Transcription:
Treatment for Vascular Ring

Melanie Cole (Host): The vascular ring is an abnormal formation of the aorta. It’s rare and accounts for less than 1% of all congenital heart problems. However, getting diagnosed and treated quickly can prevent serious complications. My guest today, is Dr. Peter Manning. He’s a Washington University Pediatric Cardiothoracic Surgeon at Saint Louis Children’s Hospital. Welcome to the show, Dr. Manning. What is a vascular ring, and what causes it?

Dr. Peter Manning (Guest): Okay, so, a vascular ring is when there are blood vessels that form a ring – or completely surround the trachea and the esophagus. And what that can result in is problems with breathing if it squeezes on the trachea too much – usually noisy breathing, or swallowing problems if it squeezes on the esophagus. It can squeeze on both at the same time.

Melanie: Is this something that would be noticed at birth or shortly thereafter, or can it sometimes hide itself until a little bit later in life?

Dr. Manning: Either situation could be possible. Probably half of patients are diagnosed in the first months of life. Very rarely, right at birth, even though everybody with this anatomy is born with this anatomy. Some children are more symptomatic than others, and then some, the symptoms are mild. The tricky thing is, though, the symptoms mimic a lot of more common problems. Many times, no one really thinks about a vascular ring when a child has some noisy breathing or is having some vomiting or problems eating or swallowing because there are many other more common reasons for these things to be happening in babies.

Melanie: What are some other symptoms, and what should a pediatrician be looking for? If you say that vomiting or some of these issues they might right away assume reflux or something along those lines. What do you want them to be looking for that would signal that this could be the situation?

Dr. Manning: It’s a tough question to answer because a lot of times the symptoms the children have with a vascular ring may not be any more pronounced or dramatic than some of the other more common problems that can lead to feeding problems like gastroesophageal reflux. I think the trick is if they’re having symptoms that are maybe more severe than one might expect, or they’re persistent, then things need to be evaluated further.

That’s commonly how we come to discover these problems. It’s not usually because the symptoms are very dramatic and dangerous, it’s just that they’re persistent. It usually leads to a little more evaluation than just trying to say, “Well, we’ll treat them with reflux medicine and see if it gets better.” A lot of times, it comes to somebody’s attention because they’re maybe having some respiratory symptoms that are hanging on, so someone gets a chest X-ray, and the radiologist notices a difference in the chest film that might suggest a vascular ring. Or, they’ll have a barium swallow study with the thought of looking for reflux, and that may show some compression of the esophagus, which would lead somebody to think, “Well, maybe this is a vascular ring, not just common variety reflux.”

Melanie: What is the Kommerell’s Diverticulum? How does it relate to the vascular ring? Is this hard for new parents to understand and visualize?

Dr. Manning: Yeah, I always find it easier to explain these problems with I can draw a picture, or use photographs. Many times, these children have undergone a more advanced testing such as a chest CT scan or MRI, where on the computer they can actually develop three-dimensional reconstructions of the child’s own anatomy that we can demonstrate on the computer. Having those visual aids honestly makes it pretty easy to explain to parents why there’s a problem, but the underlying issue is that the blood vessels formed in a little bit unusual variation from normal.

What’s really interesting is that actually, everybody actually started having a vascular ring early in the development of the aorta and blood vessels because the aorta normally starts by leaving the heart and then a portion goes around each side of the trachea and esophagus, coming back together. But part of the right arch disappears early in development, and that leaves everything swinging to the left and staying in front. It’s when there’s some variation in that normal sequence that we see a vascular ring.

Vascular rings come in two most common varieties. One is the persistence of a double arch, where neither side fully went away, and in most case, it’s the right side that stays large, and the left side gets small, but there’s still part of the aorta that wraps around each side.

The other that’s probably a little more common, is the one that is also associated with what people call a Kommerell’s Diverticulum. The aorta swings to the right instead of the left and one of the blood vessels that should come off first and stay in front comes off last and goes behind the esophagus. That’s what we call the left subclavian artery, which is the artery that supplies the left arm. The first part of that artery as it leaves the aorta is unusually fat, and that’s what we call a Kommerell’s Diverticulum. It’s there because there’s a blood vessel on the left side that completes the ring -- that is something we call the ductus arteriosus that normally closes in the first days of life and becomes just a ligament. That creates the connection that completes the circle around the trachea and esophagus.

Melanie: Dr. Manning, what is the goal of surgery, and is there a variation in treatment approaches?

Dr. Manning: Yes, the goal is to relieve the compression on the trachea and esophagus. If we envision a vascular ring as blood vessels creating an “O” or a circle around the trachea and esophagus, we can break that circle at least at one point. We’ve relieved things to a certain extent to allow the trachea and esophagus to expand out to one side and not be compressed. With the double aortic arch varieties, it’s typically on the left side, the smaller of the arches we can divide, as well as the ligamentum arteriosum. That really almost creates an aortic anatomy that’s a mirror image of normal where everything swings to the right and stays in front, rather than in a normal situation swinging to the left and stays in front.

