VATER asosiasyonu: Bir olgu sunumu
Abstract
Özet
VATER asosiasyonu (V: Vertebral anomali, VSD ve diğer kardiyak defektler; A: Anal atrezi veya fistül; T-E: Trakeaözefagial fistül ile özefagus atrezisi; R: Radial displazi, başparmak veya radial hipoplazi, preaksial polidaktili, sindaktili, renal anomali) çeşitli anomaliler ile karekterize olan bir konjenital bozukluktur. Sunulan olguda opere özefagus atrezisi ve trakeaözefagial fistül, opere vestibuler anüs ve anal atrezi, sol el başparmak yokluğu, sol el 5.parmakta klinodaktili, pektus carinatus, skolyoz, dolikosefali, kısa boyun, basık ve geniş burun kökü, kepçe kulak, sakral dimple bulunan 4 yaşında bir kız çocuğu tanımlanmıştır.
Anahtar sözcükler: VATER asosiasyonu, konjenital asosiasyon, vestibüler anüs, anal atrezi
Absract
VATER association, (V: Vertebral anomaly, VSD and other cardiac defects; A: Anal atresia or fistula; T-E: Tracheoesophageal fistula with esofageal atresia; R: Radial dysplasia, thumb or radial hypoplasia, preaxial polidactyly, syndactyly, renal anomaly) is a relatively rare pattern of multiple congenital abnormalities. İn this case, a 4-year-old female child with tracheoesophageal fistula with esophageal atresia, vestibuler anus and anal atresia, left thumb agenesis and clinodactyly in left hand fifth finger, pectus carinatus, scoliosis, short neck, broad and depressed nose, prominent ear and sacral dimple is described.
Keywords: VATER association, congenital association, vestibuler anus, anal atresiaKeywords
Kaynakça
- Quan L, Smith DW. The VATER association. Vertebral defects, Anal atresia, TE fistula with esophageal atresia, Radial and Renal dysplasia: a spectrum of associated defects. J Pediatr 1973; 82: 104-7.
- Jones KL. SMITH’S Recognizable Patterns of Human Malformation. 6th ed. Philadelphia: Elsevier, 2006; pp: 188.
- Nezarati MM, McLeod DR. VACTERL manifestations in two generations of a family. Am J Med Genet 1999; 82: 40-2.
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Ayrıntılar
Birincil Dil
İngilizce
Konular
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Bölüm
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Yayımlanma Tarihi
27 Haziran 2013
Gönderilme Tarihi
25 Kasım 2011
Kabul Tarihi
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Yayımlandığı Sayı
Yıl 2013 Cilt: 35 Sayı: 2