A case report of tuberous sclerosis with cardiac rhabdomyomas diagnosed antenatally by fetal echocardiographyn Case Report

Yıl 2009, Cilt: 44 Sayı: 4, - , 01.12.2009

Dolunay Gürses Ertürk Levent Zülal Ülger Arif Ruhi Özyürek

Öz

Tuberous sclerosis is a multisystem disease involving primarily the skin the brain the kidneys the heart and the eyes The most frequent nbsp; cardiac tumor is rhabdomyoma In our patient who was referred to our hospital due to fetal arrhythmia multiple intracardiac masses were detected by fetal echocardiography performed in the 36th week of pregnancy Cardiac rhabdomyoma was considered and the baby was investigated for tuberous sclerosis The diagnosis of tuberous sclerosis was established by the presence of multiple subependymal calcific nodules and periventricular and subcortical tubers in the postnatal magnetic resonance imaging Since the rhabdomyomas had not caused hemodynamic impairment surgical treatment was not considered The patient was followed up and we observed that rhabdomyomas regressed and arrhythmia decreased without any medical treatment We wanted to point out that the diagnosis of tuberous sclerosis should be considered in cases presenting with intracardiac masses on fetal echocardiography Turk Arch Ped 2009; 44: 146 8 Key words: Fetal echocardiography rhabdomyoma tuberous sclerosis

Anahtar Kelimeler

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Fetal ekokardiyografi ile kalpte rabdomiyomların saptandığı tuberosklerozlu bir olgu sunumu Olgu Sunumu

Öz

Tuberoskleroz başlıca merkezi sinir sistemi deri retina böbrek ve kalp olmak üzere birçok sistemi tutan bir hastalıktır En sık rastlanılan kalp tümörleri rabdomiyomlardır Gebeliğinin 36 haftasında fetal aritmi nedeniyle gönderilen hastamızda yapılan fetal ekokardiyografi ile çok sayıda kalp içi kitle saptanarak kalpte rabdomiyom düşünüldü ve tuberoskleroz açısından araştırıldı Doğum sonrası kraniyal manyetik rezonans görüntülemesinde subepandimal çok sayıda kalsifik nodüller ve beynin beyaz cevherinde periventriküler ve subkortikal tuberlerin gözlenmesi ile tuberoskleroz tanısı kesinleştirildi Rabdomiyomlar hastamızda hemodinamik bozukluğa yol açmadığından cerrahi yaklaşım düşünülmedi İzleminde rabdomiyomların küçüldüğü ve aritmisinin tedavi gerektirmeksizin gerilediği gözlendi Bu olgu sunumu ile fetal ekokardiyografide kalp içi kitle saptanan olgularda tuberoskleroz tanısının düşünülmesi gerektiği vurgulanmak istendi Türk Ped Arş 2009; 44: 146 8 Anahtar kelimeler: Fetal ekokardiyografi rabdomiyom tuberoskleroz

Anahtar Kelimeler

Fetal ekokardiyografi, rabdomiyom, tuberoskleroz

Kaynakça

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