Meet the Lamb triplets Ilyanna, Gideon, and sleepy little Willow.
“There is never a dull moment, for sure,” their mother, Trelane Lamb, told Ivanhoe.
The trio has overcome many obstacles together. At 24-weeks pregnant, Trelane was given the news.
“There’s a 30 percent chance that Gideon would make it and only I think a 70 percent chance that the girls would make it,” Trelane explained.
The preemies came into the world at 27-weeks. Gideon [came] first, then Ilyanna, both weighing just over two pounds. Willow weighed only 1 pound 13 ounces. Her fight for survival was about to begin.
“They don’t know why it happened,” Trelane said. “Because the two girls are identical and nothing is wrong with Ilyanna, but Willow had all these issues.”
It began with a rectovaginal fistula.
“They came in and said, ‘Well she doesn’t have an anus,’ and [I said], ‘what does that mean?’ They [said], ‘We are going to have check but she does not have a hole,’” Trelane explained.
At 3-days old, a colostomy was created for her anorectal malformation and a G-tube was placed after further testing revealed her esophagus was not connected to her airway.
“She had a tube down her throat that would suck out basically all of her secretions so that she could breathe because she could drown,” Trelane said.
Eight months later, Dr. Harold Lovvorn first performed surgery on her esophagus to bring together the upper and lower ends.
“If your esophagus or food tube in interrupted, obviously [you] can’t eat,” Harold N. Lovvorn, III, MD, Pediatric Surgeon, Vanderbilt University Medical Center, Nashville, Tenn., told Ivanhoe.
What he’d do next was a first in the U.S. Instead of another major surgery to connect them, he used specially designed medical magnets.
“It saves the baby a lot of additional pain, big scars, and big operations,” Dr. Lovvorn explained.
The non-invasive procedure takes 20 minutes.
“We can place them through her mouth and then through her gastrostomy tube up the lower esophagus, so that they would have their physical attraction for one another,” Dr. Lovvorn said.
“It’s just a matter of sliding it up this way,” Dr. Lovvorn explained.
The magnets wear away at the tissue between them until they connect, creating a perfectly aligned opening between the two ends of the esophagus. This allows food, saliva, and liquid to flow into Willow’s body.
“We think her prognosis is excellent,” Dr. Lovvorn said.
BACKGROUND: Esophageal atresia is a congenital defect in which the child’s esophagus does not develop in the right way. For most babies, it means they can’t eat. In most cases of esophageal atresia, the upper esophagus, instead of connecting to the lower esophagus which goes on to the windpipe and stomach, just ends. Other cases can involve simply the narrowing of the esophagus. It’s estimated the defect occurs in about one out of every 4,000 births. It’s also generally associated with other conditions as well, including tracheoesophageal fistula, heart problems, and other digestive tract complications. (Source: www.nlm.nih.gov)
SIGNS: The defect is considered a surgical emergency, and as such any of the signs need to be seen and reported as soon as possible. Some of the signs include:
* Frothy bubbles in mouth
* Blue color to the skin, especially when feeding
* Difficulty breathing
* Coughing or chocking during feeding
* A round, full abdomen
DR. LOVVORN: “The magnets are interesting because we could place them through her mouth and then threw her gastrostomy tube up the lower esophagus so that they would have their physical attraction for one another. They have to be within 4 centimeters in order to have that attraction. Fortunately her two pouches were still together so they didn’t have that much distance to overcome. They had about 7 millimeters is what we determined that they needed to overcome which was the wall of each respective esophageal pouch and scar tissue that had formed. And so really the way it works is it applies about 4 pounds of pressure between the magnets and that pressure over time gradually compresses the intervening tissue causes ischemia and within about 48 or 72 hours that ischemic tissue stuffs. Then a channel is created between the upper and the lower esophagus. So really it’s quite straightforward, we don’t have to go back reopen her chest, we don’t have to dissect the esophagus. Probably most importantly is we don’t have to remove any esophagus that had been induced to grow from her initial operations. So basically it’s a purely intraluminal way to canalize the esophagus without having to mobilize, reset, jeopardize the integrity of the remaining esophagus.”
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Harold N Lovvorn, III, MD, Pediatric Surgeon, Vanderbilt University Medical Center, talks about saving babies with magnets.
Can you tell us a little about esophageal atresia, what exactly is that?
