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Potter's Syndrome is one of several serious or fatal kidney abnormalities. In Potter's (or Potter) syndrome the baby's kidneys do not develop in the first few weeks of life in the womb. The baby's kidneys are essential for the production of amniotic fluid in the womb. If there are no kidneys, there is little or no amniotic fluid (this is known as oligohydramnios) to expand the womb around the baby and to allow the baby to grow and move. The womb remains small and in its confined space the baby's lungs cannot develop properly. Many babies with Potter's syndrome are stillborn. In those who are born alive, the immediate cause of death is failure to breathe (respiratory failure) due to underdeveloped (hypoplastic) lungs, usually one or two days after delivery. Even if this problem is treated the baby cannot survive without kidneys. (Potter's syndrome is also known as Renal Agenesis, which simply means that the kidneys did not develop).
Potter's sequence is the name given to a condition which resembles Potter's syndrome in that although the baby has kidneys, there is little or no amniotic fluid (oligohydramnios). This may sometimes be because the mothers waters have broken in mid-pregnancy, or due to developmental problems in the baby's kidneys or urinary system. In Potter's sequence, as in Potter's syndrome, the baby's lungs are compressed and cannot develop properly. The baby dies of respiratory failure within one or two days of delivery (subnote: although shorter and longer lifespans do exist).
Unilateral absence is quite common, the urethra is also absent and there is asymmetry of the bladder trigone. The other kidney is usually hypertrophied and may therefore be palpable, it is often malformed. Bilateral renal agenesis is incompatible with survival, although the patient may live for a few hours. A characteristic faces (potters face) has been described as low set and large ears, beek like nose, And wide set eyes with prominent epicanthic folds forms a wide semi circle on each side of the nose. And these can help to diagnose potters syndrome at birth.
Malformations particularly in those with hypoplastic or polycystic kidneys, renal agenesis occurs twice as often in boys and is often associated with pulmonary hypoplasia.
Amnion nodosum or vernix granulosum of the amnion occurs regularly in cases of renal agenesis and often in babies with severely hypoplastic or polycystic kidneys. It is associated with olighydramnios, presumably due to lack of urinary component of amniotic fluid.
Oligohydramnios: Too little amniotic fluid is defined as having less than 200ml at term or an AFI of less than 5cm.That means during a ultrasound the largest pocket of fluid found did not measure 1cm or greater, examining the placenta after birth show signs of amnion nodosum. Plenty of women with this problem don’t have complications. In early pregnancy there is a worry of amniotic adhesions causing deformities or constriction of the umbilical cord. It can also cause deformities, like clubfeet with not having enough space in the womb. Later in pregnancy olighydramnios is one of the signs of fetal distress. Causing compression of the cord that means baby won’t be getting enough oxygen. That is why potters babies have deformities to there face, hands and feet. So therefore the concern is not only the renal agenesis (which may be absent) but also the oligohydramnios that will lead to pulmonary hypoplasia.
Agenesis often occurs because the embryonic tissue that gives rise to the affected organ, or an adjacent embryonic tissue, is missing. At many stages in development,
One structure induces the formation of another; removing the first structure causes agenesis of the second. Other cases of agenesis have been associated with chemical exposure in the womb. Such has the drug thalidomide and phocomelia.
Agenesis can also be associated with other congenital abnormalities, in renal agenesis (potter’s syndrome) absence of one or both kidneys; the ureters can also be absent and sex organs can be abnormal. Along with agenesis of the lungs so can heart defects be seen in potter’s babies.
A short biography of Edith potter who found this syndrome he was a pathologist in Chicago for over three decades. He suggested that bilateral renal agenesis multifunctional with a recurrence risk in sibs of about 1%, they described a women with only one kidney who gave birth to 2 children with the same condition and a third child with no kidneys. ( I my self had one kidney removed in 1990 due to kidney disease since being a baby also it was duplicate).The 2 children reported were in a family later reported, as having 4 sisters affected with a syndrome that, in addition to renal hypoplasia or aplasia, Showed vaginal atresia and abnormalities. The family provided support for an autosomal dominant pattern of inheritance with incomplete penetrance and variable expressively in hereditary renal hypoplasia.
Management of renal agenesis; unilateral agenesis is compatible with life as the only one available kidney enlarges to compensate for the absent one, (this was shown on my scan as one of my kidneys were not working and had to be removed). On the other hand complete absence of kidneys is not compatible with life the foetus usually die in uterus or shortly after birth. The best management therefore is taking preventative measures as much as it is possible to prevent congenital malformations from occurring. For instance a women with uncontrolled diabetes mellitus is prone to having babies with renal agencies and other malformations. So controlling the diabetes could reduce the risk.
Development of the kidneys:
The kidneys are developed in three main stages called the pronophros, mesonephros and the metanephros (nephros means kidney). The pronophros are non functional and soon degenerate being replaced by the mesonephros which functions for a short time before they are also replaced by the metanephros the definitive kidneys.
The permanent kidneys metanephros begins to develop in the fifth week of intrauterine life, urine begins about the end of the first trimester (12th week) and continues for the rest of the pregnancy. The urine produced by the foetus is secreted into the amniotic cavity and forms part of the amniotic fluid. In the foetus, the placenta is the main organ of excretion, therefore the kidneys don’t need to become functional until after birth. Earlier in pregnancy the kidney is located in the pelvis by the 9th week of pregnancy the kidneys have attained their adults position in the abdomen. This variation in position is due to the differential increase in the growth of the abdomen. For this reason it is often observed that the kidneys have various sources of blood supply during development, which gradually degenerates as the kidneys ascend to the abdominal cavity. Not surprising the adult kidneys sometimes has aberrant blood supply due to its migratory development nature.
From the foregoing discussion it becomes apparent that complete absence of the kidneys (bilateral renal agenesis) results when the metanephric buds fail to develop while unilateral renal agenesis will result from one-sided metanphric bud absence.
