion of arterial and biliary stents. These have now become acceptable alternative therapies (Cathcart 2017).

Surgical intervention is rarely required as most cases of hemobilia can be successfully treated by interventional radiology. Surgical treatment options include:

- Ligation of the hepatic artery

- Removal of a pseudo-aneurysm of the hepatic artery

- Segmentectomy or lobectomy (Nuvuluri 2016).

Hemobilia can be potentially lethal if not diagnosed and treated in a timely manner (Zhornitskiy 2019).

Surgical success rates are approximately 90%. However, the mortality rate is relatively high at 10% (Nuvuluri 2016).

1. Berry R, Han J, Kardashian A A, LaRusso N F, Tabibian J H (2019) Hemobilia: etiology, diagnosis, and treatment. Liver Research 2 (4) 200 - 208
2. Cathcart S, Birk J W, Tadros M, Schuster M (2017) Hemobilia: An Uncommon But Notable Cause of Upper Gastrointestinal Bleeding. J Clin Gastroenterol. 51 (9) 796 - 804
3. Herold G et al (2022) Internal Medicine. Herold Publ. 455, 568
4. Kasper D L, Fauci A S, Hauser S L, Longo D L, Jameson J L, Loscalzo J et al (2015) Harrison's Principles of Internal Medicine. Mc Graw Hill Education 2084
5. Navuluri R (2016) Hemobilia. Semin Intervent Radiol. 33 (4) 324 - 331
6. Staszak J K, Buechner D, Helmick R A (2019) Cholecystitis and hemobilia. J Surg Case Rep (12) rjz350 DOI: 10.1093/jscr/rjz350.
7. Zhornitskiy, A, Berry R, Han J Y, Tabibian J H (2019) Hemobilia: historical overview, clinical update, and current practices. Liver Int. 39 (8) 1378 - 1388.