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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_1033_Библиотеки_им_академика_М_И_Перельмана
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S. A. Abel and J. R. Broucek
Epidemiology
JE is a rare subset of esophageal motility disorders. Early population studies determined prevalence rates of JE to be on the order
of 4–5% of patients with esophageal dysmotility [3, 4]. In 2018,
Kristo etal. published a cohort study of 2443 patients undergoing
surgical evaluation for gastroesophageal reux and found the
prevalence of JE to be 1.7% in that population [5]. A retrospective
cohort study of 1099 patients undergoing high-resolution manometry (HRM) revealed a diagnosis of JE in 3% of study patients,
89% of which were female [6]. A small cohort of 74 patients with
either JE or nutcracker esophagus was published in 2017 by AlQaisi etal. demonstrating similar demographics between the two
patient populations. Roughly two-thirds of patients were female
with an average BMI of 28kg/m2 and an average age in the seventh decade of life [7]. Given the low prevalence of JE, large epidemiologic studies have not been conducted.
Pathophysiology
The pathophysiology of JE has been theorized, but is poorly
understood. In contrast to DES which is thought to occur because
of a decrease in inhibitory neuronal signaling that leads to early
and rapid contractions in the distal esophagus, JE is thought to
occur because of either an increase in excitatory neuronal signaling or an increased response to excitatory signaling. This ultimately results in smooth muscle hypertrophy throughout the
esophagus. There have been several studies to demonstrate eosinophilic inltration of the muscularis propria in JE [8]. Eosinophilic
inltration may have a role in facilitating enhanced neuronal signaling; however, it is not always present on histopathology in
patients diagnosed with JE. The underlying mechanism of
enhanced signaling remains unknown.
Esophagogastric junction (EGJ) obstruction is frequently seen
in conjunction with JE.There is also limited evidence of resolution of symptoms of JE and normalization of HRM after addressing the cause of EGJ obstruction such as in one case report of JE
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developing in a patient with a laparoscopic gastric band and
resolving after deation of the band. This association suggests a
possible role of obstruction at the EGJ in the pathophysiology of
JE [9]. This role of EGJ dysfunction in the pathophysiology of JE
has been studied, and impaired EGJ relaxation in patients with JE
has been found to be a predictor of disease progression and a possible indication for early and aggressive treatment [10]. There are
several case reports and prospective studies of JE that demonstrate the potential for progression of JE to type III achalasia,
especially in the setting of impaired esophagogastric junction
relaxation [10–12]. While EGJ dysfunction and eosinophilia are
often seen in the setting of JE, not all patients with JE have these
characteristics. Furthermore, there are reports in the literature of
JE occurring after radiofrequency catheter ablation or in the setting of opioid-induced esophageal dysfunction, pointing to a more
complex pathophysiologic process [13, 14].
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Clinical Features
Symptoms
Like other esophageal dysmotility disorders, JE presents with a
spectrum of symptoms. The most common symptom of JE is dysphagia to both solids and liquids, which occurs in up to two-thirds
of patients. Noncardiac chest pain occurs in roughly half of
patients and can be either the principle presenting symptom or can
be secondary to dysphagia with the sensation of food getting
stuck in the thoracic esophagus. Gastroesophageal reux has been
described in one-third of patients and weight loss in roughly a
quarter of patients with JE [15, 16].
Patient-reported symptoms are not closely correlated with the
number of hypercontractile swallows and mean distal contractile
index (DCI). Dysphagia appears to be most closely correlated to
elevated lower esophageal sphincter (LES) pressures [17]. Chest
pain is a common reason for presentation to the emergency department. Those presenting with noncardiac chest pain will have normal investigational workups. A thorough diagnostic workup in
these patients should include esophageal studies when indicated.
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S. A. Abel and J. R. Broucek
Imaging/Tests
Upper gastrointestinal endoscopy is an integral component of any
esophageal dysmotility evaluation. It can differentiate between
organic causes of dysphagia and mechanical obstruction. It is also
a means to obtaining a full-thickness biopsy of the esophagus.
Cap-tted endoscopic mucosal resection (EMR) with peroral
endoscopic myotomy (POEM) has been described as a technique
for obtaining full-layer histopathology. One particular study using
this technique demonstrated reduced numbers of interstitial cells
of Cajal in the muscularis propria, providing a possible explanation for the uncoordinated peristalsis seen in JE [18]. A barium
esophagram and a pH study can be useful components of the diagnostic evaluation when conditions other than esophageal dysmotility are suspected.
Diagnosis of JE is conrmed on HRM [19]. While the diagnostic criteria of JE are based on HRM results, the diagnostic accuracy of HRM for major esophageal motility disorders has been
subject to some debate. Some studies would suggest that the diagnostic accuracy of HRM is higher for achalasia than JE [20]. As
an adjunct to HRM, multiple rapid swallows (MRSs) and rapid
drink challenge (RDC) can be performed. These adjuncts can
yield information about inhibitory/excitatory neuromuscular
function and latent obstruction, respectively. One study evaluating
these adjuncts reported the presence of abnormal inhibition during MRS in some patients with JE and increased stimulation after
single swallows than after MRS.An obstructive pattern on RDC
was correlated to symptoms of dysphagia. These adjuncts during
HRM may help guide therapeutic strategies [21].