Dr. Lovvorn: It’s a rare condition and esophageal atresia as a spectrum of abnormalities occurs in about one in 3000 live births. It has a variety of forms: it can be isolated, so it’s esophageal atresia alone or can be connected to the airway in either of a number of places. Either the main airway with a distal fistula where it goes right down to the stomach or more proximal as well, but when it’s esophageal atresia alone, that’s a very rare abnormality and it accounts for about 8% of all sophageal atresia cases.
What are some of the complications with that?
Dr. Lovvorn: Well, if you’re esophagus or food tube is interrupted, obviously we can’t eat, and so there’s disruption of the normal flow of food from the mouth to the stomach. It also predisposes to pooling of secretions and chronic aspiration and chronic pneumonia. And, in cases where there’s a connection between the esophagus and the airway in the form of a fistula then that predisposes to reflux of stomach acid into the airway and that can cause chronic pneumonitis and irritation of the lungs if not corrected surgically.
Can you tell be about Willow’s case.
Dr. Lovvorn: Willow is a fascinating case of esophageal atresia where she was the product of a triplet gestation born profoundly preterm and interestingly she has one fraternal brother and then an identical or monozygotic twin. There is discordance between the two identical female twins for the birth abnormalities that Willow has. The other twin Ileana is entirely normal without any appreciated or detected abnormalities, whereas Willow has a condition called VATER where she has an abnormality of her esophagus, her anus, and then also she has a spinal abnormality too.
Dr. Lovvorn: So they were born at 27 weeks gestation, and Willow weighed less than 900 grams, so she was quite tiny.
How big would that be?
Dr. Lovvorn: She would basically fit in the palm of your hand, pretty much, very fragile but thanks to the expert neonatology team that we have here at Vanderbilt, all the triplets did great and especially Willow despite the number of abnormalities that she has, the neonatology team did a great job with her recovery as well as the other two triplets.
Can you tell me some of the challenges that Willow faced when she was born?
Dr. Lovvorn: So, Willow was determined very early on to have hypersalivation and in the process of trying to clear her secretions, the nurses were unable to pass what’s called an orogastric tube a tube from the mouth to the stomach to decompress the stomach. And in the process, she had a breathing tube placed, and it was determined subsequently that she had esophageal atresia without a distal fistula, which means there was no connection from her lower esophagus to her airway. So she had a primary esophageal atresia. It was also determined that on inspection of her bottom or her perineum that she had an anal and rectal malformation of the female type called a rectal vestibular fistula and that would require colonic diversion. So, she needed two surgeries early on after birth. One was access to her stomach for feeding, for enteral nutrition, and then the second was a colostomy, a temporary colostomy until we could repair her bottom. But none of these procedures, meaning the definitive corrective operations, can be performed when the babies are that small. It’s just too risky to have complications afterwards.
What were her chances of death?
Dr. Lovvorn: Well, we categorize the babies with esophageal atresia into three groups really and there are babies who weigh above 2500 grams, those between 1500 and 2500 grams, and then those less than 1500 grams. So obviously Willow fell in the higher risk category, being the tiny baby she was. Added to that risk is if the baby has a cardiac abnormality; fortunately for Willow she didn’t have a cardiac abnormality in the sense that it compromised her cardiac function. However she did have some anomalous aortic arch developments. So her aortic arch was on the right side instead of the left side, so that made it a little bit more technically challenging to approach her esophagus.
So how do the magnets work?
Dr. Lovvorn: Well, the magnets are interesting because we could place them through her mouth and then threw her gastrostomy tube up the lower esophagus so that they would have their physical magnetic attraction for one another. They have to be within 4 centimeters in order to have that attraction. Fortunately her two pouches were still together after we approximated them surgicaly so the magnets didn’t have that much distance to overcome. They had about 7 millimeters is what we determined that they needed to overcome, which was the wall of each respective esophageal pouch and scar tissue that had formed. And so really the way it works is the magnets create about 4 pounds of pressure between the ends and that pressure over time gradually compresses the intervening tissue, causes ischemia, and within about 48 or 72 hours that ischemic tissue sloughs. Then a channel is created between the upper and the lower esophagus. So really it’s quite straightforward, we don’t have to go back to reopen her chest, we don’t have to re-dissect the esophagus. Probably most importantly is we don’t have to remove any esophagus that had been induced to grow from her initial operations. So basically it’s a purely intraluminal way to canalize the esophagus without having to mobilize, resect, and jeopardize the integrity of the remaining esophagus.
Is it like a barrier kind of thing?
