Surgical Treatment of Gastrointestinal Motility Disorders

Current Problems in Surgery 53 (2016) 503–549
Contents lists available at ScienceDirect
Current Problems in Surgery
journal homepage: www.elsevier.com/locate/cpsurg
Surgical treatment of gastrointestinal motility

disorders
Jon S. Thompson, MDa,*, Sean J. Langenfeld, MDa,
Alexander Hewlett, DOb, Amareshewar Chiruvella, MDa,
Christopher Crawford, MDa, Priscila Armijo, MDc,
Dmitry Oleynikov, MDa
Introduction
Gastrointestinal tract motility disorders have been recognized for more than 100 years but
are being diagnosed with increased frequency. Specific disorders involve each segment of the
gastrointestinal (GI) tract, including the esophagus, stomach, small intestine, and colon.
Although initial medical management is the usual course, surgical procedures to treat these
disorders are being applied increasingly. A better understanding of underlying pathophysiology
of these conditions has broadened surgical options and improved outcomes.
Esophageal motility disorders
Normal esophageal motility
The esophagus is a muscular hollow tube that originates at the level of the cricoid cartilage
and normally terminates below the diaphragmatic hiatus. It is composed of the following
4 primary layers on cross-section: the mucosa, submucosa, muscularis propria, and adventitia.
The muscularis propria gradually changes from predominant skeletal muscle in the upper
esophagus to predominantly smooth muscle in the distal two-thirds. It is composed of the inner
From the a Department of Surgery, University of Nebraska Medical Center, Omaha, NE; b Department of Internal
Medicine, University of Nebraska Medical Center, Omaha, NE; and c Surgery, Federal University of São Paulo, São Paulo,
Brazil
n
Address reprint requests to Jon S. Thompson, MD, Department of Surgery, University of Nebraska Medical Center,
983280 Nebraska Medical Center, Omaha, NE 68198-3280.
E-mail address: jthompso@unmc.edu (J.S. Thompson).
http://dx.doi.org/10.1067/j.cpsurg.2016.08.006
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504 J.S. Thompson et al. / Current Problems in Surgery 53 (2016) 503–549
circular and outer longitudinal muscle layers. Within the diaphragmatic hiatus is a 2-4-cm
section of thickened circular muscle, which comprises the lower esophageal sphincter (LES).
The LES is conceptualized as having extrinsic and intrinsic components. The intrinsic portion
itself has 2 smooth muscle elements—the semicircular clasp fibers centered along the lesser
curve and the oblique sling fibers centered along the posterolateral wall wrapping around
anteriorly and thus forming the angle of His. The extrinsic components consist of the crura and
the phrenoesophageal ligament that anchor the esophagus to the diaphragm. Esophageal
innervation consists of both parasympathetic and sympathetic nerves, with peristalsis regulated
by the parasympathetic pathway from the vagus nerve and the intrinsic enteric nervous system.
With the entry of a food or liquid bolus into the esophagus, primary peristalsis moves the
bolus down the length of the esophagus. Peristaltic contraction in the striated muscle portion of
the esophagus is dependent on activation of excitatory neurons in the vagal nucleus ambiguus.
As the peristaltic wave progresses into the smooth muscle portion of the esophagus,
preganglionic neurons are activated in the dorsal motor nucleus of the vagus that subsequently
project onto inhibitory and excitatory neurons in the myenteric plexus of the esophagus. Under
normal circumstances, the inhibitory pathway is activated to relax the lower esophagus to
promote filling downstream. The inhibitory pathways are activated by preganglionic neurons in
the caudal dorsal motor nucleus and release nitric oxide and vasoactive polypeptide to promote
deglutitive inhibition. This is followed by sequential activation of excitatory neurons by the
rostral dorsal motor nucleus, which release acetylcholine and promote esophageal contraction.
Achalasia
Achalasia is a rare primary esophageal motility disorder that is characterized by the absence
of peristalsis and a defective relaxation of the LES that results in impaired bolus transport and
food stasis in the esophagus. Achalasia is derived from the Greek word “khalasis” translated as
“not loosening or relaxing.” It is relatively uncommon, with an incidence of 1 per 100,000
individuals and a prevalence of 10 per 100,000. The disease usually presents between 30 and 60
years of age and is equally distributed between men and women, without racial predilection.1
Although the clinical presentation and symptomatology have remained unchanged over time,
the modalities for diagnosing and managing this rare but frustrating disease process have
undergone advances in recent years. Some of these advances such as high-resolution
manometry (HRM) have provided new insights into the pathogenesis and clinical manifestations
of this disorder.
Achalasia is characterized by a functional loss of myenteric plexus ganglion cells in the distal
esophagus and LES, the cause of which is yet unclear. One hypothesis is that it might be the
consequence of an inflammatory or autoimmune neurodegenerative process triggered by an
indolent viral infection (herpes and measles) in conjunction with a genetically susceptible
host.2,3 This chronic inflammation causes a degeneration of inhibitory postganglionic neurons in
the distal esophagus and LES. The degeneration of the inhibitory neurons leads to unopposed
cholinergic stimulation, resulting in impaired relaxation of the LES, with or without
hypercontractility and rapid propagation of contractions in the distal esophagus.2 Variable
expression of these abnormalities can occur in individuals, but impaired deglutitive relaxation is
the only universally required defining feature of achalasia. Infection with Trypanosoma cruzi or
Chagas disease can also result in achalasia and patients often have other features of myenteric
destruction including megacolon, cardiomyopathy, and other neurologic disorders.4
Clinical presentation
The classic presentation of most patients is with progressive dysphagia to solids and liquids
(90%) associated with regurgitation of undigested food or saliva (45%). The second most common
symptom presenting is heartburn (75%) followed by noncardiac chest pain with intake of food
(20%-40%). This was corroborated in a single-center review by Tsuboi and colleagues over a
period of 24 years (1984-2008) and found that patients with achalasia most commonly
J.S. Thompson et al. / Current Problems in Surgery 53 (2016) 503–549 505
presented with dysphagia and heartburn.5-7 Respiratory symptoms are also common because of
the decreased esophageal clearance resulting in aspiration of food or liquid. Chronic aspiration is
encountered in 20%-30% of patients and approximately 33% complain of sore throat or
hoarseness. Approximately 5%-10% of patients have unintentional weight loss.
Other clinical processes, however, can manifest with similar symptoms. These include
pseudoachalasia from tumors in the gastric cardia or those infiltrating the myenteric plexus
(adenocarcinoma of the gastroesophageal junction, metastases from pancreatic, breast, lung, or
hepatocellular cancers) or secondary achalasia from a prior tight fundoplication or a
laparoscopic gastric band. This underscores the importance of the diagnostic modalities to rule
out these possibilities and diagnose true achalasia.
Diagnostic procedures
Diagnostic procedures for most esophageal motility disorders including achalasia are: (1)
barium swallow or barium esophagram (BE), (2) esophagogastroduodenoscopy (EGD),
(3) manometry or motility study, and (4) endoscopic ultrasound to rule out pseudoachalasia
based on clinical suspicion.
Barium swallow. The classic findings of achalasia on an esophagram include a narrow

esophagogastric junction (EGJ) with a “bird's beak” appearance, aperistalsis of the esophageal
body with varying degrees of esophageal dilation, and poor emptying with an air-fluid level. In
late cases of achalasia, a characteristic sigmoid conformation of the esophagus can be visualized
within the thoracic cavity. In certain cases of spastic achalasia, distal corkscrewing of the
esophagus from nonpropagated simultaneous contractions can be present and be mistaken for a
picture of diffuse esophageal spasm.
An additional role for an esophagram is to provide an objective assessment of esophageal
emptying after therapy, as symptom relief in many patients does not parallel effective
esophageal clearance. This can be demonstrated by measuring the barium column height at 1
minute and at 5 minutes after upright ingestion of a large barium bolus. This has now come to be
known as the timed barium esophagram.8 This may be useful in follow-up to help identify
patients who may be likely to fail initial treatment and need future interventions.
Esophagogastroduodenoscopy or endoscopy. The main role of EGD in achalasia is to rule out

secondary causes such as a mechanical obstruction or pseudoachalasia as the clinical and
manometric findings can mimic true achalasia. Endoscopic findings in true achalasia can range
from a normal-appearing esophageal body to a dilated, tortuous esophagus filled with
undigested food and liquid. It is not uncommon in advanced cases to find inflamed mucosa
with erosions secondary to food stasis, pill esophagitis, or candida infection. The distal
esophagus and LES usually has a rosette-like appearance with some mild-to-moderate resistance
at the EGJ, which can be opened with light pressure from the scope. In the presence of severe
resistance and inability to pass a standard adult scope, concern should be raised for a malignant
process. Biopsies are usually not indicated if findings are consistent with achalasia. Occasionally,
biopsies may show an eosinophilic infiltrate secondary to chronic inflammation, which can be
confused with eosinophilic esophagitis. However, these can be distinguished using the classic
manometry findings and clinical presentation of dysphagia to both solids and liquids.9,10
Endoscopic ultrasound should be performed in patients with a strong suspicion for malignancy,
and especially in elderly patients with marked weight loss and a short history of dysphagia.
Esophageal manometry. Manometry remains the gold standard test to establish a diagnosis of
achalasia and other primary motility disorders. Conventional manometry using either water-
perfused or solid-state catheter systems has been replaced over the past decade with HRM.11,12
The latter uses 36 closely spaced pressure sensors at 1-cm intervals to create a dynamic
representation of pressure change along the entire length of the esophagus compared with the
conventional systems where sensors were spaced at 5-cm intervals. The data gathered from
these sensors are then plotted along a space-time continuum giving rise to an esophageal
pressure topogram also known as a Clouse plot. This seminal work by led to an improved
understanding of esophageal peristaltic activity, and eventually to the creation of a new
classification scheme for motility disorders called the Chicago classification.13
The Chicago classification is based on scoring of 10 water swallows of 5 mL performed in the
supine position using the HRM catheters. EGJ relaxation, esophageal contractile activity, and
esophageal pressurization are evaluated for each swallow. This classification was first published
in 2009 and subsequently updated in 2012.12,13 The latest version of this classification (version
3.0) was published in May 2014 by the HRM working group.14 The Chicago classification uses
more clinically based methods to group the esophageal function disorders as opposed to the
older categories of achalasia, DES, nutcracker esophagus (NE), hypertensive LES, and ineffective
esophageal motility. This classification system considers 3 metrics from HRM results to assist in
classification (Table 1).
Table 1
The Chicago classification of esophageal motility v3.0. (Reprinted with permission from Kahrilas et al.14)
Criteria
Achalasia and EGJ outflow obstruction

Type I achalasia (clank achalasia) Elevated median IRP 4 15 mm Hg*), 100% failed peristalsis
(DCI o 100 mm Hg)
Premature contractions with DCI values less than 450 mm Hg s cm satisfy criteria
for failed peristalsis
Type II achalasia (with esophageal Elevated median IRP ( 415 mm Hg*), 100% failed peristalsis, panesophageal
compression) pressurization with Z20% of swallows
Contractions may be masked by esophageal pressurization and DCI should not be
calculated
Type III achalasia (spastic Elevated median IRP ( 415 mm Hg*), no normal peristalsis, premature
achalasia) (spastic) contractions with DCI 4 450 mm Hg s cm with Z20% of
swallows
May be mixed with panesophageal pressurization
EGJ outflow obstruction Elevated median IRP ( 415 mm Hg*), sufficient evidence of peristalsis such
that criteria for types I-III achalasia are not met†
Major disorders of peristalsis (Not encountered in normal subjects)

Absent contractility Normal median IRP, 100% failed peristalsis
Achalasia should be considered when IRP values are borderline and when there is
evidence of esophageal pressurization
Premature contractions with DCI values less than 450 mm Hg s cm meet criteria
for failed peristalsis
Distal esophageal spasm Normal median IRP, Z20% premature contractions with DCI
4450 mm Hg s cm†. Some normal peristalsis may be present
Hypercontractile esophagus At least 2 swallows with DCI 4 8000 mm Hg s cm*,‡
(jackhammer)
Hypercontractility may involve, or even be localized to, the LES
Minor disorders of peristalsis (Characterized by contractile vigor and contraction pattern)

Ineffectiye esophageal motility Z 50% ineffective swallows
(IEM)
Ineffective swallows can be failed or weak (DCI o 450 mm Hg s cm)
Multiple repetitive swallow assessment may be helpful in determining peristaltic
reserve
Fragmented peristalsis Z 50% fragmented contractions with DCI 4 450 mm Hg s cm
Normal esophageal motility Not fulfilling any of the above classifications
EGJ, esophagogastric junction; IRP, integrated relaxation pressure; DCI, distal contractile integral.
n
Cutoff value dependent on the manometric hardware; this is the cutoff for the Sierra device.
†
Potential etiologies—early achalasia, mechanical obstruction, esophageal wall stiffness, or manifestation of hiatal hernia.
‡
Hypercontractile esophagus can be a manifestation of outflow obstruction as evident by instances in which it occurs in
association with an IRP greater than the upper limit of normal.
EGJ morphology and deglutitive relaxation. With HRM, the relative localization of 2 constit-
uents of the EGJ, the LES, and the crural diaphragm (CD) define the EGJ subtypes. In type 1 EGJ,
there is complete overlap of the CD and LES with no spatial separation on the Clouse plot. In type
2 EGJ, the LES and CD are spatially separated with a less than 3-cm gap and the nadir pressure
between the 2 does not decline to the intragastric pressure. In type 3 EGJ, there is more than
3-cm separation between the LES and CD with the nadir pressure equal to or less than the
intragastric pressure. In type 3A, the pressure inversion point remains at the CD and in type 3B,
the pressure inversion point is at the LES.
During swallowing, the EGJ relaxation using HRM is evaluated using the integrated relaxation
pressure (IRP). It is defined as the mean of 4 seconds (contiguous or noncontiguous) of maximal
deglutitive relaxation in the 10-second window following deglutitive upper esophageal
sphincter (UES) relaxation.
Using these data, disorders of EGJ obstruction have been subdivided into 3 achalasia subtypes
and EGJ obstruction proper (Table 1).15 To date, 3 retrospective studies have shown that subtype
II has the best prognosis followed by subtypes I and III, in that order of prognosis.
Deglutitive peristaltic vigor and pattern. Metrics used from HRM to evaluate esophageal
contractile function are the distal contractile integral (DCI) and distal latency (DL). Per the current
guidelines, a DCI between 450 and 8000 mm Hg s cm is considered normal. A contraction with a
DCI of less than 100 mm Hg s cm defines a failed contraction, and a weak contraction occurs with
a DCI between 100 and 450 mm Hg s cm.15 A DCI Z 8000 defines hypercontractility.16
The contractile deceleration point (CDP) is a key landmark to assess contraction pattern. It is
the inflexion point in the contractile front on the 30 mm Hg isobaric contour in the distal
esophagus, which signifies the termination of peristalsis and onset of ampullary emptying. It is
usually located 2-3 cm proximal to the EGJ.17,18 The DL corresponds to the interval from UES
relaxation to the CDP which in turn marks the period of deglutitive inhibition preceding
esophageal contraction at the CDP.19 A DL less than 4.5 seconds is considered abnormal and
defines a premature contraction. The contractile front velocity has been removed from the
current version of the Chicago classification because of the lack of its specificity to define
esophageal spasm.
Intrabolus pressure pattern. Intrabolus pressure is characterized for each swallow using the
30 mm Hg isobaric contour and abnormal pressurization corresponds to regions greater than
30 mm Hg. The pattern is classified as panesophageal if it spans from the EGJ to the UES,
compartmentalized if it extends from CDP to EGJ, and EGJ pressurization if restricted to the zone
between the CD and LES.
Management
Currently, achalasia is a chronic condition without cure. Treatment options for achalasia are
aimed at reducing the pressure gradient across the LES, providing symptomatic relief, improving
esophageal emptying, and preventing the development of megaesophagus. Treatment modal-
ities include pharmacologic therapy, botulinum toxin injections (Botox), pneumatic dilation
(PD), laparoscopic Heller myotomy (LHM), and peroral endoscopic myotomy (POEM).
Pharmacotherapy. Oral pharmacologic therapy is the least effective treatment for achalasia and
hence has a minor role in its management.20 Calcium channel blockers (CCB) and nitrates are the
2 commonly used medications that transiently relax the LES and facilitate esophageal emptying.
Other agents that have been used are phosphodiesterase 5 inhibitors such as sildenafil,
anticholinergics, beta-adrenergic agonists, and theophylline.
The most commonly used CCB is nifedipine and has been found to decrease LES pressure by
13%-49% and improve patient symptoms by 0%-75%. Its duration of effect is 30-120 minutes and
is used 30-45 minutes before meals in 10-30 mg doses for best response. Sublingual isosorbide
dinitrate has been effective in reducing LES pressure by 30%-65% leading to symptomatic
improvement ranging from 53%-87%. This agent is administered in 5 mg doses, 10-15 minutes
before meals and has a shorter duration of action at 30-90 minutes.1,21
The clinical response to these agents is of short duration as drug tolerance develops rapidly,
the symptomatic improvement is incomplete, and these drugs can cause undesirable side effects
of headache, hypotension, and leg edema, thus limiting their use. These drugs are therefore only
considered for patients who cannot or refuse to undergo other invasive therapies and for those
in whom even botulinum toxin has failed.
Injection of Botulinum toxin. Botulinum toxin (BTX) is a presynaptic inhibitor of acetylcholine

