Nonrandom association of 6 core abnormalities

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  V ertebral defects

  
  
  
  
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    Hemivertebrae: Best demonstrated in coronal plane, scoliosis originates at hemivertebra(e)

    
    
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    Fusion of vertebral bodies or posterior elements (block vertebrae)

  
  
  
  
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  A nal atresia

  
  
  
  
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    Absent anal dimple

    
    
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    Dilated colon that does not reach perineum

  
  
  
  
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  C ardiac anomalies

  
  
  
  
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    Ventricular septal defect is most common defect in some studies

  
  
  
  
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  T racheoesophageal fistula with e sophageal atresia

  
  
  
  
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    Stomach absent or small, look for esophageal pouch in neck

    
    
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    Polyhydramnios usually late finding (3rd trimester)

  
  
  
  
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  R enal anomalies: Majority with structural renal defect also have anorectal malformation

  
  
  
  
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    Vesicoureteral reflux with additional structural defect (27%), unilateral renal agenesis (24%), multicystic dysplastic kidney (18%), duplicated collecting system (18%)

  
  
  
  
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  L imb defects: Usually bilateral upper limbs, may be asymmetric

  
  
  
  
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    Primarily radial ray malformation with hypoplasia/aplasia of radius with radial club hand or hypoplasia/aplasia of thumbs

  
  

Perform systematic search for associated anomalies when 1 defect identified

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  Cardiac anomalies most common defect (~ 80%)

  
  
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  Esophageal atresia ± tracheoesophageal fistula in 50-60%