VACTERL association

INFORMATION

The association VACTERL is a "syndromic" association of many common multisystem anomalies. VACTERL stands for vertebral, anal, cardiac, tracheal, esophageal, renal and limbic. It occurs in 1 in 10,000 to 1 in 40,000 births.

Many patients have at least three of the above systemic associations.

Vertebral anomalies predominate (covering 60-80% of patients) and may include vertebral anastomoses, missing vertebrae (atresias) and even additional vertebral bodies. Surgery may be required to correct severe angular spinal deformities and/or spinal stenosis. Similarly, anal arthrocentesis is common, requiring immediate surgical intervention shortly after birth; it is often associated with other urogenital anomalies.

Most commonly associated with heart defects include atrial septal and ventricular septal defects (ASD/VSD), Tetralogy of Fallot; less likely but reported are Arterial Trunk and Transposition of the Great Vessel (TGV). Tracheoesophageal fistulas are common, requiring earlier surgical intervention to correct them.

Renal dysfunction includes missing or malformed kidneys and/or vesicoureteral reflux.

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