R-CPD Esophageal Findings

The details of the following photos may support the R-CPD diagnosis, though they should not be considered diagnostic. Take note that all photos are non-channel scope images; that is, the scope is not able to insufflate (blow in) air. The significance: the esophagus is typically collapsed around endoscopes inserted into them and air is pumped in through a tiny channel in order to gently expand the esophagus so that its walls can be seen. Here, the air the patient cannot belch/evacuate is doing that work for us. And the esophagus remains open for extended time, the full duration of the examination. Four findings are being evaluated and compared with normal esophagoscopy images (also without insufflated air to make the comparison valid):

1) Reflux from the lower esophagus, suggesting damage to the lower esophageal sphincter from constant upward pressure trying unsuccessfully to belch.

2) What we call an “aortic shelf,” meaning that rather than an indentation of the medial circumference of the aorta, dilation of the esophagus drapes its mucosa across the upper surface of the esophagus, making a “horizontal shelf.” Keep in mind again that this is without insufflating any air.

3) Continuous patency with very infrequent, partial “clamping” down of the lumen or, often no closure at all, suggesting that there is sustained opening pressure of unbelchable air and/or that the contractile ability of the esophagus is reduced, in similar fashion to what happens to an overly-distended urinary bladder.

4) Upper esophageal dilation in a medial-lateral axis so that the upper esophagus becomes stretched in an exaggerated “oval” rather than a more gentle oval or even “circle.”

Esophageal Stretching by Unburpable Air in R-CPD

This young man has had the classic syndrome of R-CPD lifelong. His esophageal findings at the end of a videoendoscopic swallow study are classic. The esophagus is mostly a collapsed muscular tube in young people, yet his esophagus is widely open on a continuous basis due swallowed air that he cannot burp up.

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Typical view of an esophagus (1 of 4)

Typical view of the esophagus using an ENT scope without insufflated air. Esophageal walls are collapsed and therefore too close to the lens of the scope to allow visualization of the esophageal wall.

Lower esophagus (2 of 4)

Using ENT (not GI) scope in this young man with R-CPD to view the lower esophagus, which is continuously “open” due to un-burped air. H = heart; Ao = aorta. Blue water previously administered, moves up and down between stomach and lower esophagus due to lower esophageal sphincter incompetence from years of bloating.

Stretched mid-esophagus (3 of 4)

A view in the mid esophagus: Ao = aorta; S = spine; T = trachea. The esophagus remains open continuously due to unburped air. Insufflated air is routinely not necessary in persons with R-CPD.

Esophagus stretches laterally (4 of 4)

At a moment of upward surge of air that cannot escape the upper esophageal sphincter as a burp, the esophagus stretches laterally, almost trying to get around (arrows) the non-compressible trachea (T), accentuating the “tracheal mound.” Note as well the widened lumen as compared with photo 3.

More Interesting Esophageal Findings of R-CPD (Inability to Burp)

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Stretched Esophagus (1 of 4)

Using a 3.7mm ENT scope with no insufflated air, note the marked dilation of the esophagus by swallowed air the patient is unable to belch. T = trachea; A = aortic shelf; S = spine

Tracheal Wall (2 of 4)

The posterior wall of the trachea (T) is better seen here from a little higher in the esophagus. A = aorta

Over-dilation (3 of 4)

The photo is rotated clockwise at a moment when air from below is pushed upward so as to transiently over-dilate the esophagus. Note that the esophagus is almost stretching around the left side of the trachea in the direction of the arrow.

Bronchus (4 of 4)

Now deeper in the esophagus (with it inflated throughout the entire examination by the patient’s own air), it even appears that the left mainstem bronchus (B) is made visible by esophageal dilation stretching around it.

The Esophagus Doesn’t Like Being Stretched for Years Due to Untreated R-CPD

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Lateral dilation from R-CPD (1 of 3)

In this middle-aged patient with R-CPD (inability to burp), now fully resolved (burping well for more than a year) after botox therapy. This view is pre-treatment, at mid-esophagus using an ENT scope. No air was insufflated to get this photo; the patient “has her own.” The aortic shelf is prominent, but observe the dramatic lateral dilation (arrows). S = spine; T = trachea.

Lateral dilation in the upper esophagus (2 of 3)

Now in the upper esophagus, arrows again depict the remarkable lateral dilation.

