Urinary Tract Anomalies Associated with Esophageal Atresia

By Linda Jacobs, R.N.

For parents of children born with complex congenital anomalies, it is like a bad dream to be informed of, in some cases, numerous defects in our newborn infants.  In many cases these defects seem unrelated and in completely different body systems.  Parents wonder how one defect has anything to do with another and how their child could have all these problems!

As early as 1670 there was reference to associated anomalies and esophageal atresia.  Then in 1840 a physician reported in a paper on congenital malformations that there was an obvious association between esophageal atresia and anorectal malformations.  This is quite interesting since between that time and the 1940s the significance of associated anomalies was largely overlooked or ignored. One source in 1940 even wrote that associated anomalies were relatively infrequent and unimportant!

By 1969, however, a pediatric surgery journal featured an article that reported on, and clearly recognized, the significance of associated anomalies. Since the 1960s there have been numerous contributions to the literature reviewing the incidence of associated anomalies.

Although many studies have been done identifying the types of associated defects, the types of defects and their frequency of occurrence may vary from one study to another. After cardiac abnormalities, renal abnormalities are the next most common associated defects, reported in most of these studies, with esophageal atresia.

There are numerous types of urinary tract defects that can occur in association with esophageal atresia. Most urinary tract abnormalities are not life threatening and may not cause symptoms in the newborn baby. Because a defect can sometimes be damaging the urinary tract while not presenting itself until later in life, it is important for every baby born with esophageal atresia to be evaluated to determine if there are any urinary tract defects.  This is particularly so in the case of ureteral reflux, when urine backs up the ureter from the bladder into the kidney.

One source divided urinary abnormalities into four groups according to their importance in management. There are certain defects that are familiar to parents of children with esophageal atresia since they are the ones most commonly seen in their children. These groupings are by no means meant to be all inclusive but can provide some guidelines for questioning when your child is being evaluated.

The following groupings list some urinary tract abnormalities according to their clinical significance (whether treatment is required or not).

Group I:

Abnormalities of no clinical significance (generally do not cause any problems and often go undiagnosed)

Horseshoe Kidney (uncomplicated) – This is when the two kidneys are fused together and give a horseshoe-like appearance. The vast majority of horseshoe kidneys function normally. Problems can occasionally occur with horseshoe kidneys, so these children should be monitored regularly.

Extra Ureters – When there is more than one tube coming off of a kidney and connecting to the bladder (These tubes drain the urine from the kidney into the bladder).

Group II:

Abnormalities requiring no treatment

One kidney only formed

Two kidneys formed but one is not normal and may not function

One kidney may have a simple cyst on it

Pelvic kidney – A kidney that did not form in the correct position the body.

Group III:

Abnormalities likely to require treatment

Ureteral Reflux – As mentioned above, this is when urine backs up from the bladder through a ureter (the tube that drains urine from the kidney into the bladder), back into the kidney. In some cases this can cause infections and scarring of the kidney. This can be managed in some cases with medication. However, some cases may require a surgical repair.

Urinary Tract Obstruction – This is when there is some abnormality that is preventing the urinary tract from functioning normally.  Depending upon the cause of the obstruction, surgery may be necessary to allow the urinary tract to function.

Group IV:

Abnormalities likely to lead to early renal failure or death.

Children born without any kidneys

Children born with multiple cysts on both of their kidneys

Since there is a high incidence of urinary tract abnormalities that require treatment in children born with esophageal atresia, it is very important that all of these children be screened at birth.  This can be done first with a renal ultrasound, which is painless.  If further testing is necessary, the physician may order a renal scan or some other tests specific for a suspected problem.

GLOSSARY

Urinary Tract – The kidneys, ureters, bladder, and urethra.

Kidneys – Two organs found int he lower back area.  When working well, they clean the blood and get rid of waste products and excess salt and water.

Ureters – Tubes that drain urine from the kidneys into the bladder, which in turn drains urine to the outside of the body.

Bladder – A sac-like structure in the pelvis which holds urine until it is drained to the outside of the body through the urethra.

Anomalies – Abnormalities that occur in a baby before it is born.