release from the nerve endings. The toxin cleaves the SNAP-25 protein that is involved in the
fusion of the presynaptic vesicles containing acetylcholine with the neuronal plasma
membrane.22 This inhibition of exocytosis of acetylcholine causes a short-term paralysis of
the LES. However, this treatment is limited in efficacy and is associated with an approximately
50% reduction in the basal LES pressure. The average duration of its action is approximately 3-4
months.23 The standard approach is to inject 80-100 units in 4 quadrants, just above the Z line.
Doses higher than 100 units have not been found to be effective. Although 75% of patients show
initial improvement, the therapeutic effect wears off to less than 60% at 1 year. Approximately
50% of patients relapse and require repeat treatments at 6-24 months.22,24,25
Five randomized controlled trials have compared BTX injection to PD (discussed later) and
1 to LHM. Although the initial relief from dysphagia is comparable, there is a rapid deterioration
in the group treated with BTX more than 6-12 months.26-31 The procedure is usually safe, with
minor complications such as transient chest pain in 25% of patients and reflux in less than 5%.
Serious side effects such as mediastinitis and allergic reactions are uncommon. However,
repetitive injections of BTX into the LES can incite a fibrotic reaction, obscuring the submucosal
plane thus making future myotomy difficult. Owing to these limitations, BTX injections should
be considered only in elderly patients who are not candidates for PD, LHM, or POEM.
Pneumatic dilation. PD is considered one of the most effective nonsurgical options to treat
achalasia. The principle of this procedure involves the use of air pressure for intraluminal
dilation and disruption of the circular muscle fibers of the LES. Standard throrough-the-scope
balloon dilators or bougienage do not generate enough pressure to fracture the muscularis
propria needed for symptomatic relief. The most commonly used balloon dilators for achalasia
are the nonradiopaque-graded polyethylene balloons (Rigiflex dilators). These are available in
3 diameters (30, 35, and 40 mm) and are mounted on a flexible catheter that is passed over an
endoscopic guidewire usually with fluoroscopic assistance. Under sedation and fluoroscopic
guidance, the balloon is positioned at the LES and inflated and, held for 15-60 seconds.32 After
dilation, all patients undergo a gastrografin swallow followed by a BE to exclude esophageal
perforation. All patients are instructed to seek immediate attention if they develop chest pain or
fevers after discharge, as delayed perforations have occurred with postprocedural retching or
emesis.
Several studies favor a graded approach to dilation, starting with the 30-mm balloon
followed by a subjective and objective assessment in 4 weeks.33 If the patient has continued
symptoms, the next size dilator is used, up to a maximum of 40 mm. In a review of 1200 patients
by Richterand colleagues,34 PD with the Rigiflex balloon resulted in good symptom relief in 74%,
86%, and 90% of patients, treated with 30-, 35-, and 40-mm balloons, respectively. The success
rate of a single PD was reported to be 62% at 6 months and 28% at 6 years, whereas serial dilation
resulted in symptom improvement in 90% of patients at 6 months and 44% at 6 years.32,34
Approximately one-third of treated patients would experience symptom relapse over 6 years of
follow-up.
Predictors of favorable clinical response to PD include older age (4 45 years), female sex,
narrow esophagus, LES pressure of o10 mm Hg after dilation, and type II achalasia defined by
HRM.33,35,36 The most serious complication with PD is esophageal perforation, with an incidence
of approximately 1.9%.25 Hence, all patients who undergo a PD should be counseled about the
possible need for an emergent surgical intervention in the rare event of an uncontrolled
perforation. Most perforations, however, can be managed conservatively by keeping the patient
on parenteral nutrition (PN), broad-spectrum antibiotics, and by placing a stent across the
perforation site. The incidence of gastroesophageal reflux disease (GERD) after PD is
approximately 15%-35% and is usually manageable with proton pump inhibitors (PPIs).34
PD may also be employed in patients who relapse after a surgical myotomy but usually this
group does not fare as well with PD owing to higher resting LES pressures.
Surgical myotomy. The Heller myotomy is the surgical approach most widely used to treat
achalasia. It was first introduced in 1913 by Ernst Heller. The technique started out as a
laparotomy and has evolved over time with the use of thoracoscopic and laparoscopic
techniques. Shimi and colleagues35 in 1991 described the first minimally invasive technique for
the Heller myotomy. The procedure involves essentially splitting the longitudinal and circular
muscles of the lower esophagus, LES, and gastric cardia for a minimum length of 4-5 cm over the
esophagus and 2 cm over the cardia. These lengths are defined in the Society of American
Gastrointestinal and Endoscopic Surgeons (SAGES) guidelines.36 This is accompanied by the
creation of a partial anterior (Dor) or posterior (Toupet) fundoplication to reduce the
development of GERD, which is the most frequent complication after a myotomy.
Richards and colleagues37 demonstrated the benefit of adding a fundoplication in a double-
blind randomized controlled trial comparing myotomy with and without a fundoplication. This
advantage was also shown in a study by Campos and colleagues,38 with a reduction in reflux
symptoms to 8.8% with a fundoplication vs 31.5% without a fundoplication. A recent multicenter
randomized controlled trial comparing a Dor to a Toupet fundoplication showed that both
provided similar control of reflux after LHM.39
The mean success rate of LHM at a follow-up of 35 months is approximately 89% (76%-100%),
but this rate decreases to 65%-85% after 5 years as a consequence of disease progression.38
Younger men (o 40 years), LES pressure greater than 30 mm Hg, and a straight esophagus
without tortuosities are positive prognostic factors for a successful LHM.40,41 Patients with type
II achalasia have the best outcome. There is no difference in success rates between PD and LHM
for type I and type II achalasia, but type III achalasia responds better to surgery than to PD.42
The most common complication of LHM is mucosal perforation (approximately 6.3%), which
can be usually repaired intraoperatively without untoward consequences. LHM failures or
recurrences usually manifest within 12-18 months following surgery, with the most likely causes
being: an incomplete myotomy (usually on the gastric side), late scarring of the myotomy, and an
obstructive antireflux wrap.43 Such recurrences can be treated effectively with PD and, if
ineffective, with a repeat LHM. With the advent of POEM, the avenues for repeat myotomies
after surgical treatment failures have expanded the armamentarium of physicians.
Peroral endoscopic myotomy. The first account of an endoscopic myotomy was in Venezuela in
1980 by Ortega and colleagues44 in which they described two 1-cm long myotomies performed
to a depth of 3 mm at the LES. However, this technique was not embraced for the next 26 years
until in 2007, Pasricha and colleagues45 described the use of an endoscopic myotomy in a
porcine model in which the mucosal incision was made 5 cm above the GEJ and a selective
circular myotomy was performed. In 2008 in Japan, the first human endoscopic myotomy was
performed through a submucosal tunneling technique, coined POEM. Inoue and colleagues46 in
2010 published the first series of POEM procedures in 17 patients, showing promising early
results for the treatment of achalasia.
Although POEM was initially performed only in nonsigmoid achalasia, the indications have
expanded to include other spastic esophageal motility disorders, pediatric achalasia, previous
failed surgical myotomies, and patients with previous multiple endoscopic treatments. Based on
the data collected from the iPOEMs survey, the absolute contraindications that have been
generally agreed upon include prior radiation, prior extensive esophageal mucosal resection or
ablation, severe cardiopulmonary disease, severe coagulopathy, and cirrhosis with portal
hypertension.47
The POEM procedure is performed under general anesthesia and endotracheal intubation to
decrease the risk of mucosal perforation, pneumomediastinum, pneumothorax, and subcuta-
neous emphysema, all of which have resulted with conscious sedation alone. Patients are asked
to take a liquid diet for 1-5 days before the procedure and some centers administer an empiric
antifungal agent for 3-5 days before the procedure. After suctioning the esophagus, the Z line is
identified and at approximately 10-15 cm from the Z line and an initial submucosal injection is
made to safely access the mucosa-submucosal plane to create a submucosal tunnel. During the
procedure, multiple such injections of diluted contrast agent (eg, methylene blue) are used to a
total volume of 100-150 cc. A 1.5-cm mucosal incision is then made with either a triangular tip
knife or a hybrid knife and the submucosal tunnel is carefully extended to 3 cm into the gastric
cardia. As the operator advances into the cardia, the vessel density and diameter of the tunnel
increases and the mucosa becomes thinner, lending itself to a higher risk of perforation.
Different centers across the world vary in the choice of site for the myotomy. Myotomy positions
include anterior (11- or 2-o'clock), posterior (5- or 7-o'clock), and lateral (3- or 8-o'clock).48 A
discussion of the risks and benefits associated with each of these positions is beyond the scope
of this article. It is important to note that the myotomy is always commenced approximately 1.5-
2 cm beyond the mucosal incision. The total length of a standard myotomy is approximately 10-
12 cm including the 2-3 cm of cardiomyotomy. There has been debate about performing a full-
thickness myotomy compared with a selective circular myotomy, however, a few case series
reports have shown no significant difference in clinical or physiological results. After the
myotomy is completed, hemostasis is achieved and the mucosotomy is closed either with
endoscopic clips, OVESCO clips, endoscopic suturing devices, or fully covered, self-expanding
metal stents.
Postprocedure care varies between institutions, with the length of hospital stay varying
between 1 and 5 days. Most patients are kept on a liquid diet for 2 weeks and after mucosal
integrity is confirmed by endoscopy and an esophagram, patients are advanced to a regular diet.
At a 3-6-month follow-up, manometry and pH studies may be performed to assess LES pressures
and the presence of reflux. In the initial study by Inoue and colleagues on 17 patients, the
reported success rate was 100%.46 An international prospective multicenter study by von Renteln
and colleagues49 demonstrated a 97% treatment success (defined by an Eckardt score of less than
3 and IRP of less than 15 on HRM with adequate emptying of BE) at 3 months, 88.5% at 6 months,
and 82.4% at 12 months.
As an endoscopic myotomy is not accompanied by a fundoplication, a theoretical concern
exists for increased reflux compared to LHM. In the only comparative study of LHM vs POEM by
Bhayani and colleagues50 that used 24-hour pH testing, there was no statistical difference in the
incidence of reflux (LHM 32% vs POEM 39%). However, a randomized controlled trial comparing
LHM to POEM is currently underway that would help analyze the true incidence of reflux in the
long term.
With the advent of the EndoFlip technology, physicians can assess intraprocedurally, the
physiological effect of the myotomy. This consists of a balloon filled with a prespecified volume
of hypertonic saline and 16 sensors mounted along the balloon catheter that measure electrical
impedence. Using impedence planimetry, a computer can calculate the shape of the balloon and
measure its minimal diameter at the “waist” created by the LES and in turn calculate the cross-
sectional area as well as GEJ distensibility and compliance. This may, in the future, enable a
shorter esophageal body myotomy tailored specifically to the patient.51
Comparison of therapeutic modalities

PD vs LHM. Currently, PD and LHM are the most common and effective treatment approaches for
the treatment of achalasia. A randomized controlled trial by Kostic and colleagues52 in 2007
compared PD with Rigiflex balloons to LHM with Toupet fundoplication. The study showed a
superiority of LHM with only one treatment failure in the LHM group compared with 6 in the PD
group at 12 months.52 However, there were only 51 patients in the study resulting in a small
sample size. In 2011, Boeckxstaens and colleagues53 reported the results of the European
achalasia trial, a prospective randomized controlled trial comparing PD and LHM with Dor
fundoplication. The trial included 201 patients. Therapeutic success was defined as an Eckardt
score less than 4. There was no significant difference in therapeutic success at 12 months (90%
for PD vs 93% for LHM) or at 24 months (86% for PD vs 90% for LHM). Both esophageal clearance
and LES pressure improved to similar extent in both the groups. The authors thus concluded that
either modality could be used as an initial therapy for treatment of achalasia.53 Persson and
colleagues54 in 2015 followed 53 patients who underwent either PD or LHM long-term and
showed treatment failures to be higher in patients who underwent PD at both 3 (4% for LHM vs
32% for PD) and 5 years (8% for LHM vs 36% for PD).
POEM vs LHM. At present, no published randomized controlled trials have compared POEM to
conventional therapies. There are 2 RCTs that are, however, scheduled to be completed in 2017
and 2019. Five studies have compared POEM to LHM. The largest of these is a cohort study by
Bhayani and colleagues50 that included 37 patients undergoing POEM and 64 patients
undergoing LHM. The study showed significantly better dysphagia relief after POEM both at
1 and 6 months. The study also obtained esophageal pH monitoring data in 23 of the patients
who underwent POEM and 31 of the patients who underwent LHM and demonstrated similar
rates of GERD in both the groups (39% of patients who underwent POEM vs 32% of patients who
underwent LHM).50 In 2014, a systematic review and meta-analysis of 19 studies with
objectively measured clinical outcomes, showed that POEM had a similar efficacy as LHM for the
treatment of achalasia.38 Although short-term follow-up has demonstrated equivalent efficacy
between the 2 treatment modalities across multiple studies, long-term follow-up is lacking and
must be analyzed in the upcoming studies to assess the superiority of either modality.
Conclusions
Achalasia is the most well-defined esophageal motility disorder, with different phenotypes
having different response to treatment. Definitive treatments such as myotomy or PD should be
offered to patients. However, achalasia treatment is not curative and up to 20% of patients may
need additional treatments within 5 years because of symptomatic recurrence. Approximately
6%-10% may have progression to megaesophagus or end-stage disease, with esophagectomy
being reserved as a final option in cases not responding to dilation and myotomy.55
Scleroderma and connective tissue disorders
Scleroderma is a rare autoimmune connective tissue disorder with multisystemic

manifestations that is characterized by inflammation, progressive fibrosis, and vascular and
neurologic changes.56,57 There are 2 recognized types of scleroderma: localized and systemic.
Localized scleroderma, or morphea, has minimal GI involvement and is primarily confined to the
skin and adjacent tissue. Systemic sclerosis (SSc) can be divided into limited, diffuse, and sine.
Limited scleroderma involves mainly the hands, arms, and face, and GI involvement tends to
occur late in the course of this disease. Diffuse scleroderma involves a wider area of skin
combined with internal organ involvement and principally affects the GI system, which may
precede skin complications in the course of the disease. Sine scleroderma is a rare variant of the
disease without skin involvement.57,58 SSc has a prevalence range of 0.7-53 per 100,000, varying
by country, and an incidence of 20 cases per 1 million in the United States each year.59,60 SSc has
a survival estimate rate of 74.9% and 62.5%, at 5 years and 10 years from diagnosis, respectively.59
Skin and vascular involvement in this disease are widely recognized, but the esophagus and
lung are the next most commonly affected organs in the disease. The standardized mortality
ratio of SSc, one of the highest rates among rheumatic disorders, is approximately 2.7%.59
Pulmonary hypertension and lung fibrosis are the leading causes of death, accounting for 60%-
80% of the deaths, whereas esophageal dysfunction is the most common visceral organ
complication, occurring in 50%-90% of patients.58
Esophageal scleroderma is the term used for the esophageal dysmotility seen in these
patients, and is characterized by distal aperistalsis and hypotensive LES pressure.56,58,61
Esophageal scleroderma has also been associated with Raynaud phenomenon and pulmonary
fibrosis in patients that present the CREST syndrome (calcinosis, Raynaud phenomenon,
esophageal dysmotility, sclerodactyly, and telangiectasia).58
The literature is controversial regarding what subtype of SSc produces a higher risk for
esophageal dysmotility development. Previous studies showed that diffuse SSc has more
internal organ involvement; hence, the severity of esophageal dysmotility would be greater. On
the contrary, it is also known that limited SSc has a narrow relationship with CREST syndrome,
making it more likely for patients with this variant to develop esophageal scleroderma.56
It is important to note that despite esophageal scleroderma being the most common
manifestation in SSc, similar patterns of esophageal dysfunction found in this disease are also
found in other connective tissue disorders. Among them are rheumatoid arthritis, erythematous
systemic lupus, and amyloidosis, as well as non–connective tissue disorders such as
hypothyroidism and diabetes.62-65
Pathophysiology and clinical presentation