Dr. Lovvorn: Well, yeah it’s just a natural healing response to protect her the scarring that occurs with the ischemic tissue, meanwhile the coupling of the magnets where they’ve come together that tissue dies and sloughs until the magnets are entirely together.
How big of a breakthrough would you say this is?
Dr. Lovvorn: It’s quite innovative in the sense that we have a notoriously challenging problem with long gap or isolated esophageal atresia. Isolated esophageal atresia while in some cases we can over time stage it and gradually get the ends together it requires the baby to jump through a lot of hoops. There are a number of different techniques that have been developed to try to lengthen the esophagus so that a surgeon can preserve a patient’s full esophagus. If we can’t do that at the initial operation, we have to perform a variety interesting maneuvers where we take a little piece of colon and move it up to interpose between the two esophageal pouches. Some surgeons will use small bowel, other surgeons will just replace the entire esophagus with the stomach and simply pull it up to replace the esophagus. While those other three options are good and satisfactory, they don’t restore the normal function of the esophagus, so we would prefer functionally for the child in the long-term, and as they enter into adulthood, to have all native esophageal tissue. So the magnets facilitate that process whereby there’s one initial thoracotomy to appose the tissues and then the second procedure is all endoluminal. We don’t have to re-explore her chest. We can just do the work on the inside with the magnets. It saves the baby a lot of additional pain and big scars and other open operations.
So what’s her prognosis then?
Dr. Lovvorn: Well, we think her prognosis is excellent. We have a recent esophagram x-ray study within the last week or two and the radiologist called me and said, “hey, I just did the esophagram on Willow, and it looks essentially normal, I can’t see any evidence of stenosis or anything.” That was incredibly rewarding for us. Not for our gratification but to know that this baby’s esophagus has been almost completely regenerated and restored with this approach. Now, I do have to mention that the magnets are relatively small so their coupling diameter is only about 10 French, which is maybe the size of the opening of the pen here. And what that means is there’s a natural waste like an hourglass after the magnets are finished and so we can’t leave the baby with that degree of narrowing. That waste has to be dilated. And we anticipated that, so she had routine scheduling of weekly esophageal dilation’s, four consecutive, and then she had I believe it was two more routinely interspersed as she was growing to make sure that, that area kept its full diameter, her luminal diameter with the rest of her esophagus. And now she’s a little more than a year old and I think we have accomplished a full luminal diameter without having to re-explore her chest.
So she’s eating and she’s doing everything that a one-year-old will be doing?
Dr. Lovvorn: Well that’s interesting. No, not exactly. She has a couple of complicating features for that. One, she was premature. A number of premature babies have issues with their progress of eating. Interestingly her two triplets as far as I’m aware don’t have any trouble eating. She has had some trouble, she’s had catheters in her mouth and so forth I think that is mostly just her needing to learn how to eat and swallow. She can do it, she certainly manages all of her saliva, swallows that down without any problem. Babies do have issues when they have esophageal atresia, they do have issues sometimes with peristaltic function of the esophagus. Meaning that how well the food bolus progresses down. And because it was interrupted and we’ve put it back together doesn’t necessarily mean that function always is normal. So she still has some learning to do from that perspective but I think the mother can probably give you a little better update as to where she stands with her eating.
Anything you think we missed?
Dr. Lovvorn: No, I think it’s most important at a centers such as Vanderbilt where we have a number of outstanding physicians, we have dialogue about different problems, these rare cases these aren’t things that you can just take care of at an outside community Hospital without all of the investment into resources and trying to provide the most innovative care that one can or that an institution can. I think we as a multi-disciplinary team, had a number one of our interventional radiologists help with a number of these procedures. So the multidisciplinary approach, the willingness to apply knowledge about a technique to this specific scenario or case I think is really what was innovative for this child.
While this is the first time it’s ever been done, right?
Dr. Lovvorn: Willow is the first child to have this procedure in the United States. There had been a report of nine patients performed in Argentina and Dr. Zaritzky is the physician who developed this technique. And so we contacted him and said, “hey, what has been your experience since that paper was written about”? It was about five years ago. And he said that they had not tried it here in the United States and they were looking for a case in which it could benefit a child. Magnets have been used in the past historically, they had been used and perhaps maybe not in the proper patient selection and not with this level of understanding of how they actually work. That the engineers at Cook Medical have been very diligent about researching the pressure in the force and so forth to make very safe for these little babies and it was really just a matter of identifying the proper patient in whom to try this procedure.
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