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Abdomen » Hepatobiliary
Biliary hamartomas (von Meyenburg complexes)
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Author(s) :
Danilo Sirigu, MD
 
Presentation A 23 year old woman presents with abdominal pain in right iliac fossa with suspected acute appendicitis. Ultrasound scan is negative for appendicitis but reveals a liver abnormality.

Paziente di 23 anni esegue esame ecografico per dolore addominale in fossa iliaca destra da sospetta appendicite acuta.
 
 
Caption: Video 1
Description: The ultrasound examination performed with a 4 MHz convex probe provides only a vague suggestion of many small areas of increased echogenicity within the liver. (Video 1) L’esame ecografico , eseguito con sonda convex 4 MhZ non evidenzia alcuna patologia.( Video 1 )
 
 
Caption: Video 2
Description: Using a 7.5 MHz linear probe ( Video 2 ) , multiple small hyperechoic nodular lesions are evident in the liver parenchyma, consistent with a diagnosis of biliary hamartomas. There is no evidence of dilated bile ducts. Utilizzando una sonda lineare 7.5 MhZ , ( Video 2 ) si evidenzia invece nel parenchima epatico, la presenza di numerose millimetriche formazioni iperecogene diffuse , compatibili con una condizione di amartomatosi biliare , confermata da successiva valutazione RM. Non dilatazione delle vie biliari.
 
 
Caption: Video 3. Color Doppler
Description: Vascularization of the liver appears normal. Non alterazioni della vascolarizzazione. ( Video 3 )
 
Differential Diagnosis multiple liver metastases
multiple liver microabscesses
hepatic candidiasis
 
Final Diagnosis Multiple biliary hamartomas

L’ amartomatosi biliare multipla
 
Discussion
Biliary hamartomas were first described by Von Meyenburg in 1918 and are referred to as Von Meyenburg complexes. Other descriptions used for this entity include multiple bile duct hamartomas and biliary microhamartomas

Biliary hamartomas (BH ) are uncommon benign biliary malformations composed of groups of dilated intrahepatic bile ducts embedded within a dense collagenous stroma.

L’ amartomatosi biliare multipla, conosciuta anche come Complesso di Von Meyenburg, è una rara malformazione benigna del fegato . La patologia è asintomatica ed in genere si tratta di riscontri occasionali o autoptici.
Dal punto di vista macroscopico Il complesso di Von Meyenburg è caratterizzato dalla presenza di multiple formazioni nodulari di colore bianco-grigiastro diffuse nel parenchima epatico e sottoglissoniane di dimensioni variabili tra pochi mm e 1,5 cm., che corrispondono istologicamente ad una proliferazione periportale dei dotti biliari con lume ampio , ripieno di materiale mucoso , e circondati da tessuto fibroso.
 
Case References
  1. H. von Meyenburg. Über die Zyztenleber. Beiträge zur pathologischen Anatomie und zur allgemeinen Pathologie, Jena, 1918, 64: 477-532.
  2. Saul 5H. Neoplasms of liver. In: Sternberg 55, ed. Diagnostic surgical pathology. New York: Raven, 1989:1182-1183
  3. Cotran RS, Kumar V, Collins T et-al. Robbins pathologic basis of disease. W.B. Saunders Company. (1999) ISBN:072167335X.
  4. Zheng RQ, Zhang B, Kudo M et-al. Imaging findings of biliary hamartomas. World J. Gastroenterol. 2005;11 (40): 6354-9.
  5. Lev-Toaff, AS, Bach, AM, Wechsler, RJ, Hilpert, PL, Gatalica, Z, Rubin, R. The radiologic and pathologic spectrum of biliary hamartomas. Am. J. Roentgenol. 1995 165: 309-313
Recommended additional information sources:
     http://radiopaedia.org/articles/multiple-biliary-hamartomas-1
    
     http://www.wjgnet.com/1007-9327/11/6354.pdf   (immediate download of the Zheng, Zhang and Kudo paper (2meg))
          This article is especially useful because it presents the rather wide spectrum of ultrasound findings associated with
          biliary hamartomas.  The ultrasound findings have been described as hypoechoic, hyperechoic or mixed heterogenic
          echoic structures and the frank cystic form may exhibit comet tail artifacts deep to the lesions.
    
 
Technical Details Many thanks to case Author and contributor Dott Danilo Sirigu.   His outstanding collection of video cases may be viewed at the ECOMOVIES web site  http://www.ecomovies.it/Default.asp?lang=2

 
Follow Up Diagnosis was confirmed by MRI.
 
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