There are 3 well-established phases of esophageal scleroderma progression in SSc. They are a
consequence of a cascade of oxidative stress, collagen deposition, and vascular injury resulting in
autoantibody production. In an early stage of the disease, neural dysfunction is present because
of arteriolar changes in vasa nervorum and compression of nerve fibers by collagen deposition.
As the disease progresses, esophageal smooth muscle atrophy is identified in the lower two-
thirds of the esophagus and in the LES, resulting in the first symptoms reported by the patient.
Finally, the smooth muscle is replaced by fibrosis, mainly in the circular layer, in a late course of
the disease.56,57,63,66
All of these morphologic alterations result in a classic presentation of dysphagia, heartburn,
and regurgitation reported by 50% of patients with esophageal scleroderma. However, the
absence of symptoms does not exclude esophageal disease in progression because almost 90% of
patients with SSc have esophageal abnormalities detected by additional studies, but are
asymptomatic at the time the examination was performed.63,64,67
The overall incidence of heartburn and regurgitation in these patients is 14%-71% and occurs
mainly because of LES impairment, whereas dysphagia has an incidence of 24%-82% and is a
result of strictures and abnormalities in the esophageal body peristalsis.66,68 Dysphagia occurs
initially for both solids and liquids, whereas dysphagia for solids is reported only in patients who
have some degree of stricture.62 Both dysphagia and heartburn have an incidence of 29%-66%
when calculated together.68
With time, the esophageal dysfunction in patients with SSc can lead to potential
complications. Previous studies described a 2-step theory that could explain the progression
of the disease. At first, the LES pressure allows 1-2 episodes of acid reflux per day. How-
ever, owing to poor peristaltic function, the impaired esophageal acid clearance leads to
residual acid cling in the esophageal mucosa, which takes 6-7 minutes to be neutralized by
saliva.57,61 Recurrent reflux is a major factor in the development of complications, leading
to Barrett esophagus, esophagitis, webs, strictures, and in rare cases, adenocarcinoma.
Barrett esophagus, reported in 0%-37% of patients with scleroderma esophagus, is intestinal
metaplasia of the normal esophageal squamous mucosa with properties of an esophageal
adenocarcinoma precursor. However, the risk of actual progression in SSc of 0.1%-0.6% per year is
low.61,62,68
As most patients are asymptomatic and therefore left untreated, other complications are
likely to occur.69 Esophagitis, whose incidence is 30%-40%, reflux esophageal stenosis in 3%-40%,
and stricture in 17%-29%, are some examples.62,68 The patient with esophageal scleroderma can
also develop candida esophagitis, usually because of the poor emptying of the esophagus.62
Likewise, the presence of a hiatal hernia has a narrow correlation with esophageal scleroderma
being related in 50% of patients with concomitant dilatation and short esophagus, increasing the
severity of reflux in these patients.64 Similarly, 1 previous study reported a case of esophageal-
atrial fistula, secondary to perforation in a Barrett esophagus, into the left atrium.64
Diagnostic procedures
Esophageal function in patients with SSc can be evaluated with the following63,69:
(1) manometry or motilty study, (2) BE, (3) EGD, or (4) 24-hour pH-metry.
Manometry. Manometry is the gold standard diagnostic procedure for initial evaluation of
esophageal dysmotility in patients with esophageal scleroderma, because early changes are
accurately detected.62 The overall incidence of manometric abnormalities is 70%-96% in these
patients.70
At an early stage of the disease, manometry shows nonspecific findings, including low
amplitude of contractions in the distal two-thirds, increased speed of peristaltic wave—which may
represent a compensation for low-amplitude peristaltic contractions, incoordination of peristalsis,
and hypotensive LES—used for differentiation between esophageal scleroderma and achalasia, and
failure of the LES to relax. Manometry can also be used for evaluation of the risk of recurrent reflux
esophagitis.57,62,68 Findings related to the UES pressure and upper esophagus only occur in
advanced-stage disease and are still controversial in the literature, which were reported as being
low, normal, or higher.62,70 In a mid-stage disease, manometry shows progression to aperistalsis of
the lower two-thirds of the esophagus, delayed esophageal emptying, and decreased LES pressure.
In a late stage, there is aperistalsis of the distal two-thirds of the esophagus, absent LES pressure,
and in extreme cases it may involve the proximal skeletal muscle.57
Overall, manometric findings include the presence of ineffective or absent distal esophageal
peristalsis and hypotensive LES pressurization.61,63,71 Among the manometric findings of
patients with SSc, hypotensive LES was reported in 50%-83%, whereas ineffective distal
esophageal motility was documented in 60%, absent peristalsis in 47%, and weak peristalsis in
20%. Both hypotensive LES and esophageal dysmolitility were reported in 52%-63% of
manometric studies. Involvement of the distal esophagus was found to be 70%.61,70 Moreover,
these findings were present in all patients with esophagitis or Barrett esophagus or both.61 HRM
can also be combined with manometry to enhance the diagnostic potential by detecting
esophageal bolus clearance, and to measure intrabolus pressures during esophageal peristaltic
phases.61 Manometry can be performed in patients in every stage of the disease, mainly in those
clinically asymptomatic. However, it is not the examination of choice for follow-up.62,68
Barium esophagram. BE is very helpful for esophagus anatomy evaluation and esophageal transit
ability, and it can be used as a tool for recognition of esophageal strictures, esophageal dilatation,
and eventual hiatal hernias.61-63 Typical findings would be dilation and shortening of esophagus,
resulting in a hiatal hernia.62
Cine esophagram is a video recording of a barium swallow that helps with the esophageal
function evaluation. This examination can be performed with a patient lying in an upright
position, to evaluate the degree of dilatation unaffected by gravity. As common in findings in
patients with SSc, it shows low percentage and amplitude of peristaltic contractions, with no
correlation with abnormalities in LES pressure.62
Esophagogastroduodenoscopy. EGD is used mainly to evaluate and grade esophageal mucosal

damage and to identify, rule out, or stage possible complications such as Barrett esophagus,
Candida esophagitis, stricture esophagitis, and adenocarcinoma.62,63,69,70,72 This examination is
preferably done for symptomatic patients with SSc with related reflux, who have an inadequate
response to PPI therapy, and for whom complications are suspected.61 However, its use for
adenocarcinoma screening in these patients remains controversial. The literature indicates that
patients considered at risk for adenocarcinoma development are male, Z50 years old, obese,
white, and those with long-standing GERD (often defined as 4 5 years).61
24-hour pH-metry. The 24-hour pH monitoring is a very traditional examination, which is used
to evaluate the abnormal esophageal reflux that is present in 54%-86% of patients with SSc. The
findings in patients with SSc are fewer reflux events but with significantly longer duration, with
occasional proximal reflux reported in some cases.63
Manometry is the most sensitive diagnostic procedure for evaluation of esophageal
dysmotility. However, it is considered an uncomfortable, invasive procedure, which is often
not well tolerated by patients. Therefore, it is not a good choice for screening, although it should
be performed in all symptomatic patients with SSc to rule out any esophageal abnormality.
Moreover, all patients with abnormal manometric findings should be further evaluated with an
upper GI endoscopy. BE is very well tolerated and an easier test to perform when compared with
manometry and can be used to establish or rule out esophageal dysfunction. BE has a sensitivity
and specificity of only 60% and 80%, respectively.57,62,73
Management
Treatment of esophageal scleroderma currently consists of stabilizing the primary disease
and its progression, relieving the symptoms of hypomotility, stricture, and mainly reflux, to
prevent further complications.63 As most patients are asymptomatic for a long period of time
despite pre-existing esophageal abnormalities, early diagnosis and establishment of a plan of
treatment are recommended to prevent complications in these patients.63 Treatment modalities
include behavior change, pharmacologic therapy, and surgery.
Behavior modification. Patients are encouraged to make lifestyle modifications as a first line of
treatment for esophageal scleroderma, targeting mainly improvements in reflux symptoms. It is
recommended that the patients achieve and maintain a normal weight, fractionate meals in
minimal amounts, avoid supine position within 3 hours of eating, elevate the head of the bed,
quit smoking, and reduce alcohol intake.61,63
Pharmacologic therapy. Oral pharmacotherapy can be divided into 2 major groups—antisecretory

agents and prokinetic agents. Antisecretory agents include PPI, a well-established concept of
first-line treatment of GERD.61,71 As esophageal abnormalities are extremely prevalent in
the disease and there is poor correlation with the symptoms, PPI has been recommended for
all patients with SSc, including those who are clinically asymptomatic. Despite several
PPIs being available, pharmacotherapy does not correlate with its potency; it lies otherwise in
the commitment of the patient being compliant with his treatment.61 This line of therapy is
effective in most cases, but some patients would still need adjuvant treatment, especially those
with advanced-stage esophageal scleroderma. This occurs because of the high pathologic
complexity of the disease, and because of the involvement of esophageal dysfunction, which can
lead to severe complications not only in the GI tract, but in other system as well.74 Use of
omeprazole heals esophagitis and probably helps in reversal of fibrosis by decreasing
hydroxyproline levels. Another study showed the same outcome in an EGD follow-up of
patients receiving PPI therapy.61 Nonetheless, the literature also shows that long-term PPI
therapy can lead to osteoporosis and infection, apart from not improving the risk of
bronchoaspiration, which is a severe complication, because PPI treatment has no effect on the
incompetent LES.61,74,75
For the patient whose response to PPI is not as expected, it is possible to increase the PPI
dosage to twice daily, increase the dosage (eg, to take lansoprazol 30 mg, instead of 15 mg daily),
or to switch to another PPI such as esomeprazole or rabeprazole.61 Another alternative is to
resort to prokinetic agents, such as metoclopramide, domperidone, cisapride, erythromycin, and
octreotide, which play a role in SSc-esophageal dysmotility improvement and in GERD
symptoms. Among them, a peripheral dopamine antagonist called domperidone is the most
used in patients with SSc.63,71
Cisapride and loperamide may alleviate the symptoms because of the improvement in
peristalsis, an increase in LES pressure, and amplitude of distal esophageal body peristalsis.71,76
However, association of these drugs with cardiac arrhythmias has been reported making their
use restricted owing to safety profile issues.61,63
Metoclopramide and erythromycin have been reported to improve LES pressure in patients
with esophageal scleroderma. Nevertheless, metoclopramide has been associated with neuro-
logic side effects, including potentially irreversible tardive dyskinesia, with its long-term
use.61,71
Methacholine can be used during the initial phase of the disease, acting to inhibit the smooth
muscle contraction; however, it cannot be used in advanced stages because of fibrosis of the
esophageal musculature, rendering this medication ineffective.63
Surgical therapy. A surgical approach in patients with SSc is considered in certain scenarios such
as failure of medical therapy, high severity of reflux symptoms, findings of aperistalsis or failure
of swallows, and reduced peristaltic amplitude in the distal esophagus.63,77 The main goal is to
perform an antireflux procedure, such as a partial fundoplication, as symptoms of reflux are
present in 90% of patients with SSc, with a rate of 50% of complications related to it. Surgical
outcomes of patients with SSc who underwent antireflux surgery are extremely controversial,
and this may be attributed to variability in impaired esophageal motility and disease severity
among patients.63,74,77 The biggest concern for these patients is the development or worsening
of dysphagia in a postoperative status. Studies have reported postsurgical dysphagia rates
ranging from 38%-71% with some degree of esophagitis.61,74
Studies have also reported good outcomes of antireflux surgery in these patients.63,77 It is
recognized that surgery can improve esophageal acid reflux and reduce its symptoms;
additionally, it can improve quality of life and, in some cases, reduce the dysphagia complaints
in those patients.78,79 Antireflux surgery should not be withheld from these specific patients.
Hypercontractile esophageal motility disorders
Esophageal motility disorders can be hyperactive or hypoactive. The 2 primary hyperactive

esophageal motility disorders are distal esophageal spasm (DES) and jackhammer esophagus
(JHE). DES has been referred to as either distal esophageal spasm or diffuse esophageal spasm,
although the latter term is falling out of favor, as the spasm occurs in the distal esophagus.
Neither disorder is seen in normal individuals and is typically encountered in the process of
investigating dysphagia or atypical chest pain. Abnormal esophageal manometry can result from
a primary motility disorder or can be secondary to other pathology. Primary esophageal motility
disorders have been categorized by the Chicago classification (version 3.0, see Table 1).14
It is necessary to rule out a secondary esophageal motility disorder where the underlying
cause may require different treatment. Secondary causes can range from primary or metastatic
malignancy, connective tissue diseases, or infiltrative diseases such as eosinophilic esophagitis
or amyloidosis.
These disorders have a slight female predominance and are associated with certain psychiatric
diagnoses, but the population prevalence is not well defined, as these diseases require HRM to
detect. The combined prevalence of DES, spastic achalasia, and JHE is reported to be 2%.80
Distal esophageal spasm

Diagnosis. The diagnosis of DES requires the presence of certain abnormal findings on HRM of
the esophagus. DES is defined by the presence of more than 20% premature contractions of the
distal esophagus, in the setting of a normal IRP of the gastroesophageal junction following a
swallow. A normal IRP is mandatory in the diagnosis of DES, as an elevated IRP would suggest
achalasia or mechanical obstruction.
In DES, the esophageal body contractions are of normal amplitude. An esophageal contraction
is considered premature if its DL is less than 4.5 seconds. This is a change in version 3.0 of the
Chicago classification. Prior definitions of DES incorporated the contractile front velocity, which
represents the speed of propagation of esophageal peristalsis.
There may be associated abnormalities seen on other imaging or endoscopy, but this is
sporadic. DES can be seen on a contrast esophagram, with the classical picture of a corkscrew
esophagus. Esophagography may be normal, because symptoms and contractions are
intermittent. Endoscopy is typically normal, unless other concurrent pathology is present,
although cases have been reported that depict a clear corkscrew anatomy.
There are few data to support doing esophageal pH monitoring on all patients with DES, but if
patients report symptoms consistent with reflux or demonstrate improvement on acid
suppression therapy, it is important to perform esophageal pH monitoring. A retrospec-
tive study by Crespin and colleagues81 in 2015 examined 192 patients with documented
hypercontractile esophageal motility disorders who underwent esophageal pH monitoring.
Most patients complained of heartburn or regurgitation (69%), and 103 patients were found to
have abnormal testing, with pathologic levels of distal esophageal acid exposure. Of those
patients, 66 underwent laparoscopic Nissen fundoplication. The motility disorders included
NE (13 patients), JHE (2 patients), DES (6 patients), and hypertensive LES (17 patients).
After Nissen fundoplication, they all had normal distal esophageal acid exposure and they all had
improvement in their symptoms, with symptom resolution in 28 of 38 patients. Six patients
underwent postoperative HRM and 5 of them had resolution of their esophageal motility
disorder (1 with NE, 2 with JHE, and 3 with hypertensive LES). The authors also noted that most
patients who presented with dysphagia or chest pain did not have GERD.
Etiology. The underlying etiology of DES is unclear. It is theorized that it results from
degeneration of the distal esophageal nerve plexus, similar to achalasia.80,82 This results in a
loss of inhibitory neurons in the smooth muscle and causes premature or simultaneous spastic
contractions. The proximal esophagus retains somatic innervation and typically retains normal
motility. DES is a primary hypercontractile esophageal motility disorder, but it has been
associated with other diseases such as hypertension, coronary artery disease, anxiety,
depression, pulmonary disease, and chronic pain syndromes, as well as opioid use.
The association with psychiatric disease was reported as early as 1983 by Clouse and
Lustman,83 who found that 84% of their patients with esophageal manometry abnormalities had
a lifetime diagnosis of psychiatric disease, compared to 31%-33% of patients with normal
manometry. This was further explored by Almansa and colleagues84 in 2012 in a series of
patients, finding that 45% of them were on psychotropic drugs. Patients with DES were identified
based on manometry, and their demographics, symptoms, and comorbidities were evaluated.
The median age was 71 years with a 55% female predominance. The most commonly associated
comorbidities were hypertension in 57% of patients and psychiatric disorders in 30% of patients.
The most common psychiatric disorders were depression (24%), chronic pain syndromes (22%),
anxiety (15%), and fibromyalgia (10%). The most prevalent symptom was dysphagia, present in
76% of these patients.
Morphine has been found to cause increased esophageal peristaltic velocity and impaired LES
relaxation, an effect that is reversible with naloxone. This was discovered by Penagini and
colleagues85 in a 1996 study of 8 healthy adults (age: 19-25 years, 5 of whom were male) using
conventional manometry. Baseline manometry was performed, followed by intravenous
administration of 100 mg/kg of morphine, after which manometry was repeated. A distensible
balloon was also placed in the esophagus to mimic a food bolus, looking for an inhibitory
response distal to it. The manometry performed after morphine administration showed a
significant increase in velocity of esophageal peristalsis, impaired LES relaxation, and decreased
inhibitory response of the esophagus distal to the distensible balloon. Following this evaluation,
a dose of 80 mg/kg of intravenous naloxone was given, which reversed all of these effects. The
test was repeated on another day with baseline manometry and naloxone administration, with
no significant change in any manometric parameters. This was theorized to be secondary to
failed deglutitive inhibition.
The association with opioid use is reversible in some instances, with resolution of DES after
cessation of opioid therapy. One retrospective case series performed by Kraichely and
colleagues86 in 2010 demonstrated that in a group of 15 patients with dysphagia on chronic

