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with these patients is aspiration, because that can lead to signicant morbidity and
mortality. Oropharyngeal dysphagia is a very common disease, with signicant
costs to the healthcare system. In evaluating these patients, I think the thing that's
key is to have a close relationship with your speech therapist and to understand what
the patient’s video swallow study shows.
So, if you’re worried that a patient has oropharyngeal dysphagia, the typical rst
approach will be to do a video swallow study to see if they have any penetration
above the vocal cords or if they have any aspiration. Then, if they do, what is the
consistency of the food that is aspirated? Do they have liquid aspiration? Do they
have aspiration of pudding? Do they aspirate thicker liquids? Do they have aspira-
tion for solid foods as well? And sometimes, from that standpoint, initial manage-
ment is to try to determine if there are certain foods that can be tolerated without
aspirating. If so, the speech therapist can work with the patient to see if they can
modify the consistency of some of the other foods. If the patient aspirates liquids,
then they can be taught to thicken up the liquid that they’re taking in. If they cannot
swallow any foods without aspirating, then you have to talk about whether or not
alternate delivery of nutrition is necessary, including enteral feeding or G-tube
placement to get you through the initial phases of the oropharyngeal dysphagia.
The speech therapists are good at teaching certain maneuvers that patients can do
to limit their aspiration risks. One of these is the Shaker exercise, which requires a
head lift, to try to strengthen some of the pharyngeal swallowing musculature. This
maneuver can also help with upper esophageal sphincter opening. Obviously, the
limitation of many of these exercises is that you have to have a patient who is capa-
ble of following instructions. If the patient is neurologically devastated, that's less
of an option.
Also, if you have a patient with restricted neck mobilization, because they have
had a big spinal surgery or other type of neurosurgery, this also limits your ability to
do certain swallowing exercises, including the Shaker exercises. Then, there are
things down the pipeline that hopefully will address some of these issues. We are
doing research protocols in our laboratory, working with a swallow resistance exer-
cise device, which is a pressure collar that people wear. Patients basically practice
swallowing against resistance. In some of the initial data, we're able to improve
aspiration and penetration in a subset of individuals who wear this collar, including
some with Parkinson’s disease. These are all areas of study where hopefully we can
modify the swallowing mechanism, nd a way to strengthen their musculature to
delay the disease progression, and prevent or delay aspiration.
However, in patients who have persistent aspiration, we consider PEG tubes.
Although PEG tubes are not necessarily the best thing in demented patients that
have oropharyngeal dysphagia, they may be options for patients that have ALS,
Parkinson’s disease, and some stroke patients to hopefully maintain their nutri-
tional status.
P. Sanvanson
https://t.me/medicina_free
17© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2023
W. H. Sobin et al. (eds.), Managing Complex Cases in Gastroenterology,
https://doi.org/10.1007/978-3-031-48949-5_4
Chapter 4
Achalasia
BensonMassey
Case 1 A patient is referred to your surgeon by a community gastroenterologist
requesting a fundoplication for chronic gastroesophageal reux. The gastroenter-
ologist thinks the surgeon should consider doing a Toupet procedure because his
patient, in addition to having chronic reux esophagitis, has ineffective esophageal
motility. The surgeon, concerned about the presentation, is asking you to review the
case, wondering if he has been misdiagnosed.
This is a 40-year-old man with a long history of heartburn and regurgitation.
This has been going on for a couple of years and is getting worse. He has lost about
ten pounds over the past couple of months. The patient frequently has regurgitation
after eating, and over time, the patient has transitioned to a softer and more liquid
diet. He avoids eating for several hours before bedtime. In fact, he occasionally
induces vomiting before going to bed because, on occasion, he has woken up in the
middle of the night regurgitating liquid, occasionally solid material.
His gastroenterologist did an EGD that showed some retained liquid and super-
cial ulceration in the distal esophagus, all of which he interpreted as reux esoph-
agitis. The GE junction was somewhat tight, which he interpreted as a reux
stricture. He dilated the stricture using a TTS balloon (to 18 mm), with minimal
symptomatic improvement. The patient has been taking PPIs long term, and yet his
symptoms keep getting worse.
