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):

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

Dramatic esophageal dilation from R-CPD, even in a young person!

The central problem for persons with R-CPD is (obviously) the inability to burp. Bits of air travel through the upper esophageal sphincter (cricopharyngeus muscle) which closes behind each swallow.

Even as the air builds up, expanding the esophagus and sending increasing signals of the need to burp, the air is unable to “get back out” via burping. Initial discomfort from stuck burps-in-waiting may elicit reflexive “extra” swallows that only add more air that cannot be burped.

Gradually this dilates the esophagus (and eventually the stomach, and as air traverses the GI tract, the intestines all the way to the rectum, where it must be released as flatulence). Even in young people, esophageal dilation can be dramatic, causing chest discomfort.

The images below are from the office examination of a teenager sitting in the examination chair with a small ENT scope in his esophagus. The column of air he cannot burp up is holding the esophagus widely open. No air has been insufflated through the scope. The stretched esophagus throws structures outside it into high relief as seen below.

Stretched esophagus (1 of 3)

In this view, the lower third of the esophagus is in the “always open” position seen routinely in R-CPD, due to his unburped column of air. His heartbeat can be seen from its posterior wall (H). Unusually well seen is the ridged vertebral column (VC).

Mid-esophagus (2 of 3)

In the mid-esophagus, at the lower end of the trachea (T), the segmented left mainstem bronchus (B) turns laterally to pass under the Aorta (Ao).

Stretching esophagus (3 of 3)

Again at the lower trachea, but with the view rotated to the patient’s left, note that since the trachea (T) is not compressible, the esophagus tries to stretch around it, bringing even the side of the trachea into view.

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.

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 due to trapped air — 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)

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

left mainstem bronchus is made visible — 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

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

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.

A bony spur in the spine — 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.

dilation of the upper 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

Saliva bubbles (1 of 5)

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

saliva wells up in the esophagus — 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 upper surface of the aorta — 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.

aorta shelf — 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).

Dramatic Lateral Dilation of the Upper Esophagus

lateral dilation of the throat at C6 of the spine — 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 at the mid-esophagus — 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.

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.

R-CPD Esophageal Findings

The significance

Dramatic esophageal dilation from R-CPD, even in a young person!

Stretched esophagus (1 of 3)

Mid-esophagus (2 of 3)

Stretching esophagus (3 of 3)

Esophageal Stretching by Unburpable Air in R-CPD

Typical view of an esophagus (1 of 4)

Lower esophagus (2 of 4)

Stretched mid-esophagus (3 of 4)

Esophagus stretches laterally (4 of 4)

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

Stretched Esophagus (1 of 4)

Tracheal Wall (2 of 4)

Over-dilation (3 of 4)

Bronchus (4 of 4)

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

Lateral dilation from R-CPD (1 of 3)

Lateral dilation in the upper esophagus (2 of 3)

Medial-lateral stretch (3 of 3)

Emerging Esophageal Findings

Aortic shelf (1 of 3)

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

Stretched esophagus due to unburpable air (3 of 3)

Example 2

Saliva bubbles (1 of 5)

Symptoms point to GERD (2 of 5)

Pocket in the aorta (3 of 5)

Trapped air expands the esophagus (4 of 5)

Exaggerated laterally-stretched esophagus (5 of 5)

Dramatic Lateral Dilation of the Upper Esophagus

Remarkable lateral dilation (1 of 3)

Dilated upper esophagus (2 of 3)

Aortic shelf (3 of 3)

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.

View of the mid-esophagus (1 of 2)

View of the mid-esophagus (2 of 2)

R-CPD in X-ray Images

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