opioid therapy, all 15 of them had demonstrable manometric abnormalities, including
incomplete LES relaxation and nonperistaltic esophageal body contractions. The manometry
results were reported as esophageal topography plots. Three of the 15 patients were retested
after their opioid therapy had been discontinued for at least 72 hours. Two of these 3 patients
had previously been diagnosed with DES based on their manometry. On repeat testing while off
opioid therapy, their esophageal motility was normal. These findings, in conjunction with the
work by Penagini and colleagues85 should prompt one to consider nonopioid therapy in treating
chest pain that is potentially secondary to DES, as it could exacerbate esophageal dysmotility.
Treatment. Treatment of DES is predominantly with medical therapy. CCB, nitrates,

phosphodiesterase inhibitors, and trazodone have all been used for treatment of DES, usually
without clinically significant or durable improvement. The link between esophageal dysmotility and
psychiatric disease was the underlying rationale for use of trazodone. A double-blind randomized
controlled trial with trazodone was reported by Clouse and colleagues87 in 1987 for chest pain in
patients presenting with a variety of esophageal motility abnormalities. The study group included 15
patients who received trazodone and 14 placebo patients. Most patients had a concurrent psychiatric
disorder (19/29, or 66%). Both groups of patients showed a statistically significant improvement in
chest pain frequency and severity. The trazodone group had a greater improvement in heartburn
reduction, although this group had a higher prevalence at the outset. Cessation of opioid therapy may
help in some patients, but this has not been studied in a controlled fashion.
In patients with dysphagia, BTX injections can provide relief. A double-blind randomized
controlled, crossover prospective trial by Vanuytsel and colleagues88 was performed on patients
with the diagnosis of DES or NE complaining of dysphagia and chest pain. NE was diagnosed
based on peristaltic contractions with a mean amplitude greater than 180 mm Hg in the distal
esophagus or a DCI greater than 8000 mm Hg cm s. The discrepancy in diagnostic criteria for NE
was because of a transition from conventional manometry to HRM during the study period. A
sample of 22 patients underwent endoscopy with an initial injection of 4 mL of either saline (11
patients, 5 of whom had NE) or BTX (13 patients, 2 of whom had NE) in the distal esophagus,
with repeat manometry and symptom evaluations performed at 1 month after the procedure.
Following this, patients who initially received saline injections were given BTX and the patients
who received BTX were then injected with saline. The group that received BTX first showed
improvement in their dysphagia for both solids and liquids but had no improvement in chest
pain, heartburn, or regurgitation. When this group received saline injections 1 month later, there
was no change in their symptoms. The group that received saline first did not show any
improvement after the saline injection. One month after receiving the second injection, BTX,
there was no statistically significant difference in their dysphagia scores.
Operative therapy for DES consists of performing a long myotomy with a laparoscopic or
open transabdominal esophagomyotomy, thoracoscopic or open thoracic esophagomyotomy, or
with a POEM. There are no randomized controlled trials comparing a myotomy with
nonoperative therapy. Most series involve fewer than 30 patients or a heterogeneous group of
esophageal motility disorders, but these studies generally report symptom relief of chest pain.
Leconte and colleagues89 evaluated 20 patients with presenting complaints of chest pain,
dysphagia, and regurgitation, who underwent an extended myotomy by a single surgeon for
DES. Patients had undergone a variety of preoperative treatment modalities, including CCB,
nitrates, BTX injections, or PD of the LES. Median follow-up was 50 months, with 18 of 20
patients giving “excellent or satisfactory” scores for dysphagia and 20 of 20 patients giving
“excellent or satisfactory” scores for chest pain. Clinically significant morbidity occurred in 5%,
with no deaths. This operation has the potential for serious morbidity and larger controlled
studies are warranted before this can be recommended as standard of care for DES complicated
by chest pain or dysphagia. POEM has been studied primarily for achalasia but has also been
performed on patients with DES and other non–achalasia disorders.90 However, these outcomes
have not been reported separately for DES patients.
Jackhammer esophagus
Diagnosis. JHE is the second primary esophageal motility disorder marked by hypercontractility.
Patients most commonly present with dysphagia or chest pain. Similar to DES, the diagnosis of
JHE is based on the presence of abnormal findings on HRM. Before the advent of HRM, high-
amplitude esophageal contractions were termed NE, which was the presence of any esophageal
contraction greater than 180 mm Hg. That threshold was subsequently increased to greater than
220 mm Hg to increase the specificity of the diagnosis. One study found that this change in
threshold decreased the prevalence from 9%-3% in a population of patients undergoing HRM.91
Version 3.0 of the Chicago classification defines JHE as a DCI of greater than 8000 mm Hg s cm in
Z 20% of swallows. The DCI is a measure of esophageal contractile vigor and not esophageal
contractile pattern.
Patients should undergo contrast esophagography and endoscopy to evaluate for mucosal
lesions or a secondary cause of their disease. Both studies may be normal in a patient with JHE,
but the classic esophagram shows a “rosary bead” esophagus.
Etiology. JHE replaces NE in the Chicago classification, but there are no studies that show the
extent of overlap between these 2 diagnoses. The underlying etiology for either disorder is not
well defined.
DES and achalasia are thought to be secondary to degradation of inhibitory neuronal
innervation, but this has not been observed in NE. An experimental trial with healthy volunteers
and patients with NE showed that a hypercholinergic state contributed to prolonged, high-
amplitude asynchronous contractions.92 Administration of edrophonium to healthy volunteers
induced significantly higher amplitude and duration of esophageal contraction. Patients with NE
(with a threshold of 4 180 mm Hg) were given 2 successive doses of 5 and 10 mg/kg of atropine,
with a subsequent significant decrease in esophageal contraction amplitude and duration.
JHE has now been reported to progress to type II achalasia. As with other achalasia subtypes,
there is 100% failed peristalsis and an elevated median IRP. Type II achalasia is then
differentiated from other achalasia subtypes based on panesophageal pressurization with more
than 20% of swallows. Abdallah and Fass93 published a case of a patient who had a nonspecific
esophageal motility disorder based on conventional manometry. A year later, she was found to
have dysphagia and weight loss and had HRM that documented the presence of a JHE (DCI of
10,770 mm Hg s cm). She subsequently developed worsening dysphagia and weight loss and
underwent repeat HRM a year after that, which showed findings consistent with type II
achalasia with 100% of swallows showing panesophageal pressurization.
Treatment. As with DES, management of JHE is primarily medical treatment, with many of the
same agents. CCB, phosphodiesterase inhibitors, tricyclic antidepressants, trazodone, and BTX
injections have all been studied. The use of diltiazem was trialed in a group of patients with NE
(with a threshold of 4 125 mm Hg in the distal esophagus) for treatment of noncardiac chest
pain.94 Patients experienced an improvement in their chest pain, along with a concurrent
decrease in esophageal contraction amplitude.
BTX injections have been studied for use for NE in a mixed group of patients with DES and
NE, as discussed in the DES segment above. Patients who received BTX injections had improved
dysphagia scores, but results were not reported separately for DES and NE patients.
POEM has been used for the treatment of NE in small series. Kristensen and colleagues95
performed a long-segment esophagomyotomy for NE in 3 patients. All 3 presented with
dysphagia and were evaluated using a scoring system. The esophageal myotomy was 12-15 cm
long and the gastric myotomy was 3-4 cm long. During a 1-year follow-up, there was significant
improvement in the patients' symptom scoring scales. There were no operative complications,
but all 3 patients were readmitted at different intervals, 2 because of abdominal pain and
vomiting and 1 because of fever and odynophagia.
Given the reported incidents of progression of JHE to achalasia, it is appropriate to repeat
testing the patients whose symptoms progress to worsening dysphagia, as they may ultimately
require an esophagomyotomy for dysphagia. In addition, as discussed previously with regard to
treatment for DES, Crespin and colleagues81 performed laparoscopic Nissen fundoplication for
patients with primary esophageal motility disorders and concomitant gastroesophageal reflux.
There were 2 patients with JHE and 1 with NE. All 3 had improvement in their manometry, 2 of
which returned to normal values. Patients with NE or JHE should be evaluated for
gastroesophageal reflux and treated for it, if needed.
Gastric motility disorders
Normal gastric motility
In general, the proximal stomach serves as a temporary reservoir for meals, whereas the distal
stomach churns and mixes food with digestive juices.96-98 Once the distal stomach has processed
the solid food to the appropriate size and consistency, the outflow is regulated by the pylorus into
the duodenum that feeds back regulating gastric emptying. Normal gastric emptying is a complex
series of events that requires appropriate function and coordination of multiple interrelated
processes, including the interstitial cells of Cajal, the smooth muscle of the gut, afferent and
efferent neurons, and the sympathetic and parasympathetic nervous system. Gastric peristalsis is
regulated by slow wave activity that is paced by the interstitial cells of Cajal located along the
midportion of the greater curve of the stomach. Enteric neurons initiate smooth muscle activity.
Gastric smooth muscle activity is controlled by myogenic, neural, and hormonal influences.
In the fasting state, migrating motor complexes (MMC) occur, which clear ingested material
from the lumen of the stomach and intestine. The pylorus opens in response to these contractions
to allow emptying. Within minutes of ingesting a meal, the MMC give way to the fed state of
gastric motility. The proximal stomach stretches to accommodate the contents of the meal and
allow adequate mixing with gastric contents. This gastric accommodation occurs primarily via
stimulatory vagal input. More distally, there is an absolute increase in magnitude of the gastric
contractions. Peristalsis begins at the midstomach at the site of the gastric pacemaker and
progresses toward the pylorus. As peristaltic waves reach the pylorus, the pylorus narrows down
to a few millimeters in size, to allow only liquids and small particulate matter to funnel out of the
stomach and the rest of the solid material to remain for further digestion and breakdown.
The regulation of gastric emptying starts with accommodation of the proximal stomach. If
this compliance is reduced, there would be an increased intragastric pressure and accelerated
emptying. Gastric emptying is influenced by the composition of gastric contents with liquids
emptying more rapidly. Solid-phase gastric emptying, which can be measured with a technetium
99m-labeled scrambled egg study, occurs over approximately 120 minutes with a half-life of 60-
90 minutes. The gastric emptying rate is primarily controlled by caloric intake with the rate of
gastric emptying being tightly regulated to allow 1-4 Kcals per minute into the proximal
intestine. Decreased temperature of the luminal contents delays emptying, whereas increased
temperature accelerates emptying. Similarly, isotonic fluids empty more quickly than either
hypertonic or hypotonic contents. The duodenum itself controls the emptying of the stomach by
its own-paced peristaltic activity and a unique antroduodenal coordination that is vagally
mediated. In addition, the passage of nutrients into the lumen of the ileum would slow gastric
emptying, a reflex termed as the ileal brake.
Disordered gastric motility results in a spectrum of dysfunction ranging from abnormally
slow transit, termed “delayed gastric emptying” or “gastroparesis,” to abnormally rapid gastric
emptying, resulting in the “dumping syndrome.” Both the conditions can be associated with a
variety of symptoms, including nausea, vomiting, and abdominal pain. Diarrhea may also be a
feature of rapid emptying.
Delayed gastric emptying
Delayed gastric emptying is defined as abnormally slow gastric transit in the absence of
physical obstruction and is associated with a chronic symptomatic disorder termed
Table 2
Causes of gastroparesis.
Postviral
Systemic disease
Diabetes mellitus
Neurologic disorders
Connective tissue disease
Postsurgical
Vagotomy
Gastric resection
Medications
Idiopathic
gastroparesis.99,100 This condition is estimated to occur in approximately 4% of the population;

80% of affected individual are women. Gastroparesis can be caused by a variety of medications
and drugs, often a postsurgical condition, and it can be associated with a variety of medical
conditions that influence neuromuscular function of the stomach (Table 2). However, many
cases are idiopathic, including those that are postviral.
Diabetes mellitus is the most common systemic cause of gastroparesis. Gastroparesis occurs
in 20%-50% of diabetic patients, usually in the later stages of disease, and is more common with
type 1 diabetes. Diabetes causes suppression of phase 3 of the MMC as well as an increase in
pyloric contractility. Chronic hyperglycemia appears to result in both vagal injury and direct
myopathy of the gastric antrum. Gastroparesis makes glycemic control difficult and conversely
poor glycemic control worsens symptoms of gastroparesis. Other neuropeptide abnormalities
have been seen in diabetic gastroparesis including changes in nitric oxide and increases in
oxidative stress.
Postsurgical gastroparesis is another common cause. Any operation that compromises the
vagus or disrupts gastric anatomy can cause gastroparesis (Table 3).101 Truncal vagotomy can
result in gastroparesis by altering fundal accommodation, reducing peristalsis in the distal
stomach, and inhibiting pyloric relaxation. It also leads to loss of phase 2 of the MMC. Vagal
injury can occur from a variety of procedures, including thoracic procedures, fundoplication, and
gastric bypass. Roux limb construction may also delay gastric emptying, the so-called Roux
syndrome.
Idiopathic gastroparesis is also a common etiologic group. It is a heterogeneous group of
patients, predominantly female. Obesity may be present. One-half of patients have an acute
onset that may be associated with an infectious prodrome suggesting a viral etiology. Previous
cholecystectomy may also be a risk factor in this group.
Table 3
Surgical procedures causing gastroparesis.
Procedure Incidence
Thoracic
Lung transplantation 6%
Esophagectomy 12%
Abdominal
Vagotomy
Without drainage 30%-50%
With drainage o5%
Fundoplication 5%
Gastrectomy 7%
Gastric bypass o5%
Pancreatectomy 20%
Patients with gastroparesis can have a variety of symptoms, including nausea and vomiting,
bloating, early satiety, and abdominal pain. These are exacerbated by ingestion of solid meals. It
is seen that 10%-30% of patients with gastroesophageal reflux have delayed gastric emptying.102
Abdominal pain occurs in 20% of patients and may be related to sensory nerve dysfunction.
There may be the associated finding of abdominal distension. Eventually the symptoms reduce
oral intake with resultant malnutrition. However, symptoms can wax and wane with episodic
flares. This disorder has significant effects on quality of life, morbidity, and mortality.103
Symptom severity is often assessed by the Gastroparesis Cardinal Symptom Index.104 There are 3
subscales—nausea and vomiting, postprandial fullness and early satiety, and bloating, with
reference to the preceding 2 weeks.
The gold standard for diagnosis of gastroparesis is the 4-hour radionuclide colloid gastric
emptying study.99,100 This is usually administered as an isotope-labeled, low-fat, scrambled egg
meal. Greater than 60% retention at 2 hours or greater than 10% retention at 4 hours confirms
the diagnosis. Concomitantly, outlet obstruction needs to be ruled out by endoscopy or a
contrast study. The presence of retained material in the stomach, especially phytobezoars,
indicates the presence of delayed gastric emptying. Liquid gastric emptying can also be assessed
by scintigraphy. A variety of other radiologic, functional, and motility studies have been
employed (eg, wireless capsule motility study and radioactive CO2 breath test), but are currently
not being used routinely.99,100 Manometric evaluation of the pylorus is now feasible. A delayed
gastric emptying test confirms gastric dysmotility but does not confirm that symptoms are due
to gastroparesis. Unfortunately, there is not a good correlation between gastric emptying and
symptoms.103
Management of gastroparesis begins with efforts to determine the etiology.100 Optimal
medical therapy for any predisposing conditions (eg, glucose-controlled diabetic patients)
should be undertaken. Goals of therapy include symptom relief and maintenance of nutrition.
Altering the diet with small, frequent meals that are low in fat or fiber may also reduce
symptoms. Postpyloric enteral feeding may become necessary. Prokinetic and antiemetic
therapies are the mainstay of medical management. Metoclopramide, erythromycin, and
domperidone are the useful prokinetics. Pharmacologic stimulation of gastric accommodation
may be beneficial with agents like buspirone.105,106 Long-term benefits are unclear. Neuro-
modulation with gabapentin and tricyclic antidepressant has moderate effect on the above-
mentioned vagal and enteric neuropathies affecting increased nausea, abdominal fullness,
and pain.
Surgical therapy of gastroparesis