Now, with the right aortic arch with an aberrant left subclavian artery – that’s the variety that has this Kommerell’s Diverticulum – if you divide the ligament, which goes from the Kommerell’s to the pulmonary artery – that used to be the ductus arteriosus – you’ll open the ring, but you’re still leaving blood vessels that are surrounding the esophagus and trachea about two-thirds to three-quarters of the way around. That’s been the typical treatment that most people have employed, but what people have found out in following these patients, some of them will have persistent symptoms when you only open things on one side, and there are still blood vessels wrapping around two-thirds of the way.

The alternative is to add an additional step to the operation where we can remove the Kommerell’s Diverticulum -- the fat, first part of the subclavian artery -- and then move that left subclavian artery from behind the esophagus to the front and attach it to another blood vessel that’s coming off of the aorta -- something we would call reimplantation of the left subclavian artery. In doing that extra step, which is something that I prefer to do routinely in these cases, we think we’re lessening the chances that they’ll ever need a reoperation due to recurrent symptoms, which has been seen in a certain percentage of people when that step is left out.

Melanie: Do you take this different approach – are other cardiologists and cardiothoracic surgeons taking the same approach?

Dr. Manning: Yeah, I think some are. I think the majority are not. For many years, the traditional surgical treatment for these patients with a Kommerell’s Diverticulum, particularly for the younger children, would be just to divide the ligament and make sure there's plenty of space off to one side and see if they have recurrent symptoms. Many of them won’t. Many of them will do just fine with that approach. But, if you do have recurrent symptoms, then it’s a second operation that sometimes can be more difficult when somebody’s already been in that area operating before.

The addition of this step is part of the first operation. Certainly, we wouldn’t consider it if it was relatively complex or it added risk to the procedure, but it’s usually a pretty straightforward thing to add on to the first operation – when you’re there for the first time when the anatomy is all fresh and not previously dissected. Because the risks are relatively low, I usually have taken that approach. I know a handful of other surgeons who have a lot of experience with vascular ring surgery around the country also lean towards doing this more routinely.

Melanie: Are there any new advancements that you’d like to discuss or research being done to improve treatment outcomes?

Dr. Manning: No, I think the one thing that’s interesting is that it’s usually looked at as a relatively simple problem once you’ve identified it. The key is in primary caregivers, or the first line of consultants that are sometimes asked to look in on these children -- like gastroenterologists or ENT surgeon – is to keep a high level of suspicion for something like this so that we pick up on it early before it becomes a more chronic problem. But usually, the thing that I like to emphasize is just have a high index of suspicion that something else is going on if a child is having persistent symptoms or symptoms that are out of normal range for what they would normally see.

The treatment, really, has not evolved a whole lot other than the addition of this Kommerell’s Diverticulum resection and reimplantation as a more routine practice. It’s certainly, in my experience, it’s far easier to do when a child is younger. Their anatomy may be smaller, but it’s a lot closer together, and it’s easier to reach with our usual approach with going between the ribs. There’s some variations of anatomy that put a child at a higher risk for having recurrent symptoms in the future.

I think the addition of the extra step is likely to also reduce that risk in the future. There’s unfortunately, not a lot of long-term follow-up for patients with vascular rings because the vast majority of them do fine, and there’s not one group or specialty that tends to follow them routinely. They tend to be followed primarily by their primary pediatrician or family practitioner, so people haven’t done a lot of investigation in the long-term outcomes of the different approaches. That’s something that would be interesting to look into in the future.

Melanie: And what can a pediatrician or other provider expect from your team at Saint Louis Children’s Hospital, after referring a patient to you?

Dr. Manning: First, we may want to do some additional evaluation studies. Most kids, by the time this has been discovered, have already had most of the testing that they would need for moving ahead with planning surgery. Typically, any child with symptoms related to one of these should have an operation. The anatomy is not going to go away, and I usually like to think the younger they are, the more time that they have to remodel. We’ll meet with the family and review these studies that have been done. If there are any additional studies that need to be done, we may suggest those. Sometimes they can be done as an outpatient. Sometimes, some things like bronchoscopy -- which can be helpful, also, to look at the airway – can be done at the time of the operation to fix the vascular ring. We’ll typically meet with the family and go over all of this information and counsel them on the different surgical options, and what our recommendation is for managing them.

Then, the operation itself in most situations is done with an outpatient admission. They come into the hospital on the day of the surgery. The operation itself takes about two hours, and most kids, even the youngest all the way up to the handful of teenagers that I’ve dealt with, usually stay in the hospital about two or three days after the surgery. Other than some restrictions while they’re healing their incisions, they’re not put on a whole lot of restrictions afterward as they’re convalescing.

Melanie: Thank you, so much, for being with us today, Dr. Manning. A physician can refer a patient by calling Children’s Direct Physician Access Line at 1-800-678-HELP, that’s 1-800-678- 4357. You’re listening to Radio Rounds with Saint Louis Children’s Hospital. For more information on resources available at Saint Louis Children’s Hospital, you can go to SaintLouisChildrens.org, that’s SaintLouisChildrens.org. This is Melanie Cole. Thanks, so much for listening.