Potters Syndrome - Bilateral Renal Agenesis

|Potter's Sydrome is defined as a condition with insufficiant amniotic fluid.  It can be caused by many different kidney defects.  There are a few different types of Potters Sydrome: Classic Potters is the result of Bilateral Renal Agenesis (the lack of any kidney development), Type I is referred to as Autosomal Recessive Polycystic Kidney Disease.  Type II is Renal Dysplasia, Type III is Autosomal Dominant Polycystic Kidney Disease.

Bilateral Renal Agenesis  is a rare but serious condition where both fetus' kidneys failed to develop during gestation, this is the condition our son Gabriel had. We were told that this condition is approximately 1 in 4000. Unilateral Renal Agenesis is the absense of a kidney and is much more common.it is not life threatening in most cases. Bilateral Renal Agenesis, having no kidneys,  causes a lack of amniotic fluid which prevents many vital organs from forming. For Gabriel this caused his lungs to be dramatically underdeveloped, it also caused his digestive track to be underdeveloped and disfunctional.

The fact that the lungs cannot develope without the amniotic fluid causes the child to go into resperatory failure upon birth making it impossible to survive. Bilateral Renal Agenesis(BRA) is 100% fatal in all cases.

Many times, after a fatal diagnosis, parents will chose to terminate the pregnancy. If chosen to continue the pregnancy, some babies pass away while in utero, this happens most often due to the baby putting pressure on its own umbilical cord. If a baby does survive to full term, occasionally the baby is too weak to survive the labor and delivery process, or the baby may put too much pressure on its cord during a contraction or delivery. Too much pressure would cause the heart rate to drop dramatically sometimes causing it to stop all together.  If the baby has made it beyond labor and delivery, they have no way of predicting how long the baby will survive outside of the womb, some only live a few moments, some live up to 4 hours.

When a BRA baby is born, many times the pressure from being in the womb can cause visual malformations. Disconfigured face, limbs and head is fairly common. (however, Gabriel appeared perfect when he was born.) Often the baby remains blue in color due to lack of oxygen, and can sometimes be smaller than normal.  Because there is serious lack of lung formation, Potters Syndrome babies tend to have a very small chest. For Gabriel, his lungs hadn't developed beyond 18 weeks or so, so his chest never expanded as a normal baby's would have.

Bilateral Renal Agenesis can sometimes be a genetic disorder, although they don't think this is the case for us.  Sometimes it is just simply "bad luck". If the condition is genetic, than there is a 25% risk of it reoccuring. If it is determined that the condition is not genetic than the risk factor is extremely low for reoccurance.
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