Operative management of gastroparesis is reserved for severe, persistent symptoms that are
refractory to medical management. Surgical options range from placement of a gastrostomy or
jejunostomy tube to a total gastrectomy (Fig 1). Operations usually address one or more of the
pathophysiologic mechanisms, including autonomic dysfunction, impaired gastric accommoda-
tion, antral hypomotility, pylorospasm, and duodenal dysmotility (Table 4).106 The choice of
surgical therapy is determined by etiology and patient factors such as obesity and presence of
associated symptoms (eg, GERD) (Fig 2).
GI tubes. The placement of gastric or jejunal tubes is often the first step in surgical management.
These tubes can be placed via percutaneous, endoscopic, or surgical techniques.107,108
Gastrostomy tubes have been employed to vent the stomach and improve nausea and
vomiting.107 However, the benefits are controversial and some have recommended restricting
their use to more generalized motility disorders.98 Gastrostomy tubes can be converted to a
gastrojejunal tube with use for feeding as well as to relieve symptoms. Proximal dislodgment is a
risk with repeated vomiting. A jejunostomy tube is the preferred technique for prolonged
nutrition in patients with inadequate oral intake because of the small tube size and durability of
postpyloric feeding. Although jejunostomy tube placement improves symptoms and nutrition in
more than one-half of patients, there is a significant morbidity associated with placement.109
A B C
D E
Fig. 1. Surgical options for gastroparesis: (A) gastric neurostimulator, (B) pylorplasty, (C) sleeve gastrectomy, (D) gastric
bypass, and (E) gastrectomy.
A temporary nasojejunal tube trial of enteral feeds has been advocated by some before
permanent insertion to exclude those with small bowel dysmotility. Furthermore, patients with
gastroparesis may have associated intestinal dysmotility that may limit benefit.
Gastric neurostimulation. Electrical stimulation of the stomach has been investigated for several
decades.110,111 Initial studies of gastric stimulation employed long-duration and low-frequency
multiple channel–pacing electrodes that sought to entrain gastric slow waves, normalize gastric
arrhythmias, and accelerate gastric emptying.110 Such devices are still under investigation but
have technical constraints. Subsequently, it was shown that stimulating with a frequency several
Table 4
Operations for gastroparesis and potential mechanisms.
Autonomic Impaired gastric Antral Duodenal

dysfunction accommodation hypomotility Pylorospasm dysmotility
Gastric neurostimulation þ ?
Pyloroplasty þ
Sleeve gastrectomy þ
Gastric bypass þ þ þ þ
Gastrectomy þ þ þ þ
Gastroparesis
Failure medical therapy
Malnutrition
Obesity Mild to moderate symptoms
Gastrostomy or No GERD
GERD Postsurgical DM, idiopathic
gastrostomy tube
Gastric bypass Sleeve gastrectomy Pyloroplasty Gastric neurostimulation
Gastrectomy
Fig. 2. Surgical management of gastroparesis. DM, diabetes mellitus; GERD, gastroesophageal reflux disease.
times higher than normal (3 cycles per minute) and with low energy, and a short duration would
improve symptoms of gastroparesis.
Currently, the Enterra gastric neurostimulator (Medtronic, Minneapolis, MN) is the only Food
and Drug Administration–approved device.110-129 It was approved as a humanitarian device
exemption for treatment of drug refractory nausea and vomiting due to diabetic or idiopathic
gastroparesis in 2000. Its use has subsequently been expanded to postsurgical patients.
Approximately 1500 procedures are performed annually, an 8-fold increase over the past
decade.128 It uses a low-energy, high-frequency, short-duration pulse to stimulate the gastric
enteric nervous system.
The device can be placed via laparotomy or laparoscopy. Two electrodes are placed in the
muscularis propria approximately 1 cm apart on the greater curve, 10 cm proximal to the
pylorus, which places them near the native pacing zone on the greater curve of the stomach.
They are 1.5 cm in length and are secured by sutures and plastic discs to prevent dislodgment.
These leads are then led to a pocket in the anterior abdominal wall and connected to a pulse
generator. The generator is sutured to the fascia. The leads must be positioned carefully and
cannot be transluminal, so endoscopy is performed at the time of placement to monitor for this.
The device is programmed to standardized parameters at initial placement (rate ¼ 14 Hz, pulse
width ¼ 330 ms, current ¼ 5 mA, cycle on time ¼ 0.1 second, and cycle off time ¼ 5.0 seconds).
Adjustments are then made beginning 3 months postoperatively based on symptom improve-
ment. Generally, the current is increased. The life expectancy of the battery is up to 10 years,
depending on settings used. In one long-term study, 20% of patients underwent generator
exchange with a mean interval of 63 months.118
There are several potential surgical complications. Generator pocket infection, skin erosion,
flipped generator, or pain at the site can occur. Gastric leads can break, dislodge, or perforate into
the stomach. Intestinal obstruction from the leads has been reported. Removal of the generator
or leads occurs for infection (6%), lack of improvement (2%), and lead dislodgment or
complications (5%). Overall, 15%-25% of patients require further operation for complications.
The mechanism of gastric neurostimulation's effects on nausea and vomiting continues to be
investigated.110 It does not control gastric arrhythmias or consistently improve gastric emptying.
Potential mechanisms include increased vagal nerve activation, fundic relaxation, and effects in
centers for nausea and vomiting in the thalamic and caudate nuclei.110,111 There is preliminary
evidence that long-term gastric electrical stimulation causes improvement in basal unstimulated
gastric frequency.126
The World Anti-Vomiting Electrical Stimulation Study (WAVESS), a double-blind crossover
study whereby the Enterra system was activated or shammed starting at the time of surgery, was
the pivotal early study demonstrating efficacy.112 There was overall a significant reduction in
vomiting frequency with the device on. Subsequently, gastric electrical stimulation has been
shown to improve nausea and vomiting, reduce total symptoms and improve quality of life,
improve nutritional status, decrease use of medications, and decrease medical costs in various
studies.113-121 Long-term observational studies indicate better than 50% symptomatic improve-
ment with mean follow-up of 56 months and lasting up to 10 years.113 Symptomatic
improvement was greater in diabetic (60%) and postsurgical patients (59%) than idiopathic
patients (49%). However, gastric emptying time did not consistently change. Abdominal pain is
the symptom least likely to improve. Predictors of having a better outcome include etiology
(diabetes mellitus), and lack of preoperative narcotic dependence, psychiatric disorders, and
presence of gastric dysrhythmias.117 Previous cholecystectomy may also improve the outcome.
Obese patients may not respond as well.121 Overall, the clinical efficacy remains unclear and the
outcomes may be related to severity of initial symptoms.127
There are several surgical approaches to patients who fail to respond to gastric neuro-
stimulation. Zehetner and colleagues123 converted 25% of patients to subtotal gastrectomy for
treatment failure, with 87% symptom improvement. Timratana and collleagues121 performed
gastric bypass on obese patients who failed neurostimulation. Placement of a new gastric
electrical stimulation system in patients who had an initial satisfactory response has also been
reported.119
Several recent developments might improve outcomes. Endoscopic placement of electrodes
to permit temporary gastric stimulation has been reported.124 It has been suggested that this
technique might have a role in predicting outcome of permanent placement. Based on evidence
that pyloroplasty accelerates gastric emptying, it has been proposed to combine pyloroplasty
with gastric neurostimulation.129
Pyloric procedures. Manometric studies in gastroparetic patients have demonstrated prolonged

periods of increased pyloric tone and phasic contractions, termed pylorospasm.100 Clinical
assessment is fairly difficult. Endoscopy and contrast studies are not reliable. Manometry of the
pylorus has been employed most frequently, but its availability is limited. The endoluminal
functional lumen imaging probe (EndoFLIP) that uses impedance planimetry to estimate
distensibility and compliance of the pylorus has recently been described.130,131 Several strategies
have been employed to address pyloric dysfunction.130-132
Botox inhibits acetylcholine release leading to temporary muscle relaxation. Thus, endoscopic
injection into the pylorus has been performed. However, endoscopic Botox injection for
pylorospasm does not appear to be effective. Although initial small open-label studies of Botox
injection (100-200 units) into the pylorus improved gastric emptying and symptoms,
subsequent randomized studies have shown no improvements in symptoms compared with
placebo.132-134
Endoscopic transpyloric stent placement is another option.130,135 Fully covered, self-
expandable metallic stents have been employed. Although short-term improvement has been
suggested in a small group of patients, efficacy and long-term durability must be further
assessed. Stent migration occurred in one-half of patients.130 Although this therapy may not
represent a long-term strategy for relief of symptoms, it may permit identification of patients
who would benefit from pyloroplasty.
Pyloroplasty has been employed for decades as a gastric drainage procedure after vagotomy.
There have now been several large series reported in patients with gastroparesis.137,138
Shada and colleagues138 reported 177 laparoscopic Heineke-Mikulicz pyloroplasty
procedures performed in patients with delayed gastric emptying. A total of 105 patients
(58%) had a concomitant fundoplication. The morbidity rate was 7%, with 4 reoperations and
2 confirmed leaks. Gastric emptying studies were performed in 40% of patients postoperatively
with improvement noted in 86%. In all, 11% of patients underwent subsequent procedures for
persistent symptoms, most frequently gastric neurostimulator placement or gastrectomy. There
were significantly improved symptoms. These investigators suggest that pyloroplasty should be
the initial procedure for mild-to-moderate delayed gastric emptying. Mancini and colleagues137
reported results of 46 patients with gastroparesis undergoing pyloroplasty. GES improved in 90%
and symptoms improved significantly over 6-12 months of follow-up. Toro and colleagues139
reported outcomes of 50 patients undergoing pyloroplasty. Symptoms improved in 82% and GES
improved in 96% with mean follow-up of 3 months. Overall, 10% of patients required further
operation.
Pyloroplasty is now often performed at the time of gastric neurostimulator place-
ment.129,130,137-139 In a retrospective study, 49 patients underwent gastric neurostimulator
placement and 26 had concomitant pyloroplasty. Gastric emptying studies were improved in
64% of patients who underwent pyloroplasty compared with those in 7% of controls, but
symptom improvement was similar.129
As gastroparesis is commonly associated with GERD, there is interest in treating both the
conditions. Fundoplication combined with pyloroplasty has been performed for combined GERD
and gastroparesis. Masqusi and Velanovich140 reported symptom improvement in 80% of
patients with improved gastric emptying. Farrell and colleagues141 found that although
fundoplication alone improved delayed gastric emptying in 38% of patients, the addition of a
pyloroplasty improved gastric emptying in 70%. Khajanchee and colleagues142 also found that
GERD improves even with delayed gastric emptying and dyspeptic symptoms are improved by
fundoplication, but significantly better with pyloroplasty.
Endoscopic peroral pyloromyotomy has recently been reported.130,136 The antral mucosal is
incised permitting submucosal division of the pyloric sphincter. This has been successfully
performed in a small number of patients with short-term improvement in symptoms. This may
provide a less invasive approach to pylorospasm.
Sleeve gastrectomy. Longitudinal gastric resection, or sleeve gastrectomy (SG), was initially
performed for ulcer disease, but has recently become popular as a bariatric procedure. Initially, it
was part of duodenal switch procedures, then as the first step of a 2-stage operation in high-risk
patients, and now as a primary bariatric procedure.143 SG has also been employed for gastric
ischemia or benign and malignant lesions on the greater curve.144 It is now being proposed as a
procedure for treatment of gastroparesis.145-147
SG can be performed by open or laparoscopic approaches. SG is performed by mobilizing the
greater curve of the stomach and then performing longitudinal resection, usually with a stapler,
beginning 5 cm proximal to the pylorus and ending near the gastroesophageal junction. The
stapler is typically applied alongside a calibrating bougie or endoscope of 40-60 Fr. This removes
approximately 80% of the stomach and results in a 100-120-mL gastric pouch, with the goal to
minimize the formation of proximal or distal dilated pouches.
SG can affect gastric physiology via several mechanisms.148 The gastric pacemaker is excised.
There is greater intraluminal pressure and less distensibility in the gastric tube. Excision
of most part of the body and fundus results in decreased parietal cell mass and decreased level of
hormones such as ghrelin. This also eliminates the receptive and propulsive activities
of the fundus. Although the goal is to maintain functional integrity of the antral pump, this
might be impaired. Experimental studies suggest there is less vagal denervation with SG than
GBP.149
Several studies have demonstrated that gastric emptying of both solids and liquids is
accelerated after SG.150-153 Nearly all have been performed in obese patients, who may have pre-
existing motility abnormalities. SG also accelerates small intestinal motility.154
SG has been associated with increased symptoms of GERD.155 This may be related, in part, to
marked narrowing or strictures of the gastric channel and development of a proximal fundal
pouch.156 Other symptoms (fullness, early satiety, or pain) may also occur but do not correlate
well with gastric emptying studies.157 The dumping syndrome also occurs.154
There have been a few small clinical series reported with SG. Meyer and colleagues145
performed SG on 9 obese patients with gastroparesis. All had symptomatic improvement with
more than 1-year follow-up. Body mass index (BMI) decreased by 3 kg/m2 and 3 of 4 patients
had normal gastric emptying. Bagloo and colleagues147 performed SG in 4 diabetic patients with
gastroparesis. Three patients (75%) had symptomatic improvement on short-term follow-up. Le
Page and Martin146 reported 4 patients who underwent SG at the time of hiatal hernia repair and
fundoplication for gastroparesis. They found a 67% improvement in gastric emptying and
resolution of symptoms. The surgical technique was modified to leave the proximal 1-2 short
gastric vessels and the corresponding fundus to permit fundoplication. The gastric tube was
more generous and the antrum preserved. BMI ranged from 19-32 kg/m2 and was preserved up
to 24 months postoperatively. These results suggest that SG is a useful approach to gastroparesis,
particularly in overweight patients without GERD.
Gastric bypass. Gastric bypass (GBP) is another surgical option for treatment of
gastroparesis.158,159 This procedure addresses several of the pathophysiologic mechanisms
(Table 4). GBP has been primarily considered an option in obese patients. An advantage over
gastrectomy is the potential reversibility of this procedure.
There have been 2 recent reports with GBP for gastroparesis. Sun and colleagues158
performed GBP in 7 morbidly obese patients with refractory gastroparesis, including 4 of whom
who had undergone prior gastric neurostimulator placement. A total of 70% of patients reported
symptom improvement at a mean follow-up of 20 months. Two patients with persistent
symptoms underwent remnant gastrectomy with improvement in 1 patient. BMI decreased by a
mean of 8 kg/m2. Symptomatic improvement was comparable to 20 obese patients undergoing
gastric neurostimulator placement.
Papasavas and colleagues159 similarly reported GBP in 7 morbidly obese patients with
gastroparesis, including 2 gastric neurostimulation failures. At 8 months of follow-up, total
symptom score was improved but 3 patients continued medication. Mean BMI decreased by
9 kg/m2. Gastroesophageal reflux resolved in 5 of 6 patients in whom it was present
preoperatively.
Gastrectomy. Refractory gastroparesis has long been treated by subtotal (70%) or complete
gastrectomy.160-167 Gastrectomy is usually considered in the most severe cases in which other
treatment modalities have failed. The long-term results may be best in patients who have
previously undergone a partial gastrectomy or vagotomy rather than those with diabetic or
idiopathic gastroparesis. Although gastrectomy deals with the pathophysiologic defects of
gastroparesis, there are concerns about continual symptoms, nutrition, and morbidity and
mortality.
There have been several reports of gastrectomy. Eckhauser and colleagues162 reported 81
patients with postsurgical gastroparesis undergoing completion gastrectomy. In all, 80% had
long-term symptom improvement with mean follow-up of 56 months. Speicher and
colleagues163 reported 44 patients undergoing completion gastrectomy for postsurgical gastric
atony. Complications occurred in 36% and there was 1 death. There was significant improvement
in symptoms and ability to take oral nutrition. A total of 78% felt they were in better health with
mean follow-up of 5.6 years. Postoperative morbidity was 36% and mortality was 2%. Forstner-
Barthell and colleagues164 reported completion gastrectomy in 62 patients had undergone
vagotomy with 5-year follow-up. Overall, the operation was successful in 43% of patients.
Chronic nausea, need for PN, and retained food at endoscopy preoperatively were negative
prognostic factors. Patients with post–fundoplication gastrectomy have a particularly poor
outcome after gastric resection.166
Bhayani and colleagues160 demonstrated the feasibility of laparoscopic gastrectomy. They
reported 35 patients with refractory gastroparesis. Nearly one-half had undergone previous
pyloroplasty and a similar number fundoplication. The percentage of patients who had improved
symptoms was 70%. The cause of gastroparesis was postoperative (43%), diabetic (34%), and
idiopathic (23%). Zehetner and colleagues123 had a similar favorable experience in 44 patients.
Experience with gastrectomy for diabetic gastroparesis is more limited. Watkins and
colleagues161 reported 7 patients with diabetic gastroparesis who underwent subtotal (70%)
gastrectomy. Symptoms were relieved in 85% with 6-year follow-up. Zehetner and colleagues123
reported laparoscopic subtotal gastrectomy in 12 patients with diabetic gastroparesis.
A special circumstance is gastroparesis of the gastric conduit after esophagectomy. The role of
routine pyloromyotomy or pyloroplasty in this setting is controversial.168 Currently, endoscopic
dilation or Botox injection and even gastric neurostimulation are treatment options.169-171
Rapid gastric transit
Rapid gastric transit is associated with a spectrum of postprandial symptoms referred to as

