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Hi everyone, I'm Kim Pribben.

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And I'm Em Gootee.

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We are research fellows at Cincinnati Children's Hospital.

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Today we will learn more about the diagnosis and management of pyloric stenosis with Dr.

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Alex Bondoc, a pediatric surgeon from Cincinnati Children's.

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Pyloric stenosis is a condition in infants where the pyloric muscle becomes thickened

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or hypertrophied, thus creating a much narrower passage between the stomach and the small

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intestine.

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This muscle thickening can block food from passing from the stomach into the small intestine

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and prohibits digestion.

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The classic symptom is projectile vomiting after feeding, followed by signs of hunger,

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which can lead to severe dehydration and weight loss.

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In today's podcast, we're going to review in detail to understand better how to diagnose

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and manage these patients both medically and surgically.

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Let's start with the case.

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Our patient is a seven weeks old male with no past medical history, whose parents reported

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five weeks of vomiting.

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He was initially breastfed, but due to persistent vomiting, the parent tried three different

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formulas without any improvement in the patient's symptoms.

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His vomiting would only occur after feedings and was described as large volume, projectile

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and not bloodier bilious in nature.

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His lab work would show a chloride level of 88 millimoles per liter and a bicarbonate

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level of 35 millimoles per liter.

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So what are the potential medical and anatomical conditions we would want to consider?

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In a child this age, what would be potentially the most common, most likely diagnosis of

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a baby that spits up?

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Reflux.

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G-E-R. Reflux, right?

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Reflux is really common in the first year of life.

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A few other things you may consider initially is a cow milk allergy, gastroenteritis, or

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maybe even a viral infection.

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Those are by far the most common things that you would see in an infant this age.

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You would then want to investigate less common anatomical causes like pyloric stenosis, proximal

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obstructions, or gut duplication, pylorespasm, duodenal webs, or pyloric atresia.

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One of the most important differential diagnosis assessments for this patient is to determine

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if the vomiting is bilious or non-bilious.

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In our patient, the vomiting was non-bilious.

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Don't forget that bilious vomiting should be considered as an emergency in an infant.

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So what would your initial diagnosis of this patient be?

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We'll say let's focus on pyloric stenosis now.

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What are the risk factors for pyloric stenosis?

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There are several risk factors to consider when diagnosing an infant.

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For example, being a firstborn child or a male dramatically increases the infant's

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chances of developing pyloric stenosis.

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Other factors like family history, maternal diabetes, being bottle fed, prematurity, and

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even birth via c-section or exposure to certain antibiotics such as erythromycin can also

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play a role.

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It is also important to remember that pyloric stenosis usually affects babies between 2

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and 8 weeks of age, but can occur any time from birth to 6 months.

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So it's a relatively rare diagnosis, about 4 to 5 in a thousand live births, and the

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preponderance is heavily male.

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And then there have been lots of papers about other risk factors or epidemiologic findings

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related to pyloric stenosis, one of which is socioeconomic status.

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The Journal of Pediatric Surgery published a study in 2017 that took a retrospective

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approach, examining the data from 2007-2012.

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This study reviewed 584 patients with pyloric stenosis and found that African American infants

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presented with higher bicarbonate levels and lower chloride levels.

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That same study also suggested that babies that come from uninsured families also had

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lower chloride and higher bicarb levels upon presentation, and they often had longer times

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between the diagnosis and the operating room, though this delay may be due to the need to

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correct electrolytes.

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And is it a cause and effect relationship?

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Perhaps not, but we're talking adversity.

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This is a patient population you have to be aware of.

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Perhaps they're not seeking care as soon or they've been placed in the waiting room,

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they're not being seen as fast.

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Hard to say.

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When looking at other risk factors, a large-scale study out of Denmark and Holland found that

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pyloric stenosis is 87% heritable.

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This makes knowing the family's history crucial for diagnosis.

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It is particularly interesting that researchers were able to identify a molecular diagnostic

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marker.

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They can now trace specific genetic changes that may indicate the risk for pyloric stenosis.

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After reviewing a patient's history, the next step is a physical exam and ordering labs

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and imaging.

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A key physical exam finding is what is often called a palpable olive in the upper right

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quadrant, which is actually the palpable stenose muscle.

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And now Dr. Bondoc will tell us what are some tips on how to find the stenosed area.

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As you're rolling the hypertrophied muscle over the retroperitoneum or even over the

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spine, so they tell you to warm up your hands, have mom or dad holding them tight so that

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they're not upset.

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And then maybe sometimes you can elicit it, especially if it's particularly large.

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All right.

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So once you're done with the physical assessment, the next step is reviewing the lab results.

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You really want to pay close attention to those electrolyte panels because signs of dehydration

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in those panels are very common and are part of the diagnostic criteria.

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Typically, you'll see issues like hyperboloidemia, hypokloremia, and elevated bicarb levels.

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Addressing these levels as soon as possible is really important as it can become life

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threatening very quickly.

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The cause of these abnormal labs are related to the chronic vomiting and poor nutritional

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intake.

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The loss of chloride and hydrogen ions can lead to hypokloremic metabolic alkalosis.

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Bicarbonate also becomes elevated and the excess is excreted in the urine, which obligates

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sodium loss to maintain electro-neutrality.

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As water loss follows sodium excretion, the patient becomes even more hemodynamically

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unstable.

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There is an association with Gilbert syndrome and pylorextinoosis as well.

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We've had some patients incidentally diagnosed with that disorder when they came in for pylorextinoosis,

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when they had their labs checked.

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Next, we would want to review the imaging on the patient.

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The common practice is to order a standard, non-invasive, non-ionizing ultrasound to help

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the diagnosis.

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When reviewing the ultrasound, the team would want to measure the pyloric channel and the

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muscle thickness.

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Any muscle thickness greater than 4 millimeters and a pyloric channel length greater than 15

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millimeters would suggest the patient has pylorextinoosis.

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Once we have a diagnosis, the next big step is fluid and electrolyte resuscitation, especially

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since these patients can have severe metabolic alkalosis.

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In a 2008 and subsequent 2016 follow-up study published in the Journal of Pediatric Surgery,

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they were able to really identify the specific fluid resuscitation care pathways needed to

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stabilize these patients.

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The treatment plan also reduced the number of blood draws and did not delay the time

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to surgery, which is pretty impressive.

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In this study, the researchers suggest administering a bolus of isotonic saline followed by maintenance

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IV fluids.

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This can be a dextrose 5% and half normal saline, but they wanted you to add 20 mEq

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per liter of potassium chloride.

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It is also important to avoid nasogastric compression because you really want to avoid

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exacerbation of the metabolic alkalosis.

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So after fluid resuscitation, let's say that the baby isn't stable enough to go to the

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operating room.

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How can you medically manage pylorextinoosis?

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You could aggressively observe the patient.

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But atropine is a traditional medical management.

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And it's essentially fallen out of favor because how good pediatric anesthetists are and there

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isn't necessarily a high importance rate between congenital anomalies that would make a patient,

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a baby, a high anesthetic risk.

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When considering all of this and the fact that it is a 45 minute curative operation

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that can be done safely and quickly, you are likely choosing that option.

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In this case, our patient was stabilized and was able to go to the OR.

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Now let's talk a little bit about the surgical management of pylorextinoosis.

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First question would be, do you order pre-op antibiotics?

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According to a 2023 study from Journal of Pediatric Surgery, antibiotic prophylaxis

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may be unnecessary for a pyloromyotomy and may carry unnecessary long-term side effects.

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Next, you'll want to decide if your pyloromyotomy will be done laparoscopically or as an open

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procedure.

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But before we review the surgical setup and technique, Dr. Bondak has an interesting piece

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of history about the pyloromyotomy procedure.

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The first pyloromyotomy was done actually the year before by someone else, but Ramstedt

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was the one who popularized it and a spreader bears his name.

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He used a coffee spoon and he actually intended to do a pyloroplasty but ended up doing a

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myotomy only because the baby got unstable or they could do it appropriately, so he only

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did the myotomy.

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So that's how we figured out the myotomy would be curative.

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That's a pretty interesting use of a coffee spoon.

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Now let's talk more about the pre-op and the surgical procedure.

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Once we choose our approach and get the baby into the OR, the next step is to ensure that

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there is gastric suction in prior to induction.

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We didn't put an NG in, so we were paying the price for that, but what we typically

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will do or what some of the old-school anesthetists and I would assume that they've taught the

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fellows and their junior partners is you will suction the baby while awake in three different

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positions.

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Head down, turn to the right, turn to the left to make sure you've sucked out that

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big atonic bag.

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Why is this step so important?

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Because again, you relax the baby, you have no NG, the baby vomits and aspirates.

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So it's very specific to paroxysmosis because of what's happened metabolically, physiologically

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with the stomach.

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In this patient, Dr. Bondock chose to proceed with a laparoscopic procedure.

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Let's walk through some important surgical techniques and tips.

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Positioning wise, you would want to actually turn the baby perpendicular to the long axis

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of the bed and plan to operate from the baby's feet.

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The surgeon would start by making three incisions, one in the umbilicus, one in the right upper

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quadrant and the other in the upper left quadrant.

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You have the scope at the belly, at the umbilicus and then two stab incisions, right?

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So what size instruments are we using?

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Three millimeter instruments.

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So again, just think about all these things that if someone opens the five millimeter

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instruments that means you got to make a big incision.

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It is important that the full extent of the bovey tip is in the patient and the surgeon

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is able to turn the bovey while moving it.

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You will want to identify the theoretical anatomic demarcation of the pylorus and duodenum

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by identifying the pyloric sphincter and the duodenar jejunal junction.

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You will also generally see one well-defined vein with the rest around it looking like

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regular gastroepiploic vessels.

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If you see where it bows, you can usually see the bulging of the muscle.

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So how do you know how long to make your myotomy?

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This is why you should know how long the channel is because if it's 15, 18 millimeters, that

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three millimeter Maryland grasp or when you open it completely is 13 millimeters.

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If you only make a 13 millimeter myotomy on a 15 or an 18 millimeter channel, you're

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going to have an incomplete myotomy.

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Now that the stenosis has been found intraoperatively, what techniques are used to address it?

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One of the most common, that's the way I was taught to do it, is you take both ends

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of the grasp or the slit, score the pylorus, the cirrhosis surface of the pylorus, find

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one way in with the bovey blade, twist it so you crack the muscle and then you can get

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this spreader.

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And it's got that serrated, gentle jaw on both sides because then it grips the muscle

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and spreads it apart.

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And then you grab both ends of the separated muscle and it's supposed to move independently

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of each other.

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One thing to note is if the myotomy is incomplete, you might feel resistance as you grip the

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muscle or they may still move as if they're connected.

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It also might be a little bloody because it's all done bluntly and oftentimes you'll actually

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see the mucosa bulging through the hole.

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The other thing sometimes we can do is when there's blood or serous fluid, I'll have

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the anesthetist put air down a suction catheter and theoretically looking for bubbles of air

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if you've violated the mucosa.

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I also lay a piece of omentum on top of there.

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Oh right, what about an open pylotomy?

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What technique does Dr. Bondoc prefer?

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So the way I was taught was to do it through a peri-umbilical incision and then you tunnel

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up because remember again, another nice thing about working on babies is you can make a

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small incision but you can stretch their skin and move it around so that you can get all

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the way up to the right upper quadrant through a peri-umbilical incision.

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It is also common practice to use a transverse incision.

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Alright, so what complications and risks would you tell the family about when you get consent

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for either of these procedures?

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When talking with the families, it is important to share that while the outcomes are generally

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really good, some of the risks of the procedure would be an incomplete myotomy, mucosal perforation,

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aspiration and maybe wound infection.

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Other weird things about this operation are incisional hernias actually because you've

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made a stab and typically they don't close the fascia on those stab incisions in the

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left and right upper quadrant.

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I've seen two omental hernias so I always try to close it with at least one little micro-stitch.

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And what about the complication rates?

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The rates of complication after pylormyotomy.

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They're either equivalent or better actually with open because I think to me, lap pylormyotomy

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is an experience and a feel operation because like we said, there's no magical way to

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know exactly how complete your myotomy is because I definitely lose my nerve going towards

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the duodenum and sometimes people will stop short.

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But overall, the risks are rare.

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So what are some post-op care instructions we can tell the families and the nurses after

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surgery?

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I tell them that it is very common for babies to vomit afterwards and it doesn't necessarily

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mean that the operation didn't work.

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And why does this happen?

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My theory is that the stomach is still atonic, right?

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It's not going to remodel itself immediately.

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Sometimes if you're challenging the baby, they still will vomit and that's okay.

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Now once you get into days and complete intolerance of feats, then you have to worry about something

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like an incomplete myotomy.

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Yes, that makes perfect sense because the stomach is getting back on track.

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We would want the patient to take in nothing by mouth initially and then introduce feats

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very slowly.

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And some researchers have demonstrated that following a more flexible protocol, such as

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reducing formula or volume if a baby vomit six times but continuing to feed smaller amounts

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and then increasing as tolerated allows for greater flexibility and decreases the length

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of hospital stay.

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I think it's really important to educate not only the family but also the pediatric

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care team on the hospital floors that some vomiting is expected and is actually acceptable.

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Just follow the protocol and keep trying smaller amounts of feeds and monitor closely.

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It is also important to monitor the patient for apnea and signs of pain.

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Overall, this procedure is a very well tolerated operation.

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Finally, looking at the studies on adults who had a pyloric myotomy as kids, it's

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pretty clear that they don't tend to have major issues like nausea, vomiting, reflux

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or other gastrointestinal problems later in life.

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This suggests that long-term follow-ups are usually not necessary, which is really reassuring

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for both patients and the doctors.

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In this episode, we discussed how to diagnose and manage the care of children with pylorectal

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stenosis.

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Here are a few things to consider.

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During the initial patient presentation, it is really important to get a good family history,

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review electrolyte panels and ultrasound results.

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When determining the next steps in the patient's care, try to get the patient's fluids and

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electrolytes balanced.

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It is really important for avoiding morbidity or mortality by stabilizing them for surgery.

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Surgery can be done either laparoscopically or as an open procedure with similar outcomes.

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And lastly, make sure that the care teams and families understand post-op care.

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Remember, the patients may vomit and it might be expected.

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Adjusting the amount and frequency of feedings will help speed up the recovery.

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Thank you for listening this episode.

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We will cast MD along with Cincinnati Children's Hospital, sharing knowledge to improve child

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health around the globe.

