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VACTERL anomalies in patients with esophageal atresia: an updated delineation of the spectrum and review of the literature

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Abstract

The VACTERL complex refers to anomalies of the bony spinal column (V), atresias in the gastrointestinal tract (A), congenital heart lesions (C), tracheoesophageal defects (TE), renal and distal urinary tract anomalies (R) and limb lesions (L). The incidence of each of these components has not been precisely quantified in the recent literature and the full array of anomalies within each systemic class of the VACTERL complex has not been well described. Therefore, we reviewed our most recent 20-year experience of patients born with esophageal atresia to comprehensively delineate and accurately describe the type and incidence of associated lesions. A retrospective review was then conducted on all patients diagnosed with esophageal atresia between 1985 and 2005. Patient demographics recorded included gestational age, weight and gender. The specific types of lesions were carefully cataloged. The outcome measure recorded was survival. One hundred and twelve patients were diagnosed with esophageal atresia were identified during the study period. The gestational age range was 28–41 weeks with an average of 36.5 weeks. Average birth weight was 2,557 g (range 1,107–3,890). A male predominance was seen with 62 males and 50 females. The overall survival was 92.9%. The categorical breakdown of anomalies were vertebral (24.1%), atresia (14.3%), cardiac (32.1%), tracheoesophageal fistula (95.5%), urinary (17.0%), skeletal (16.1%) and other (10.8%). VACTERL anomalies are common in patients with esophageal atresia, however, they appear to have little impact on overall survival.

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References

  1. Quan L, Smith D (1973) The VATER association. Vertebral defects, anal atresia, T-E fistula with esophageal atresia, radial and renal dysplasia: a spectrum of associated defects. J Pediatr 82:104–107

    Article  PubMed  CAS  Google Scholar 

  2. Smith D (1974) The Vater association. Am J Dis Child 128:767

    PubMed  CAS  Google Scholar 

  3. Temtamy S, Miller J (1974) Extending the scope of the VATER association: definition of the VATER syndrome. J Pediatr 85:345–349

    Article  PubMed  CAS  Google Scholar 

  4. Barnes J, Smith W (1978) The VATER association. Radiology 126:445–449

    PubMed  CAS  Google Scholar 

  5. Barry J, Auldist A (1974) The Vater association; one end of a spectrum of anomalies. Am J Dis Child 128:769–771

    PubMed  CAS  Google Scholar 

  6. Kim P, Mo R, Hui C (2001) Murine models of VACTERL syndrome: role of sonic hedgehog signaling pathway. J Pediatr Surg 36:381–384

    Article  PubMed  CAS  Google Scholar 

  7. Spilde T, Bhatia A, Mehta S, Ostlie D, Hembree M, Preuett B, Prasadan K, Li Z, Snyder C, Gittes G (2003) Defective sonic hedgehog signaling in esophageal atresia with tracheoesophageal fistula. Surgery 134:345–350

    Article  PubMed  Google Scholar 

  8. Spilde T, Bhatia A, Ostlie D, Marosky J, Holcomb G III, Snyder C, Gittes G (2003) A role for sonic hedgehog signaling in the pathogenesis of human tracheoesophageal fistula. J Pediatr Surg 38:465–468

    Article  PubMed  Google Scholar 

  9. Mandhan P, Quan Q, Beasley S, Sullivan M (2006) Sonic hedgehog, BMP4, and Hox genes in the development of anorectal malformations in ehtylenethiourea-exposed fetal rats. J Pediatr Surg 41:2041–2045

    Article  PubMed  Google Scholar 

  10. Giampietro P, Raggio C, Reynolds C, Ghebranious N, Burmester J, Glurich I, Rasmussen K, McPherson E, Pauli R, Shukla S, Merchant S, Jacobsen F, Faciszewski T, Blank R (2006) DLL3 as a candidate gene for vertebral malformations. Am J Med Genet 140:2447–2453

    Article  PubMed  CAS  Google Scholar 

  11. Shinkai Y, Tsuji T, Kawamoto Y, Kunieda T (2004) New mutant mouse with skeletal deformities caused by mutation in delta like 3 (Dll3) gene. Exp Anim 53:129–136

    Article  PubMed  CAS  Google Scholar 

  12. Yang C, Soong W, Jeng M, Chen S, Lee Y, Tsao P, Hwang B, Wei C, Chin T, Liu C (2006) Esophageal atresia with tracheoesophageal fistula: ten years of experience in an institute. J Chin Med Assoc 69:317–321