The gastroenterologist has previously sent patients to this surgeon for anti-reux
surgery with great results. He knows that the surgeon likes to have manometry done
beforehand. His GI practice still uses older water-infusion manometry catheters,
and his experience doing manometry is somewhat limited. He goes ahead and
B. Massey (*)
Division of Gastroenterology and Hepatology, Department of Medicine, Medical College of
Wisconsin, Milwaukee, WI, USA
e-mail: bmassey@mcw.edu
https://t.me/medicina_free
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performs the procedure, after placing the manometry catheter without using uo-
roscopy. His interpretation of the study is that there is clearly decreased pressure in
the esophagus with limited, if any peristalsis. He is unable to detect a lower esopha-
geal sphincter zone. He interpreted this to mean that the LES was nonfunctional,
blown-out.
When the patient comes to see you, he brings the manometry tracing which you
review. Looking at the tracing, you suspect that the probe never passed the LES and
never entered the stomach. Without being able to evaluate the LES, you are not sure
if you are dealing with GERD in a patient with ineffective motility or if the patient
may have Type 1 achalasia.
How often do you see patients being diagnosed with GERD, who actually
have achalasia?
First, it can be very difcult to distinguish GERD from achalasia by history, and
there is a lot of overlap, particularly in early achalasia. We use dysphagia for solids
and liquids as a marker of achalasia, but early on in achalasia there may just be
dysphagia for solids. This may be very hard to distinguish from the symptoms of
someone with a peptic stricture or even something like eosinophilic esophagitis. So,
the symptom overlap may confuse the diagnosis early on.
Second, because, achalasia is several orders of magnitude less frequent than
GERD, anyone who’s coming in with esophageal symptoms, even if they seem a
little weird, like dysphagia for solids and liquids, they're still more likely to have
GERD than they are achalasia, just a priori without doing any investigation at all.
Interestingly, the reliability of the clinical history for diagnosing GERD is poor.
If you look at the Diamond study [1], done some years ago, which looked at primary
care doctors trying to determine whether a patient had GERD based on symptoms,
their decision-making was not much better than ipping a coin (sensitivity and
specicity of 63%, based on the history when you’re using actual acid exposure as
the gold standard). Gastroenterologists don’t do much better. The sensitivity and
specicity of their history are about 67% and 70% which still mean that about a
third of the time they got it wrong [1].
So, with GERD and achalasia both being tricky diagnoses, unless someone pres-
ents with advanced achalasia where they’re having really obvious features, such as
an esophagus that is massively lled with liquid in a manner that really gets your
attention, I think it’s really a bit of a challenge.
There are a few clinical clues that may help distinguish the two. First, the patient
described here is regurgitating. When you have achalasia and you regurgitate, the
stuff tastes the same coming up as when it goes down. When GERD patients regur-
gitate, it will taste sourer, unless the patient is on vigorous PPI therapy for reux, in
which case the regurgitation may not taste acidic.
An endoscopic technique to help determine whether achalasia is present is to
inate the esophagus with a lot of air, watch if the GEJ opens up, and then inate
the stomach with a lot of air and retroex and see if the GE junction opens up. In
achalasia, the GEJ stays shut; in GERD, it tends to open wide. In fact, if you look
from below, in retroex, it’s hard to keep the stomach insufated in GERD
because so much air is leaking back up through the opening (that’s only in very
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end-stage conditions like systemic sclerosis, where there is a very hypoten-
sive LES).
So, it’s a pretty accurate sign if you see the GEJ opening up, and the patient is
belching air, that the patient doesn’t have achalasia (if they’ve not already had
surgery).
Another thing you can do, if you’re unsure whether you’re dealing with GERD
or achalasia, is to combine your endoscopy with a BRAVO study. If your EGD is
unrevealing and the Bravo returns negative, then you’re not dealing with GERD,
and you have to really start thinking about another diagnosis like achalasia.
You decide to repeat the manometry yourself. You pass the probe into the stomach
with uoroscopic guidance. On the tracing, you note absent peristalsis, but the IRP
is only borderline elevated, with some decreased relaxation. You still think this is
likely achalasia, but you want more convincing evidence.
What other tools can help dene whether the patient has achalasia?
