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HEMOBILIA & BILHEMIA

Introduction

  • Key Point: Hemobilia and bilhemia occur when blood and bile mix abnormally due to a connection (fistula) between the bloodstream and the biliary system.

Hemobilia

  • Cause: Fistula between the hepatic artery and bile ducts. Blood enters the biliary tree.
  • Mechanism: High biliary pressure can facilitate the flow.

Bilhemia

  • Cause: Fistula between the biliary tract and the portal/hepatic veins. Bile enters the bloodstream.
  • Mechanism: Often due to high pressure in the bile ducts (e.g., from obstruction).

Additional Notes

  • Both conditions are considered rare, but can be serious if left untreated.
  • Understanding the causes, how they happen, and their signs is important for quick diagnosis and treatment.

Pathophysiology and Clinical Manifestations

Core Concept: Both conditions stem from abnormal connections (fistulas) between the bloodstream and the bile ducts.

  1. Hemobilia
    • How it happens:
      • Fistula between the hepatic artery (or less often, portal/hepatic veins) and bile ducts.
      • High-pressure blood enters the lower-pressure bile system.
    • Classic Presentation (Quincke's Triad):
      • Upper GI bleeding (melena or hematemesis)
      • Jaundice
      • Right upper quadrant pain
      • Note: The full triad is uncommon, but any combination of these symptoms, especially after liver trauma or procedures, should raise suspicion.
    • Other Complications:
      • Blood clots in the bile ducts can cause obstruction, choledocholithiasis, cholangitis, cholecystitis, or pancreatitis.
    • Lab Findings: Anemia, elevated liver enzymes (mainly bilirubin and alkaline phosphatase).
  2. Bilhemia
    • How it happens:
      • Fistula between bile ducts and portal/hepatic veins.
      • High biliary pressure (often due to obstruction) pushes bile into the bloodstream.
    • Symptoms:
      • Rising serum bilirubin without matching elevation of liver function tests (this distinguishes it from some other causes of jaundice).
      • Major risk: Septicemia from infected bile entering the bloodstream.

Etiology (Causes)

Shifting Trends:

  • Historically, accidental trauma was the main cause. Now, most cases are iatrogenic (caused by medical procedures).

Iatrogenic Injuries (over 70% of cases)

  • Highest Risk: Percutaneous procedures involving the liver:
    • Percutaneous Transhepatic Biliary Drainage (PTBD)
    • Percutaneous Transhepatic Cholangiography (PTC)
  • Factors Affecting Risk: Needle size, approach side (left/right), condition of the bile ducts, blood clotting ability.
  • Other Procedures:
    • Liver biopsies (lower risk)
    • ERCP (endoscopy)
    • RFA (radiofrequency ablation of tumors)

Accidental Trauma (now <5% of cases)

  • Blunt Trauma: Deep liver damage, or later formation of pseudoaneurysms.
  • Penetrating Trauma: Immediate injury to both blood vessels and bile ducts.
  • Key Point: Hemobilia can appear weeks or months after trauma.

Neoplasms (5-10% of cases)

  • Primary liver cancers (hepatocellular carcinoma, cholangiocarcinoma, etc.)
  • Metastases to the liver
  • Even benign lesions can rarely cause this
  • Important: Hemobilia can be the first sign of an undetected malignancy.

Vasculopathy & Coagulopathy

  • Vascular Malformations: Arterial aneurysms (most common), etc.
  • Bleeding Disorders: Hemophilia, platelet disorders, etc.

Inflammatory & Infectious Conditions

  • Gallstones: Prolonged obstruction can lead to inflammation and fistulas.
  • Cholecystitis: Both with and without stones.
  • Parasites: Liver flukes, roundworms, etc. Liver flukes and roundworms such as Clonorchis sinensis, Fasciola hepatica, and Ascaris lumbricoides have all been implicated in hemobilia.111–114 In addition, Echinococcus infectionscan also cause hemobilia secondary to local inflammation, caus- ing vascular and biliary damage adjacent to areas of hydatid cyst growth.115

