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307

Esophagogastric Junction
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Outow Obstruction
KellyM.Herremans, J.ChristianBrown,
andAlexanderL.Ayzengart
Case Scenario
67-year-old woman presented with a 3-year history of dysphagia
(mostly to solids), associated with occasional regurgitation of
undigested food and substernal chest pain. Her workup included a
diagnostic upper endoscopy, timed barium esophagram, and highresolution manometry. Manometry was consistent with esophagogastric junction outow obstruction and demonstrated elevated
lower esophageal sphincter integrated relaxation pressure of
18.8mmHg (Fig.22.1). Peristalsis was normal with 100% bolus
clearance. No evidence of intraluminal mucosal pathology, strictures, or external compression was noted on endoscopy. Timed
barium esophagram showed retention of liquid contrast at 1min
and eventual passage of the barium tablet after 5min, but no evidence of hiatal hernia, gastric volvulus, or esophageal diverticulum.
22
K. M. Herremans · J. C. Brown · A. L. Ayzengart (*)
Department of Surgery, University of Florida Health,
Gainesville, FL, USA
e-mail: kelly.herremans@surgery.u.edu
© Society of American Gastrointestinal and Endoscopic Surgeons
(SAGES) 2023
A. D. Patel et al. (eds.), The SAGES Manual of Physiologic
Evaluation of Foregut Diseases,
https://doi.org/10.1007/978-3-031-39199-6_22
309

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Fig. 22.1 Findings of EGJOO on HRM
Introduction
K. M. Herremans et al.
Esophagogastric junction outow obstruction (EGJOO) is classied as a major esophageal motility disorder by the Chicago
Classication of Esophageal Motility Disorders, version 4.0, and
is dened by recently rened ndings on high-resolution manometry (HRM) and its associated protocol incorporating patient positioning and provocative swallowing. EGJOO is characterized by
an elevated integrated relaxation pressure (IRP) with preserved or
weak peristalsis, such that the criteria of achalasia are not met.
IRP is considered elevated when above the threshold of 15mmHg,
albeit this is technology-specic.

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311
Etiology
Findings of EGJOO are not uncommon and are reported in up to
14% of HRM studies. It is more commonly found in women (51–
88%) and the average age of diagnosis is between 56 and 69years
old. Etiologies of EGJOO vary signicantly and include mechanical, inammatory, medication-related, and functional causes.
Secondary causes of EGJOO include a wide range of pathologies
(Table22.1). Functional, or idiopathic, EGJOO is diagnosed once
secondary causes have been excluded.
Table 22.1 Secondary Causes
Secondary Causes of EGJOO
Mechanical Inammatory Medication-related
• Hiatal hernia
• Gastric volvulus
• Distal esophageal
– Stricture
– Schatzki ring
– Web
– Diverticulum
• Esophageal varices
• Vascular
compression
• Malignancy
• Postsurgical
• Reux esophagitis
• Eosinophilic
esophagitis
• Systemic sclerosis
• Amyloidosis
• Chronic opioid
use
• Antipsychotics

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K. M. Herremans et al.
Clinical Presentation
Patients diagnosed with EGJOO present with a variety of symptoms. The most common symptom prompting HRM evaluation is
dysphagia (46%), but other symptoms include chest pain (32%),
heartburn (20%), nausea, and vomiting (19%). However, patients
typically present with a combination of symptoms. EGJOO may
also be found incidentally on routine preoperative evaluation for
anti-reux procedures. Therefore, large volume foregut centers
may report higher incidence of EGJOO.
Diagnosis
The diagnosis of EGJOO is made based on HRM ndings of an
elevated median IRP above 15mmHg with intact peristalsis. It is
differentiated from achalasia based on the presence and degree of
peristalsis. IRP measurement is representative of the deglutitive
esophagogastric junction relaxation and is recorded as a median
pressure (mmHg) of ten test swallows.
Additional information from HRM studies may be utilized to
help support the diagnosis of EGJOO and identify patients who
would benet from intervention. Findings of delayed bolus transit
combined with patient symptoms of dysphagia and chest pain are
highly predictive of clinically relevant EGJOO. Elevated distal
contractile integral (DCI) >11,000 mmHg-cm-s in addition to
elevated IRP is predictive of symptom persistence after lower
esophageal sphincter (LES) directed therapy. Though further
research is needed, panesophageal pressurization during rapid
drink challenge is found to be related to increased severity of
symptoms and likelihood of progression to achalasia.
Additional methods for evaluation of EGJOO include endoscopy, timed barium esophagram (TBE), computed tomography
(CT), and endoscopic functional luminal imaging probe
(EndoFLIP). If not previously performed, endoscopy should be
completed to assess for secondary causes of outlet obstruction.
Thorough endoscopic evaluation, including forward and retro-

