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[Liver hemangiomatosis in Osler's disease]
M Selmaier1, K Cidlinsky, C Ell
1Medizinische Klinik I mit Poliklinik, Universität Erlangen-Nürnberg.
Insights
This case study highlights a rare variant of hereditary hemorrhagic telangiectasia presenting as calcified hepatic hemangiomatosis. Early diagnosis and targeted treatment of gastrointestinal angiodysplasias are crucial for managing bleeding complications.
Area of Science:
- Gastroenterology
- Hepatology
- Genetics
Background:
- Calcified hepatic hemangiomatosis, a rare liver condition, can present with significant shunt volumes leading to heart failure.
- Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) is a genetic disorder characterized by abnormal blood vessel formation, often leading to bleeding complications.
Abstract:
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 subsequent years there were several bleedings from peptic ulcers. Ultrasonography revealed an increase in liver size and the previously diagnosed calcification. 14 years later the patient was again hospitalized because of increasing weakness and stress dyspnoea; the haemoglobin level was 5.5 g/dl. In addition to the florid gastric and duodenal ulcers, angiodysplasias were for the first time demonstrated in the stomach, duodenum and sigmoid colon, as were telangiectasias in the face and echo-dense round foci in the spleen. After transfusion of red cell concentrates and healing of the peptic ulcers under a 14-day treatment with omeprazole (20 mg two times daily by mouth) and amoxycillin (750 mg three times daily by mouth) the haemoglobin level increased at first (10 g/dl), then tarry stools were once again noted. The condition stabilized after laser coagulation of all accessible gastrointestinal angiodysplasias. The isolated calcified hepatic haemangiomatosis, diagnosed 14 years previously, is most likely a rare variant of hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease).