Today our guest is Dr. Jose Pairo, a pediatric surgeon at Cincinnati Children's, which has
the nation's highest volume photoscopic center.
Dr. Peiro also specializes in neonatal and fetal malformations, and he is the endoscopic
fetal surgery director in Cincinnati Children's Fetal Care Center.
My first question is, why do we even think about fetal surgery and why do we offer it
to our patients?
The answer is very easy.
It's just because we have prenatal diagnosis.
So in other words, we can detect things in utero very early in gestation.
And fortunately, we have very good tools for prenatal diagnosis.
The most important is the prenatal ultrasound.
The conventional ultrasound at mid-gestation, around 20 weeks, will be the best.
So most of the obstetricians and MFMs can catch most of the malformation at that point.
So the rate of the detection of this malformation is very high in the developed countries.
Obviously, with ultrasound, we have the 3D and the 4D modalities that basically we can
see the surface.
In this case, we can detect if it's a cleft lip plate or things like that.
And we can also see the movement, which helps us analyze how these babies move their legs,
knees, and the flex and extension rates of the ankles, which is important for conditions
like spina bifida.
With Doppler, we can analyze the modinamic status of the baby, taking the flows in the
umbilical artery, in the ductus venosus, and also in the middle cerebral artery.
We can have a good assessment of how the baby is doing.
Finally, we have a fetal MRI.
MRI can contribute a lot to define the diagnosis after the ultrasound.
Basically defining on the brain, chest, and abdomen, providing a good health for the find
the condition and counseling of the parents.
So we know that we will detect many of these malformations and we know that some of them
will be lethal or will have postnatal severe disabilities.
So that's the goal of fetal intervention, just try to rescue or improve these conditions.
So what happens after the detection?
For instance, in gastrosclerosis, I have good experience just moving a little bit earlier
so we can reduce the injury on the bowel so we have less serositis and better recovery
in these babies.
Let's talk a little bit about what kind of surgeries they do at Fetal Care Center here
at Cincinnati Children's.
Open fetal surgery is the most similar to neonatal surgery.
So we have the patient in our hands.
We can operate in a fetus the same as we operate in a preemie.
The only problem is we need to open the uterus.
Fetal surgeons use dissolvable staples to limit the bleeding and maintain stability.
But an opening in the uterus produces a scar and that also can activate the dynamics because
the uterus is very irritable.
And sometimes, even though we give a lot of tocolysis to the mom, preterm delivery happens
a few days after the surgery even if the surgery was a success.
For that reason, all the fetal surgeons were trying to go away from fetal surgery, trying
to look for minimally invasive surgery.
So in what conditions a fetal surgeon might offer fetal surgery?
Open fetal surgery still is used for large solid masses in the chest, for sacro-cociate
thratomas and also for spina bifida.
Let's start with fetal lung lesions.
For example, CPAM.
It's very easy to identify because we can see a multi-cystic component in the lungs of
these fetuses by the ultrasound.
Obviously, we need to be very careful about the microcystic, the solid CPAMs, because
the growth is very fast.
And sometimes you can see how the mass can grow and make a shifting of the mediastinum
compression on the other lung and also the heart.
So the next consequence of this rapid growth will be hydropts.
We can see ascites in the abdomen, pleural effusion in the lungs, the scalp edema.
So it's going to be a complete onasarca.
So hydrosfetalis is very close to be complete a fetal demise.
That is the usual outcome of these babies if we don't do nothing.
So how we can anticipate the babies are having risk for hydropts and demise and not.
We use the CVR.
CVR is calculated by the volume of the CPAM, width times height times length times 0.523
and divided by the head circumference.
The cut-off is 1.6.
Everything that measures less than 1.6 is very, very low risk of developed hydropts.
On the contrary, more than 1.6, we have high risk of hydropts and complications.
And for this condition, the first line of therapy is steroids, maternal intramascular
betamethasone, like the one we use for lung maturation in a preemie.
Steroids can be injected to mom in one, two, or even three rounds weekly.
So we can decrease the risk by decreasing the CVR.
It's a good rescue in more than half of these babies.
So the other half, or 40%, probably don't respond very well.
The only possibility before 30 weeks is just to put a shunt if it's a cystic component.
But if it's a solid, the only way is to do open fetal surgery and do a lobectomy.
The other condition, sacro coccidial teratoma, is pretty similar to this.