the dumping syndrome.172-175 The primary mechanism of these symptoms is the transit of
unprocessed or poorly processed hyperosmolar gastric contents into the proximal small bowel
rather than the actual speed of gastric emptying.96,97 This can result from bypass or functional
impairment of the pyloric sphincter. Vagotomy may also contribute. Early dumping occurs 15-30
minutes after meal ingestion, when the hyperosmolar luminal contents enter the proximal
intestine. This leads to a sudden fluid shift in the intestine with associated nausea and vomiting,
diarrhea, diaphoresis, hypotension, and occasionally syncope. Serotonin release also leads to
further peripheral and mesenteric vasodilatation. Late dumping occurs less frequently and
results from the swift uptake of glucose and other sugars from the small bowel. This results in
hyperglycemia, stimulating the release of insulin, with a subsequent rebound hypoglycemia
occurring 45-60 minutes after the meal.
The dumping syndrome has been recognized for more than 100 years. Most cases of dumping
syndrome have been related to partial gastrectomy and pyloroplasty (Table 5). However, most
gastric procedures can lead to this complication. In the current era of gastric surgery, it is often
associated with the gastric bypass performed for morbid obesity.172 A total of 40% of patients
undergoing GBP or SG have this complication. Reduced gastric compliance can also result in
accelerated emptying. Vagotomy may also play a role.176
Currently, the radionuclide colloid scintigraphy using the isotope-labeled 2% scrambled egg
meal is used to diagnose this condition. Greater than 50% transit of luminal contents in 1 hour
establishes the diagnosis. Previously, a barium gastric emptying study was used to support the
diagnosis. A glucose challenge test may also be performed.172
The initial management of rapid gastric transit includes dietary modifications encouraging
solid intake and avoiding hyperosmolar intake. Small high-protein, low-carbohydrate meals
spread out over 6 meals a day is the standard recommendation. Patients should avoid drinking
with meals. A variety of medications may also be helpful, such as the alpha-glucose–based
inhibitor acarbose, which slows carbohydrate digestion and blunts hyperglycemia and
hypoglycemia, and the long-acting somatostatin analogue octreotide, which slows gastric
emptying and blunts the hormonal response.
Table 5
Surgical procedures causing dumping syndrome.
Esophagectomy
Nissen fundoplication
Roux-en-Y gastric bypass
Vagotomy
Antrectomy
Gastrojejunostomy
Pyloroplasty
Surgical therapy for dumping syndrome

In severe cases, refractory to medical treatment, there may be surgical options to decrease
rapid gastric transit. This would be determined by previous operative procedures. However,
overall these procedures often have unpredictable outcomes and may have significant
morbidity.
One strategy is to try to restore more normal gastric anatomy. There have been attempts at
direct pyloric reconstruction after previous pyloroplasty.173 Conversion of Billroth type II
procedures to type I either directly or with an interposed intestinal segment is another
alternative. Takedown of a gastric bypass, restoring continuity with the distal stomach, is
another option.
Another approach is to use intestine distal to the stomach to slow gastric emptying.
Historically, the surgical option employed has been the use of reversed intestinal segments to
slow gastric transit.177-180 As Roux-en-Y reconstruction may delay gastric emptying (Roux stasis
syndrome), converting a loop gastrojejunostomy to a Roux-en-Y should alleviate dumping
syndrome.
Dumping syndrome after bariatric procedures is a challenging problem. In these patients,
maintaining weight loss is an important goal. Revisional procedures have taken several
approaches. One is reconstruction of the gastric reservoir. This might include conversion to a
SG.181-183 Addition of a restrictive intervention (band around pouch, revision of pouch) has also
been employed. Insertion of a short antiperistaltic loop is another possibility.
There have been several surgical strategies to reduce the occurrence of dumping syndrome
after a variety of surgical procedures. Pylorus-preserving gastrectomy is one such approach.
A technique to reconstruct the pylorus with a gastric flap valve at the time of Billroth I
anastomosis has recently been described.184
Small intestinal motility disorders
Normal intestinal motility
Digestion of nutrients in the small intestine is facilitated by contractile movements of the

smooth muscle layers, which provide antegrade propulsion of the luminal contents combined
with mixing action through segmentation.185-187 There is organized, periodic motor activity,
even in the absence of luminal nutrients. Regulation of these activities is complex and involves
the intrinsic neural network as well as the connections to the central nervous system. There are
smooth muscle cells present in both the longitudinal and circular muscle layers as well as in the
muscularis mucosae. They display spontaneous electrical depolarizations and are able to
propagate this signal in neighboring smooth muscle cells. The dominant intestinal pacemaker
that initiates these contractions is localized in the circular muscle of the duodenum as interstitial
cells of Cajal. These periodic depolarizations occur 11-13 times per minute in the proximal small
intestine and decrease to 8-10 times per minute in the ileum. As the rate of contractions is
higher in the proximal gut, the transit time in the distal intestine is longer than that of the
proximal portion.
The contractile activity of the small intestine is under a variety of regulatory mechanisms,
including myogenic, neural, and chemical control. This smooth muscle contractile activity is
influenced by both extrinsic autonomic (parasympathetic and sympathetic) neural activity from
the central nervous system and the intrinsic neurons of the enteric nervous system.
Parasympathetic innervation comes via the vagus nerve, which has both afferent and efferent
fibers. Sensory fibers account for 80% of the vagal fibers. Sympathetic innervation rises from the
thoracic and lumbar spinal nerves. Stimulation of the vagus fibers produces contractile activity
in the upper part of the small intestine. Electrical stimulation of the mesenteric sympathetic
nerves inhibits small intestinal contractions. The enteric nervous system is made up of
interconnected neural plexuses and ganglia. Most neurons controlling contractile activity in the
small intestine have their cell bodies in the myenteric plexus. The myenteric reflex results in
relaxation of the gut distal to a point of excitation. Chemical control involves the stimulation or
innervation of smooth muscle activity by neurocrine, paracrine, or endocrine factors. There is
also a relationship between the immune system of the gut and enteric nervous system.
Contraction of the small intestine can be divided into individual phase contraction, organized
groups of contractions, and special propulsive contractions. Individual phase contractions are
the basic motor activity in the small intestine. They occur in both fasted and fed states.
Contractions are more regular and coordinated in the proximal part of the gut. Rapidly migrating
contractions can occur with a velocity as fast as 30 cm/s over distances up to 200 cm within the
intestine. The MMC is a cyclic pattern of phasic contractile activity that occurs in the fasting
state. The MMC originates in the proximal portion of the small intestine and migrates to the
distal ileum with a cycle time of 90-120 minutes. This functions to cleanse the small intestine of
residual food and enteric secretions and minimizes bacterial growth. The MMC appears to derive
from the enteric nervous system.
Peristalis involves coordinated circular and longitudinal contractions resulting in propulsion
of luminal contents. Pendular contractions occur in longitudinal muscles and cause a back and
forth movement. Segmental contractions occur in circular muscle, causing a mixing rather than
propulsive function. There are also special propulsive contractions. Retrograde giant contrac-
tions can result in a long-duration retrograde contraction initiated from the midportion of the
intestine with a propelling of intestinal contents back to the stomach. This is a motor activity
correlative of vomiting. Giant migrating contractions are also called prolonged propagated
contractions. Large-amplitude long-duration contractions occur near the ileocecal junction. The
primary function may be to return refluxed fecal contents from the ileum back to the colon.
Thus, more frequent giant migrating contractions can be associated with diarrhea.
Disruption of normal intestinal motility can lead to a variety of problems. Abnormally slow
transit can be temporary (ie, ileus) but may be permanent, termed intestinal pseudo-
obstruction. Abnormally rapid transit can also occur, particularly after operations such as
intestinal resection and ileostomy.
Slow intestinal transit
Intestinal pseudo-obstruction
Chronic intestinal pseudo-obstruction (CIPO) is a heterogeneous clinical syndrome that has
diverse causes and varied clinical presentations.188-198 The initial description in 1958 included 13
patients who underwent laparotomy for presumed intestinal obstruction and no mechanical
obstruction was found.188 Only 4 had undergone previous laparotomy. CIPO can be distinguished
from ileus by its chronic nature and by the presence of an underlying disease process involving
the intestine muscle, mesenchyme (ICC), or enteric nervous system. However, such abnormal-
ities may not always be obvious on a routine histologic evaluation. These changes may be diffuse
throughout the GI tract and are often just one component of a more generalized systemic illness
(Table 6). Although some developmental conditions (eg, Hirschsprung disease) are well
recognized, most of the causes are acquired. There appears to be a female predominance and
one-half of patients have a genetic basis.
Abnormalities of both the extrinsic and the intrinsic enteric nervous systems result in a
spectrum of altered motility that includes loss of normal peristalsis and transit, disruption of
normal cyclical events, spontaneous, uncoordinated contractile activity, and abnormality of
sphincter function. In general, myopathic conditions cause low-amplitude pressure activity and
hypomotility, whereas neuropathic pseudo-obstruction is characterized by excessive and
uncoordinated activity. These can be associated with marked dilatation in any part of the
intestinal tract. One-half of patients have motility disorders of the stomach and colon as well.
CIPO can occur in patients of all ages. Recurrent attacks of nausea, vomiting, cramping,
abdominal pain, and abdominal distension are variable in their frequency, intensity, and
duration. Both constipation and diarrhea can be present. Distension may eventually become
permanent and the resultant anorexia and malabsorption lead to progressive weight loss and
Table 6
Classification of intestinal pseudo-obstruction.
Primary
Neuropathy
Myopathy
Mesenchymopathy
Secondary
Collagen vascular disease
Diabetes
Neurologic disorders
Other
Idiopathic
malnutrition. Bacterial overgrowth is a common associated problem. If pain is a dominant

feature, narcotic bowel syndrome should be considered.195
The nonspecific symptoms and lack of definitive diagnostic testing make diagnosis of CIPO
difficult. These patients often undergo laparotomy for a presumed mechanical obstruction with a
diagnosis being made postoperatively. Conversely, mechanical obstruction that is mistakenly
diagnosed as CIPO can lead to inappropriate treatment with disastrous consequences. CIPO
should be suspected when there is a long duration of changing symptoms. Alternating
constipation and diarrhea and underlying diseases are the factors commonly associated with
pseudo-obstruction. Mechanical obstruction must be excluded through radiologic, manometric,
and endoscopic assessment. The presence of small intestinal diverticulosis suggests the
diagnosis of pseudo-obstruction, as one-fourth of patients would have this condition.198
Full-thickness biopsy of the small intestine may permit definitive histologic study.193
Radiologic study is necessary to eliminate the possibility of mechanical obstruction and to
localize the disease process. Contrast studies may demonstrate anatomical abnormalities such as
bowel dilatation and diverticulosis as well as prolonged transit time. Radionuclide studies can be
useful for evaluating gastric emptying and intestinal transit time. Wireless motility capsules are
useful for determining transit time.199 This technology assesses gastric and colonic transit
as well.
Therapy for CIPO includes identification and treatment of any underlying causes. Prokinetic
therapy can be attempted, but has variable success. Bacterial overgrowth should be sought and
treated with the appropriate antibiotic therapy. One-half of patients require PN. In patients
requiring PN, the survival rate is 78% at 5 years, 75% at 10 years, and 68% at 15 years.197
Surgical therapy. More than one-half of patients undergo exploratory laparotomy owing to
confusion about diagnosis. Careful preoperative evaluation should decrease the number of
unnecessary operations performed to rule out mechanical obstruction.200 If operation is
undertaken and no mechanical obstruction is found, a full-thickness biopsy should be
considered. Other operative therapy in this situation is often not effective. Although resection
or bypass of an apparently localized process is tempting, surrounding areas are often involved
and operation can exacerbate the motor dysfunction. This is especially true of patients with
underlying systemic lupus erythematosus and SSc.201,202
There are several patterns of pseudo-obstructive complications that require different surgical
approaches (Fig 3). Localized disease is often present initially. Megaduodenum is a problem in
several disorders. Both side-to-side duodenojejunostomy and gastrojejunostomy have been
performed, but with variable results. The duodenum can be defunctionalized by antrectomy and
vagotomy to relieve symptoms as well. Intestinal tapering, either by simple imbrication or
excision along the antimesenteric border, can be used for a localized dilated small bowel.
Diffuse intestinal disease is a more challenging problem. Venting gastrostomy, enterostomy,
or cecostomy tubes, and long-term nutritional support have generally been used to palliate
Suspected intestinal pseudo-obstruction
rule out mechanical obstruction
pseudo-obstruction mechanical
treat underlying disease appropriate operation

symptom control
nutrition support
localized disease diffuse disease

tapering venting tubes
intestinal bypass subtotal enterectomy
intestinal resection
parenteral nutrition intestinal transplantation
Fig. 3. Management of intestinal pseudo-obstruction.
patients with diffuse disease.203-205 Patients with extensive diverticulosis may require resection
of these segments to prevent bacterial overgrowth or the development of pneumoperitoneum.
Although CIPO cannot be cured surgically, operation is often undertaken. Sabbagh and
colleagues206 reported outcomes of 63 adult patients with CIPO undergoing operation. A total of
43% of initial operations were exploratory laparotomy alone. Another 43% underwent various
resections and the other 14% underwent ostomy creation. Volvulus, perforation, and recurrent
obstruction were frequent problems. Prolonged postoperative ileus was common. Patients
underwent an average of 3 procedures including many repeat laparotomies and resection. The
overall morbidity rate was 58% and the mortality rate was 8%. Therefore, operation should be
considered cautiously.
Dysfunctional bowel may eventually need to be resected, but symptoms usually recur and
short bowel syndrome (SBS) can result (Table 7).206-210 The morbidity and mortality rates for
subtotal enterectomy are high. Sabbagh and colleagues206 reported 10 patients undergoing
subtotal resection leaving a 20-cm proximal intestinal remnant with jejunocolic anastomosis.
The 30-day mortality rate was 10%. In all, 60% of patients died during follow-up, most with
underlying disease. One patient had PN-related liver disease. In surviving patients, oral intake
was improved and PN was reduced. Lapointe207 reported 8 PN-dependent patients who
underwent subtotal resection with residual intestinal length of 28 cm. Two patients who had
initial ileal anastomosis required conversion to jejunocolic anastomosis. All stayed PN
dependent but improved symptomatically. Mean survival was 6 years. One patient died of liver
failure.
Intestinal transplantation may eventually be an option in some patients in whom massive
resection has been performed or is anticipated.211,212 Approximately 10% of intestinal transplants
are performed for motility disorders in adults. The overall outcome appears to be similar to
transplantation for other etiologies.
Rapid intestinal transit
Short bowel syndrome

Operations designed to prolong intestinal transit time and thereby improve absorption for
SBS have been performed clinically for several decades. However, the short-term and long-term
Table 7
Experience with subtotal enterectomy for intestinal pseudo-obstruction.
Intestinal Follow- Morbidity Mortality Liver failure

