Zespół wad wrodzonych ze złożoną wadą serca i zaburzeniami krzepnięcia sugerujący spektrum oczno-uszno-kręgowe – opis przypadku (original) (raw)

Pediatria Polska, 2015

Abstract

Abstract Oculo-auriculo-vertebral spectrum (OAVS) is a multisystem developmental anomaly that affects one in every 5600–26,500 births. The case of a 13-month-old girl with typical symptoms of OAVS and successfully corrected coexistent double outlet right ventricle with outflow tract obstruction is presented. Additionally deficit of VII, IX and XII coagulation factors was diagnosed prior to surgery as well as hemiparesis, right kidney agenesis and cleft palate. The authors stress the need for detailed and extended coagulologic laboratory examinations to precisely calculate operative risk and to establish appropriate therapeutic protocol.

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