What are bile duct hamartomas?

What are bile duct hamartomas?

Biliary duct hamartomas, otherwise known as Von Meyenburg complexes, are dilated cystic bile ducts, which are embryologic remnants that failed to involute during the period of embryogenesis. They are typically small (less than 5mm) and present as multiple lesions scattered throughout the liver.

What causes biliary hamartoma?

Biliary hamartomas are congenital bile duct malformations that contain cystic dilated bile ducts surrounded by fibrous stroma and occur due to failure of embryonic involution. They are also called Von Meyenburg complexes (VMC’s) named after the person who first described them.

What is Von Meyenburg complex?

Von Meyenburg Complexes (VMCs) is a rare clinicopathologic entity, consisting of small (<1.5cm), usually multiple and nodular cystic lesions. VMCs typically cause no symptoms or disturbances in liver function and thus in most instances they are diagnosed incidentally.

What makes up the biliary tree?

The biliary tree (also known as the biliary tract or biliary system) is comprised of the gallbladder, liver and bile ducts. These structures produce, store, and release bile to the small intestines, where it aids in the digestion of lipids.

Do hamartomas grow?

Hamartomas are noncancerous growths that can appear anywhere on the body. While seen as harmless, these benign tumors can grow to large sizes and cause pressure on surrounding tissues. Depending on where they grow externally or internally, hamartomas can cause life-threatening symptoms.

What is congenital hepatic fibrosis?

Congenital hepatic fibrosis (CHF) is an inherited disease that both the liver and kidneys. The condition can cause the liver, kidneys and bile ducts to form abnormally before birth and may also cause fibrosis and scarring on the liver.

What are the symptoms of cysts on the liver?

Symptoms of liver cysts can include:

• distended or protruding stomach.
• feelings of abdominal fullness or bloating.
• abdominal pain, particularly in the upper right quadrant.
• heartburn.
• nausea and vomiting.
• shoulder pain.

What is VMC in liver?

Multiple bile duct hamartoma [von Meyenburg complex (VMC)] is a benign liver malformation that includes biliary cystic lesions with congenital hepatic fibrosis causing ductal plate malformations (1–7). It is generally asymptomatic and tends to be identified either at autopsy or during histological examinations (1).

What are the common signs and symptoms of biliary disease?

Symptoms of possible biliary disease

• Jaundice (yellowing of the skin and whites of the eyes)
• Abdominal pain, especially in the upper right side of the abdomen under the rib cage.
• Nausea or vomiting.
• Loss of appetite, which may result in weight loss.
• Fatigue.
• Fever or chills.
• Itching.
• Light brown urine.

What are the symptoms of bile duct problems?

Symptoms

• Abdominal pain in the upper right side.
• Dark urine.
• Fever.
• Itching.
• Jaundice (yellow skin color)
• Nausea and vomiting.
• Pale-colored stools.

Should hamartoma be removed?

Surgical excision is the definitive treatment. Hamartomas have the same malignant potential as normal breast tissue, unless greater than 6 cm, and thus do not require any additional treatment than one would for adenoma or other benign lesions.

How do you get rid of hamartoma?

If you begin to experience seizures, doctors may prescribe anticonvulsants to reduce episodes. If you don’t respond to medication, surgical removal of the hamartoma may be required. However, surgery is an invasive procedure that can cause life-threatening complications, depending on the size and location of the growth.

What are the treatment options for bile duct hamartomas?

Bile duct hamartomas are benign lesions, with no malignant potential. No specific treatment is necessary for these lesions. Matthew M. Yeh MD, PhD, in Practical Hepatic Pathology: a Diagnostic Approach (Second Edition), 2018

How is bile duct hamartoma (BDH) diagnosed on MRI?

On MRI, BDH have low signal intensity on T2-weighted images and high signal intensity on T2-weighted images. Bile duct hamartomas need to be distinguished from malignant neoplasms in the liver such as metastatic adenocarcinoma or cholangiocarcinoma (CC), especially during frozen section consultation.

What is bile duct hamartoma ( von Meyenburg complex)?

LINDA D. FERRELL, in Surgical Pathology of the GI Tract, Liver, Biliary Tract, and Pancreas (Second Edition), 2009 Bile duct hamartoma, also termed von Meyenburg complex, is a type of ductal plate malformation. These lesions often occur as part of the spectrum of polycystic disease of the liver or other organs.

What is multifocality in bile duct hamartomas?

Multifocality is common. Microscopically, bile duct hamartomas consist of numerous small to medium-sized ductules, which are typically more dilated than normal ducts and usually separated by dense collagen ( Fig. 47-18 ). These lesions are usually located in, and at the periphery of, portal tracts.