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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_1401_Библиотеки_им_академика_М_И_Перельмана.pdf
106
A. M. Brown and A. D. Pryor
residual pressure [8]. In a small study with 11 patients, Sildenal leads to improved
manometric ndings in 9 patients, but symptomatic improvement in only 4 patients,
of which 2 of the 4 had signicant side effects requiring cessation of the medication
[9]. Additional medications including aminophylline and terbutaline have been
poorly studied [4].
Achalasia is best treated with surgical or endoscopic therapies. Pneumatic balloon dilation (PD), endoscopic botulinum toxin injection, Per Oral Endoscopic
Myotomy (POEM), and heller myotomy (HM) have all been shown to be effective
for relief of symptoms of achalasia [10]. While POEM and laparoscopic HM have
been shown to be efcacious in treating symptoms of achalasia, there may be higher
post-procedural acid exposure in the esophagus following POEM [11].
Esophagogastric Junction Outflow Obstruction
Esophagogastric junction outow obstruction (EGJOO) is dened as an elevated
IRP (>15mmHg), with sufcient evidence of peristalsis such that criteria for types
1–3 achalasia are not met [1].
EGJOO can be further divided into two major categories, functional and mechanical EGJOO.Functional EGJOO is suspected in patients in which no mechanical
cause is identied for the obstruction [12]. This is generally treated similarly to
achalasia, with surgical and endoscopic interventions [13–15]. Botulinum toxin
injection with pneumatic balloon dilation has had short-term successful outcomes [12].
Conversely, mechanical EGJOO has an underlying etiology that should be
treated based on the cause. Common etiologies include eosinophilic esophagitis,
which can effectively be treating with proton pump inhibitors (PPI) monotherapy as
the rst-line treatment, as well as corticosteroids [16]. Obesity caused EGJOO
results from increased intra-abdominal pressures, and can be effectively treated
with weight loss. Chronic daily opioid exposure has been shown to be present in
almost a third of all EGJOO patients as dened by HRM [17]. Cessation of opioids
may help mitigate the symptoms of esophageal dysfunction in these patients when
there is not another cause identied. Figure10.3 shows the topographic representation of EJGOO on HRM.
Hypercontractile Esophagus
Hypercontractile esophagus, also known as jackhammer esophagus, is dened by at
least two swallows with DCI >8000mmHg/s/cm. Hypercontractility may involve,
or even be located to the lower esophageal sphincter [1].
Treatment for this condition is extremely varied, and given its low prevalence,
few large studies have looked at treatment efcacy. A recent study by Kahn etal., in
10 Motility Disorders: Medical Modalities
Fig. 10.3 Esophagogastric
outlet obstruction on HRM
[12]. Impaired EGJ
relaxation with normal
peristalsis. (Image used
with Permission from
Dr.Alexandra Guillaume
and Stony Brook
University Hospital GI
Motility Laboratory)
107
81 patients with jackhammer esophagus, found that treatment options included
endoscopic dilation, Botox injection, PPI, surgical or endoscopic myotomy, calcium
channel blockers, hyoscyamine, tadalal, tricyclic antidepressants, peppermint oil,
benzodiazepines, baclofen, and trazodone. These authors found that nonpharmacologic treatment (endoscopic treatment or myotomy) had signicantly higher rates of
symptomatic improvement, but this effect dissipated on long-term follow-up. No
single pharmacologic agent proved to be superior [18]. Other research has found
that pharmacological relaxation of the smooth muscle with phosphodiesterase-5
inhibitors or anticholinergic agents has shown symptomatic improvement [19].
Surgical and endoscopic options have also been tried with moderate results. Similar
to esophageal spasm, POEM may be an effective tool for signicant improvements
in chest pain and dysphagia in this patient population [20]. Figure10.4 shows HRM
of hypercontractile esophagus.
Distal Esophageal Spasm
Distal esophageal spasm is dened by a normal IRP and with ≥20% premature
contractions (DL <4.5s) with a DCI >450mmHg/s/cm. Some normal peristalsis
may be present [1, 2].
Pharmacologic treatment options have limited efcacy. Options include concentrated peppermint oil, nitrates or phosphodiesterase-5 inhibitors, calcium channel
blockers, tricyclic antidepressants, endoscopic botulinum toxin injection, pneumatic dilation, and myotomy (surgical or endoscopic) [21]. In a small study with
108
Fig. 10.4 Hypercontractile esophagus on HRM
[2]. DCI >8000mmHg/s/
cm in at least 20% of
swallows with a normal
DL. (Image used with
Permission from
Dr.Alexandra Guillaume
and Stony Brook
University Hospital GI
Motility Laboratory)
A. M. Brown and A. D. Pryor
eight patients, peppermint reduced the number of simultaneous contractions found
on manometry [22]. Endoscopic and surgical options have traditionally had limited
impact, but there is some evidence that the POEM may be a viable option for the
treatment of chest pain and dysphagia in these patients, although the data is limited
[20]. Distal esophageal Spasm is represented on HRM in Fig.10.5.
