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TABLE 2 Dysphagia Scoring Scale
https://t.me/med1917
Class Dysphagia Scoring Scale
0 Able to consume normal diet
1 Dysphagia with certain solid foods
2 Able to swallow semisolid foods
3 Able to swallow liquids only
4 Unable to swallow saliva (complete dysphagia)
Data from Ogilvie AL, Dronfield MW, Ferguson R, Atkinson M. Palliative
intubation of oesophagogastric neoplasms at fiberoptic endoscopy. Gut.
1982;23:1060–1067.
esophageal perforations, variceal bleeding, postsurgical anastomotic
leaks, and achalasia has increased by more than 50% in the past
decade.
Malignant Esophageal Disease
Malignant Esophageal Strictures
Malignant esophageal dysphagia results from luminal narrowing
either by intramural growth of primary esophageal tumors or
extrinsic compression from mediastinal and airway tract neoplasms.
Primary EC remains one of the most lethal cancers of the alimentary
tract. Because of its insidious course, clinically apparent disease is
typically synonymous with advanced stage. In these patients, relief
of dysphagia and resumption of oral intake provides an invaluable
improvement in QOL and, in some cases, survival prospects. Since
its inception, ES has been regarded as the first-line therapy for the
palliation of malignant dysphagia. SEMS provides prompt relief
and the opportunity to optimize patients’ nutritional status. Interest
in regionally targeted radiation modalities such as brachytherapy
has transformed the clinical approach to malignant dysphagia. A
randomized controlled trial (RCT) comparing SEMS to single-dose
brachytherapy demonstrated that, although stenting resulted in earlier relief of dysphagia, brachytherapy provided longer-lasting relief,
higher QOL scores, and significantly less morbidity as evidenced by
a rate of major complication of 13% compared with 25% for SEMS.
Despite these findings, the initial enthusiasm for brachytherapy
is fading due to the frequently noted need to insert rescue stents.
Brachytherapy may be considered for carefully selected patients with
mild to moderate dysphagia who have a longer life expectancy. ES
thus remains the first line of intervention for patients with moderate
to severe dysphagia who are not candidates for other modalities or
patients with recurrent dysphagia after brachytherapy. Fully covered SEMS (fc-SEMS) are the stents of choice for the management
of malignant strictures. Recent studies have demonstrated a lower
rate of tumor ingrowth compared with uc-SEMS and a lesser risk
of stent migration when compared with SEPS. Although there is
no difference in outcomes between fc-SEMS and partially covered
SEMS (pc-SEMS), the latter is often subject to tumor ingrowth at
its uncovered distal and proximal ends, thus making for challenging
endoscopic retrieval.
Malignant Esophageal Fistulas
Malignant esophageal fistulas result primarily from esophageal
tumor infiltration into surrounding structures, such as the trachea,
mediastinum, pleura, and proximal abdominal cavity, and less
commonly from extrinsic infiltration of respiratory tract tumors or
chemoradiation-induced tumor necrosis. Regardless of the mechanism, temporary suspension of oral intake, drainage of involved
spaces, and endoscopic insertion of a fc-SEMS are frequently recommended. The Amplatzer atrial septal defect (ASD) occluder has
ESOPHAGUS
FIG. 2 Left to right, Self-expandable plastic stent, partially covered self-ex-
panding metal stent (pc-SEMS), double-layer fully covered SEMS (fc-SEMS).
also been used off-label for closure of trachea-esophageal fistulas in
limited studies. Currently, successful fistula closure rates are more
than 70%, and recent evidence suggests a modest survival advantage
once closure is achieved. The early appeal of concurrent stenting of
the tracheobronchial tract (Fig. 2), or “dual stenting,” has been largely
overshadowed by a higher risk of highly lethal intermural and vascular erosions due to synergistic pressure necrosis. Despite the scarcity
of data available regarding the most appropriate stent for malignant
fistulas, fc-SEMS are the modality of choice by consensus.
