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VATER-association

Medical expert of the article

Pediatric geneticist, pediatrician
, medical expert
Last reviewed: 05.07.2025

Epidemiology of the VATER association

1.6 per 10,000 newborns.

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Reasons for the VATER association

Most cases are sporadic, with no indication of teratogenic or environmental factors. A few familial cases have been described.

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Symptoms of VATER association

The spectrum of congenital defects is very wide and more than 2/3 of these defects are localized in the lower body segment (defects of the distal intestine, genitourinary anomalies, pelvic bones and lower limbs). Defects of the upper body segment include esophageal atresia, various defects of the radial structures, congenital heart defects and upper respiratory tract defects.

Symptom complex of congenital malformations:

  • Vertebral defects (congenital spinal defects - non-fusion of the arches, butterfly vertebrae) - 70%;
  • Anal atresia - 80%;
  • Tracheoesophageal fistula - 70%;
  • Renal defects - kidney defects or Radial defects - aplasia/hypoplasia of the radial structures of the hand - 65%.

Diagnosis of VATER association

Diagnostic criteria: presence of 3 of the 5 congenital malformations listed above with a normal chromosomal set.

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What tests are needed?

Treatment of VATER association

Symptomatic treatment.

Peculiarities of patient management

If a newborn has a combination of anal atresia and anomalies of the spine or hand, a thorough examination is carried out for the presence of a tracheoesophageal fistula.

If a newborn has a combination of esophageal pathology and radial structures of the hand, it is necessary to exclude congenital kidney defects.

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Forecast

Determined by the spectrum and severity of identified congenital defects. Intelligence is preserved.


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