Chief of Pediatric Surgery at the Rocky Mountain Hospital for Children, Dr. Steven Rothenberg talks about fixing a premature baby’s separated esophagus with a new procedure.
Interview conducted by Ivanhoe Broadcast News in 2022.
Tell me about the first time you met Harper.
ROTHENBERG: Met Harper almost two years ago, and she was a severely premature infant. She was 23 weeks gestation and shouldn’t have even survived delivery. She was tiny, she was around 300 grams, which is about a third of a pound and she was just barely hanging on to life.
So just with those stats, that’s a hard life to survive.
ROTHENBERG: Without anything else going on, her survival was probably less than 50 percent.
And then she had another problem?
ROTHENBERG: She was born with something called esophageal atresia. That’s where the esophagus, which is your swallowing tube and your trachea, which is your airway tube, develop out of a common channel. In her case that didn’t completely develop and so the two ends of her esophagus were separated by quite a bit, she could not swallow whatsoever. She would never be able to eat with that.
How long until you could do something?
ROTHENBERG: She was too small to do the operation, to try and bring the two ends of the esophagus together. There are different ways of managing this that people around the country use. Even if she’d been full term, it would have probably been a month to six weeks before we would have tried to bring the two ends together. She was so small,so we couldn’t do anything. Normally, what we do in the interim while we’re waiting for the babies to grow a little bit to try and stretch the two ends together is we put something called a gastrostomy button into their stomach. It’s a device that goes directly through the skin and into the stomach so that we can feed them.
Is that what people know as a feeding tube?
ROTHENBERG: Yes. Basically, this is called a gastrostomy tube. A feeding tube can go down through the mouth but this is only so we could access it, but she was so small I couldn’t do that. I needed to let it grow for about a month before I could even put a tube into her stomach so we could feed her so in the meantime, we just had to feed her through her veins.
How long did it take until you could go in for that first surgery?
ROTHENBERG: I think we had to wait probably for about two months.
Was that every day not knowing if she was going to make it?
ROTHENBERG: Yes, especially during the first month. Then once she got that, she was to the size of what we normally see in a premature infant. She was getting a little bit bigger, about two pounds and she was growing and seemed to be doing quite well, considering how premature she was. We let it grow till she weighed about three kilos, which is about seven point five pounds. We knew that the gap between the two ends of her esophagus is something we call a long gap so that means that we talk about it in terms of centimeters or vertebral bodies but she had a gap of about six to seven centimeters or vertebral bodies, which is quite a long gap between the two ends.
How big is that?
ROTHENBERG: It’s quite a gap and when there is a short gap, we usually can mobilize the two ends of the esophagus in surgery and stretch them to get them together. Even with those longer gaps, we’ve developed techniques to in most cases be able to get those together. As opposed to many places in the world, we do all of this minimally invasively so it’s all done through a couple of small quarter-inch incisions with a special scope and special instruments.
And you went in for that first surgery but you couldn’t quite get it together?
ROTHENBERG: Right. It wasn’t surprising. Again, she had about seven vertebral body gap, which is quite long. We’ve got the two ends almost together, but there was still about a one-inch gap between the two ends. The technique we use now is bringing those together under a little bit of tension and then we come back in a few days. Generally that tension is much less than we can often get the two ends together then in order to seal them together.
Is that what you did. You were waiting for it?
ROTHENBERG: Yes. We did the first surgery and it went perfect. She did great for about 36 to 48 hours and then she got incredibly sick. We know that she has some systemic disease and pulmonary hypertension, so high blood pressure around her heart and lungs is when she gets stressed out. It causes a very bad reaction and she got incredibly sick. The surgery itself took less than an hour, but she got so sick that it was impossible to operate on her again. Only to the credit of our neonatal team, the doctors and the nurses, did she survive all of this, it was a heroic effort. After about a month, there were a lot of things that went on the interim, but she was stabilized and started to get to the point where we could start thinking about doing the surgery again.
Did you do another surgery?
ROTHENBERG: I did. It was about six weeks after the initial surgery and again, normally we would have done it four or five days later. I went back in and mobilize the two ends and this time I could get them just touching. Normally what I would have done is sewing them together. There was a little bit of tension and I didn’t want to risk trying to do it under that tension but I was so worried that the same thing was going to happen to her again. I was afraid not to sew the two ends together and I went ahead and did that, but the two ends were really under too much tension and I could only get about two-thirds of it connected and I was still stuck with a a big hole in the part of the esophagus. I did something called a plural flap, but I took part of the lining of her chest wall and laid it over like a band-aid to cover it in the hopes that the two ends would stay together and it would heal and we’d at least have her esophagus in one piece.
Then there was trouble again?
ROTHENBERG: Then there was trouble again and then she got sick again, not as sick as the first time, but sick enough so that it took a couple of weeks for her to recover.
What was your next step because you still had a hole? And your whole goal is to get these kids to?
ROTHENBERG: We let her heal from all that and we did a study and what it showed is that the two ends, were about a centimeter. It’s a third of an inch to a third to a half inch apart. The part and it was in continuity now, had a tube through it but there was a very tight stricture there. I knew that that was a lot of times if we have a very short stricture, we could dilate those up. We put a balloon inside from inside the mouth down in, and we could blow in and dilate that, and gradually the inner lining of the esophagus, what we call the mucosa grows over that and allows it to heal and you can treat this stricture that way.