67
Gastroesophageal Junction Tumors
Palliation of gastroesophageal junction (GEJ) strictures by endoscopic means is technically challenging, owing to intrinsic anatomic
peculiarities. Unlike the upper and middle esophagus, the GEJ features an acute angle and varying luminal diameters, which may interfere with satisfactory stent fixation. ES at this location is frequently
associated with an increased risk of acid reflux, distal migration,
and ineffective palliation. The higher rate of complications is often
compounded by the overall poorer survival ascribed to GEJ tumors
and has long been a basis for the argument against stenting of these
lesions. In clinical practice, pc-SEMS are considered the stent of
choice for GEJ strictures, as their bare ends allow for better anchorage. The introduction of modified fc-SEMS featuring progressive step
flaring of the distal end (conforms better to the gastric opening) and
one-way reflux valves at the distal end theoretically favors improved
treatment of GEJ lesions. A recent RCT by Persson etal. comparing
modified fc-SEMS with pc-SEMS in 95 patients with malignant GEJ
strictures showed no difference in stent migrations, although there
was a trend toward better relief of dysphagia with fc-SEMS.
Bridge to Surgery
Preoperative ES in patients with locally advanced EC undergoing
neoadjuvant therapy may allow for nutritional optimization before
surgical resection. Although several small studies have demonstrated
improved oral intake, weight stabilization, and QOL when SEMS,
SEPS, or BDS were used as a bridge-to-surgery in a neoadjuvant
setting, a retrospective study by Mariette et al. reported worse
oncologic outcomes with SEMS. A lower rate of complete resection
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(71.0% vs. 85.5%; P = 0.041), shorter disease-free interval (6.5 vs.
9.0 months; P = 0.040), and worse 3-year overall survival (25% vs.
44%; P = 0.023) were noted in the preoperative SEMS group. One
possible explanation resides in the fact that SEMS-induced mural
inflammation may distort the tissue planes and interfere with the
completeness of resection. There is currently no evidence regarding
oncologic outcomes with preoperative stenting during neoadjuvant
therapy for SEPS and BDS. Of note, although stent migration rates
in this setting are upward to 50%, they may represent a favorable
response to neoadjuvant therapy.
Benign Esophageal Disease
Benign Refractory Esophageal Strictures
The mainstay in the treatment of benign esophageal stricture is periodic endoscopic balloon dilation. However, for a subset of patients,
dysphagia will persist despite repeated dilation, thus requiring more
aggressive interventions. RBES are most commonly encountered
after caustic injuries, radiation to the chest or mediastinum, postsurgically after esophagodigestive anastomoses, or after endoscopic
interventions such as per-oral endoscopic myotomy (POEM) and
endoscopic mucosal resection. Although no study has, to date,
evaluated the impact of dilation time on outcomes, proponents of
temporary stenting for RBES purport that continued radial pressure
on the esophageal lumen may result in sustained luminal patency.
The challenge in selecting a stenting modality for RBES resides
in the transient nature of the intervention. The ideal stent needs
to be easily retrievable and yet resistant to migration. To this end,
fc-SEMS are the most commonly used. Although initially showing
great efficacy, Polyflex, the only FDA-approved SEPS for RBES, was
discontinued in the United States owing to a disappointing safety
profile. Its insertion resulted in migration rates up to 50% and a rate
of severe stent-related complication nearing 25%. There are currently
no RCTs evaluating outcomes for various stent designs in the management of RBES. A recent meta-analysis by Thomas etal. examined
the performance of fc-SEMS in 199 patients with RBES. The efficacy
of fc-SEMS for palliation was 46.2% with a migration rate of 26.4%
and a successful retrieval rate of 87%. BDS has shown similar efficacy
to fc-SEMS and has the added advantage of not requiring retrieval.
However, BDS has a higher rate of stent-related complications such
a retrosternal pain and bleeding. In brief, ES with either fc-SEMS or
BDS is an intervention of last resort in benign esophageal strictures,
reserved for carefully selected patients with RBES.
