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Scimitar syndrome in a case with VACTERL association

Published online by Cambridge University Press:  06 June 2014

Christian J. Fritz ,
Heiko M. Reutter  and
Ulrike Herberg
Christian J. Fritz
Affiliation:
Department of Neonatology, Children’s Hospital, University Hospital of Bonn, Germany
Heiko M. Reutter
Affiliation:
Department of Neonatology, Children’s Hospital, University Hospital of Bonn, Germany Institute of Human Genetics, University of Bonn, Germany
Ulrike Herberg*
Affiliation:
Department of Pediatric Cardiology, Children’s Hospital, University Hospital of Bonn, Bonn, Germany
*
Correspondence to: Dr med. U. Herberg, Abteilung für Kinderkardiologie, Zentrum für Kinderheilkunde, Universitätsklinikum Bonn, Adenauerallee 119, 53115 Bonn, Germany. Tel: +49 228/287-33448 bzw. -33333; Fax: +49 228/287-33360; E-mail: Ulrike.Herberg@ukb.uni-bonn.de
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Abstract

VACTERL association and Scimitar syndrome are rare congenital diseases. In this study, we report on a neonate with prenatal suspicion of VACTERL association and small left-sided cardiac structures, which, only on postnatal angiography, could be revealed to be part of a Scimitar syndrome. As this is the second reported case of VACTERL association and Scimitar syndrome, the presence of Scimitar syndrome should be considered in the prenatal and postnatal evaluation of VACTERL association.

Keywords

Scimitar syndromeVACTERL associationcongenital abnormalitypulmonary veinanorectal malformation
Type
Brief Reports
Information
Cardiology in the Young , Volume 25 , Issue 3 , March 2015 , pp. 606 - 609
Copyright
© Cambridge University Press 2014 

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References

1. Takawira, FF, Omar, F. Unusual variant of scimitar syndrome associated with an absent right pulmonary artery, stenosis of the inferior vena cava, hemi-azygous continuation and the VACTERL association. Cardiovasc J Afr 2013; 24: e3–e6.Google Scholar
2. Quan, L, Smith, DW. The VATER association. Vertebral defects, anal atresia, T-E fistula with oesophageal atresia, radial and renal dysplasia: a spectrum of associated defects. J Pediatr 1973; 82: 104–107.Google Scholar
3. Temtamy, SA, Miller, JD. Extending the scope of the VATER association: definition of the VATER syndrome. J Pediatr 1974; 85: 345–349.Google Scholar
4. Solomon, BD. VACTERL/VATER association. Orphanet J Rare Dis 2011; 6: 56.Google Scholar
5. Opitz, JM. The developmental field concept. Am J Med Genet 1985; 21: 1–11.Google Scholar
6. Martínez-Frías, ML, Frías, JL, Opitz, JM. Errors of morphogenesis and developmental field theory. Am J Med Genet 1998; 76: 291–296.Google Scholar
7. Gudjonsson, U, Brown, JW. Scimitar syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2006; 9: 56–62.Google Scholar
8. Ruggieri, M, Abbate, M, Parano, E, et al. Scimitar vein anomaly with multiple cardiac malformations, craniofacial, and central nervous system abnormalities in a brother and sister: familial Scimitar anomaly or new syndrome. Am J Med Genet 2003; 116: 170–175.Google Scholar
9. Levent, M, Esen, D, Memnune, A, et al. Eponym Scimitar syndrome. Eur J Pediatr 2010; 169: 1171–1177.Google Scholar
10. Seale, AN, Carvalho, JS, Gardiner, HM, et al. Total anomalous pulmonary venous connection: impact of prenatal diagnosis. Ultrasound Obstet Gynecol 2012; 40: 310–318.CrossRefGoogle ScholarPubMed