The oligohydramnios sequence describes the effects of insufficient amniotic fluid production. These are serious malformations that develop due to low amniotic fluid levels during embryogenesis. The disease is fatal.
What is an oligohydramnios sequence?
The oligohydramnios sequence describes the effects of low amniotic fluid production during pregnancy. The embryo cannot develop normally due to the cramped space conditions due to the small amount of amniotic fluid. Serious malformations of the internal and external organs occur. The syndrome was first described by the American pathologist Edith Potter in the absence of kidneys on both sides (bilateral renal agenesis). It is also known as Potter syndrome. See acronymmonster for Definition of Lumbar Spine Syndrome in English.
Potter performed about 5000 autopsies on 3 female and 17 male embryos or newborns. However, since the malformations are not limited to the absence of the kidneys, they are referred to as oligohydramnios sequences. The lack of amniotic fluid during pregnancy is also known as oligohydramnios. By definition, this is the case when the amount of amniotic fluid falls below 200 to 500 ml.
The amniotic fluid index is less than 5.1 cm or the amniotic fluid deposits are less than 2 cm between the placenta (placenta), uterine wall and fetus. Oligohydramnios occurs in up to four percent of pregnancies and can have multiple causes. Only rarely does an oligohydramnios sequence develop from this.
The cause of an oligohydramnios sequence is the lack of amniotic fluid. The most common cause of premature rupture of membranes is the loss of amniotic fluid. However, this is not the cause of an oligohydramnios sequence because the loss of amniotic fluid happens just before birth. The oligohydramnios sequence can only develop in long-term oligohydramnios. This is mainly caused by reduced urine production by the embryo.
Furthermore, strong growth delays in the baby can lead to this. Functional disorders of the placenta caused by high blood pressure or nicotine consumption also sometimes cause oligohydramnios. Other reasons can be obstructive urinary tract diseases or chromosomal aberrations. As a result of the insufficient amount of amniotic fluid, there is a lack of space for the embryo, since it can only develop within the amniotic fluid.
These cramped conditions result in unusual growth conditions for the fetus. All malformations are due to this. However, in some cases, existing kidney or urinary tract malformations can also limit urine production and urine excretion. This results in reduced amniotic fluid production, which in turn impedes the development of the remaining organs.
Overall, it is therefore not entirely clear whether the clinical picture of the oligohydramnios sequence can also be genetically determined or is really only based on growth disorders due to the oligohydramnios. There may also be several causes.
Symptoms, Ailments & Signs
The oligohydramnios sequence is characterized by severe internal and external dysplasia. The most important symptom is the malformation of the urogenital tract. In particular, the two kidneys are affected. They are either missing completely (renal agenesis) or they are severely underdeveloped. In addition, there is hypoplasia (underfunctioning) of the lungs. In addition to clubfeet, other deformities of the extremities or the spine can occur.
The malformations on the face, known as the so-called Potter facies, are particularly noticeable. These dysmorphisms are similar to those in Down syndrome. The auricles are specially shaped for low-set and flat ears. The cartilage substance of the auricles is missing. Another symptom is a medial epicanthus. This is a double crease similar to the Mongolian crease at the inner corner of the eye.
In addition, the distance between the eyes is comparatively large, which is referred to as hypertelorism. Finally, the lower jaw is underdeveloped. It therefore appears shortened. The prognosis of the disease is very poor. Due to renal agenesis alone, death occurs either before the child is born or shortly thereafter.
Diagnosis & course of disease
Potter’s syndrome or oligohydramnios sequence can be diagnosed before birth with a prenatal ultrasound scan. From the 17th week of pregnancy, Potter’s syndrome is clearly recognizable. It can be determined by the significant delay in growth and the unusual forced posture due to the lack of space due to the lack of amniotic fluid.
Because of the oligohydramnios sequence, the children suffer from very severe malformations and deformities. In severe cases, the children can be born dead or die shortly after birth. As a rule, the parents and relatives of the child also suffer from severe psychological problems and depression.
Furthermore, the patient’s kidneys can be missing completely, so that the child is directly dependent on dialysis. Down syndrome can also occur and significantly reduce the quality of life of those affected. The lower jaw is shortened and there are also hearing problems and visual disturbances. In most cases, however, the child dies shortly after birth as a result of the oligohydramnios sequence.
Unfortunately, treatment of the oligohydramnios sequence after birth is not possible. However, the complaint can be diagnosed relatively early, so that an early treatment of this disease is possible. The deficits in the amniotic fluid can be compensated so that the malformations can be avoided. The oligohydramnios sequence poses no health risk to the mother and there is no reduction in life expectancy.
