What is VACTERL Association

Around 50% to 80% of babies born with VACTERL Association have some  form of kidney problem, although this may not cause any problems immediately  after birth. 

These abnormalities can vary in severity with some patients requiring monitoring and/or treatment [1].

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Kidneys that formed differently before birth

Renal agenesis (missing kidney)
Renal hypoplasia (small kidney)
Renal dysplasia (abnormal kidney tissue)
Horseshoe kidney (two kidneys joined together)
Ectopic kidney (kidney often lower in the pelvis)
 

Problems with the bladder and the passage of urine

Hydronephrosis (swelling of the kidney due to urine build-up)

Duplicated collecting system (extra or abnormally shaped ureters)
Ureteropelvic junction (UPJ) obstruction (blockage where the kidney meets the ureter)
Hydroureter (swelling of the ureter)

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Problems with the bladder

Vesicoureteral reflux (VUR) – urine flows backward from the

bladder toward the kidneys.

Posterior urethral valves (blockages in the urinary tract,

especially in boys)
Neurogenic bladder (bladder control problems due to spine/vertebral defects)
Bladder outlet obstruction.

 

Kidney related differences can affect daily life

Kidney and urinary differences may lead to:

• Urinary tract infections (UTIs)

• High blood pressure

• Trouble emptying the bladder

• Reduced kidney function over time

 

How Kidney and Urinary Issues Are Checked

At diagnosis and during follow-up, doctors may recommend:

• Kidney ultrasound – to check kidney size, position, and structure

• Urine and blood tests - to check kidney function

• Blood pressure monitoring 

 

A special bladder x-ray test called a voiding cystourethrogram may be recommended if there is:​

• Swelling of the kidney(s) - Hydronephrosis​

• Recurrent Urinary Tract Infections - UTIs

• Urine backflow to the kidney(s) - Vesicoureteral Reflux

• Blockage in the urine passage, near the bladder - Posterior Urethral Valves

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Early screening helps catch problems before symptoms appear.

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N.B. descriptions of the Terms used here can be found in the Glossary section​.