VACTERL association is a “syndrome-like” association between many frequently occurring multi-system anomalies. VACTERL stands for Vertebral, Anal, Cardiac, Tracheal, Esophageal, Renal and Limb. It occurs in 1 in 10,000 to 1 in 40,000 live births.
Many patients have at least three of the above system associations.
Vertebral anomalies predominate, (involving 60-80% of patients), and may include fusion of vertebrae, missing (atresia) vertebrae, or even extra vertebral bodies. Surgery may be required to correct severe angular spinal deformities and/or severe spinal stenosis. Similarly, Anal atresia is frequently present, requiring immediate surgical intervention shortly after birth; this is also frequently associated with other genito-urinary tract anomalies.
The Most commonly associated cardiac defects include atrial-septal and ventriculo-septal defects (ASD/VSD), Tetralogy of Fallot; less likely but reported are Truncus Arteriosus and Transposition of the Great Vessels (TGV). Tracheal-esophageal fistulas are frequently present, requiring surgical attention early in life to correct.
Renal abnormalities include missing or mis-shaped kidneys and/or severe vesiculo-urethral reflux.
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