Medial-lateral stretch (3 of 3)

Opening of the esophagus is constant, due to the patient’s retained air, but as air goes downward transiently, the lumen size is reduced, almost accentuating the medial-lateral “stretch” of the esophagus. * denotes the same place in photos 2 and 3, for reference.

Emerging Esophageal Findings

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Aortic shelf (1 of 3)

A view of the mid-esophagus in a young person (early 30’s). The esophagus is kept open by the patient’s un-burped air. Note the “aortic shelf” at A, delineated by dotted lines.

Bony spur emerges due to stretched esophagus (2 of 3)

A moment later, additional air is pushed upwards from the stomach to dilate the mid-esophagus even more. A bony “spur” in the spine is thrown into high relief by the stretched esophagus.

Stretched esophagus due to unburpable air (3 of 3)

A view of the upper esophagus (from just below the cricopharyngeus muscle sphincter) shows what appears to be remarkable lateral dilation (arrows) caused over time by the patient’s unburpable air. Dilation can only occur laterally due to confinement of the esophagus by trachea (anteriorly) and spine (posteriorly), as marked.

Example 2

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Saliva bubbles (1 of 5)

Saliva bubbles in the lower esophagus. Note that no air has been insufflated to obtain this view.

Symptoms point to GERD (2 of 5)

Approximately 0.5 second later, saliva wells up from below while the esophagus itself remains unchanged (Red dots in photos one and two mark identical reference points). Either abdominal wall or stomach wall compression lifts a column of stomach contents superiorly (retrograde) in the esophagus, explaining significant rates of GERD symptoms in this group, especially after treatment, which allows air to evacuate and might potentiate a higher rise of the material in the stomach/ lower esophagus.

Pocket in the aorta (3 of 5)

Seen here is what almost appears like a pocket (arrow) at the upper surface of the aorta (A). Compare with the next photo.

Trapped air expands the esophagus (4 of 5)

The patient’s unburped air further expands the esophagus and makes more of a “shelf” of the upper surface of the aorta (A).

Exaggerated laterally-stretched esophagus (5 of 5)

Now visualizing the upper esophagus, just below the cricopharyngeus muscle (UES): It appears that there is exaggerated lateral “stretch” of the esophagus. It cannot stretch posteriorly due to immovable spine (S) nor can it expand anteriorly due to the non-collapsible trachea (T). Consequently, it can only dilate laterally (arrows).

Abdominal Distention of R-CPD

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Gastric Air Bubble (1 of 3)

This abdominal xray of an individual with R-CPD shows a remarkably large gastric air bubble (dotted line), and also excessive air in transverse (T) and descending (D) colon. All of this extra air can cause abdominal distention that increases as the day progresses.

Bloated Abdomen (2 of 3)

Flatulence in the evening and even into the night returns the abdomen to normal, but the cycle repeats the next day. To ask patients their degree of abdominal distention, we use pregnancy as an analogy in both men and women. Not everyone describes this problem. Most, however, say that late in the day they appear to be “at least 3 months pregnant.” Some say “6 months” or even “full term.” In a different patient with untreated R-CPD, here is what her abdomen looked like late in every day. Her abdomen bulges due to all of the air in her GI tract, just as shown in Photo 1.

Non-bloated Abdomen (3 of 3)

The same patient, a few weeks after Botox injection. She is now able to burp. Bloating and flatulence are remarkably diminished, and her abdomen no longer balloons towards the end of every day.

Can’t Burp: Progression of Bloating and Abdominal Distention – a Daily Cycle for Many with R-CPD

This young woman has classic R-CPD symptoms—the can’t burp syndrome. Early in the day, her symptoms are least, and abdomen at “baseline” because she has “deflated” via flatulence through the night.  In this series you see the difference in her abdominal distention between early and late in the day.  The xray images show the remarkable amount of air retained that explains her bloating and distention.  Her progression is quite typical; some with R-CPD distend even more than shown here especially after eating a large meal or consuming anything carbonated.

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Side view of a bloated abdomen (1 of 6)

Early in the day, side view of the abdomen shows mild distention. The patient’s discomfort is minimal at this time of day as compared with later.

Front view (2 of 6)

Also early in the day, a front view, showing again mild distention.

