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  • Acronym stands for:

    • V: vertebral defects (e.g. hemivertebrae, fused or butterfly vertebrae, or extra vertebrae)

    • A: anal or other intestinal atresia

    • C: cardiac defects (VSD & tetralogy of Fallot are the most common) 

    • TE: tracheoesophageal fistula (TEF)

    • R: renal malformations (e.g. horsheshoe kidney, renal agenesis, vesicoureteral reflux, hypospadias, dysplastic kidney, & cryptorchidism

    • L: limb defects

  • Not all features are always present, but TEF is accepted as essential for the diagnosis, along with at least one other major defect in one of the five other categories

  • Most patients are developmentally normal 



  • Pediatric patient considerations 

  • Risk of regurgitation & considerations of TEF 

  • Risk of cardiac malformations, especially VSD & TOF

  • Renal & limb defects 






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