VACTERL syndrome / association

What is Vacterl Syndrome

The acronym for a constellation of BIRTH DEFECTS that tend to occur in coincidence with each other. Doctors consider a baby who has three or more of the defects to have the VACTERL association, and will examine the baby closely for the other defects in the constellation. These defects include

• V: vertebral (spinal) anomalies
• A: ANAL ATRESIA (also called imperforate ANUS)
• C: cardiac (HEART) anomalies
• T/E: tracheoesophageal fistula (may also occur as tracheal fistula and ESOPHAGEAL ATRESIA)
• R: renal (kidney) anomalies
• L: limb anomalies

Causes of VACTERL syndrome

Occurring often enough that some doctors believe it, too, is part of the constellation, is a single ARTERY UMBILICAL CORD (the normal umbilical cord has two arteries), which sometimes appears in the acronym as a final S (VACTERLS). VACTERL occurs sporadically (in a nonhereditary pattern); researchers do not know what causes it, nor do they understand the connections among the various defects. Some of the birth defects can be life threatening, such as the HEART malformation tetralogy of Fallot (a complex of four serious heart defects). Treatment, often surgery, attempts to correct the congenital anomalies.

See also CONGENITAL ANOMALY; CONGENITAL HEART DISEASE.

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