Laparoscopic Findings in Hereditary Haemorrhagic Telangiectasia (Osler-Weber-Rendu Disease)

Summary

The laparoscopic findings observed in eight patients with hereditary haemorrhagic telangiectasia are reported. Clinical signs or laboratory data suggestive of liver involvement were present in all cases and constituted the main indication for laparoscopic examination. Characteristic vascular lesions distributed at random were found in four cases. Numerous oval, round or polygonal areas of 0.5 to 1.5 cm in diameter were observed on the external surface of the liver. They were of a reddish or pink colour, flat or slightly raised, and made up of a dense vascular meshwork. In one patient macronodular cirrhosis was associated with the vascular lesions. In the remaining four cases laparoscopic examination did not show striking vascular changes, although the liver biopsy revealed a fatty liver in three cases and micronodular cirrhosis with intense inflammatory activity, steatosis and Mallory bodies in one case. In these cases the accompanying hepatic lesions could probably not be aetio-pathogenically related to Osier's disease.

Key words:

Laparoscopy - Telangiectasia - Osier's disease