What are biliary hamartomas?
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 liver hamartoma?
Mesenchymal hamartoma of the liver is a benign tumor of infancy characterized by an admixture of epithelial structures in a loose connective tissue stroma with fluid accumulation suggestive of lymphangiomatous channels.
What is Von Meyenburg disease?
von Meyenburg complex is a common type of liver tumour seen in approximately 3-6% of the population. Some may appear before birth during the time in fetal development when the liver and bile ducts are first forming. Most bile duct hamartomas are called sporadic because they have no known cause.
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?
Pulmonary hamartomas grow slowly, and most of them are smaller than 4 cm, although they may reach 10 cm in diameter. The tumors are usually solitary, although multiple tumors in the Carney triad have been reported.
Is polycystic liver disease curable?
The only definitive treatment of PLD, used in only the most severe cases, is liver transplant [2, 8, 33]. Medication to slow down cyst growth and fluid secretion in the liver (somatostatin analogs, namely octreotide and lanreotide) is also useful in reducing liver volume [7, 10].
How common is Alagille syndrome?
The estimated prevalence of Alagille syndrome is 1 in 70,000 newborns. This figure is based on diagnoses of liver disease in infants, and may be an underestimation because some people with Alagille syndrome do not develop liver disease during infancy.
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).
Is biliary atresia congenital?
Biliary atresia is a blockage in the tubes (ducts) that carry bile from the liver to the gallbladder. This congenital condition occurs when the bile ducts inside or outside the liver do not develop normally. It is not known why the biliary system fails to develop normally.
What will happen if the bile duct is blocked?
When the bile ducts become blocked, bile builds up in the liver, and jaundice (yellow color of the skin) develops due to the increasing level of bilirubin in the blood. The possible causes of a blocked bile duct include: Cysts of the common bile duct.
Do hamartomas cause pain?
Splenic hamartomas, while rare, trigger symptoms in more women than men. Hamartomas found on the spleen can cause pain and discomfort in the abdominal region.
How common are hamartomas?
Incidence. Many people have never heard of hamartomas, but they are relatively common tumors. Lung hamartomas are the most common type of benign lung tumor, and benign lung tumors are relatively common. 6 Breast hamartomas are responsible for roughly 5 percent of benign breast masses.
What is a biliary duct hamartoma?
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 are the long-term consequences of multiple biliary hamartomas (mbH)?
Aside from the possible risk of malignant transformation (see Associations above), multiple biliary hamartomas are a benign asymptomatic condition with no long-term consequences, and no treatment is required.
What are the signs and symptoms of a basal biliary hamartoma?
Biliary hamartomas are hypoattenuating and often show no enhancement 7. Occasionally an enhancing nodule or rim may be identified in a small number of lesions. Only a limited number of the many lesions that are actually present can be seen as grape-like clusters of abnormal vascularity with contrast persisting into the venous phase 10.
Who first described the pathology of biliary hamartomas?
Hans von Meyenburg (1887-1971) was a German-born Swiss pathologist, who first described the pathology of biliary hamartomas in an article published in 1918 13,16 . General imaging differential considerations include: 1. Chung EB. Multiple bile-duct hamartomas.