We can detect in the ultrasound and also in the MRI very easily.
It's important to analyze if this component is more cystic or more solid.
It are more dangerous because they're more rapid growth and also the vascularization
that we can analyze with the dopplers.
The classification of Altman that we use postnatally can also serve in the analysis of these MRIs.
Just to tell you what type of teratoma is.
And also we can decide if we need to do something or not.
The other condition that they're working a lot at Cincinnati Children's is spina bifida
and myelomeningocele.
So open fetal surgery is the gold standard.
Still we are offering, but you will see that there are other innovations on the road.
It is required to operate on the first day of life, just to avoid infections and protect
the spinal cord as much as possible.
The problem came from early in the embryo, usually at four weeks of embryonic time.
Normally, the spinal cord is protected by the different layers, but in spina bifida,
it is not protected.
It's completely facing the outside and also open like a book.
That's the first hit, the malformation itself.
But the most important thing is the second hit.
This neural tissue is not prepared to be in contact with the amniotic fluid.
We know the amniotic fluid is rich in meconium, in enzymes that progressively will damage,
will destroy this supposed spinal cord and the nerves.
Also during the delivery, the spinal cord can almost be destroyed.
So it is very important to say that this condition is very progressive in utero.
So we are losing all the neurons that should help this baby to move their legs.
So what happens when we do neonatal surgery for spina bifida?
We need to assume the sequela of this malformation, that basically in the lower part of the body.
We have fecal incontinence, urinary incontinence, sexual dysfunction, orthopedic anomalies,
and also motor impairments, even needing a wheelchair at some point, depending on the
level of the injury.
But also in the head, because the compression of the brain stem produce obstruction.
You can see like a plaque in a bottle, the brain retains that fluid and the build-up
produce hydrocephalus.
At 24 weeks, half of the fetuses have already hydrocephalus.
And for the others, 95% of the time, they will have hydrocephalus later in the pregnancy.
Hydrocephalus is important because these babies will need derivation, some kind of ventricle
peritoneal chanting with all the problems that produce.
And also that generates migration disorders, atherotopia, second in these babies.
To summarize, we can say that the pathophysiology of spina bifida makes the spinal cord exposed
to amniotic fluid, have second anural damage and hydrocephalus.
So how can we stop that?
So we can avoid amniotic fluid touching the nerves and we can avoid this leak of CSF.
That's a rationale of open fetal surgery and also whatever repair in spina bifida.
That's why the mom's trial started just to try to compare prenatal surgery with postnatal
surgery using the same technique they use in neonates.
Using the dura, the layers and the skin, sometimes using patches of the skin.
And then the results show that these babies have a significant benefit from having peritoneal
surgery.
Surgeons reduced at least half of the need for chanting.
They improved the mental scores and the motor outcomes.
In most of the cases, they revert completely the high brain herniation.
For duraplasty, we introduce a rollet patch through the cannula.
So with one stitch, we can maintain in position as a dura patch.
After that, we try to close the skin primarily or sometimes we need to put a patch.
What we can say is that Dr. Pera and his team are creating less maternal morbidity and they're
able to allow the moms to deliver their babies vaginally.
Next one is the exit procedure, which is another kind of open fetal surgery happens at the
time of delivery.
And it's a very interesting strategy that we use when we detect something that can produce
neonatal asphyxia or difficulties for intubation.
We have many indications and cases.
Most likely neck masses that are compressing the trachea, but also others like congenital
obstructions or severe micrognathia may have a benefit from this technique.
We can achieve between one hour and three hours, maintaining the oxygenation from mom
through the placenta to the core to the baby.
So it's a controlled situation.
We don't need to rush to secure the airway.
Obviously we need to paralyze and anesthetize the fetus.
Maternal monitoring and stability is also important.
And we use inhalational anesthetics for uterine relaxation.
Cases like dratomas in the neck, sometimes even in an exit, you can do a straightforward
intubation.
Sometimes you can use rigid instruments or laryngoscopy.
And sometimes even with that, it's not possible.
With the exit procedure, we have time to do a partial resection of the theratoma, identify
the trachea and then intubate the baby.
So then once the intubation is done, we don't need to be more on placental support.
So we can clamp the core and do the surgery in next door OR so we can complete this theratoma
resection.
In summary, in summary, fetal surgery is a very important procedure.
So we need to start, what it looks like to do in the inst Mountains, where we really