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exed views, is necessary to evaluate for secondary causes such
as hiatal hernia, postsurgical changes, strictures, rings, gastric and
esophageal tumors, and esophagitis. Endoscopic ultrasound may
also be used to further elucidate obstructive etiologies if suspicious ndings are noted on endoscopy. TBE serves a dual purpose
in the evaluation of EGJOO, as it may be used to rule out secondary causes, but may also be used to indicate clinically relevant
disease. This test is particularly useful in differentiating EGJOO
from untreated achalasia. Addition of a barium tablet may be used
to supplement barium esophagram. Delay in passage of barium
tablet further indicates functional or anatomical obstruction. CT
imaging may be used to evaluate secondary causes of
EGJOO. However, CT should be considered on an individual
basis as it has been found to yield little additional information.
Endoscopic FLIP topography is a relatively new method that has
been employed to assess esophageal motility. The EndoFLIP
probe uses high-resolution impedance planimetry to measure the
relationship between luminal dimensions and distensive pressure
during volumetric distension. It may be utilized trans-nasally in
an awake patient, but also can be used as a quick 5-min adjunct to
endoscopy or in the intraoperative setting. Despite limited adoption in clinical settings, EndoFLIP may be used as a complementary tool to assess EGJ dynamics. It is recommended that data
from multiple testing modalities should be evaluated and utilized
on a case-by-case basis in order to determine the etiology of
patient’s EGJOO.
313
Management
EGJOO is a heterogeneous disorder composed of both mechanical and functional motor abnormalities. As a result, management
strategies for the treatment of EGJOO are as diverse as its underlying causes. Appropriate diagnosis, as identied during clinical
evaluation, denes the practical treatment modality. However,
thorough review of these individual clinical entities and their
associated treatment outcomes is beyond the scope of this chapter.

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A portion of manometrically identied EGJOO lacks a discrete
mechanical obstruction and is classied as functional, or idiopathic, EGJOO (iEGJOO). Some indicate that iEGJOO is a variant phenotype of achalasia; the cardinal feature of impaired lower
esophageal sphincter relaxation occurs, but with intact peristalsis.
Although tailoring the therapeutic modality to the underlying etiology is likely critical to achieving successful long-term treatment
outcomes, a paucity of evidence exists to support an optimal
approach.
For patients with incidentally found EGJOO on HRM, watchful waiting is an appropriate option as many resolve without intervention. Differentiating between patients with nil-to-mild
symptoms and moderate-to-severe symptoms helps to delineate
between further management options. In patients with mild or
atypical symptoms, expectant management may be recommended
initially, as overall spontaneous resolution rate is approximately
40%.
Patients with signicant dysphagia and severe chest pain are
less likely to have symptom resolution without intervention.
Manometric ndings of DCI >11,000mmHg-cm-s, delayed bolus
transit, intrabolus pressure greater than 24mmHg, and panesophageal pressurization on rapid drink test help to distinguish clinically signicant disease. Delayed barium passage as well as
retention of barium tablet during further workup may also assist
in identifying patients who would benet from intervention.
K. M. Herremans et al.
Treatment
Clinical evidence regarding the treatment of EGJOO remains
sparse as a result of the rarity of this disease. However, due to the
overlap with achalasia, multiple therapeutic options have been
applied with varying success. If a patient’s presentation includes
the aforementioned severe symptoms or evaluation ndings, targeted lower esophageal sphincter (LES) therapy is warranted and
treatment options must be tailored to patient’s symptoms, comorbidities, surgical candidacy, and physician expertise.

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315
Pharmaceutical treatments have been explored with mixed outcomes. Medications frequently trialed are calcium channel blockers, PPI’s, nitrates, antispasmodics, and prokinetics. Initiation of
pharmacologic treatment is a practical starting point given their
safety prole and known outcomes in achalasia. However, it has
been reported that only 50% of patients with EGJOO exhibit any
response to therapy. Acotiamide is a new prokinetic medication
that has been heralded for its preliminary results in functional
dyspepsia and subsequently EGJOO with up to 83% of patients
showing symptomatic improvement. In the United States, it
remains in clinical trials at the time of this publication.
Botulinum toxin may also be injected into the LES. The effect
is transient and decremental with subsequent injections.
Botulinum toxin injections are inferior to myotomy and should be
reserved for poor surgical candidates as resultant scarring from
injections may obscure esophageal surgical planes if additional
interventions need to be carried out.
Bougie dilation may be reserved for EGJOO secondary to
inammatory or peptic strictures, but pneumatic dilation is preferred in functional EGJOO.Pneumatic dilation is a safe, effective option for patients with clinically signicant EGJOO. Of
note, age less than 40 and male gender were associated with lower
rates of sustained symptomatic improvement following pneumatic dilation. However, clinical improvement is typically
achieved through repeat dilations. Pneumatic dilation is noninferior to myotomy in the treatment of EGJOO, though patient
characteristics should be considered.
Heller myotomy, performed laparoscopically or robotically, is
a successful and sustainable treatment option in EGJOO with
90–100% success in symptom resolution. Classically, a fullthickness myotomy is created at least 5–6 cm proximal and
2–3cm distal to the esophagogastric junction. At the time of operation, a partial fundoplication is typically performed to reduce
postoperative GERD. Heller myotomy remains an excellent
option for the treatment of EGJOO in patients who are deemed
appropriate surgical candidates.

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K. M. Herremans et al.
Peroral endoscopic myotomy (POEM) is a relatively new technique that may be used to perform LES myotomy in EGJOO.In
this procedure, a long submucosal tunnel is created using a combination of hydrodissection and electrocautery, followed by an
endoscopic myotomy of the circular muscle bers. The myotomy
length can extend beyond 15cm proximal and 3cm distal to the
esophagogastric junction and can be tailored to the underlying
esophageal disease. Short-term data are promising (>90% success
rate), but future long-term outcomes require further analysis.
Notably, patients undergoing POEM have signicant rates of
symptomatic reux and esophagitis following the procedure.
Conclusion
Given the heterogeneity of EGJOO and its unclear clinical signicance, a progression of treatment modalities from the least
invasive to the most invasive (i.e., surgery) is a reasonable therapeutic approach (Fig.22.2). An eventual progression to the POEM
Fig. 22.2 Treatment Algorithm
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