Author # Jejunocolostomy Jejunoileostomy length (cm) up (y) (%) (%) (No.)
Sabbaugh and 10 9 1 20 – 80 10 1
colleagues206
Lapointe207 8 1 7 28 6 – 0 1
Mughal and 3 2 1 20 1.3 33 0 0
Irving209
Noel and 1 0 1 120 3 0 0 0
colleagues210
efficacy of these procedures remains unclear.213-218 They are often performed at the time of
ostomy closure or other surgical interventions that might themselves independently improve
intestinal function.217,218 These adjunctive procedures are also frequently performed early after
resection during the adaptive phase of SBS. Thus, it is difficult to determine whether further
improvements in intestinal absorption and nutritional status are because of the surgical
procedure or the normal adaptive process. Three procedures (reversed intestinal segments,
colon interposition, and intestinal valves) have been attempted in sufficient numbers to deserve
consideration (Fig 4).
Reversing segments of intestine to slow intestinal transit has been reported most extensively
for SBS.213-218 The antiperistaltic segment functions by inducing retrograde peristalsis distally,
which disrupts the motility of the proximal intestine. In addition, myoelectrical activity in the
distal remnant is slowed by the disruption of the intrinsic nerve plexus. Reversed segments also
alter the hormonal milieu normally found after massive resection.219 Experimental studies of
antiperistaltic segments generally demonstrate slowed intestinal transit, improved absorption,
reduced weight loss, and prolonged survival after intestinal resection.219,220
For the antiperistaltic segment to slow intestinal transit without causing complete functional
obstruction, several technical details are important. The optimal length of the reversed segment
would appear to be approximately 10-12 cm in adults.213-218 A satisfactory vascular arcade to the
segment must be identified and complete rotation of the mesentery is avoided during reversal to
prevent intestinal ischemia. The reversed segment should be created as distal as feasible in the
small intestinal remnant to get the benefit of proximal absorptive surface. Depending on
anatomy, this may be proximal to an ostomy or a colonic anastomosis.
More than 80 patients with SBS have undergone antiperistaltic segments. Of them, 90% were
adults.213-218 The length of the segment has varied from 5-15 cm in adults in these reports.
Clinical improvement has been reported in 80% of patients with SBS in these anecdotal reports,
with slowed intestinal transit and increased absorption demonstrated. Long-term function of
Fig. 4. Techniques for slowing intestinal transit: antiperistaltic segment (left), intestinal valve (center), colon
interposition (right). (Reprinted with permission from Thompson JS, et al Current Problems in Surgery. New York:
Elsevier; 2012 p. 85.)
antiperistaltic segments has been demonstrated radiographically. However, our experience and
that of others suggests only a 50% long-term clinical benefit.214-218 Transient obstructive
symptoms, intestinal ischemia, and anastomotic leak are potential early problems that have
been reported. Small intestinal bacterial overgrowth is a theoretical long-term concern. This
procedure has been reported in patients with Crohn disease with acceptable outcomes and it
does not appear to influence recurrence rates.215,217,220 In patients with mesenteric scarring or
residual intestinal disease, reversed intestinal segments may not be feasible. Obviously, caution
must be exercised in patient selection.
The effect of surgically constructed valves on intestinal motility may involve several potential
mechanisms similar to reversed segments. They create a partial mechanical obstruction and
disrupt the normal motor pattern of the small intestine. They may prevent retrograde reflux of
colonic contents in patients with retained colon.213,221 Intestinal valves and sphincters have been
shown to prolong intestinal transit time, increase absorptive capacity, and improve survival after
intestinal resection in experimental studies. However, the results have been inconsistent.
Effective valves usually induce some dilation of the proximal intestine and may cause, at least
transiently, obstructive symptoms. Necrosis of the valve and complete obstruction due to
narrowing and intussusception are potential complications. Durability of the sphincter function
of valves is also a concern.
Intestinal valves have been created using several techniques. These include external
constriction of the intestine, segmental denervation, and intussuscepting intestinal segments
to increase intraluminal pressure, with the latter being employed most frequently. Studies
suggest that intussuscepted or nipple valves should be 2 cm in length if created retrograde and
4 cm if the valve is prolapsed antegrade. We have generally created a retrograde sphincter
similar to that employed in the continent ileostomy procedure but only 2 cm in length.221
However, there is little experience to recommend one technique over another.
Intussuscepted valves have been reported in 25 adults and 1 infant as primary treatment of
SBS.213,222,223 In all, 24 patients improved markedly, one patient had questionable benefit, and
the other patient required takedown of the valve. Important clinical end points have been
improved diarrhea and maintenance of body weight. However, in one study nipple valves failed
anatomically in one-third of patients followed up for more than 5 years, again raising the issue
of durability.
Interposing a colonic segment within the small intestinal remnant to retard intestinal transit
has been performed in either an isoperistaltic or antiperistaltic fashion. Isoperistaltic
interposition is performed proximally and functions by slowing down the rate at which
nutrients are delivered to the distal small intestine.213 Similar to the reversed small intestinal
segment, antiperistaltic colon interposition is performed distally. In addition to their effect on
intestinal transit, interposed colonic segments absorb water, electrolytes, and nutrients. In
experimental studies, isoperistaltic colon interposition generally resulted in slower transit time,
less weight loss, and improved survival after resection, but results with antiperistaltic colon
interposition have been less consistent. The length of colon interposed seems to be less critical
for efficacy than with reversed segments of small intestine.
Colon interposition has been reported clinically in a dozen patients with SBS. Of them, 11
were with isoperistaltic interpositions.213,214,224 The length of the interposed colon segments
varied between 8 and 24 cm. Most patients (92%) were infants younger than 1 year of age. All
patients were PN dependent before the procedure. Only one-half of the patients demonstrated
sustained clinical improvement. The other half, including one with an antiperistaltic colon, did
not improve and subsequently died of sepsis or hepatic failure. Colonic stasis with bacterial
overgrowth is suspected to have contributed to these outcomes. Overall, this experience
suggests that isoperistaltic colon interposition may have some merit, but there are no long-term
studies.
In summary, procedures designed to slow intestinal transit should be applied cautiously in
patients with SBS with demonstrated rapid transit. Although we generally use an 80-90-cm
remnant as the cutoff, others would consider the procedure in patients with as little as 40 cm of
intestinal segment.217 These procedures are best considered only after maximal intestinal
adaptation has occurred. However, the opportunity to perform them may come earlier in the
clinical course. Antiperistaltic segments should be used in patients with longer remnants so that
the 10-cm segment used still leaves sufficient intestinal remnant for absorption. Valves should
be considered in patients with shorter remnants because less bowel is used. Colon interposition
has largely been restricted to children in whom the small intestinal remnant is much shorter.
Overall, these procedures are applicable to only a small proportion of patients with SBS (o 5% in
our experience) and their long-term efficacy remains uncertain.
Ileostomy diarrhea
Normally, 1-1.5 L of fluid enters the colon from the small intestine each day. In the early
postoperative period after ileostomy creation, patients demonstrate a dramatic excretion of
water and sodium.225 These losses typically decrease over several days, decreasing to less than
1 L per day within 1-2 weeks. If intestinal resection has not been performed, ileostomy output
typically remains less than 1 L per day and not associated with significant malabsorption of
nutrients, vitamin deficiency, or metabolic abnormalities. However, in some patients this output
persists for 1 or 2 months and in a small number of patients there is a chronic elevated ileostomy
output. Ileostomy diarrhea is a persistent output greater than 2 L per day.226,227
In patients with ileostomy diarrhea with a persistently high output, it is important to exclude
partial small bowel obstruction because of either ileostomy stenosis or because of proximal
lesions such as adhesions, intra-abdominal abscess, recurrence of disease (eg, Crohn disease), or
previous resection. Bacterial overgrowth and medication-induced diarrhea are other factors that
should be considered. Thus, the initial management of ileostomy diarrhea is aimed at evaluating
the presence of these factors. However, in most patients no identifiable cause will be found and
empiric treatment is indicated. Dietary substances known to increase ileal fluid should be
avoided. These primarily involve fruits, juices, and baked beans. Fiber supplementation may also
be useful, including both insoluble and soluble fiber. Oral rehydration therapy may also be
useful. A variety of pharmacologic treatments are also available. Antimotility agents such as
loperamide, diphenoxylate, and opiates can be employed. Other potential therapies including
serotonergic agents such as alosetron and antisecretory agents such as octreotide, H2
antagonists, PPIs, and clonidine may also be used.
There has been a long-term interest in surgical treatments for persistent ileostomy diarrhea.
These would be employed when other causes of ileostomy diarrhea have been ruled out, medical
therapy has been used, and significant disability occurs from the fluid loss and need to change
the appliance frequently. These primarily involve reversed or antiperistaltic loops of intestine to
reduce transit in ileostomy output similar to their use in SBS.228-230 These are primarily
anecdotal reports and our experience for this indication is also limited. Recently, a modified
ileostomy with a preserved ileocecal valve has been described in an attempt to ameliorate
ileostomy diarrhea.231
Colonic motility disorders
Normal colonic motility
There is a wide variation of what patients consider to be “normal” bowel habits. Within a
normal colon, motility is greatly influenced by diet, fluid consumption, medications, time of day,
sleep cycles, physical and emotional stress, and anatomical variation.232,233 In general, the
propagation of contents through the colon relies on alternating waves of contraction and
relaxation, with an average net transit rate of 1 cm/h.234 Defecation involves not only delivery of
stool to the rectum but also a complex and coordinated effort of the anorectum, which is
discussed elsewhere. However, the colon continues to participate in motility during defecation,
as there is an associated increase in propagating pressures as the colon attempts to fill and
distend the rectum.234 The normal timing and extent of defecation is arbitrary, and often relies
on the related definitions of “abnormal.” Normal colorectal function results in soft, formed
bowel movements that occur, without straining, at least every 1-2 days.
A brief review of physiology is necessary to frame the discussion of motility disorders. The
main physiological roles of the colon are the following: (1) bacteria-moderated fermentation of
complex carbohydrates and proteins, (2) absorption of water and nutrients, and (3) transport of
feces to the rectum.232 The production of short-chain fatty acids by bacteria within the colon is
an essential component of colonic health and function, and can be affected by the microbiome
and diet. Similarly, the efficiency of water and nutrient absorption has a major influence on
colonic motility. A healthy colon typically receives 1.5-2 L of fluid from the small bowel every
day, but reabsorbs approximately 90%.233 Changes in sodium and water resorption can lead to
changes in the volume and consistency of stool delivered to the rectum.
It is noteworthy that anorectal physiology is an integral component of GI motility and
defecation. Anorectal disorders such as obstructive defecation are quite common, and contribute
greatly to common motility issues such as constipation. A recent volume of Current Problems in
Surgery explored the topic of pelvic floor disorders elegantly and in great detail, and those efforts
are not repeated in this section.235
Functional disorders of the colon are very common, and include rapid transit, slow transit,
and the inability to evacuate. The severity of complaints extends from mild discomfort to
unacceptably poor quality of life and complete inability to work.
Delayed colonic transit
Acute colonic pseudo-obstruction (Ogilvie syndrome)

Acute colonic pseudo-obstruction was first described by Sir William Heneage Ogilvie in 1948,
where he presented a 2-patient case report of “large intestine colic due to sympathetic
deprivation.”236 Both of these patients had retroperitoneal malignancies, and died shortly after
presentation. The condition was subsequently given the eponym “Ogilvie syndrome” (OS) and is
frequently encountered in modern surgical practice.
OS involves acute dilation of the colon in the absence of an anatomical obstruction, and is
thought to be secondary to a loss of autonomic tone.237 The cause is often nondistinct and
multifactorial, but tends to be a visceral response to another stressor rather than a primary
colonic process. Examples of underlying causes are opiate consumption, electrolyte abnormal-
ities, trauma, and underlying infection. Patients often present with moderate-to-severe
abdominal distention and imaging that reveals a diffusely dilated colon. If left untreated, OS
can progress to colonic perforation, sepsis, and death.
The careful surgeon treats OS as a diagnosis of exclusion, and before initiating therapy, one
must exclude the possibility of a large bowel obstruction. This is typically accomplished with a
water-soluble enema, but careful endoscopy is also appropriate.232 In addition, toxic megacolon
related to Clostridium difficile infection must also be excluded, as it carries similar radiologic
characteristics, but is usually associated with a more toxic patient, leukocytosis, and thickening
of the bowel wall.
Once this is accomplished, most cases will respond to conservative therapy with bowel rest,
correction of electrolytes, elimination of polypharmacy, and treatment of the underlying
illnesses. When these measures are unsuccessful, the patient should be given intravenous
neostigmine, an acetylcholinesterase inhibitor that carries a 91% clinical response rate.238 It is
noteworthy that neostigmine is known to cause cramping, nausea, vomiting, and bradycardia, so
the patient should be within a monitored environment when given this medication. It is the
author’s preference to place the patient on a heart monitor, dilute 2 mg of neostigmine with
normal saline for a 5-10 mL overall volume, and administer the drug very slowly to avoid
significant bradycardia. It is also helpful to have atropine at the bedside to counteract clinically
significant bradycardic events.
Colonoscopic decompression of the colon can be used as a primary alternative to neostigmine
in patients with cardiac disease who are deemed unsuitable for the medication, or it can be
employed as a backup for neostigmine failures. Although it is generally safe and well tolerated, it
does little to improve the parasympathetic tone of the colon, and recurrent pseudo-obstruction
occurs in 20%-40% of patients.232,239
Percutaneous interventions for OS. The placement of a decompressive cecostomy tube for
refractory OS is well described, but there is little supporting evidence for its use, and most
studies consist of case reports and small case series. However, most case series report a high rate
of clinical success.240 The benefit of a cecostomy tube compared to surgery is the avoidance of a
general anesthetic. Several approaches have been described, but the cecostomy tube is typically
placed by the interventional radiologist, or is placed endoscopically with a technique similar to
percutaneous endoscopic gastrostomy placement.240 A complication rate of approximately 42%
(mostly due to minor complications) has been reported for this technique, most of which were
minor.205
Surgery for OS. If patients have progressed to perforation, emergent laparotomy is necessary to
remove the perforated colon and wash out the feculent peritonitis. As the cecum is often the site
of perforation, a common approach is cecectomy with creation of an ileostomy. Although
creation of a “venting” mucus fistula is also common, it is not necessary as long as the colon has
been decompressed. Emergency surgery for perforation is associated with a mortality rate of
40%-50%.237
For patients without perforation, surgical intervention is reserved for a failure to improve
with conservative measures. There is surprisingly little literature, or even expert opinion, on the
proper algorithm and surgical approach to OS. However, it is clear that surgery should be
reserved as a last resort. Because of the multifactorial and recurrent nature of OS, the treatment
team must be extremely patient, and not proceed with surgery out of frustration. Using this
approach, surgery is exceptionally rare for OS.
When a patient does require surgery for nonperforated OS, most experts recommend a
“venting stoma” consisting of a cecostomy or loop colostomy.241 Total abdominal colectomy has
also been described, either with an ileostomy or ileorectal anastomosis, but there is no evidence
for this approach, and removal of the entire colon is quite morbid in a patient who is already
very sick. Even when the colon is not perforated, surgical intervention carries a 30% mortality
rate,232,237 so the surgeon should try to be as noninvasive as possible. A laparoscopic approach is
usually feasible, although technically difficult owing to the degree of colonic dilation.
Chronic constipation
Constipation is an extremely common complaint among patients young and old, with a
nationwide prevalence of 2%-27%.242,243 Although this section summarizes the surgeon’s
approach to constipation, 2 excellent resources for further review are the 2007 practice
parameters from the American Society of Colon and Rectal Surgeons (ASCRS)244 and the second
edition of the ASCRS textbook.245
Constipation is more common in women than men, and the incidence increases with
aging.245 The ROME III criteria to define constipation are listed in Table 8, and include straining,
hard stools, and less than 3 bowel movements per week.246
Colonic evaluation for chronic constipation

Once again, a proper evaluation starts with a detailed history and physical examination, as
constipation is affected by diet, work habits, medical problems, operations, and medications. The
surgeon should assess the patient for worrisome symptoms such as an acute change in bowel
habits, anorexia, weight loss, and hematochezia. The physical examination is usually normal, but
patients with more severe disease may demonstrate abdominal distention or tenderness, or
both. A detailed anorectal examination is warranted to evaluate for hard stool in the rectum and
other pathologies of the pelvic floor.
Table 8
ROME III criteria* for functional constipation.
1. Must include 2 or more of the following:

a. Straining during at least 25% of defecations
b. Lumpy or hard stools in at least 25% of defecations
c. Sensation of incomplete evacuation for at least 25% of defecations
d. Sensation of anorectal obstruction/blockage for at least 25% of defecations
e. Manual maneuvers to facilitate at least 25% of defecations (eg, digital evacuation and support of the pelvic floor)
f. Fewer than 3 defecations per week
2. Loose stools are rarely present without the use of laxatives
3. Insufficient criteria for irritable bowel syndrome
n
Criteria fulfilled for the last 3 months with symptom onset at least 6 months before diagnosis.
As constipation is common, and often responds well to medical therapy, extensive diagnostic
studies are not initially warranted. Instead, they are reserved for failures of initial medical
therapy. However, most experts do recommend a colonoscopy for patients with new-onset
constipation to rule out obstructing lesions and inflammatory diseases.245
Empiric medical therapy

