COPYRIGHT: G. Brancatelli

Transcription

1 Fibroysti liver diseases Giuseppe Branatelli University of Palermo

2 Fibroysti liver diseases - definition Group of related lesions of liver and biliary trat Caused by abnormal development of embryologi dutal plate

3 Fibroysti liver diseases - embriology Dutal plate: a ylindrial layer of ells surrounds the portal vein branhes during 1 st week of gestation Bile duts are formed by partial involution + remodelling of the dutal plate Anomalies may develop at various stages of remodelling: Timing or stage determines the linio pathologial disorder

4 Fibroysti liver diseases Small intrahepati bile duts 10% Congenital hepati fibrosis

5 Congenital hepati fibrosis Clinio-path Results in periportal fibrosis Usually progresses to irrhosis by adolesene (varieal bleeding, splenomegaly)

6 Congenital hepati fibrosis g Small right lobe Atrophy of seg IV Hep C irrhosis (different patient) Enlarged segment IV

7 Congenital hepati fibrosis * * * * Hyperplasti nodules Collateral vessels Hep C irrhosis (different patient) 37-year-old woman

8 Fibroysti liver diseases Small intrahepati bile duts 10% Congenital hepati fibrosis Biliary hamartomas

9 Biliary hamartomas Synonyms: bil. mirohamartomas, von Meyenburg omplexes Clinio-path: Usually multiple small (<15 mm) masses lined by biliary epithelium Rarely symptomati Often mistaken for ysts or metastases

10 Biliary hamartomas 80 y/o woman -asymptomati COPYRIGHT: G. Branatelli

11 Biliary hamartomas Epithelial-lined Contains bile Not in ommuniation with duts

12 Fibroysti liver diseases Small intrahepati bile duts Congenital hepati fibrosis Biliary hamartomas Medium-size intrahepati bile duts 40% Autosomal polyysti liver disease

13 Autosomal Dominant Polyysti Liver Disease Clinio-path: Multiple (innumerable) ysts (few mm to >12 m) May be inherited with or without kidney or other organ involvement Often auses huge hepatomegaly, but rarely affets liver funtion

14 Polyysti liver disease

15 Polyysti liver disease COPYRIGHT: G. Branatelli

16 Fibroysti liver disease Small intrahepati bile duts Congenital hepati fibrosis Biliary hamartomas Medium-size intrahepati bile duts Autosomal polyysti liver disease Large intrahepati bile duts 70% Caroli

17 Caroli s disease Abnormal duts retain ommuniation with biliary tree Often assoiated with hepati fibrosis Often develop intrahepati aluli Often present with holangitis, jaundie Can progress to irrhosis Can develop holangioarinoma

18 Caroli s disease Dot sign COPYRIGHT: G. Branatelli

19 Caroli s disease aluli

20 Caroli s disease Evolution to biliary irrhosis

21 Fibroysti liver disease Small intrahepati bile duts Congenital hepati fibrosis Biliary hamartomas Medium-size intrahepati bile duts Autosomal polyysti liver disease Large intrahepati bile duts Caroli Large extrahepati bile duts 90% Choledoal yst

22 Choledohal yst g Gallbladder Choledohal yst Cysti dut 56-year-old woman

23 Choledohal yst note aberrent entry of CBD into side of pan dut

24 Autosomal Congenital polyysti hepati liver fibrosis disease Caroli Biliary hamartomas Choledoal yst

25 COPYRIGHT: G. Branatelli Fibroysti liver disease (47 y/o woman) Cong Hep Fibrosis +biliary hamartomas +polyysti kidneys

26 25-year-old woman ongenital hepati fibrosis Caroli COPYRIGHT: G. Branatelli