Absent Contractility
Absent contractility is characterized by 100% failed peristalsis with a normal
IRP. Achalasia should be considered when IRP values are borderline and when
there is evidence of esophageal pressurization [1].
Absent contractility has been shown to be associated with systemic sclerosis. A
recent study by Aggarwal etal. showed that in 122 patients with systemic sclerosis
who underwent HRM, 60% of patients had absent contractility [23]. There is no
available treatment to restore or improve peristalsis that has been well-proven.
Treatment of an underlying sclerotic condition and symptomatic management of
associated gastroesophageal reux disease (GERD) remain the mainstay of management. This begins with PPI therapy, but symptom control has been attempted
with antireux surgery, or gastric drainage procedures, although the data is limited
[2]. Absent contractility is seen in Fig.10.6.
10 Motility Disorders: Medical Modalities
Fig. 10.5 Distal
esophageal spasm on HRM
[2]. Premature contractions
(DL<4.5s) in at least 20%
of swallows. (Image used
with Permission from
Dr.Alexandra Guillaume
and Stony Brook
University Hospital GI
Motility Laboratory)
109
Fig. 10.6 Absent
contractility on HRM [2].
Aperistalsis in the setting
of a normal LES relaxation
with an IRP <10mmHg.
(Image used with
Permission from
Dr.Alexandra Guillaume
and Stony Brook
University Hospital GI
Motility Laboratory)
110
A. M. Brown and A. D. Pryor
Minor Disorders ofPeristalsis
Ineffective Esophageal Motility
Ineffective esophageal motility is dened by ≥50% ineffective swallows. Ineffective
swallows can be either failed or weak with a DCI <450mmHg/s/cm [1].
Treatment is aimed at the management of gastroesophageal reux, with proton
pump inhibitors with or without pro-kinetic agents. In a small study by Jeong etal.,
with 17 patients, only 41.2% of the patients had either a complete or satisfactory
response to PPI treatment [24]. Mosapride, a prokinetic serotonin receptor agonist,
signicantly increases peristaltic contractions in healthy volunteers, and along with
other prokinetic agents, are an area of future study [25]. Additional pharmacologic
interventions have been studied without success. The anxiolytic buspirone did not
lead to improvement, and treatment of GERD may be helpful when this disorder is
secondary to reux [2, 26]. HRM images of ineffective esophageal motility are seen
in Fig.10.7.
Fragmented Peristalsis
Fragmented peristalsis is dened by ≥50% fragmented contractions with a DCI
>450mmHg/s/cm [2].
Similar to ineffective esophageal motility, treatment is aimed at the management
of GERD.Jeong etal., in only seven patients, found that 85.7% of patients symptomatically improved to treatment with PPIs [24]. Fragmented peristalsis is represented on HRM in Fig.10.8.
Fig. 10.7 Ineffective
esophageal motility on
HRM [2]. Failed or weak
peristalsis in at least 30%
of swallows. (Image used
with Permission from
Dr.Alexandra Guillaume
and Stony Brook
University Hospital GI
Motility Laboratory)
10 Motility Disorders: Medical Modalities
Fig. 10.8 Fragmented
peristalsis on HRM [2].
Fragmented peristalsis
with a 7cm gap. (Image
used with Permission from
Dr.Alexandra Guillaume
and Stony Brook
University Hospital GI
Motility Laboratory)
References
111
1. Kahrilas PJ, Bredenoord AJ, Fox M, etal. International High Resolution Manometry Working
Group. The Chicago classication of esophageal motility disorders, v3.0. Neurogastroenterol
Motil. 2015;27:160–74.
2. Schlottmann F, Patti MG.Primary esophageal motility disorders: beyond achalasia. Int J Mol
Sci. 2017;18(7):1399.
3. Zaninotto G, Bennett C, Boeckxstaens G, etal. The 2018 ISDE achalasia guidelines. Dis
Esophagus. 2018;31(9):1–29.
4. Lake JM, Wong RKH.Review article: the management of achalasia– a comparison of differ-
ent treatment modalities. Aliment Pharmacol Ther. 2006;24(6):909–18.
5. Bortolotti M. Medical therapy of achalasia: a benet reserved for few. Digestion.
1999;60(1):11–6.
6. Triadalopoulos G, Aaronson M, Sackel S, etal. Medical treatment of esophageal achalasia.
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7. Gelfond M, Rozen P, Gilat T.Isosorbide dinitrate and nifedipine treatment of achalasia: a clini-
cal, manometric and radionuclide evaluation. Gastroenterology. 1982;83:963–9.
