Sonographic findings in hepatic involvement of hereditary haemorrhagic telangiectasia.
OBJECTIVE
Hereditary haemorrhagic telangiectasia, or Rendu-Osler-Weber disease, is an autosomal-dominant disorder characterised by angiodysplastic lesions (telangiectases and arteriovenous malformations) which affect many organs including the skin, lungs, gastrointestinal tract, and brain. A broad spectrum of vascular and structural changes have been reported. Our objective was to systematically examine the prevalence of sonographic findings in hepatic involvement in patients with hereditary haemorrhagic telangiectasia (HHT).


METHODS
We studied 22 consecutive patients with hereditary haemorrhagic telangiectasia by ultrasonography in combination with colour-Doppler and pulsed wave-Doppler for liver involvement. The clinical diagnosis of HHT was based on the Curaçon criteria.


RESULTS
Sixteen of the 22 patients had signs of hepatic involvement including prominent common hepatic artery (14 of 16), dilatation of the intrahepatic part of the hepatic arteries (15 of 16) and intrahepatic AV-shunts (16 of 16). Ectasia of the hepatic vein, fibrotic parenchymal changes, left accessory hepatic artery and focal hepatic lesions were found less frequently.


CONCLUSIONS
Diagnosis of liver involvement in HHT can be made by sonography with colour-Doppler. The main features of this involvement include prominent common hepatic artery, dilatation of the intrahepatic part of the hepatic arteries and intrahepatic AV-shunts.
Ocran
K
K
Universitätsmedizin Berlin (Campus Charité Mitte) Medizinische Klinik, Abt. Gastroenterologie, Hepatologie und Endokrinologie, Berlin, Germany. ocran@charite.de
Rickes
S
S
Heukamp
I
I
Wermke
W
W
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001166:Arteriovenous Shunt, Surgical / Q000379:methods; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D008107:Liver Diseases / Q000000981:diagnostic imaging* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D015203:Reproducibility of Results; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000000981:diagnostic imaging* / Q000235:genetics; D014465:Ultrasonics; D018615:Ultrasonography, Doppler, Color / Q000379:methods