When you’re dealing with someone with absent contractility, the question is as
follows: Is this achalasia or is this a scleroderma-like dead esophagus? Is that pos-
sibly borderline high IRP in supine swallows really due to high deglutitive residual
tone in the LES? To help determine whether an elevated IRP may be clinically
important, you should also do the manometry sitting up as well as supine. If the IRP
is normal on swallows while sitting, the high IRP during supine swallows should be
disregarded.
When the IRP is also elevated during sitting swallows, there can still be a con-
cern that this reects muscle activity of the diaphragmatic crura (particularly in
patients with obesity or lung disease) or could reect a structural problem (e.g., a
paraesophageal hernia or tight fundoplication wrap). This is where provocative
pharmacologic testing during manometry using amyl nitrite can help with the diag-
nosis. Amyl nitrite completely abolishes smooth muscle tone in the esophageal
body and LES for around 30s. If the nadir pressure at the esophagogastric junction
after amyl nitrite falls over 10mmHg lower than the IRP on swallows, then you
have impaired LES relaxation. If there is really no change with amyl nitrite, then the
cause of the pressure is not smooth muscle tone in the LES.
Do you rely on the timed barium esophagram to help make a diagnosis of
achalasia?
No, I don’t think that the timed barium esophagram is as helpful in making a
diagnosis of achalasia as it is in measuring the response to therapy. The timed bar-
ium esophagram can show you a delay in esophageal emptying, but it can’t differ-
entiate whether the delay is due to decreased esophageal contractions or to EGJOO
or a combination of the two. We feel we get much more information by doing an
impedance study at the time of manometry. Using impedance, if liquid is not empty-
ing normally, I can determine whether it relates to decreased squeeze of the esopha-
geal body, or increased resistance at the GEJ, or elevation of gastric pressure (in an
obese patient there is elevation of intragastric pressure that may delay esophageal
emptying). I can also assess the height of the uid column remaining in the esopha-
gus at different times, when the patient is in a seated position.
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After you evaluate your manometry and impedance studies, you are con-
vinced the patient has achalasia. How do you approach the management of the
different types of achalasia?
For patients with spastic achalasia, the type 3 achalasia, we often try pharmaco-
logic therapy rst, a calcium channel blocker, like nifedipine or sublingual hyoscya-
mine, and many of them will get relief from pharmacology alone. In a number of
these patients, the dysphagia is caused more by spastic contractions than outow
obstruction. We nd that a number of these patients will respond to low-dose sub-
lingual hyoscyamine. Even though it doesn't normalize the motility, they get symp-
tomatic relief without needing to do an intervention on the GEJ and spastic
contractions in the esophageal body.
In type 2 achalasia, I would not try pharmacologic therapy because it will
decrease pan-pressurization and you need that pressure to help empty the esophagus.
I would also avoid doing a POEM in type 2 because you still have intrinsic motil-
ity that can help empty the esophagus in a non-peristaltic fashion, and you destroy
that with a POEM. When you’re doing a POEM, you are essentially creating a
scleroderma-like esophagus, and you’re getting rid of all motility. What you need in
a type 2 is to get rid of the EGJOO either with a Heller myotomy or pneumatic dila-
tion. There is usually enough remaining intrinsic esophageal motility that the reduc-
tion in LES tone produced by pneumatic dilation should be sufcient to relieve
symptoms in type 2. However, the reduced esophageal body contractility in type 1
(end-stage achalasia) usually requires a more complete ablation of the LES tone,
which necessitates a myotomy.
In type 1, I am worried less about a POEM ablating esophageal body contractil-
ity, because it is already essentially absent. However, the concern for increased
reux after POEM remains.
People talk about pan-pressurization (in type 2 achalasia) as if it is a bad thing. I
think it is a good thing, a driving force that can help to empty the esophagus. So, if
the esophagus is pan-pressurized to 30mm of mercury, and you can reduce the pres-
sure at the LES to 10mm of mercury, now you’ve got a 20-mm mercury driving
pressure to empty out the esophagus. The difculty comes when there’s not suf-
cient esophageal squeeze pressure and then you have to really obliterate all the tone
at the LES, allowing things to empty by gravity.