Diagnostic Modalities

  • Key Point: Choice of test depends on the suspected cause of bleeding.
  • Gold Standard: Angiography
    • Directly visualizes blood vessels.
    • Shows contrast flowing into the bile ducts, bleeding sites (extravasation), and pseudoaneurysms.
    • Can be both diagnostic AND therapeutic (allows for coiling or stenting to stop bleeding).
    • Typically, angiography is under- taken in a systemic manner by cannulating the branches of celiac trunk, followed by the hepatic arterial branches and, if needed, the superior mesenteric artery.
  • CT Angiography:
    • Similar to angiography, shows contrast leaks.
    • Also provides information about other abdominal organs.
  • Important Note: Angiography (both direct and CT) are most helpful when there is ACTIVE bleeding happening at the time of the test.
  • Endoscopy:
    • Upper endoscopy: Looks for blood at the ampulla (where bile duct meets the intestine).
    • ERCP: Examines the bile ducts directly, can see blood clots, and potentially remove them.
  • Endoscopic Ultrasound (EUS): Specialized ultrasound probe for detailed views.
  • Bilhemia: Often only detected with ERCP, showing contrast flowing from the bile ducts into veins.
  • Additional Imaging
    • Ultrasound & MRCP: Not ideal for direct diagnosis, but can show signs like dilated bile ducts or pseudoaneurysms.

Management: Key Principles

  1. Stop The Bleeding: Key goal to prevent blood loss.
  2. Restore Bile Flow: Prevent complications like cholangitis (bile duct infection).
  3. Treat the Underlying Cause: If this is possible (e.g., tumor).

Treatment Options

  • Minor Hemobilia:
    • Conservative - often resolves on its own by adjusting bile duct drains, treating clotting issues.
  • Major Hemobilia
    • Endovascular Intervention: First-line treatment.
      • Embolization: Blocking the bleeding vessel with coils or other materials. Very successful, but carries a risk of bile duct injury.
      • Stenting: Covering the damaged area with a stent, especially useful for larger artery injuries.
    • Endoscopic Intervention: Can be both diagnostic and therapeutic
      • Control bleeding (for some causes) with injections, cauterization, etc.
      • Place stents to block off fistulas.
      • Most importantly: Clear blood clots from the bile ducts to restore flow.
    • Surgery: Last resort, for severe uncontrolled bleeding or when the cause itself needs surgery (e.g., tumor removal).
  • Special Note: Bilhemia
    • Focus on clearing biliary obstruction to reduce pressure.
    • Endoscopy is usually the main treatment option.

Hemobilia [DT]

Hemobilia:

  • Glisson: Guessed the possibility of hemobilia.
  • Sandbolm: Coined the term hemobilia.
  • Quincke: Described the triad of hemobilia, which includes gastrointestinal (GI) bleeding, jaundice, and right upper quadrant pain.
  • Most common cause: Trauma. Tumors are relatively uncommon causes.
  • Percutaneous cholangiography: Associated with intrabiliary hemorrhage, which is usually minor and self-limiting.
  • Ultrasonography: Nonspecific for diagnosing hemobilia.
  • Arteriography: Remains the best diagnostic method for hemobilia.
  • Severe hemobilia: Best treated with arteriographic embolization.
  • Surgical intervention: Reserved as a last resort or for cases recognized intraoperatively.

True statement regarding hemobilia:

  • a) Termed by Glisson
  • b) Characterized by Quincke's triad which includes GI bleed, fever, and jaundice
  • c) Trauma is the most common cause (Correct answer)
  • d) Spontaneous resolution is rare

Explanation:

  • Trauma is the most common cause of hemobilia. Quincke's triad consists of GI bleeding, jaundice, and right upper quadrant pain, not fever.

Hemobilia following PTBD (Percutaneous Transhepatic Biliary Drainage):

  • Tube repositioning or upsizing is generally sufficient to tamponade the bleeding.
  • First check: Ensure the proximal side hole of the catheter is within the duct and not in the transhepatic tube tract.
  • Next step: If venous bleeding persists, consider upsizing the biliary drainage catheter.

Which of the following statements about hemobilia are true?:

  • a) Tumors are the most common cause.
  • b) The primary treatment of severe hemobilia is surgical.
  • c) Percutaneous cholangiographic hemobilia is usually minor (Correct answer)
  • d) Ultrasonography usually reveals a specific diagnosis

Explanation:

  • Hemobilia following percutaneous cholangiography is usually minor and self-limiting. Tumors are not the most common cause, and surgery is typically reserved as a last resort.

