Von Meyenburg Complexes
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A bile duct hamartoma or biliary hamartoma, is a benign tumour-like malformation of the liver. They are classically associated with polycystic liver disease, as may be seen in the context of polycystic kidney disease, and represent a malformation of the liver plate.


Cause

It is a developmental anomaly in which abnormal tissues are present at normal site. Due to failure of regression of embryonic biliary duct.


Diagnosis

File:Histopathology of a bile duct hamartoma, high magnification.jpg, Histopathology of a bile duct hamartoma, high magnification, H&E stain. It shows typical features of bile duct hamartoma: Topic Completed: 23 November 2020. Minor changes: 23 November 2020
- Small to medium sized, irregularly shaped bile ducts lined by bland cuboidal epithelium (may also be flattened).
- Prominent intervening collagenous stroma.
- Bile ducts containing eosinophilic debris (may also contain inspissated bile) File:Von Meyenburg complex cropped.tif, von Meyenburg Complex in ultrasound. Numerous little cysts with ringdown artefacts.
At
CT scan A computed tomography scan (CT scan; formerly called computed axial tomography scan or CAT scan) is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers ...
s, bile duct hamartomas appear as small, well-defined hypo- or isoattenuating masses with little or no enhancement after contrast administration. At MRI, they appear hypointense on T1-weighted images, iso- or slightly hyperintense on T2-weighted images, and hypointense after administration of gadolinium based contrast-agent. On imaging, multiple hamartomas may look similar to metastases or microabscesses.


Treatment

Observation as there is increased risk of cholangiocarcinoma.


Eponym

The eponymous terms ( von Meyenburg complex, ''Meyenburg complex'') are named for
Hanns von Meyenburg Hanns von Meyenburg (actually ''Walter'' but called ''Hanns''; 6 June 1887, in Dresden – 6 November 1971) was a Swiss pathologist. Biography Hanns von Meyenburg was the son of Swiss sculptor Victor von Meyenburg (1834–1893)01384 Viktor von ...
.H. von Meyenburg. Über die Zyztenleber. Beiträge zur pathologischen Anatomie und zur allgemeinen Pathologie, Jena, 1918, 64: 477-532.


Additional images

Image:Bile duct hamartoma intermed mag.jpg, Micrograph of a bile duct hamartoma. Trichrome stain. Intermediate magnification Image:Bile duct hamartoma high mag.jpg, Micrograph of a bile duct hamartoma. Trichrome stain, high magnification Image:Von Meyenburg complex low mag.jpg, Low magnification micrograph of a bile duct hamartoma. Trichrome stain. Image:Von Meyenburg komplex.jpg, Gross pathologic appearance of a large bile duct hamartoma.


See also

* Cholestasis *
Ground glass hepatocyte In liver The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In h ...
* Mallory body


References

Congenital disorders of digestive system {{neoplasm-stub