However, when you obliterate all tone at the GEJ, you also knock out your anti-
reux barrier. With a Heller, you can restore some of that by also performing a Dor
or Toupet anti-reux procedure, but with a POEM you can’t. I have some concerns
about the POEM long term. I think there’s the potential for a lot of reux damage
that can accumulate over time, including Barrett’s esophagus and possibly associ-
ated neoplasia.
One of the things to watch out for post-achalasia treatment is that patients will
feel great when you see them 1 or 2 months after their initial therapy. Some gastro-
enterologists mistakenly tell their patients not to come back unless they’re having
problems. That’s not a good idea. Many patients, especially after a POEM, will have
signicant reux. Many patients will put on a lot of weight since they’re able to eat
a lot better now that their obstruction is relieved. As a result, they develop even more
B. Massey
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reux related to weight gain, and many will develop reux esophagitis and even
reux strictures. Also, pills are more likely to lodge unawares in the esophagus of
these patients, so they are more prone to pill injury from NSAIDS, etc. Therefore, I
think you have to monitor these patients long-term and continue to re-educate them
regarding potential problems.
I also think that with the surgical myotomy in type 2 achalasia, the surgeons should
try to disrupt as little of the muscle in the esophageal body above the LES as possible.
There is an understandable tendency to err on the side of cutting higher into the
esophageal body, to avoid the disaster of an incomplete myotomy of the LES.We’re
starting to see an increased number of patients who have a blown-out esophagus,
years after their surgery because too much esophageal muscle has been destroyed.
Case 2 An 82-year-old woman is referred to you for dysphagia. She has had dys-
phagia for 6 months that has been getting progressively worse. It was initially for
solids, but now she has dysphagia for liquids as well. Her community gastroenter-
ologist did an EGD that showed a corkscrew esophagus. The LES was somewhat
tight and seemed to have a birds-beak appearance. The scope easily passed the zone
of resistance at the LES and popped into the stomach. He did a TTS dilation of the
LES, and the swallowing improved for about a week, and then, swallowing got worse.
He suspects the patient may have a variant of achalasia and wants your opinion.
He thinks she probably is a candidate for Botox. While he thinks a pneumatic dila-
tion would help her, he is reluctant because of her age and asks your opinion.
You examine the patient. She is somewhat frail and fairly immobile. She is reluc-
tant to have esophageal manometry.
Would you ever inject Botox without a manometry documenting achalasia?
I don’t want to empirically treat someone for achalasia without manometry.
There are different presentations and different types of achalasia. Some have spastic
contractions, and some have a dead and scleroderma-type esophageal body. You
manage these differently.
For the patient who complains that they don’t want to go through an esophageal
manometry because they are afraid of the catheter placement being painful, we will
place the catheter at the time of endoscopy while they are sedated, so it should not
be a problem.
Do you ever see 80-year-old patients presenting with a new diagnosis of
achalasia?
I do see achalasia being diagnosed in patients who are in their 70s and 80s. Of
course, many patients have had their disease for years before the diagnosis is made.
I’ve seen some patients where I have looked back in their old X-ray jacket and saw
a dilated esophagus on a CAT scan done years before symptom onset that was not
appreciated at the time.
Of course, when you diagnose achalasia in elderly, frail patients there is a reluc-
tance to send them to surgery because of the higher risk of postoperative complica-
tions. They’re also reluctant to have the patient get a pneumatic dilation for the same
reason, because there’s a 2% risk of potential perforation. So, you inject Botox and
the patient does great.
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However, when you decide to treat the older patient with Botox because it is less
invasive are you just delaying the inevitable? They do great for, at best, a year, and
then you inject them again. After a while, you’ve injected a lot of Botox down there,
the patient develops antibodies, and the Botox stops working. In addition, all that
Botox may or may not cause brosis which surgeons aren't too happy about.
So, if the patient survives long enough to outlast the Botox what are you going to
do? You’ve got to do something, but it’s going to be at higher risk. When you decide
whether you should start Botox on an elderly patient, that is a challenging decision
that your present self has to make, that your future self is going to have to live with.