FIG. 3 Acquired tracheoesophageal fistula. (Courtesy Dr. Richard K. Freeman, MD.)
involved space, broad systemic antibiotic therapy, intravenous fluid
administration, and temporary diversion of oral secretions are often
employed to optimize clinical outcomes.
Achalasia
The role of ES in the treatment of achalasia is evolving. Over the last
3 decades, the introduction of temporary stenting using retrievable
SEMS has renewed interest in this modality. In 2010, a long-term follow-up of a prospective comparison of pneumatic dilation and SEMS
of various diameters in 120 patients with achalasia showed a higher
clinical remission rate at 13 years (83.3% vs. 0) when 30-mm SEMS
were used compared with pneumatic dilation. Although surgical and
endoscopic interventions such as Heller myotomy and POEM are the
mainstays of definitive therapy for achalasia, temporary retrievable
stenting with SEMS may be an option for patients who are not surgical candidates (Fig. 5).
Benign Perforations, Anastomotic Leaks, and Fistulas
Benign esophageal perforations (BEP) can be classified as spontaneous, such as in Boerhaave’s syndrome, iatrogenic, or traumatic.
As for fistulas, contamination of surrounding mediastinal or pleural
spaces by esophageal luminal contents can have dire consequences,
triggering highly lethal inflammation and infection. Early determination of the etiology and severity of the leakage is crucial, as these
considerations will guide therapeutic management and determine
outcomes. A contained perforation or anastomotic leak in an otherwise clinically stable patient may be successfully treated with conservative measures. In these patients, nil per os, intravenous hydration,
nasogastric drainage, and broad-spectrum antibiotics are the mainstays of therapy. In patients with noncontained leaks, ES may allay
the potential morbidity of surgical intervention and is now increasingly used (Figs. 3 and 4). As with traditional surgical approaches,
the goal of therapy in using stents in these patients remains complete
drainage of the contaminated spaces, successful sealing of the leak,
diversion of oral secretions, and prevention of widespread infection.
In a recent meta-analysis comparing SEMS to surgical intervention
in patients with esophageal leakage, Persson etal. reported a higher
success rate (88% vs. 83%) and a lower mortality rate (7.5% vs.
17%) when SEMS were used. We recommend the use of covered
stents—either fc-SEMS or pc-SEMS—for the management of hemodynamically stable patients with BEP. Concurrent drainage of the
Refractory Esophageal Variceal Bleeding
Fully covered SEMS may be used for refractory esophageal variceal
bleeding. There are specially designed SEMS with a wide diameter
(at least 25 mm) for this indication. There are multiple small studies that have been published with encouraging results. In a recent
meta-analysis, technical success was achieved in 97% of cases and
bleeding was controlled in 96%. However, one small trial comparing
SEMS to balloon tamponade showed no difference in survival. All
studies performed for this indication have reported adverse events
that include stent migration and ulceration.
PROCEDURE
Meticulous planning is necessary when considering ES. Although
both conscious sedation and general anesthesia are comparable, the
latter would best serve a patient at high risk for aspiration. Preprocedural endoscopic evaluation of the lesion should be comprehensive.
The luminal diameter, location, and size of the lesion of interest
must be carefully assessed and documented. Evaluating the friability of the luminal tissue is equally important, as it could portend
a greater risk than usual of bleeding, pressure necrosis, or mural
erosion after the procedure. Endoscopic predilation of the lesion
is no longer routinely performed but could be considered for the
small subset of patients in whom the severity of the stenosis might
ESOPHAGUS
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69
AB
FIG. 4 (A) Upper gastrointestinal (GI) series with water-soluble oral contrast medium demonstrating extraluminal extravasation of contrast medium from
the left side of the esophagus approximately 2 cm below the level of the carina, consistent with esophageal perforation. (B) Repeat upper GI series with
water-soluble oral contrast medium in the same patient as in (A) demonstrates a distal esophageal stent in good position. There is no evidence of contrast
medium extravasation to suggest a persistent leak.
