Potter sequence is a rare disorder that is sometimes called oligohydramnios sequence—a
term that helps define it because oligo- means low and -hydramnios means amniotic fluid.
So in Potter sequence there's basically very little amniotic fluid and this makes
the uterus a pretty hostile place for the fetus to grow.
Typically, around the 20th week of gestation, the kidneys, ureters, and urethra develop
and start producing and excreting urine, and this urine becomes the major source of amniotic
fluid.
Various conditions like renal agenesis, which is when one or both kidneys are missing, or
atresia of the ureter or urethra, which is where those parts of the urinary tract are
obstructed, can lead to an inability to produce or excrete urine and therefore leads to oligohydramnios.
Other common causes can include amniotic rupture, which is the leakage of amniotic fluid, or
uteroplacental insufficiency, which is where there's low blood flow from the placenta
meaning the fetal organs—which includes the fetal kidneys—see less blood flow, which
in turn leads to decreased urine production.
When there's very little amniotic fluid, a couple of things happen as a result.
First, amniotic fluid is crucial for the development of the fetal lungs, by both helping the airways
physically stretch out as well as contributing amino acids like proline, which helps with
the formation of connective tissue and collagen in the lung.
With less amniotic fluid, though, there's pulmonary hypoplasia.
Hypo meaning under, and -plasia means formation, so the lungs basically remain underdeveloped.
Not only that though, with less amniotic fluid, there's less space in the amniotic sac,
and so the fetus is literally compressed into a smaller space, which causes developmental
abnormalities like a flattened face, wrinkly skin, widely separated eyes with epicanthal
folds, low-set ears, as well as limb abnormalities like clubbed feet.
Potter sequence usually affects boys, since they have a higher rate of urinary tract obstruction
anomalies, like posterior urethral valves.
Diagnosis of Potter sequence is usually done with ultrasound during the second trimester
of pregnancy.
Unfortunately, though, by the time this condition is diagnosed, the syndrome is usually very
advanced and most cases result in a stillbirth, which is the delivery of a fetus that has
already died.
When babies do survive, the pulmonary hypoplasia leads to severe respiratory problems which
often leads to death shortly after birth.
Alright, as a quick recap, Potter sequence, is an often fatal condition that primarily
affects newborn males, and it's caused by oligohydramnios, which is low or absent amniotic
fluid.
Potter itself is actually a mnemonic, and it stands for Pulmonary hypoplasia, Oligohydramnios,
Twisted skin, for the wrinkles, Twisted face, Extremity deformities, and Renal agenesis.
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