A little bit like a stent?
ROTHENBERG: Yes. We put it in a balloon dilated and it opens it up. Then sometimes it tends to stricture down again, so sometimes we have to do it multiple times. However, I knew hers, it was just scar tissue and there wasn’t enough lining of the esophagus to allow it to heal and I didn’t want to go back into it. Normally I would just go back in and cut out that part of her esophagus but again, I didn’t want to go back into her chest because she convinced me that every time I operated on her that she was going to try to die. So it seemed like a bad idea. There are some surgical magnets that have been developed to try and help hook the two ends of the esophagus together. We haven’t routinely use those. In fact, we hadn’t used it at all, but there was data coming out suggesting that in certain cases it might be a good option. I contacted the company and said, “I want to put a magnet in the upper pouch and in the lower pouch to bring the two ends together and ablate this stricture.” They said, well, “It’s not really what this is indicated for.” From here, we had a lot of discussions about it and we had to get special permission because it was outside of what they’d gotten FDA approval for. We got permission from everyone and we had to put in a special study at our institution. It took almost eight weeks to go through all of that and get permission. When we finally did, we took her to the operating room, but instead of going into her chest, we put one magnet down her mouth and into the upper part of her esophagus above the stricture. Then we put the other magnet through her gastrostomy tube side, the inner stomach, and we pulled it because we had continuity across it. We could basically put a guide wire when we pulled it up and put it in the lower part, so now we had a magnet here and a magnet here and there. They were about a half inch apart. Then ver the next few days, the magnets gradually attracted each other and met, which is the idea behind the magnets. The tissue in between was ablated and now we had the two ends of the esophagus together, and then you just pull the magnets out through the mouth.
It’s like you tricked Harper.
ROTHENBERG: Yeah, I did. I finally got the upper hand. Even after that, she still had the same reaction, but this time, it only lasted a couple of days.
Now, she can swallow, she can eat?
ROTHENBERG: Obviously, she went home just after her first birthday. She spent the first year of her life, granted she was born five months premature, the first year of her life in the hospital. She went home just after her first birthday. I just saw her back two weeks ago, and she’s taking about half of her food by mouth. She still gets food through the gastrostomy tube. She has a lot of learning to do, and a lot of catch-up, and she has a twin and you really have a good comparison to see how she’s progressing and doing, but It’s amazing. The fact that she is alive and able to eat is truly a miracle.
You spend a year with this little girl, do you feel like she’s basically a part of your family and you’re saving a family member?
ROTHENBERG: Yes. You get attached when you have that much investment, and you work so hard and not just me. If you talk to the nurses up in the NICU, and eventually, she was in the pediatric intensive care unit because she got too big for the NICU. Ttalk to the doctor who took care of her, they all have an investment in her and her folks. She has great folks and that’s so much to undertake, especially when you have another child, another twin. They were incredible, so yes, she’s part of our Rocky Mountain Hospital family.
And should she as she grows, grow normally now and catch up and be fine?
ROTHENBERG: As best I can tell, you always worry with a premature that they may have neurologic sequelae growth, but she’s almost as big as her twin. There’s no question that she’s going to be able to eat everything by mouth. We haven’t had to do anything to her esophagus in almost a year. She did get, one or two dilations after because the magnets are small, so they make a small communication. But we haven’t had to do anything at all so from a surgical standpoint. Hopefully, I’m done with her and she’s done with me. Now, it’s just a matter of developmentally catching up and growing, but yes, I’m very optimistic.
How long did you leave the magnets in?
ROTHENBERG: The magnets, in her case, took about three days to come together. We pulled them out, I think on the fourth day.
So it keeps it together just by making that contact, it keeps it together?
ROTHENBERG: Yes.
How is that?
ROTHENBERG: What happens is that it’s a compression, so the two ends, normally they leave them in 5 to 7 days. I think we may have left hers in five days. What happens is when the magnets get together, it compresses the tissue together, and causes what we call necrosis, which is that tissue to die and go away from compression. But that also causes an anastomosis, so the tissues meld to each other.
That quickly? I would think it would take months.
ROTHENBERG: No.
Anything I’m missing? Do you think she the smallest to undergo this and survive?
ROTHENBERG: Yes. I’m pretty sure I did a search and I can’t find it, and I’ve traveled all over the world teaching people about esophageal atresia. I’ve talked to people about Harper, but I’m pretty sure she’s the smallest baby ever born with esophageal atresia to survive. I believe all these surgeries should be done minimally invasively, otherwise you end up doing a relatively large incision on the kid’s chest. Those patients grow up to have significant morbidity from having the muscles of the chest either cut or stretch, and they develop scoliosis, chest wall deformities, shoulder girdle weaknesses, and we know that up to 40 percent of patients who get a thoracotomy as an infant will have those problems. One of the things that Rocky Mountain is known for is that we pioneered all these techniques to do them minimally invasively. The hardest part of that operation is sewing the two ends together and that’s what I’ve spent the last 20 years trying to teach people to do. It may be that all we need to do is mobilize the two ends minimally invasively to get them close, and then let the magnets hook them together, make the anastomosis. This is the hardest part, which is what I think keeps a lot of pediatric surgeons and other institutions from doing this operation. If we could make it easy to hook the two ends together, we might be able to eliminate the thoracotomies that are being done around the world.
END OF INTERVIEW
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