So, they’re now ve years older, and the Botox is not working. You have to do some-
thing more substantial, but now they’re only a poorer surgical candidate.
In this case the patient received a TTS dilation that helped very briey.
What do you think about doing an empiric TTS dilation in a patient with
achalasia?
I actually think it is a good idea. A number of patients with achalasia also have a
stricture at the GEJ.Empirically, dilating the stricture can improve their dysphagia,
and some of them may not need further treatment for their achalasia for a long time.
It may be hard to tell that they’ve got a stricture down there, and you can’t see the
stricture because the LES isn’t relaxing. However, I do have any number of achala-
sia patients who, in addition to their achalasia, have a stricture at the GE junction.
You won’t know it unless you pass a 60 French bougie across there or inate a TTS
20-mm balloon.
I’ve had some patients with achalasia that probably had the condition for 10 or
20 years without bothersome symptoms, and then, they get a stricture, and that’s
the thing that tips them over. If I dilate the stricture, they can live with the achala-
sia. I wouldn’t do that in a 20-year-old, but a 70-, 80-year-old, if you can nd a
stricture and dilate it, and they get symptomatic relief I wouldn’t treat their acha-
lasia any further, although they still need to be followed and their symptoms
monitored.
We don’t really know how Botox works to relieve symptoms in achalasia. We
assume that it works on the motility of the sphincter, but is it possible that it works
more on the sensory component? I had a guy this last year who was in his nineties
with well-documented achalasia. We treated him with Botox, and his symptoms of
dysphagia and regurgitation got dramatically better, but then we noticed that he also
had iron deciency anemia. I’m not too excited about working up iron deciency
anemia in a 90-year-old, but then, 2 or 3 weeks later he started having black stool.
He was on an antiplatelet agent for heart disease, and his blood count was dropping
more. So, we felt we had to do a colonoscopy, but we didn’t nd anything. So, what
was causing this dark stool? Did he have something concerning down in his
small bowel?
At that point, he’s saying his swallowing is doing great, because of the Botox,
and so we decided to do a capsule endoscopy, which he swallowed without dif-
culty. It was a 12-h capsule, and then, we went to read the images and the capsule
sat in the esophagus for 11h. Then, it nally came out, but he felt no dysphagia at
all. Clearly, the esophagus wasn’t emptying correctly, and he just didn’t feel it.
B. Massey
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23
Case 3 A 64-year-old woman with a history of small cell lung cancer that has been
in remission is referred with dysphagia. Dysphagia started about a month earlier
and rapidly worsened. She is now having marked difculty swallowing solids and
liquids.
A CT scan shows a mildly dilated esophagus but no suggestion of metastatic
disease in the region of the esophagus or stomach. An EGD is performed that shows
a birds-beak-type LES with increased resistance, but eventually the scope success-
fully pops through. No lesions are seen in the esophagus and cardia, etc. A TTS
dilation is performed without prolonged clinical benet. You suspect the patient has
pseudoachalasia.
What are some of the markers that make you suspect pseudoachalasia?
Historical markers for pseudoachalasia include older age, more rapid onset of
symptoms, and more substantial weight loss. A clue during endoscopy is that it may
be more difcult to get the scope through the GEJ.In idiopathic achalasia, the GEJ
may not open with air insufation alone, but you can easily advance the scope
through the sphincter into the stomach. One of the markers of pseudoachalasia is
signicant difculty advancing the scope into the stomach. There may be tumor
inltrating the GEJ which may be difcult to visualize.
Are there manometric distinctions that can help differentiate between idio-
pathic achalasia and pseudoachalasia?
Idiopathic achalasia is very uncommon, pseudoachalasia is extremely uncom-
mon, and pseudoachalasia as a paraneoplastic phenomenon is extremely rare. To tell
you whether there are manometric ndings that can help distinguish pseudoachala-
sia from achalasia is difcult because there are so few cases to evaluate. It might be
possible there will be different responses to pharmacologic provocative maneuvers,
but at this point we really have insufcient data to tell, and there are so few cases
out there.