Dierential Diagnosis
The differential diagnosis for the most common presenting symptom of JE, dysphagia to both solids and liquids, includes structural and functional etiologies. Structural conditions include
different benign or malignant obstructing masses, esophageal ring
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or web, or esophageal stricture. Esophageal strictures occur secondary to peptic ulcer disease, caustic injury, radiation, prior surgery, or ischemia. Functional conditions include achalasia,
ineffective or absent esophageal motility, scleroderma, infectious
or eosinophilic esophagitis, or DES. Patient’s presenting with
chest pain would need to be evaluated for both cardiac etiologies,
as well as noncardiac etiologies including peptic ulcer disease,
gastroesophageal reux disease, esophagitis, and hypercontractile
esophageal motility disorders such as DES.
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Diagnosis
Evaluation
When a patient presents with symptoms suspicious for esophageal dysmotility, a systematic approach to diagnosis is necessary.
First, it is important to identify any strong risk factors for esophageal disease such as a history of relevant radiation therapy, caustic
injury, surgery, or cancer. Patients with these risk factors should
be evaluated rst with an esophagram. This may detect an
obstructing lesion, prompting further workup to rule out malignancy. Although rare, midesophageal pulsion diverticula have
been described in patients with JE [22, 23]. Such a nding on
esophagram should not change the focus of further investigation.
Other nonspecic ndings on esophagram may include multifocal
contraction and delayed esophageal emptying, and in patients
with EGJ dysfunction, a narrowed gastroesophageal junction may
be evident (Fig.20.1). The next appropriate step in diagnosis is to
perform endoscopy with esophageal biopsies. This may reveal an
esophageal web, a stricture, a diverticulum, a mass, or it may
reveal infectious or eosinophilic esophagitis. If endoscopy with
biopsies reveals no abnormalities and a contrast swallow study
has already been performed, the next step in diagnostic workup is
to perform HRM, upon which the diagnosis of JE is based. Once
the diagnosis of JE is conrmed, a thorough systemic workup
should be considered. One study demonstrated that 25% of
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S. A. Abel and J. R. Broucek
Fig. 20.1 Barium esophagogram showed a constricted and curling of the
esophageal lumen resembling a corkscrew. (Sirinawasatien A, Sakulthongthawin P.Manometrically jackhammer esophagus with uoroscopically/endoscopically distal esophageal spasm: a case report. BMC Gastroenterol. 2021
May 17;21(1):222. doi: 10.1186/s12876-021-01808-3)
patients with JE had systemic inammatory diseases or synchronous neoplasias at the time of diagnosis [24].
Diagnostic Criteria
The Chicago classication of esophageal motility disorders is an
international consensus that has evolved in recent years, rst
being published in 2009 and later being revised in 2012 and 2014.
It relies on high-resolution esophageal pressure topography,
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derived from the combination of HRM and Clouse plots, to calculate the vigor of each distal esophageal contraction. This calculation accounts for the amplitude, duration, and vertical length of
each esophageal contraction and is reported as the DCI.
The Chicago Classication v3.0 is the most updated version of
this classication scheme, dening JE as >20% of observed swallows having a DCI >8000mmHg (Fig.20.2). JE can occur at any
level of the esophagus, including the LES.The diagnosis of nutcracker esophagus, which had been dened by DCI readings
between 5000 and 8000mmHg, was removed due to lack of clinical signicance [2].
The integrated relaxation pressure (IRP) is dened as the 4-s
mean EGJ pressure measured over 10s of relaxation following
upper esophageal sphincter relaxation with swallowing. The IRP
is used to determine the presence or absence of an EGJ outlet
obstruction and can help differentiate between different esopha-
Fig. 20.2 High-resolution esophageal manometry representative of the
patient’s swallows. The median integrated relaxation pressure (IRP) was borderline high at 15.5mmHg, a distal latency (DL) was normal at 6.5s, and the
mean distal contractile integral (DCI) was elevated to 14,458mmHg-s-cm.
High-amplitude peristaltic esophageal contraction with a DCI>8000mmHgs- cm represented the hypercontractile (JH) esophagus. (Sirinawasatien A,
Sakulthongthawin P. Manometrically jackhammer esophagus with uoroscopically/endoscopically distal esophageal spasm: a case report. BMC Gastroenterol. 2021 May 17;21(1):222. doi: 10.1186/s12876-021-01808-3)
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geal dysmotility disorders. For example, JE is characterized by a
markedly elevated DCI with a concomitantly elevated IRP.This
differs from spastic achalasia which does not present with an elevated IRP [2].
A key component of the diagnostic evaluation for JE is to distinguish it from DES.Both conditions affect the distal esophagus;
however, DES is characterized by premature (distal latency [DL]
<4.5s) but functional (DCI≥450mmHg·s·cm) contractions. JE
is characterized by vigorous contractions with normal latency [2].