    Article  PubMed  Google Scholar 

  13. Garne E, Nielsen G, Hansen O, Emmertsen K (1999) Tetralogy of Fallot. A population-based study of epidemiology, associated malformations and survival in western Denmark 1984–1992. Scand Cardiovasc J 33:45–48

    Article  PubMed  CAS  Google Scholar 

  14. Stevenson R (1974) The association of extra vertebras and tracheoesophageal anomalies. Birth Defects Orig Artic Ser 10:147–148

    PubMed  CAS  Google Scholar 

  15. Janssen F, Kemperdick H (1974) Spinal and costal abnormalities in congenital atresia of the esophagus. Z Kinderheilkd 117:275–280

    Article  PubMed  CAS  Google Scholar 

  16. Bond-Taylor W, Starer F, Atwell J (1973) Vertebral anomalies associated with esophageal atresia and tracheoesophageal fistula with reference to the initial operative mortality. J Pediatr Surg 8:9–13

    Article  PubMed  CAS  Google Scholar 

  17. Touloukian R, Keller M (1988) High proximal pouch esophageal atresia with vertebral, rib, and sternal anomalies: an additional component to the VATER association. J Pediatr Surg 23:76–79

    Article  PubMed  CAS  Google Scholar 

  18. Uehling D, Gilbert E, Chesney R (1983) Urologic implications of the VATER association. J Urol 129:352–354

    PubMed  CAS  Google Scholar 

  19. Inatomi J, Miyazaki O, Fujimaru R, Iijima K (2005) Renovascular hypertension complicated with VATER association. Pediatr Nephrol 20:1174–1176

    Article  PubMed  Google Scholar 

  20. Apold J, Dahl E, Aarskog D (1976) The Vater association: malformations of the male external genitalia. Acta Paediatr Scand 65:150–152

    PubMed  CAS  Google Scholar 

  21. Cho S, Moore S, Fangman T (2001) One hundred three consecutive patients with anorectal malformations and their associated anomalies. Arch Pediatr Adolesc Med 155:587–591

    PubMed  CAS  Google Scholar 

  22. German J, Mahour G, Woolley M (1976) Esophageal atresia and associated anomalies. J Pediatr Surg 11:299–306

    Article  PubMed  CAS  Google Scholar 

  23. Kotsios C, Merei J, Hutson J, Graham H (1998) Skeletal anomalies in the adriamycin-exposed prenatal rat: a model for VATER association. J Orthop Res 16:50–53

    Article  PubMed  CAS  Google Scholar 

  24. Xia H, Migliazza L, Montedonico S, Rodriguez J, Diez-Pardo J, Tovar J (1999) Skeletal malformations associated with esophageal atresia: clinical and experimental studies. J Pediatr Surg 34:1385–1392

    Article  PubMed  CAS  Google Scholar 

  25. Ratan S, Rattan K, Ratan J, Sodhi P, Bhatia V (2005) A neonate with anorectal malformation with rare limb defects report of a case. Pediatr Surg Int 21:825–828

    Article  PubMed  Google Scholar 

  26. Ein S, Palder S, Filler R (2006) Babies with esophageal and duodenal atresia: a 30-year review of a multifaceted problem. J Pediatr Surg 41:530–532

    Article  PubMed  Google Scholar 

  27. Louhimo I, Lindahl H (1983) Esophageal atresia: primary results of 500 consecutively treated patients. J Pediatr Surg 18:217–229

    Article  PubMed  CAS  Google Scholar 

  28. Driver C, Shankar K, Jones M, Lamont G, Turnock R, Lloyd D, Losty P (2001) Phenotypic presentation and outcome of esophageal atresia in the era of the Spitz classification. J Pediatr Surg 36:1419–1421

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Surgery, The Children’s Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA

    Scott J. Keckler, Shawn D. St. Peter, Patricia A. Valusek, KuoJen Tsao, Charles L. Snyder, George W. Holcomb III & Daniel J. Ostlie

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  1. Scott J. Keckler
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  2. Shawn D. St. Peter
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  3. Patricia A. Valusek
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  4. KuoJen Tsao
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  5. Charles L. Snyder
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  6. George W. Holcomb III
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  7. Daniel J. Ostlie
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Correspondence to Shawn D. St. Peter.

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Keckler, S.J., St. Peter, S.D., Valusek, P.A. et al. VACTERL anomalies in patients with esophageal atresia: an updated delineation of the spectrum and review of the literature. Pediatr Surg Int 23, 309–313 (2007). https://doi.org/10.1007/s00383-007-1891-0

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  • DOI: https://doi.org/10.1007/s00383-007-1891-0

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