The Potter's Syndrome. What do you mean versus?
In our previous discussion we just talked about unilateral renal agenesis
now let's go and talk about bilateral renal agenesis.
The story not so good, not so good. Let me walk you through this, you'll like this.
I'm here to give you clinical information,
I'm trying to give you how you can identify your patient, the quickest
so you can come up with the proper diagnosis and implement management when necessary.
The management here, not a whole lot that you can do really, and what's happening?
Well, here both the kidneys are missing.
When? In-utero. In-utero. So, the fetus within the pregnant women is missing both kidneys.
I need you to think of amniotic fluid and its circulation has been very, very, primitive and simple for learning purposes, okay?
Now if you wanna know more, then, you do that on your own time but for right now
for the sake of time, we need to make sure that we go through this effectively.
Okay, so what's happening? Amniotic fluid is what you're thinking of.
This is a fetus. This is a fetus that is going to be consuming the amniotic fluid which contains the nutrients that it requires.
It is then going to "urinate" the amniotic fluid. It is a continuous cycle, a continuous cycle.
Now, what does it mean to have amniotic fluid in your placenta? You call this amnios, right?
Amnios, and you're gonna have two different clinical manifestation upon ultrasound
oligo or too much, poly. So poly or oligo.
Well, you do an ultrasound on this pregnant women and you find that the amniotic fluid, amnios is too little.
What is -- which one is too little? The poly or the oligo? Oligo is too little, isn't it?
You've heard of oliguria, oligomenorrhea, oligohydramnious. So you have oligohydramnious. So, what happened?
Both the kidneys aren't present in the fetus. Not good. So what's happening?
You don't have as much amnioitic fluid that's being "urinated" out.
Think of it as such please so that you never missed this question.
Now, what happens? Now, the devastation begins. What do I mean by that?
This amniotic fluid is not present, the placenta is now crushing the fetus, crushing it.
My world is caving in upon me, everywhere. So, what does that mean?
Flatten faces, recessed chin, low set ears. What about the lungs? Good, hypoplasia.
Welcome to Potter's Sequence.
Not a single thing that you need to memorize here except for maybe the name Potter
but everything else, bilateral renal agenesis, the end of the story.
Oligohydramnios, you have a very small world that this fetus is living in to the point that it's crushing the baby
you're going to have, what we've just talked about where you have flatten faces, recessed chin,
flattened nose and we have pulmonary hypoplasia. Death, not good.
Potter's Syndrome, so what's happening along with this?
Well, I need you to think of development of the kidney as being two stages, okay?
What does diverticulum mean to you? An out pouching.
Tell me a couple of important places in pathology where diverticulum becomes quite common for us?
Well, might have a diverticulum in the esophagus and that's called Zenker diverticulum.
You might have a diverticulum down in the intestine for a couple of reasons
maybe it's Meckel's diverticulum and that would be a remnant of vitelline duct, are you with me?
Or you could have a diverticulum because in the western diet we have lots of consumption of meat
with perhaps only a little bit of fiber, so you're sitting there going -- all of the time, of constipated.
That's a lot of pressure that you're putting in the intestine, could I be any more dramatic.
So what happens in the intestine?
Down by the left lower quadrant you might have, an out pouching diverticulum.
So what is a diverticulum? My question stands, is an out pouching.
So, with the kidney you'll have the ureteric bud.
I want you to close your eyes, think of the ureter, and that is being developed by the metanephric diverticulum.
It is an actual out pouching, ureteric bud that is then going to come in
and it is going to hook up with the blastema, that's your metanephrogenic blastema.
Now, what does that mean to you? The ureteric bud which is the diverticulum,
at least get that out of the way, is going to give rise to the distal portion of the nephron.
So that will be your ureter, that will be the calices, and that will be all the stuff that is going on in the pelvis.
Whereas, the metanephrogenic blastema is going to give rise to the proximal portion of the nephron.
What may then happen if the two are not going to properly meet? And there you have it.
You have a diverticulum which is then going to punch a whole into the blastema
and as long as those two are going to communicate, you have a perfectly formed communicative nephron.
What if it doesn't happen?
Well if it doesn't happen then the kidney is not functioning properly, it is that simple as that.
I need you to know from embryo and congenitally, these two structures and what it represents,
please, as amazing as to how many different times that it will come into play.
Now, at some point when we get into what we're seeing here with the letters
and such for example here's letter A and that then gives you the two different components that are not united.
What two components? The diverticulum at the aortic bud a.k.a. and then the blastema.
And by the time it gets to letter D,
you'll notice that there's perfect communication between the diverticula and the blastema.
That is all that I wish for you to take out of this particular illustration.
If you wanna know further detail, then here it is for your preference.