VIDEO:
Ultrasound : non homogeneous hepatic parenchymal echostructure, characterised by the presence of multiple hypoechoic nodular masses with a maximum diameter of 1 cm.
VIDEO CT :
Contrast-enhanced CT of the liver: multiple hypodense and nonenhancing lesions distributed throughout the liver without a distinctive distribution pattern.
CLINICAL CASES
PRESENTATION
Biliary hamartomas were first described by von Meyenburg in 1918 and are referred to as Von Meyenburg complexes. Biliary hamartomas (BH ) are uncommon benign biliary malformations composed of groups of dilated intrahepatic bile ducts embedded within a dense collagenous stroma.
The pathogenesis of this malformation is unknown. Some authors believe it results from the altered evolution of the embryonic bile ducts, some malformation is involved in the genesis of biliari hamartomas : congenital hepatic fibrosis, and liver cysts in ADPKD, and Caroli disease.
The major clinical importance of BH is the propensity for misdiagnoses as multiple metastases, microabscesses, C. albicans, and extrapulmonary P. carinii infections.
On sonography, BH have been described as innumerable tiny hypoechoic or hyperechoic foci measuring less than 10 mm and distributed uniformly throughout the liver .
Differences in echogenicity may be due to the size of the dilated bile duct component, which, at a certain size, would behave like other microcystic structures and show echogenicity. Below this size, their complex internal structure might attenuate the echo signal.
On CT, BH are depicted as multiple round, small hypoattenuating and nonenhancing areas distributed throughout the liver without a distinctive distribution pattern.
As regards the prognosis is described in literature the possibility of a transformation into cholangiocarcinoma.
FINAL DIAGNOSIS :
Bile Dcuct Hamartomas:Von Meyenburg complexes
REFFERENCES :
1) Chung EB: Multiple bile-duct hamartomas.Cancer 96: 26-287, 1970.
2) Thommesen N: Biliary hamartomas (Von Meyenburg complexes) in liver needle biopsies. Acta Pathol Microbiol Immun Scand 9: 86-93, 1978.
3) Burns CD, KHuhns JG, Wieman TJ:Cholangiocarcinoma in association with multiple biliary microhamartomas. Arch Pathol Lab Med 114: 1287-1289, 1990.
4) Eisenberg D, Hurwitz L, Yu AC: CT and sonography of multiple bile-duct hamartomas simulating malignant liver disease (case report). AJR 147: 279-280,1986.
5) Martinoli C, Cittadini G, Rollandi GA et al: Case report: imaging of bile-duct hamartomas. Clin Radiol 45: 203-205,1992. 6) Tan A, Shen J, Hect A: Sonogram of multiple biliary hamartomas.
J Clin Ultrasound 17: 667-669, 1989.
Bile Dcuct Hamartomas:Von Meyenburg complexes
Autori:
Dr Danilo Sirigu *;
* Servizio di Radiologia, Azienda Ospedaliera Brotzu, Cagliari