HEMANGIOMATOSIS OF THE LIVER IN HEREDITAR Y HEMORRHAGIC TELANGIECTASIA (OSLER-WEBER-RENDU DISEASE)

Heart failure occurred in a 50-year-old woman as a result of calcified haemangiomatosis of the liver with a high shunt volume. In the subseq uent years there were several bleedings from peptic ulcers. Ultrasonog raphy revealed an increase in liver size and the previously diagnosed calcification. 14 years later the patient was again hospitalized becau se of increasing weekness and stress dyspnoea; the haemoglobin level w as 5.5 g/dl. In addition to the florid gastric and duodenal ulcers, an giodysplasias were for the first time demonstrated in the stomach, duo denum and sigmoid colon, as were telangiectasias in the face and echo- dense round foci in the spleen. After transfusion of red cell concentr ates and healing of the peptic ulcers under a 14-day treatment with om eprazole (20 mg two times daily by mouth) and amoxycillin (750 mg thre e times daily by mouth) the haemoglobin level increased at first (10 g /dl), then tarry stools were once again noted. The condition stabilize d after laser coagulation of all accessible gastrointestinal angiodysp lasias. The isolated calcified hepatic haemangiomatosis, diagnosed 14 years previously, is most likely a rare variant of hereditary haemorrh agic telangiectasia (Osler-Weber-Rendu disease).