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Biliary Diseases - Types, Symptoms, and Treatment

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Biliary diseases are disorders of the bile duct, which can be inflammatory, congenital, or neoplastic. Different types of biliary diseases are explained below.

Medically reviewed by

Dr. Ghulam Fareed

Published At January 23, 2023
Reviewed AtJuly 11, 2023

Introduction:

Diseases that affect the bile duct (the tract that carries bile from the liver to the gallbladder and the intestine) are called biliary diseases. They can be congenital, such as choledochal cysts, inflammatory such as cholangitis, and malignancy, like adenoma biliary papillomatosis.

What Are the Different Types of Biliary Diseases?

1. Choledocholithiasis: It occurs when gallstones block the bile duct leading to an obstruction in the bile flow. The stones can have near-water, fat, or soft-tissue attenuation with peripheral calcification. The stones can be detected on CT (computed tomography) scan, however, ERCP (endoscopic retrograde cholangiopancreatography) is considered highly accurate for detecting small choledocholithiasis.

The symptoms include jaundice, clay-colored stools, and dark urine. The stones also block the enzymes from the pancreas leading to gallstone pancreatitis, which can cause left upper abdominal pain, vomiting, and nausea. The therapeutic procedure for choledocholithiasis is ERCP.

2. Choledochal Cyst: It involves cystic dilatation of any part of extrahepatic bile ducts. Most cases are diagnosed in childhood, and about 20 % manifest in adults. Choledochal cysts are classified by Todani modification of the Alonso-Lej classification, which is based on the morphological changes in the bile ducts:

  • Todani Type I: A single cystic dilatation of the extrahepatic bile duct (EBD).

  • Todani Type II: True diverticula of the extrahepatic bile duct seen.

  • Todani Type III: Choledocholocele or astasia of an intramural extrahepatic bile duct.

  • Todani Type IV: Multiple cystic dilatations with extrahepatic and intrahepatic components.

  • Todani Type V (Caroli Disease): Cystic dilatation of only intrahepatic bile ducts (IBD).

The choledochal cyst can lead to complications such as stone formation, cholangitis, liver abscess, bile peritonitis, and cholangiocarcinoma.

3. Cholangitis: It is the inflammation of the bile duct system, which can be either bacterial or sudden. It can also be acute (short-length) or chronic (long-term infection). The different types of cholangitis are:

  • Recurrent Pyogenic Cholangitis: Characterized by strictures of the peripheral ducts with reduced branching and abrupt tapering, giving an “arrowhead appearance” with excessive dilatation of the extrahepatic and central bile ducts. Sonography, CT (computed tomography), and MRCP (Magnetic resonance cholangiopancreatography) aid in the diagnosis of the disease. They also help to intervene the pathway of infection.

  • Suppurative Cholangitis: It is a potentially life-threatening infection that arises when bile duct obstruction is combined with bacterial contamination. Approximately 80 % of cases of suppurative cholangitis have undrerlying choledocholithiasis. Marked liver parenchymal enhancement is observed in the arterial-phase in patients with acute inflammation. CT or MRI helps to diagnose the inflammation.

  • IgG4- Related Cholangitis: Infiltration by abundant IgG4- positive plasma cells causes this type of cholangitis. Unlike primary sclerosing cholangitis, multifunctional strictures in IgG4-related sclerosing cholangitis are long, continuous, and associated with restenotic dilatation.

  • Primary Sclerosing Cholangitis: It is a progressive disease showing inflammation and fibrosis of the bile duct and is associated with inflammatory bowel disease. The diagnosis can be made by cholangiographic findings, which include diffuse, multifocal strictures, which are short-segmented with mild dilatation in the intrahepatic and extrahepatic bile ducts. It gives a “beaded appearance” in some cases.

4. Neoplasms: They can be either benign or malignant tumors developing in the bile duct.

  • Benign Tumors: Some of the common benign tumors are:

  1. Hamartoma: It is also known as the Meyenburg complex. It manifests as innumerable cysts, typically less than 1.5 cm in size and is distributed on both lobes of the liver.

  2. Biliary Cystadenoma (Biliary Mucinous Cystic Neoplasm): These are rare, multilocular cystic tumors of biliary origin. They appear to have cystic content in a well-marginated capsule along with internal septa, and mural nodules. Malignant cystadenocarcinoma resembles cystadenomas, but the former tends to have a thicker internal septum and contain polypoid projections.

  3. Intraductal Papillary Neoplasm: Intraductal papillary neoplasm of the bile duct is a premalignant tumor that can progress to carcinoma. Imaging features show intraductal tumors and bile duct dilatation, which is caused by mucin secretion. Mucin hypersecretion causes expansion of the ampulla of Vater.

  • Malignant Tumors: Cholangiocarcinoma (CC) is an adenocarcinoma of bile ducts. According to the classification proposed by the Liver Cancer Study Group of Japan (LCSGJ) based on macroscopic growth patterns, cholangiocarcinoma can be classified as:

  1. Mass-Forming: It manifests as a large non-encapsulated mass of irregular contour, which shows early peripheral rim enhancement.

  2. Periductal-Infiltrating: Present as a narrowed perihilar duct with irregular wall thickening showing delayed enhancement and upstream bile duct dilatation.

  3. Intraductal Growing: It appears as a papillary or polypoid tumor within the bile duct lumen and has superficial mucosal growth patterns.

Bismuth-Corvette System of Classification: Depending on the positions and areas involved, the tumors are classified as:

  • Type I: Tumor involves only the common hepatic duct below the first junction.

  • Type II: Tumor involves the first confluence but without the second one.

  • Type III: Tumor involving either of the one confluences.

  • Type III-A: Involves the right confluences.

  • Type III B: Involves left confluences.

  • Type IV: Tumor involves both confluences.

What Are the Symptoms of Biliary Diseases?

There are a few symptoms that can be common to all biliary dysfunctions:

  • Jaundice: Yellowing the skin and whites of the eyes (sclera).

  • Abdominal pain, particularly in the upper right side of the abdomen below the rib cage.

  • Loss of appetite leads to weight loss.

  • Nausea or vomiting.

  • Fatigue and weakness.

  • Fever or chills.

  • Light brown-colored urine.

  • Clay-colored stools.

What Are the Treatment Options for Biliary Diseases?

  • Medication: The prescribed medication will treat the bile obstruction and infections by increasing the bile flow and getting rid of the infections.

  • Hepatoportoenterostomy: It is a surgical procedure that drains bile from the liver when the bile duct is obstructed.

  • Endoscopic Retrograde Cholangiopancreatography: This is a diagnostic and therapeutic procedure. It helps to identify as well as remove gallstones from the bile duct.

  • Cholecystectomy: It is a surgical procedure for the removal of gallbladder through single incision laparoscopy.

  • Resection: It is done for cases with neoplastic tumors; surgical resection followed by chemotherapy and radiotherapy is the treatment choice. In cases of neoplasms, the treatment plan depends on the staging of the tumor.

Conclusion:

Biliary diseases affect the bile duct, which carries bile from the liver to the gallbladder and intestine. Most biliary diseases cause bile obstruction, which will lead symptoms like abdominal pain, nausea, vomiting, and jaundice. Since the disease can be congenital, inflammatory, and neoplastic, the treatment also varies according to its nature. Some diseases, like benign or malignant tumors, can be life-threatening, so early diagnosis and treatment are essential.

Dr. Ghulam Fareed
Dr. Ghulam Fareed

Medical Gastroenterology

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biliary diseases
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