Black aorta in a patient with alkaptonuric ochronosis

Abstract

Tedavide, aynı seansta sol ana karotid arter bifürkasyonundan sol internal karotid artere uzanan endarterektomi ve 8 mm ringli greft kullanılarak sol karotikosubklavyen baypas yapıldı. Hasta ameliyattan sonra problemsiz olarak dördüncü gün taburcu edildi.Spinning Wheels (çıkrık) sendromu, koroner subklavyen çalma sendromunun değişik bir formu olarak farklı anatomik görünümlerde karşımıza çıkabilir. Proksimal subklavyen arter darlığı ve KABG'nin birlikte bulunması nadir olmasına rağmen, KABG planlanan hastalarda aortik arkın görüntülenmesi olası morbiditeleri önleyebilir. Black aorta in a patient with alkaptonuric ochronosis Alkaptonürik okronozis'li bir hastada siyah aort Alcaptonuria (black urine disease) is a rare inherited genetic disorder of phenylalanine and tyrosine metabolism with hiperpigmentation of skin, sclera, cartilages, degenerative ochronic arthropathies. Other important consequences of alcaptonuric ochronosis are aortic valve stenosis and urinary tract involvement.A 62-year-old male patient with unknown alcaptonuria was admitted to our institution (Fig. 1, Typical dark spots on the sclera and a dark skin lesion and collected urine was black). Echocardiography showed aortic stenosis with a mean gradient of 55 mmHg. Coronary angiography demonstrated 2-Vessel-Disease.Aortotomy revealed typical ochronotic pigmentation of a severely calcified aortic valve and aortic intima. The patient underwent aortic valve replacement with a 25-mm aortic valve prosthesis and the left internal mammarian to the left anterior descending artery and single venous to the right coronary artery bypass grafting (Fig. 2, 3).The postoperative course was uneventful. The patient was discharged on the fifth postoperative day.This extremely rare illness, detected at the time of surgery, has only been described in few papers in the cardiovascular surgery.From the anesthesiologist's point of view, there is a severe risk of difficult airway because of an advanced stiffness of the cervical spine and a reduced mouth opening in these patients.From the surgeon's point of view, there is more risk during cannulation of the ascending aorta, because the discolored aortic lesions are usually more mobile than in other patients. In addition, after opening the aorta and resection of the calcified aortic valve, some black calcium particles can move into the left ventricle cavity and be invisible, so that there is more emboli risk than in other patients.