When should you go to the doctor?
The far-reaching consequences of the lack of amniotic fluid are dramatic for the developing child. Numerous malformations occur, as a result of which the child dies. The possible causes of an oligohydramnios sequence have not been clarified, multicausal causes are obvious.
Although an oligohydramnios sequence can be diagnosed prenatally and as part of a routine ultrasound examination. However, the presence of an oligohydramnios sequence can only be recognized after the 17th week of pregnancy. But then it is too late for an abortion. However, if the gynecologist recognizes the lack of amniotic fluid in time, it can be compensated for. In this case, the child is likely to be born healthy or with only minor consequential damage.
The unborn child often dies before birth if there is a pronounced oligohydramnios sequence. Sometimes it dies shortly afterwards. The affected child suffered such severe damage due to the lack of space in the amniotic sac that it would not be able to survive even with the best medical help. In some cases, children are born alive but with severe kidney damage. You need dialysis immediately. Such children learn that doctor visits are part of their short lives.
Treatment & Therapy
In the case of a pronounced oligohydramnios sequence, therapy is unfortunately no longer possible. The prognosis is 100 percent fatal. If the infant is not already born dead, it will die within a short period of time due to kidney and lung malformations. However, the underlying cause of the oligohydramnios sequence (the oligohydramnios) can be treated during pregnancy. As already mentioned, oligohydramnios is characterized by a reduced amount of amniotic fluid.
Evidence of this comes from an unusually small uterus and reduced fetal movement. This already points to cramped spatial conditions. Sometimes it is enough for the pregnant woman to drink enough to bring the amount of amniotic fluid back to normal. This is the case when only temporary causes have led to the reduction in amniotic fluid. However, if the amount of amniotic fluid cannot be compensated for by drinking, then the child will develop abnormally.
In this case, there is still the possibility of compensating for the amount of amniotic fluid with an amniotic infusion. An amniotic infusion is an amniotic fluid replenishment from the outside. The amniotic fluid is filled with a glucose-saline solution using a catheter or needle. This measure takes place under ultrasound control. However, if the child is in poor general condition, the treatment of choice is the premature delivery of the child. However, an induction of lung maturation should be initiated beforehand.
Outlook & Forecast
If insufficient amniotic fluid is produced during pregnancy, it can be a death sentence for the embryo. Too little amniotic fluid means that the unborn child cannot develop properly. It would be severely deformed by the oligohydramnios sequence, or die in childbirth.
The oligohydramnios sequence appears to be multifactorial. Most children with deformities and consequential damage caused by this die at birth or a short time later. Those that survive longer have severe damage and malformations in many organ systems and limbs. Among other things, the kidneys are missing. That alone prevents many of the children from surviving. The survivors are dependent on dialysis. They also have other severe deformities.
Nowadays, however, prenatal examinations can determine whether there is enough amniotic fluid or not. If a deficiency is detected, the amount of amniotic fluid can be increased. As a result, the serious consequential damage caused by the oligohydramnios sequence can be avoided. The problem, however, is that this examination only makes sense after the 17th week of pregnancy, because the doctors cannot determine the lack of amniotic fluid beforehand. It is then already too late for an abortion.
However, if the lack of amniotic fluid is detected at this point, most children can be born healthy and with a good prognosis. Some have slight deformities.
To prevent an oligohydramnios sequence, constant attention must be paid to ensuring sufficient amniotic fluid volume during pregnancy. If signs of oligohydramnios appear, appropriate amniotic fluid replenishment measures should be taken immediately.
You can do that yourself
Since oligohydramnios sequence is a fatal disease, self-help measures are very limited. In order to prevent an oligohydramnios sequence, care must be taken to ensure that the amniotic fluid volume is sufficiently high during pregnancy. Sometimes it is enough if the pregnant woman compensates for an increased loss of liquid by drinking.
If an oligohydramnios sequence has already been diagnosed, the life expectancy of the unborn child is already very low due to possible lung and kidney damage. Since this represents an exceptional psychological situation for the parents and relatives, it is important to offer sufficient support. As a person affected, it can help to open up to such offers of support, which are often offered by hospitals and psychological services. Your own social environment should not be neglected in such a situation. An intact social environment can help to deal with the stress of an oligohydramnios sequence and its consequences.
The first signs of depression should be taken seriously and acted upon. Alternative therapies and relaxation methods such as yoga and meditation can help to gain new courage even during the difficult time of the illness of one’s own child or that of a relative.