Greater Distention (3 of 6)

Late in the same day, another side view to compare with photo 1. Accumulation of air in stomach and intestines is distending the abdominal wall.

Front view of bloating stomach (4 of 6)

Also late in the day, the front view to compare with photo 2, showing considerably more distention. The patient is quite uncomfortable, bloated, and feels ready to “pop.” Flatulence becomes more intense this time of day, and will continue through the night.

X-ray of trapped air (5 of 6)

Antero-posterior xray of the chest shows a very large stomach air bubble (at *) and the descending colon is filled with air (arrow).

Side view (6 of 6)

A lateral view chest xray shows again the large amount of excess air in the stomach and intestines that the patient must rid herself of via flatulence, typically including through the night, in order to begin the cycle again the next day.

Shortness of Breath Caused by No-Burp (R-CPD)

Persons who can’t burp and have the full-blown R-CPD syndrome often say that when the bloating and distention are particularly bad—and especially when they have a sense of chest pressure, they also have a feeling of shortness of breath. They’ll say, for example, “I’m a [singer, or runner, or cyclist or _____], but my ability is so diminished by R-CPD.  If I’m competing or performing I can’t eat or drink for 6 hours beforehand.”  Some even say that they can’t complete a yawn when symptoms are particularly bad.  The xrays below explain how inability to burp can cause shortness of breath. 

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X-ray of trapped air (1 of 2)

In this antero-posterior xray, one can see that there is so much air in the abdomen, that the diaphragm especially on the left (right of xray) is lifted up, effectively diminishing the volume of the chest cavity and with it, the size of a breath a person can take.

Side view (2 of 2)

The lateral view again shows the line of the thin diaphragmatic muscle above the enormous amount of air in the stomach. The diaphragm inserts on itself so that when it contracts it flattens. That action sucks air into the lungs and simultaneously pushes abdominal contents downward. But how can the diaphragm press down all the extra air? It can’t fully, and the inspiratory volume is thereby diminished. The person says “I can’t get a deep breath.”

Dramatic Lateral Dilation of the Upper Esophagus

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Remarkable lateral dilation (1 of 3)

This photo is at the level of (estimated) C6 of the spine (at S). This person has known cervical arthritis, accounting for the prominence. Opposite the spine is the trachea (T). Note the remarkable lateral dilation (arrows) in this picture obtained with with no insufflated air using a 3.6mm ENF-VQ scope. It is the patient’s own air keeping the esophagus open for viewing.

Dilated upper esophagus (2 of 3)

At a moment when air from below further dilates the upper esophagus, the tracheal outline is particularly well-seen (T) opposite the spine (S). The “width” of the trachea indicated further emphasizes the degree of lateral dilation, which is necessary because spine and trachea resist anteroposterior dilation.

Aortic shelf (3 of 3)

Just for interest, at mid-esophagus, the familiar aortic “shelf” is seen. Again, this esophagus is being viewed with a 3.6 mm scope with only the patient own (un-burped) air allowing this view.

Dramatic Dilation of the Esophagus in a Person with R-CPD due to Buildup of Swallowed Air that He Cannot Belch to get Rid of.

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View of the mid-esophagus (1 of 2)

This view in the mid-esophagus was obtained with a 3.6mm scope without an air channel. The dilation is from the patient’s own unbelchable air. Note quite major lateral dilation of the esophagus, indicated by concentric dotted lines and arrows. Dilation is not possible in the direction of unyielding spine (S) and trachea (T).

View of the mid-esophagus (2 of 2)

A view that shows more clearly the indentation of trachea (T). Persons with this much dilation of esophagus often complain as much of chest pressure as they do abdominal bloating. This man has experienced “large” reduction of R-CPD symptoms after botulinum toxin injection into his upper esophageal sphincter (cricopharyngeus muscle).
R-CPD in X-ray Pictures: Misery vs. Crisis from Inability to Burp
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R-CPD in X-ray Images

Why do persons with R-CPD experience such daily misery? These X-ray images provide the explanation, as well as the rare “abdominal crisis” in this group is also explained.

In a new video format, Dr. Bastian will discuss various photo essays found across Laryngopedia, and provide in-depth descriptions on their origins and what is going on behind the scenes during the time of capture.

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