Pol. Merkur. Lek (Pol. Med. J.), 2011, XXXI/181: 045-047 Maximize

Pol. Merkur. Lek (Pol. Med. J.), 2011, XXXI/181: 045-047

Title: Excision of the rectum due to carcinoma in patient with hereditary hemorrhagic teleangiectasia

Authors: Gajda S., Szczepanik A.M., Misiak A., Szczepanik A.B.

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Excision of the rectum due to carcinoma in patient with hereditary hemorrhagic teleangiectasia

Gajda S.1, Szczepanik A.M.2, Misiak A.1, Szczepanik A.B.1

1Institute of Hematology and Transfusion Medicine in Warsaw, Poland, Department of General and Hematological Surgery; 2Praski Municipal Hospital in Warsaw, Poland, Second Department of Internal Medicine

Hereditary hemorrhagic telangiectasia (HHT) also known as Rendu-Osler-Weber disease, is a rare, autosomal dominant disorder of the fibrovascular tissue. Clinically, it is characterized by the triad of symptoms of mucocutaneous telangiectasias and arteriovenous malformations of visceral organs, recurrent hemorrhages from vascular changes at different localization, and familial occurrence. A coexistence of HHT syndrome and malignant neoplasms in various sites, including large bowel, is suggested. The authors describe the case of 67-year old woman with HHT with massive bleeding from digestive tract in history and persistent chronic sideropenic anemia. In upper gastrointestinal endoscopy performed in regional hospital multiple telangiectasias in the stomach were found. These were diagnosed as the possible bleeding source therefore colonoscopy was not performed. After twelve months in diagnostic colonoscopy due to chronic anemia carried out in our Institute, rectal carcinoma was found. The patient were subjected to surgery – lower anterior excision of the rectum. Intra operatively and in the postoperative period complications were not observed.
Conclusion. In every case of bleeding from digestive tract and also in chronic anemia in patients suffering from Rendu-Osler-Weber disease, beside the endoscopy of esophagus, stomach and duodenum, full visualization of the large bowel is necessary to exclude malignant neoplasm. In the presented case no colonoscopy performed in the regional hospital delayed the carcinoma diagnosis by twelve months.

Key words: hereditary hemorrhagic telangiectasia, rectal carcinoma, rectal excision

Pol. Merk. Lek., 2011, XXXI, 181, 45