8. Bortolotti M, Mari C, Lopilato C, etal. Effects of sildenal on esophageal motility of patients
with idiopathic achalasia. Gastroenterology. 2000;111:253–7.
9. Eherer AJ, Schwetz I, Hammer HF, etal. Effect of sildenal on oesophageal motor function in
healthy subjects and patients with oesophageal motor disorders. Gut. 2002;50(6):758–64.
10. Kahrilas PJ, Bredenoord AJ, Carlson DA, etal. Advances in management of esophageal motil-
ity disorders. Clin Gastroenterol Hepatol. 2018;16(11):1692–700.
11. Sanaka MR, Thota PN, Parikh MP, et al. Peroral endoscopic myotomy leads to higher rates
of abnormal esophageal acid exposure than laparoscopic Heller myotomy in achalasia. Surg
Endosc. 2019;33(7):2284–92.
12. Samo S, Qayed E.Esophagogastric junction outow obstruction: where are we now in diagno-
sis and management? World J Gastroenterol. 2019;25(4):411–7.
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13. Van Hoeij FB, Smout AJ, Bredenoord AJ, etal. Characterization of idiopathic esophagogastric
junction outow obstruction. Neurogastroenterol Motil. 2015;27:1310–6.
14. Scherer JR, Kwiatek MA, Soper NJ, etal. Functional esophagogastric junction obstruction
with intact peristalsis: a heterogenous syndrome sometimes akin to achalasia. J Gasgtrointest
Surg. 2009;13:2219–25.
15. Perez-Fernandez MT, Santander C, Marinero A, et al. Characterization and follow-up
of esophagogastric junction outow obstruction detected by high resolution manometry.
Neurogastroenterol Motil. 2016;28:116–26.
16. Molina-Infante J, Lucendo AJ.Proton pump inhibitor therapy for eosinophilic esophagitis: a
paradigm shift. Am J Gastroenterol. 2017;112:1770–3.
17. Babaei A, Szabo A, Shad S, etal. Chronic daily opioid exposure is associated with dyspha-
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2019;31(7):e13601.
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esophageal sphincter-dependent and esophageal sphincter-independent jackhammer esophagus. Neurogastroenterol Motil. 2019;31(2):e13507.
19. Hong YS, Min YW, Rhee PL.Two distinct types of hypercontractile esophagus: classic and
spastic jackhammer. Gut Liver. 2016;10:859–63.
20. Filicori F, Dunst CM, Sharata A, etal. Long-term outcomes following POEM for non- achalasia
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2018;131(9):1034–40.
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in diffuse esophageal spasm. J Clin Gastroenterol. 2001;33:27–31.
23. Aggarwal N, Lopez R, Gabbard S, et al. Spectrum of esophageal dysmotility in systemic
sclerosis on high-resolution esophageal manometry as dened by Chicago classication. Dis
Esophagus. 2017;30(12):1–6.
24. Jeong J, Kim SE, Park MI, etal. The effect of anti-reux therapy on patients diagnosed
with minor disorders of peristalsis in high-resolution manometry. Korean J Gastroenterol.
2017;69(4):212–9.
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A. M. Brown and A. D. Pryor
Esophageal Motility Disorders
11
MichaelJureller andErinMoran-Atkin
Overview
Esophageal motility disorders are broad and present at various times in their natural
course. This can make an exact diagnosis challenging. These various pathologies
unfortunately have no denitive cure. All treatments, medical or surgical, are based
on palliation and symptom relief [1].
The goal of this chapter is to provide an overview of the latest recommendations
in the diagnosis, workup, and management in a spectrum of esophageal dysmotility
syndromes.
History
The rst account of surgical disease of the esophagus dates from 3600 BC to
2500BC to the famed ancient Egyptian “Edwin Smith Papyrus,” in which there is a
description of “a gaping wound of the throat penetrating the gullet,” and the repair
of a cervical esophagus with assumingly a muscle ap, “Thou shouldst bind it with
fresh meat the rst day. Thou shouldst treat it afterwards with grease, honey, (and)
lint every day, until he recovers” [2, 3].