One study, however, described two distinct subgroups of
JE.Classic JE was characterized by hypercontractility and normal
distal latencies, and spastic JE was characterized by hypercontractility and short distal latencies. This study found that classic
JE patients responded to anticholinergic agents, while spastic JE
patients did not [25].
S. A. Abel and J. R. Broucek
Management
Initial Management
The initial approach to a patient with a new diagnosis of JE is
medical management. JE, however, appears to be heterogeneous,
and the nuances of management can be difcult to perfect. The
rst component of medical management has long been pain control medications. These include conventional medications such as
NSAIDS and opioids, as well as anxiolytics, benzodiazepines,
and low-dose tricyclic antidepressants [26].
Given that the pathogenesis of JE is thought to involve an
imbalance between inhibitory and excitatory signaling; the second component of medical management has long been smooth
muscle relaxants such as nitrates, calcium channel blockers, and
phosphodiesterase inhibitors. In limited studies, nitrates have
demonstrated both clinical and manometric efcacy in patients
with esophageal spasm [27]. Studies have demonstrated efcacy
of calcium channel blockers in up to nearly 2/3 of patients [28].
Phosphodiesterase inhibitors have been shown to reduce contractile amplitude and velocity in patients with motility disorders
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[26]. Anticholinergics are also an option as some studies have
demonstrated cholinergic nerve excitation in association with the
characteristically strong contractions seen in JE [29].
Systemic steroid administration and antiallergic medications
have demonstrated efcacy in the management of JE in patients
with eosinophilic esophagitis or eosinophilic inltration on histopathology [28]. One particular study not only found resolution of
symptoms with systemic steroid administration, but also improved
esophageal muscularis propria thickening, and normalized manometric ndings [30]. Another study found resolution of dysphagia, but not chest pain with high-dose systemic steroid use [31].
Other literature, however, found that while systemic steroids
reduced the number of peripheral blood eosinophils and resulted
in the disappearance of eosinophils from the epithelium, there
was persistent eosinophil inltration into the muscular layer,
ongoing hyperperistaltic contractions on HRM, and no resolution
of symptoms [32].
Beyond these mainstays of medical management are some less
proven options. Botulinum toxin has been considered as a means
of reducing muscle contractility in some patients; however, it has
not been well studied in this patient population [33]. There are
case reports that demonstrate efcacy of the herbal medication
Shakuyakukanzoto in the treatment of JE because of its antispasmodic effect on smooth muscle [28, 34]. It has also been demonstrated to inhibit gastrointestinal motility on endoscopy when
sprayed into the lumen of the intestinal tract [35].
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Persistent Disease
JE patients who fail to improve with maximal medical therapy
may require surgical or endoscopic management. The goal with
such management is to decrease the hyperperistaltic contractions
and/or EGJ outlet obstruction that drives symptoms. This can be
accomplished with a Heller myotomy or with pneumatic dilatation. These techniques have demonstrated variable degrees of success; however, POEM is quickly emerging as an effective
alternative.
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POEM is a relatively new intervention for which robust data,
especially as it pertains to JE, are still emerging. Presently, there
is no expert consensus and no ofcial guidelines for the use of
POEM in the treatment JE.There are several small prospective
studies as well as systematic reviews and meta-analyses that demonstrate the safety and efcacy of this therapeutic modality in the
treatment of spastic esophageal disorders, including JE in both the
adult and pediatric populations [36–41]. Existing data demonstrate comparable short-term and midterm results when compared
to laparoscopic Heller myotomy and superior results when compared to pneumatic dilatation [42].
Some existing literature on the use of POEM in the treatment
of JE supports extending the myotomy through the LES to reduce
the risk of postprocedural ineffective esophageal motility and the
risk of progression to achalasia [38]. This is especially true in
patients with JE and impaired esophagogastric junction relaxation
[43]. Other studies would suggest that variations in the total myotomy length do not have a signicant effect on clinical success,
and POEM may cause irreversible brotic change, particularly in
the LES muscle, which predisposes patients to postprocedural
gastroesophageal reux [44, 45]. There is no consensus for myotomy length; however, consideration of more aggressive management may be prudent for JE patients presenting with dysphagia
secondary to EGJ outlet obstruction, and intermittent bolus clearance on impedance exam, as these patients are at risk of further
deterioration of esophageal function [43].
S. A. Abel and J. R. Broucek
Editors’ Note
Jackhammer
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285
Jackhammer esophagus. 66-year-old female with complaints of chest pain,
dysphagia, and GERD.Manometry reading: The study of the LES reveals a
shortened length and a hiatal hernia. The LES pressure is normal at 28mmHg
and relaxes completely. The esophageal body study demonstrates normal
peristalsis with high contraction amplitudes. Bolus transit is normal. Overall
the patient has a hiatal hernia and jackhammer esophagus. *Note the normal
bolus transit and normal LES relaxation in the face of hypercontractility in
the esophageal body
References
1. Roman S, Pandolno JE, Chen J, Boris L, Luger D, Kahrilas
PJ.Phenotypes and clinical context of hypercontractility in high-resolution esophageal pressure topography (EPT). Am J Gastroenterol.
2012;107(1):37–45. https://doi.org/10.1038/ajg.2011.313. Epub 2011
Sep 20.
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