Case 4 A 43 year-old man presents to his community gastroenterologist with dys-
phagia of one-month duration. He has dysphagia for solids and liquids. He has
been taking high doses of Percocet for months after a back injury. The gastroenter-
ologist does an EGD that suggests achalasia. He does a manometry that is consis-
tent with achalasia. He sends the patient to you because he refers all his pneumatic
dilations to you.
Do you approach the diagnosis of achalasia differently in patients on
opiates?
If you look at patients with type 3 achalasia and patients with EGJOO, there is an
increased number of people in these cohorts who take high doses of narcotics. If you
look at their responses to pharmacologic provocative testing during manometry, the
responses to amyl nitrite and CCK are different in patients who have opiate-
associated achalasia from those patients with idiopathic achalasia.
We only see this clinical picture in patients who are taking high doses of narcot-
ics, at least 30 milliequivalents of morphine daily, not just one or two Percocet a
day. Even before we knew about the connection of achalasia with opiates, we
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realized that patients on opiates did not get as good a result with achalasia therapy
as patients who were not on opiates. The symptomatic outcomes were especially
poor in people who appeared to have type 3 achalasia who were treated with abla-
tive therapies.
On the other hand, we have seen that if you can get these patients completely off
opiates, many of them will get total relief from their symptoms. So, that becomes
the goal.
For whatever reason, we are seeing fewer of these patients than before, perhaps
because community gastroenterologists have been made aware of this connection
and they’re explaining to their patients that the opiates are the problem. Anyway,
when we see patients like this, we will not refer them for surgery until they’ve gone
off narcotics. If they do that, symptoms will resolve in most patients.
One thing about my approach to treating opiate-induced esophageal dysmotility
is that I don’t want to be overly aggressive in patients who have symptoms that are
simply a nuisance and not a threat to life (e.g., not having substantial weight loss or
recurrent pneumonias). I am loath to recommend a POEM to someone whose condi-
tion is potentially reversible upon stopping opiates or dropping to a lower dosage,
because the POEM creates a scleroderma-like esophagus, something that can’t be
reversed.
Reference
1. Dent J, Vakil N, Jones R, Bytzer P, Schöning U, Halling K, Junghard O, Lind T.Accuracy of
the diagnosis of GORD by questionnaire, physicians and a trial of proton pump inhibitor treat-
ment: the Diamond Study. Gut. 2010;59(6):714–21.
B. Massey
https://t.me/medicina_free
25© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2023
W. H. Sobin et al. (eds.), Managing Complex Cases in Gastroenterology,
https://doi.org/10.1007/978-3-031-48949-5_5
Chapter 5
Other Motility Disorders oftheEsophagus
andAchalasia
FrancisEdeani
Case 1 The patient is a 35-year-old woman who presents with a history of
occasional chest pain which is clearly related to meals, denitely worse when
eating. The pain is described as an ache or a cramp. She also has the sensation of
food sticking intermittently, both solids and liquids. Her symptoms are exacerbated
when she is stressed. Her physical exam is normal. An EKG is normal. She is treated
with PPIs for one month without relief.
How do you evaluate a patient with chest pain and dysphagia during
swallowing?
Dysphagia to solids and liquids suggests a motility disorder of the esophagus.
We generally start with the simplest test—an esophagram. This is noninvasive, pro-
vides a “roadmap” and can give a general idea about esophageal motility, shows the
presence of strictures or rings, and may provide some information about the muco-
sal lining. Challenge with a barium tablet or marshmallow can sometimes reproduce
the patient’s symptoms and can demonstrate stasis of the food bolus.
The gold standard test for esophageal motility disorders is esophageal manome-
try. EGD is typically performed as a complementary test to assess for lesions due to
reux esophagitis, eosinophilic esophagitis, infectious esophagitis (such as CMV,
HSV, candida in immunocompromised patients), esophageal strictures, rings or
webs, and malignancy. A tight esophagogastric junction during EGD may suggest
the presence of an esophageal outow obstruction motility disorder. If the EGD
with biopsies is normal, esophageal manometry is performed. In patients with high
probability for esophageal motility disorder as determined by ndings on
F. Edeani (*)
Division of Gastroenterology and Hepatology, Department of Internal Medicine,
Medical College of Wisconsin, Milwaukee, WI, USA
e-mail: fedeani@mcw.edu
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