Later, circa AD 0 Chinese scripts detail patients with esophageal cancer and
associated dysphagia and dysmotility [4]. The rst documented treatment of esophageal dysphagia, thought to be achalasia, was recorded in 1679 by Thomas Willis in
which he described using a sponge-tipped whale bone to assist in passage of food
M. Jureller (*) · E. Moran-Atkin
General Surgery, Monteore Medical Center/Albert Einstein College of Medicine,
Bronx, NY, USA
e-mail: mjurelle@monteore.org
© Springer Nature Switzerland AG 2021
N. Zundel et al. (eds.), Benign Esophageal Disease,
https://doi.org/10.1007/978-3-030-51489-1_11
113
114
M. Jureller and E. Moran-Atkin
bolus lodged in the esophagus [5, 6]. Later, in 1913, Heyrovsky published the rst
open surgical approach to “idiopathic dilation of the esophagus” in which he
described a series of patients in which he performed an anastomosis of the distal
esophagus to the gastric fundus [7]. Shortly afterward, Heller, De Bruine
Groeneveldt, and Zaaijer described the esophagocardiomyotomy [8], which has
since been modernized to what we refer to as the Heller myotomy. Since that time,
minimally invasive techniques using endoscopy and robotics have emerged, which
we will explore in this chapter. Presently, treatment for esophageal dysmotility syndromes ranges from behavioral, to pharmacologic, to endoscopic and surgical.
Initial Workup andDiagnosis
History andPhysical Exam
As with all ailments, proper diagnosis begins with the careful history and physical
examination of the patient. Most patients will complain of chest pain, and thus it is
important to rule out acute coronary syndrome while proceeding with a workup.
Particular attention should be paid to habits pertaining to diet and associated symptoms including chest pain and weight loss. Points to question in detail are any symptoms of dysphagia, retrosternal chest pain, immediate postprandial regurgitation,
and halitosis [9, 10]. With achalasia, patients may complain of retrosternal pains
when ingesting cold liquids and cold substances such as ice cream, which sit statically in the distal esophagus. If dysphagia is present, what is its quality? Has the
dysphagia been progressive and does it favor solids or liquids? If gastric bloating,
distension, and delayed postprandial emesis are endorsed, gastroparesis may be
present. Upper respiratory tract complaints may be present as well, which are similar to those with gastrointestinal (GI) reux disease such as cough, asthma, and even
pulmonary brosis [11].
The Eckardt scoring system (Fig. 11.1) is traditionally used for patients with
dysphagia and is a good and validated subjective marker for the need for treatment
and can be followed postoperatively [12–15].
Physical examination, while important, is likely to be unremarkable. With the
exception of signs of weight loss such as cachexia, temporal wasting, and thinning
Score
Symptom
Dysphagia
Regurgitation
Chest pain
Weight loss
(kg)
Fig. 11.1 Eckardt score graded 0–12 for subjective measurement of severity of dysphagia
0
None
None
None
0
1
Occasional
Occasional
Occasional
<5
2
Daily
Daily
Daily
5–10
3
Every meal
Every meal
Several times
per day
>10
11 Esophageal Motility Disorders
of the thenar eminences, an examination is likely to be negative. It is of utmost
importance to examine nodal basins, as esophageal and gastric cancers should be a
part of the initial differential diagnosis. The ndings of enlarged cervical, supraclavicular or periumbilical lymphadenopathy will drastically change the further
workup and management.
115
Initial Testing
Upper GI Fluoroscopy
Fluoroscopic evaluation is the rst test of choice and should be obtained on all
patients being assessed for upper GI motility disorders. Contrast-enhanced video
uoroscopy should be performed prior to endoscopy to evaluate for diverticulum,
since endoscopy in this setting can possibly result in perforation. Video uoroscopy
allows for visualization of esophageal dilation, length, the presence of diverticula or
a hiatal hernia, as well as gastroesophageal reux. Several pathognomonic signs can
be present on an esophagram, most famously the “bird’s beak” (Fig.11.2) appearance of the esophagus at the lower esophageal sphincter [16].
The Rezende classication (Fig.11.3), sorted between I and IV, has typically
been used to communicate the extent of esophageal dilation and tortuosity [17].
EGD
Esophagogastroduodenoscopy (EGD) should be performed on all patients for all
suspected esophageal motility disorders and most other pathologies of the foregut.
There are multiple utilities for EGD including, importantly, its assessment for carcinoma. Additional pertinent ndings on EGD are for the caliber and mucosal quality of the esophagus, if a hiatal hernia is present and the concomitant presence of
Helicobacter pylori [11]. Biopsies should always be taken of any suspicious esoph-
ageal, gastric, or duodenal lesions.
Endoscopic ndings particularly indicative of achalasia are numerous. In 2012,
the Japan Esophageal Society established several typical ndings including the dilation of the esophageal lumen, retained food bolus in the distal esophagus after their
midnight fast, whitish thickening along the mucosa—a combination of adhesive
debris from food and candida—functional stenosis of the gastroesophageal junction, and abnormal contractions of the esophageal body [18] (Fig.11.4). An additional nding, a so-called “Pinstripe pattern” can also been seen in up to 60% of
patients and is characterized by the longitudinal wrinkling of esophageal [19]
(Fig.11.5). On passage of the endoscope of the gastroesophageal junction, a typically popping sensation may be felt as the endoscope overcomes the pressure of the
lower esophageal sphincter.