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VACTERL Association
KEY FACTS
Terminology
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Nonrandom association of anomalies involving multiple organ systems
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V ertebral/vascular
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A nal atresia/auricular
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C ardiac
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T racheoesophageal fistula
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E sophageal atresia
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R enal/radial ray/rib
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L imb
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VACTERL association diagnosed when ≥ 3 of above malformations present; causative gene unknown
Imaging
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Actively seek other features of VACTERL association when 1-2 components present
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Initial imaging in suspected cases: Radiographs & US
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Radiographs: Spine & limbs (if limb anomaly present on physical exam)
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US: Head, spine, renal/bladder, echocardiography
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Further imaging depends on clinical exam & initial imaging findings
Clinical Issues
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Incidence of VACTERL: 1/10,000 to 40,000 liveborns
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Children with VACTERL: 72% have 3 anomalies, 24% have 4 anomalies, 8% have 5 anomalies
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Cardiac: 40-80%
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Renal: 50-80%
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Anal: 55-90%
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Tracheoesophageal: 50-80%
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Vertebral: 60-80%
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Limb: 40-50%
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Diagnostic Checklist
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Consider VACTERL in child with vertebral & other anomalies
Frontal radiograph in a newborn shows vertebral segmentation anomalies
. The nasogastric tube (NG) could not be advanced beyond the upper esophagus
due to esophageal atresia (with the bowel gas indicating an associated tracheoesophageal fistula). Mild central pulmonary vascular congestion
is secondary to a VSD. There is partial sacral agenesis
in this child with an ARM.
. The nasogastric tube (NG) could not be advanced beyond the upper esophagus due to esophageal atresia (with the bowel gas indicating an associated tracheoesophageal fistula). Mild central pulmonary vascular congestion is secondary to a VSD. There is partial sacral agenesis in this child with an ARM.
Longitudinal US images of the left (top) & right (bottom) renal fossae in the same patient show a solitary left kidney
.
.
Frontal radiograph in a newborn shows vertebral segmentation anomalies
& partial agenesis of the sacrum
. The NG tube could not be passed beyond the upper esophagus due to esophageal atresia
(with the lack of abdominal gas indicating the absence of a tracheoesophageal fistula).
& partial agenesis of the sacrum . The NG tube could not be passed beyond the upper esophagus due to esophageal atresia (with the lack of abdominal gas indicating the absence of a tracheoesophageal fistula).
Lateral view from a VCUG in the same child shows a rectourethral fistula
from the rectal pouch
to the posterior urethra
. Left vesicoureteral reflux
is also seen in this child with an ARM.
from the rectal pouch to the posterior urethra . Left vesicoureteral reflux is also seen in this child with an ARM.
TERMINOLOGY
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