Biliary Hamartoma

 a.k.a. von Meyenburg complex


image Asymptomatic and of no clinical concern





IMAGING




• Multiple, near water density/intensity liver lesions < 15 mm in diameter
image Varied enhancement based on cystic and solid components

image No communication with biliary tree

• US: Small and well-circumscribed lesions
image Often have echogenic walls with small fluid content

• US shows much more echogenicity and fewer cystic lesions than anticipated based on prior CT or MR


TOP DIFFERENTIAL DIAGNOSES




• Autosomal dominant polycystic liver disease
image Larger, more numerous cysts in liver and other organs

• Multiple simple hepatic cysts
image Fewer cysts of varying size; no mural nodules

• Caroli disease
image Central dot sign on CECT and MR

image ERCP and MRCP: Communicating bile duct abnormality

• Multiple/solitary, small metastatic lesions
image More complex and varied in size

• Opportunistic infection (microabscesses)
image Must be considered in immunosuppressed patient with fever


DIAGNOSTIC CHECKLIST




• No further evaluation needed when seen as isolated finding in healthy, nononcologic patient

• Should be considered as likely diagnosis in setting of innumerable small, slightly complex “cysts” in healthy patient

• Lesions appear more echogenic than expected on sonography

image
(Left) Axial T2WI MR shows innumerable tiny bright foci throughout the liver image, representing biliary hamartomas. This patient also had evidence of congenital hepatic fibrosis on imaging and liver biopsy, both part of the congenital hepatic and renal fibropolycystic disease spectrum.


image
(Right) MRCP shows small spherical cyst-like lesions image that do not communicate with the (normal) biliary tree. This feature helps to distinguish biliary hamartomas from Caroli disease.

image
(Left) Sonographic image shows innumerable tiny echogenic foci image throughout the liver and 1 of ∼ 10 cyst-like lesions image, though even these have small foci of echogenicity within the wall. MR on this patient showed many more cystic-appearing biliary hamartomas.


image
(Right) The branching, angulated glands in biliary hamartomas are lined by a single layer of flattened cuboidal epithelium. These glands may expand or rupture to produce small “cysts.” There is no nuclear atypia. (Courtesy S. Kakar, MD.)


TERMINOLOGY


Synonyms




• Bile duct microhamartoma

• von Meyenburg complex


Definitions




• Uncommon benign malformations of biliary tract


IMAGING


General Features




• Best diagnostic clue
image Multiple near water density/intensity liver lesions < 15 mm in diameter

• Location
image Subcapsular or intraparenchymal in location

image Scattered throughout both lobes of liver

• Size

• Irregular spherical

• Usually multiple to innumerable


CT Findings




• NECT
image Density of lesions depends on predominance of cystic or solid component
– Predominantly cystic: Water density

– Predominantly solid (fibrous stroma): Soft tissue attenuation

• CECT
image Cystic components remain near water density
– No enhancement of contents

image Solid (fibrous stroma) components enhance
– Usually become nearly isodense to liver

image Punctate calcifications may be seen


MR Findings




• T1WI
image Hypointense (both cystic and solid lesions)

• T2WI
image Very hyperintense (cystic components)

image Intermediate intensity (solid lesions)

image Heavily T2WI: Remain hyperintense (equal to fluid)
– 

• T1WI C+
image No enhancement of cystic components
– ± nodular enhancement of fibrous nodules

• MR cholangiography (MRC)
image Markedly hyperintense lesions

image No communication with biliary tree


Ultrasonographic Findings




• Grayscale ultrasound
image Multiple small and well-circumscribed lesions

image Fibrotic parts of lesions are very echogenic
– Reflect solid and cystic components

– Fluid or cystic component is sonolucent
image ± posterior acoustic enhancement

– US shows much more echogenicity and fewer cystic lesions than anticipated based on prior CT or MR

Nov 16, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Biliary Hamartoma

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