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TUBEROZ SKLEROZLU BİR OLGUDA KARDİYAK RABDOMYOM

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Abstract (2. Language): 
Cardiac rhabdomyoma in a case with tuberous sclerosis. Tuberous sclerosis (TS) is a mul-tisystem disease, involving primarily skin, brain, kidneys, heart and eyes. The most frequent cardiac tumour is rhabdomyoma. TS was considered in our patient in whom intracranial vent-ricular asymmetry and intracardiac masses were observed in fetal ultrasonography performed in the 3İst week of the pregnancy. Since one of the intracardiac obstruction in outlet of the left ventricle, fetal echocardiography was repeated every two weeks. The diagnosis of TS was es-tablished by cortical tubers, subependimal nodules and linear white matter lesions in postnatal magnetic resonance screening.
Abstract (Original Language): 
Tüberoz skleroz (TS) başlıca deri, beyin, böbrek, kalp ve gözü tutan multisistemik bir hastalıktır. En sık rastlanan kardiyak tümör rabdomiyomlardır. Otuzbirinci gebelik haftasında yapılan fetal ultrasonografisinde intrakraniyal ventriküler asimetri ve intrakardiyak kitleler saptanan vakada TS düşünüldü. İntrakardiyak kitlelerden birisi sol ventrikül çıkış yolunda minimal obs-trüksiyona neden olduğu için iki haftalık aralarla fetal ekokardiyografi tekrarlandı. Postnatal manyetik rezonans görüntülemedeki kortikal tüberler, subependimal nodüller ve lineer ak madde lezyonları ile TS tanısı kesinleşti. Seri ekokardiografik kontrollerde rabdomiyomların küçüldüğü ve obstrüksiyonun azaldığı görüldüğü için cerrahi yaklaşım düşünülmedi. Böyle hastalarda kitlelerin tedavisinde agresif davranılmayıp, dikkatle izlemenin uygun olacağı kanısına varıldı.
102-104

REFERENCES

References: 

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