Management of portal venous bleeding following PTBD:

First step should be:

  • a) Catheter side hole repositioning (Correct answer)
  • b) Upsizing of catheter
  • c) Embolization of catheter tract
  • d) Angiographic embolization

Explanation:

  • The first step in managing portal venous bleeding following PTBD is repositioning the catheter side hole to ensure it is intraductal. If bleeding persists, upsizing or other measures may be considered.

Hemobilia Vs Bilhemia Vs Hemosuccus Pancreaticus [DT]

Hemobilia Key Points:

  • Always consider hemobilia in the differential diagnosis (DDx) of upper GI bleeding, especially in patients with a history of liver trauma or biliary tree instrumentation.
  • Increased risk of procedural hemobilia with:
    • INR > 1.3
    • Platelet count < 60,000
  • Left-sided PTBD is more commonly associated with hemobilia.
  • Minor intraductal bleeding during liver resections can cause confusing postoperative jaundice.
  • Hemobilia after transplant is often secondary to percutaneous biopsy.
  • Most common primary vascular disorder causing hemobilia: Hepatic artery aneurysms.
  • Vascular lesions associated with hypertension can cause hemobilia, e.g., gallbladder apoplexy (hemorrhage into the gallbladder).
  • Gallstones can cause hemobilia when large stones penetrate the cystic artery or invade adjacent vessels or viscera.
  • Significant hemobilia occurs due to an abnormal fistula between a hepatic artery branch and the biliary tree.

Sandblom’s Triad for Hemobilia:

  1. Upper abdominal pain
  2. GI hemorrhage (GI bleeding)
  3. Jaundice

Diagnostic and Management Points:

  • Only 10% of endoscopies are diagnostic, showing blood at the Ampulla of Vater.
  • ERCP may reveal clots in the bile ducts.
  • Best investigation: Selective arteriography of the hepatic artery.

  • Treatment:

    • Transarterial embolization is the treatment of choice for significant hemobilia.
    • Surgery (ligation of right or left hepatic artery) may be necessary in refractory cases.
    • Portal venous fistula can often be treated by upsizing the biliary stent, which provides sufficient tamponade of the bleeding tract.

Bilhemia:

  • Definition: Bile entering the bloodstream through a communication with the hepatic veins or portal vein.
  • Cause: Occurs due to obstructive jaundice, where the pressure in bile ducts is higher than venous pressure, leading to bile leakage into the bloodstream.
  • Diagnostic tools:
    • Scintigraphy: Useful in making the diagnosis.
    • ERCP: The best means to show the fistula.
  • Treatment:
    • Endoscopic sphincterotomy (ES) or percutaneous transhepatic drainage.
    • Fistula occlusion through angiography.

MCQ Discussion - Bilhemia

Question: Regarding Bilhemia, all are true except:

a) Selective arteriography - diagnostic

b) Patients with rapidly increasing jaundice with elevated direct bilirubin without increase of liver enzymes

c) Treatment aims at release of distal obstruction

d) Fistula may close spontaneously

Answer: A

  • Selective arteriography is diagnostic for hemobilia, not bilhemia.

Key Points:

  • Bilhemia presents with rapidly increasing jaundice and elevated direct bilirubin without a significant rise in liver enzymes (Answer B is true).
  • Treatment focuses on releasing the distal obstruction to resolve the condition (Answer C is true).
  • In some cases, fistulas may close spontaneously but most often require treatment (Answer D is true).

Hemosuccus Pancreaticus:

  • Definition: Hemorrhage into the pancreatic duct and from there, through the ampulla of Vater into the GI tract.
  • Associated conditions: Can occur secondary to acute or chronic pancreatitis, pancreatic cystic neoplasms, or pseudoaneurysms.
  • Four key signs:
    1. Pain
    2. Raised amylase levels
    3. Upper GI bleeding
    4. History of acute or chronic pancreatitis
  • Treatment:
    • Selective angioembolization is the first line of treatment.
    • If surgery is necessary, the procedure of choice is distal pancreatectomy.