For most patients, small changes in lifestyle and nonessential medications can effectively
alleviate constipation. Regular exercise has been shown to relieve constipation in middle-aged
patients.247 As dehydration is often contributory, patients are also counseled on adequate water
intake, and avoidance of excessive alcohol and caffeine, as these both have diuretic effects that
can worsen dehydration. Several diet changes can be implemented, with the most effective
approach being the adoption of a high-fiber diet. It is recommended that patients ingest 25-30 g
of fiber per day. The western diet can often be low in fiber, and patients may have more success
when they supplement their diet with fiber powders. Exercise, increased water intake, and fiber
supplementation are very simple and are effective techniques to improve constipation.
Laxatives. Most pharmacies and grocery stores are stocked with medications advertised to
resolve acute constipation episodes. Many of these prescription and over-the-counter laxatives
exist, and have demonstrated variable success in the long-term management of constipation. A
reasonable approach to these drugs is stepwise implementation, as patients may have variable
responses and side effects. It is best to start with the gentlest agents that carry the lowest risk of
physical dependence.
Docusate sodium is a common and well-tolerated stool softener that acts by lowering the
surface tension at the oil-water interface. Docusate can be used long term with minimal side
effects.245 Mineral oil is another well-tolerated stool softener that coats the stool surface
preventing fluid loss.
Polyethylene glycol is an osmotic laxative that causes water retention in the colon, and it is the
most common osmotic agent recommended for safe, daily use.245 Typical dosing is 17 g mixed with
240 ml of liquid, which can be consumed 1-3 times per day, and titrated for desired effect.
Stimulant laxatives such as senna, bisacodyl, and magnesium citrate are acceptable for short-
term use, but tend to cause dramatic, loose stools leading to intermittent dosing. They can also
lead to melanosis coli and physical dependence. Long-term use of stimulant laxatives should be
avoided.
Over the last few years, there have been dramatic advances in pharmacologic therapy for
constipation. Lubiprostone and linaclotide have been developed as a class of secretagogues
increasing water secretion by stimulating colonic chloride channels. These are effective in
chronic constipation and opioid-induced constipation.
Diagnostic testing
When constipation persists despite adequate medical therapy, the surgeon should proceed
with testing to evaluate for colonic inertia and obstructive defecation. As stated previously,
pelvic floor testing has been extensively covered in Volume 52 of Current Problems in Surgery.235
In general, pelvic floor testing includes anorectal manometry and defecography, and we are not
repeating the interpretation of results in this article.
Sitzmark study. In 1969, Hinton and colleagues248 first described the evaluation of slow-transit
constipation by ingesting radiopaque markers and then obtaining a subsequent abdominal X-ray
to document progression of markers through the colon. At that time, the authors not only looked
at serial abdominal films, but also collected and X-rayed the stool as well, with specially
designed bags to avoid odor during transport.
In modern practice, the stool examination has been omitted, but many variations in the
timing and quantity of ingested markers still exist. One of the simplest approaches is to advise
the patient to continue fiber and water intake, but avoid all laxatives and enemas for 48 hours
before ingestion. Commercially available capsules (Sitzmarks)249 that contain 24 markers are
then consumed. A single abdominal radiograph is obtained 5 days later to assess for the number
and location of residual markers.
If all of the radiopaque markers are absent on X-ray, the patient is unlikely to have colonic
inertia. If there are residual markers concentrated solely in the rectosigmoid area, the patient
most likely has obstructive defecation. If the patient retains more than 20% of the markers (5 of
24), and they are distributed throughout the colon, the patient most likely has colonic inertia.245
Surgical treatment of constipation

When an appropriate evaluation is done, and the patient is compliant with medical therapy,
progression to elective surgery for constipation is very uncommon. It is important to note again
here that surgery for constipation is rarely helpful in the background of untreated or
undertreated pelvic floor dysfunction.
Antegrade enemas. The antegrade continence enema (ACE) was first described in 1990 by
Malone and colleagues250 in a case series of 5 pediatric patients with fecal incontinence. The
same surgical principles have since been applied to adults as well, not only for fecal incontinence
but also for slow-transit constipation.
The technique has gone through several modifications in search of the most functional
continent stoma, beginning with Malone’s appendiceal transection and reimplantation with
appendicocecostomy.250 The modifications that followed were numerous, and are outlined and
referenced in a 2015 systematic review.251 In summary, techniques that employed the
unmodified appendix had issues with reflux of stool and air through the conduit, and those
that constructed a valve had high rates of stenosis and difficult intubations.252 Currently, there is
no consensus, but many surgeons use a laparoscopic approach with either tube cecostomy and
cecopexy253 or delivery of the unmodified appendix through the abdominal wall.254
It should be noted that most ACE series are small, with large variations in reported outcomes.
Despite high rates of reported success, approximately one-third of ACE patients require colectomy
for several reasons, including treatment failures and stoma complications.244,254
Colectomy. Once all conservative measures have been exhausted, colectomy may be considered
for colonic inertia. Historically, surgeons believed the redundant sigmoid colon was to blame for
constipation, and recommended a segmental colectomy to alleviate symptoms. However,
segmental colectomy is associated with failure rates as high as 100%,255-258 and there is no role
for segmental colectomy in the modern treatment of colonic inertia.
The current surgical approach to colonic inertia involves removal of the entire colon.
Semantics are important when describing this technique, as many studies describe a “subtotal”
colectomy when they are truly performing a total abdominal colectomy with ileorectal
anastomosis (TAC-IRA). For the sake of this discussion, a subtotal colectomy (STC) involves
removal of most colon, along with an ileosigmoid anastomosis. STC is tempting because of the
perceived decrease in diarrhea and fecal incontinence when a portion of the sigmoid colon is
spared, but it is unfortunately also associated with very high rates of treatment failure and
recurrent constipation.245 In general, outcomes after STC are inferior to TAC-IRA, and it is
recommended that the entire sigmoid colon be removed.255
Total proctocolectomy with ileoanal pouch has also been described, typically as a salvage
procedure after a failed TAC. The supporting literature for restorative proctocolectomy in the
treatment of constipation is very sparse, in the form of small case series,259,260 and the success
rates remain low, with one-half of the patients ultimately requiring pouch excision in one
series.261 For most patients, removal of the rectum will have a deleterious effect on function with
little to no improvement in symptoms when compared with TAC-IRA. When colectomy fails to
alleviate constipation, the surgeon should focus more on untreated pelvic floor disease than on
the possible benefits of proctectomy.
The technique for TAC-IRA is surgeon dependent, but always involves resection of the entire
colon, typically with a low ligation of major vessels and sparing of the omentum. A stapled or
hand-sewn ileorectal anastomosis can be performed with similar efficacy. For the stapled
technique, the traditional approach is a side-to-end anastomosis, placing the anvil through the
antimesenteric side of the ileum 4 cm proximal to the bowel edge, and then closing the end
portion with a linear staple line. The stapler is then passed through the anus and rectum for a
double-stapled technique. This approach is thought to counteract some of the size mismatch
between the 2 segments of bowel. However, it should be noted that this has been poorly studied,
and alternative techniques including end-to-end and antiperistaltic side-to-side anastomoses
appear to have similar functional outcomes and leak rates.262,263
Total abdominal colectomy can be performed safely in a minimally invasive fashion, either
with a purely laparoscopic approach or a hand-assisted laparoscopic (HAL) approach. A 2008
randomized controlled trial comparing laparoscopic approach to HAL approach for total
colectomy reported similar clinical outcomes and similar length of incisions for the 2 techniques,
but a significantly shorter operative time with the HAL approach (127 7 31 vs 184 7 72
minutes; P ¼ 0.015).264 However, laparoscopic technique and expertise have continued to evolve
over the last 8 years, and it is the author’s opinion that either approach is acceptable based on
surgeon preference.
Outcomes after TAC-IRA are difficult to interpret, as many studies report on a heterogeneous
group of patients, where the indications for surgery are not well defined. The technique is also
not standardized, and the methods for follow-up range from detailed office examinations with
validated scoring systems to mailed questionnaires with poor response rates. Another important
point is that colonic inertia is typically not an isolated diagnosis, and patients may harbor other
functional and psychiatric disorders that can affect success rates for surgery. That being said, the
2 known risk factors for poor outcome after TAC-IRA are coexisting pelvic floor disorders and a
history of sexual trauma.265
Pooled functional outcomes from 32 studies were reported in 1999.255 TAC-IRA is typically
able to alleviate constipation with a median success rate of 86% (range: 39%-100%), and a 9%
median incidence of recurrent constipation (range: 0%-33%).245,255 However, it is unclear as how
many of these patients continue to require laxatives. The rate of fecal incontinence after TAC-IRA
is poorly quantified, but ranges from 0%-52% (median ¼ 14%), with diarrhea reported in 0%-46%
of patients (median ¼ 14%). Persistent abdominal pain was reported in 0%-90% of patients
(median ¼ 41%), and a range of 0%-28% of patients ultimately required an ileostomy owing to
poor function (median ¼ 5%).
Postoperative quality of life scores are typically lower in patients with TAC-IRA compared
with the general population, although preoperative scores for these patients are not available.266
Despite the abovementioned outcomes, a poll of 75 patients reported that 93% would undergo
TAC-IRA again.267
Although TAC-IRA carries the highest success rate for alleviation of colonic inertia, the
outcomes are far from perfect, so the clinician must employ a careful patient selection process,
and ensure that all nonoperative measures have been truly exhausted. In addition, it is essential
that the surgeon have a detailed preoperative discussion with the patient to manage
expectations and outline risks for morbidity and long-term functional compromise.
Sacral neuromodulation. Placement of a sacral nerve stimulator (SNS) is currently one of the
first-line surgical options for urinary and fecal incontinence.268 It involves continuous, low-
amplitude electrical stimulation of the S3 nerve root, with resultant neuromodulation of the
colon and pelvic floor.269
Long-term data are still emerging for patients who undergo placement of a SNS for
constipation, but the treatment shows promise, not only for colonic inertia but also for patients
with coexisting obstructive defecation. Like other surgical treatments for constipation, the key
factor is appropriate patient selection. Two recent case series reported that 48%-58% of patients
who underwent the initial “test” phase of percutaneous nerve evaluation had a favorable
response and underwent permanent device implantation.269,270 Among those patients who
underwent SNS placement, there were significant decreases in constipation scores, and these
results were sustained at a median of 25.6 and 37 months (range: 6-96 and 4-92 months,
respectively). Reported success rates for those with a permanent SNS were 61% and 90%.
Rapid colonic transit
Once again, patient perception of “diarrhea” varies greatly. Rapid small bowel transit has
already been discussed in great detail, so this area would focus on colonic causes of rapid transit.
Acute diarrheal illnesses require supportive care only. Chronic diarrhea is defined by the
American Gastroenterological Association as loose stools with or without increased frequency of
stools for more than 4 weeks.271
Colonic evaluation for chronic diarrhea

Like most disorders, a proper evaluation starts with a detailed history and physical
examination. The astute practitioner can assess for sick contacts, recent travel, past and present
illnesses, prescription and over-the-counter medications, operations, exposure to radiation,
weight loss or deconditioning, and drug and alcohol intake. Physical examination can assess for
signs of dehydration, malnourishment, surgical scars, areas of tenderness, and many other
important clues to the cause of diarrhea. Possible etiologies for chronic diarrhea are extensive,
and are listed in Table 9.
After history and physical examination, routine laboratory work is obtained including
complete blood count, comprehensive metabolic panel, thyroid stimulating hormone, and
erythrocyte sedimentation rate. Stool analysis can determine if there are bacterial or parasitic
infections or both, malabsorption, or GI bleeding. Although its use has been questioned, most
experts still agree that a colonoscopy is also warranted to rule out secretory masses, partial
obstructions, mucosal inflammation, pseudomembranes, and microscopic colitides.272
If patients have received a thorough evaluation without the discovery of a reversible
pathology, empiric medical management is appropriate. The agents employed to slow colonic
transit are similar to those for small bowel pathology, and include fiber supplementation,
loperamide, and tincture of opium. An additional agent with benefit specific to the colon is
cholestyramine, a bile acid sequestrant that counteracts bile malabsorption related to the
dysfunction or surgical absence of the terminal ileum. Cholestyramine binds the bile salts,
allowing them to be secreted in the feces, and preventing the typical effect on colonic sodium
and water excretion.273,274
Surgical management of diarrhea

Surgical options for chronic diarrhea are limited. If the patient has an identifiable cause of the
diarrhea, such as inflammatory bowel disease or ischemic colitis, then surgical removal of the
diseased colon is beneficial. If there are functional tumors elsewhere in the body, such as
gastrinomas and VIPomas, they can also be removed with symptomatic improvement. However,
when there is not a well-defined target for resection, surgeons are left focusing on symptom
control.
Table 9
Common colonic causes of chronic diarrhea.
Irritable bowel syndrome

Infectious colitides
Clostridium difficile colitis
Parasitic infection
Bacterial and viral gastroenteritis
Inflammatory bowel disease

Ischemic colitis
Medications
Diet and lifestyle issues

Malabsorption
Microscopic colitis
Rapid small intestinal transit may be treated with a variety of operations as detailed in the
previous section. However, there are no data supporting valve formation in the colon, and
colonic J-pouches are done as reconstruction after low anterior resection, where they provide
functional improvements but no changes in transit time.275
With an absence of suitable alternatives, the primary surgical treatment of refractory
diarrhea is fecal diversion. This is done for patients with associated fecal incontinence or severe
perianal excoriation where their symptoms are having a significant effect on their quality of life.
If the colon is the known cause of diarrhea, then proximal diversion with a loop ileostomy is a
suitable choice. This bypasses the colon, and allows for easier eventual stoma reversal if
desired.276 If the colon has no known pathology, and a permanent diversion is desired, then an
end sigmoid colostomy is appropriate. Most stomas can be created laparoscopically. It is
noteworthy that regardless of the chosen site of stoma placement, it is essential that the stoma
have a good profile above the skin, as an ileostomy or colostomy that is flattened or retracted
would lead to significant pouching problems in the background of ongoing diarrhea.
Conclusion
Functional disorders of the colon are typically responsive to medical therapy, and surgery for
either slow or fast transit is uncommon. When operative intervention is being considered, the
surgeon must always focus on careful patient selection and preoperative management of patient
expectations. When done correctly, surgery for functional colonic disease carries high rates of
clinical success, although some level of patient dysfunction typically persists.
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Surgical Treatment of Gastrointestinal Motility Disorders

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Current Problems in Surgery 53 (2016) 503–549

Contents lists available at ScienceDirect

Current Problems in Surgery

journal homepage: www.elsevier.com/locate/cpsurg

Surgical treatment of gastrointestinal motility

Esophageal motility disorders

Normal esophageal motility

Barium swallow. The classic ﬁndings of achalasia on an esophagram include a narrow

Esophagogastroduodenoscopy or endoscopy. The main role of EGD in achalasia is to rule out

Achalasia and EGJ outﬂow obstruction

Major disorders of peristalsis (Not encountered in normal subjects)

Minor disorders of peristalsis (Characterized by contractile vigor and contraction pattern)

Normal esophageal motility Not fulﬁlling any of the above classiﬁcations

Injection of Botulinum toxin. Botulinum toxin (BTX) is a presynaptic inhibitor of acetylcholine

Comparison of therapeutic modalities

Scleroderma and connective tissue disorders

Scleroderma is a rare autoimmune connective tissue disorder with multisystemic

Pathophysiology and clinical presentation

Esophagogastroduodenoscopy. EGD is used mainly to evaluate and grade esophageal mucosal

Pharmacologic therapy. Oral pharmacotherapy can be divided into 2 major groups—antisecretory

Hypercontractile esophageal motility disorders

Esophageal motility disorders can be hyperactive or hypoactive. The 2 primary hyperactive

Distal esophageal spasm

colleagues86 in 2010 demonstrated that in a group of 15 patients with dysphagia on chronic

Treatment. Treatment of DES is predominantly with medical therapy. CCB, nitrates,

Gastric motility disorders

Normal gastric motility

Delayed gastric emptying

gastroparesis.99,100 This condition is estimated to occur in approximately 4% of the population;

Surgical therapy of gastroparesis

Autonomic Impaired gastric Antral Duodenal

Failure medical therapy

Gastric bypass Sleeve gastrectomy Pyloroplasty Gastric neurostimulation

Pyloric procedures. Manometric studies in gastroparetic patients have demonstrated prolonged

Rapid gastric transit

Rapid gastric transit is associated with a spectrum of postprandial symptoms referred to as

Surgical therapy for dumping syndrome

Small intestinal motility disorders

Normal intestinal motility

Digestion of nutrients in the small intestine is facilitated by contractile movements of the

Slow intestinal transit

malnutrition. Bacterial overgrowth is a common associated problem. If pain is a dominant

Suspected intestinal pseudo-obstruction

rule out mechanical obstruction

treat underlying disease appropriate operation

localized disease diffuse disease

parenteral nutrition intestinal transplantation

Fig. 3. Management of intestinal pseudo-obstruction.

Rapid intestinal transit

Short bowel syndrome

Intestinal Follow- Morbidity Mortality Liver failure

Colonic motility disorders

Normal colonic motility

Delayed colonic transit

Acute colonic pseudo-obstruction (Ogilvie syndrome)

Colonic evaluation for chronic constipation

1. Must include 2 or more of the following:

Empiric medical therapy

Surgical treatment of constipation

Rapid colonic transit

Colonic evaluation for chronic diarrhea

Surgical management of diarrhea

Irritable bowel syndrome