A B
FIG. 5 Endoscopic view of deployed and expanded self-expanding metal stent (A) and self-expandable plastic stent (B).
prevent stent insertion. Particular attention should be paid to stent
size. The internal diameter of the stent should be slightly larger than
the lesion of interest, thus allowing for adequate radial force to be
applied. An inadequately small stent diameter may increase the risk
of migration, whereas an oversized stent may lead to perforation and
excessive pressure necrosis. The length of a stent should be at least
4 cm longer than that of the lesion, overlapping it both proximally
and distally by at least 2 cm. However, the location of an esophageal
obstruction may necessitate modification to the general technique
for stent deployment. Most notably, stent placement in the proximal
esophagus is known to be challenging. In patients with esophageal
strictures near the upper esophageal sphincter (UES), the placement
is adjusted so that the proximal margin of the stent is below the UES
(i.e., the proximal stent margin may be <2 cm). Stent placement in
the distal esophagus or across the GEJ may also be challenging. In
these instances, it is important to avoid an excessively long stent so
that the distal margin of the stent does not result in contact with the
contralateral gastric wall and lead to ulceration or perforation.
ES can be achieved using either fluoroscopic guidance, endoscopic guidance, or both. One technique is the Seldinger technique,
in which a guidewire is endoscopically advanced into the esophagus
2 to 3 cm past the distal end of the lesion, the endoscope is removed,
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and the stent delivery system is advanced over the guidewire. The
endoscope is then reinserted, and the stent positioned and deployed
under direct visualization. Caution should be used to ensure that
the stent is not fully deployed until satisfactory positioning has been
achieved. Using fluoroscopy, the guidewire is inserted using a catheter. Once a satisfactory position has been secured, a small amount
of water-soluble contrast medium is injected through the catheter to
delineate the stricture. Radiopaque markers are then used to delineate the location and length of the stricture on the patient’s skin.
Next, after the catheter is removed, the delivery system is advanced
over the guidewire, and the stent is deployed under continuous fluoroscopic monitoring.
The development and increased availability of through-the-scope
(TTS) SEMS has allowed for this technique to be performed by
advancing the stent catheter under direct visualization through the
accessory channel of a therapeutic double channel upper endoscope.
After deployment, the stent expands against the stricture and
the mucosa at the margins of the stricture. This anchors the stent
in place and helps prevent stent migration. Repeat endoscopy or an
upper gastrointestinal series 24 to 36 hours postdeployment is often
performed before resumption of oral intake. The stent is expected
to completely expand within 48 to 72 hours. When oral intake is
resumed, patients are discharged with written diet instructions based
on results of stent placement and the expected gradual expansion of
the stent. Patients are typically advised to consume liquids only for
24 to 48 hours and then to advance to a soft solid diet as tolerated, as
well as to adhere to long-term avoidance of fibrous foods such as raw
vegetables and large pieces of meat.
COMPLICATIONS
Close to 30% of patients undergoing an esophageal stent placement
experience complications (Table 3). These events are often classified
as early or late (Table 4). Events of mild to moderate severity such
as retrosternal pain or reflux symptoms typically resolve within 1
or 2 weeks postprocedure. Persistence of retrosternal pain beyond
2 weeks may indicate the need for endoscopic removal of the stent.
Recent studies have shown improvement of reflux symptoms with
TABLE 3 Overview of Adverse Events Related
to Esophageal Stent Placement for Different
Indications
Malignant
Dysphagia
Complications
(n = 1017)
SERIOUS ADVERSE EVENT (%)
Major bleeding 8.0 3.0 1.3
Aspiration pneumonia 5.0 1.3 0.7
Perforation 2.0 1.3 1.0
ADVERSE EVENTS (%)
Retrosternal pain 30.0 4.3 0.5
Reflux symptoms 7.0 2.6 0.5
Recurrent dysphagia 31.0 29.0 20.0
Stent migration 11.0 24.5 16.5
Tissue ingrowth/overgrowth 14.0 2.2 2.7
Food obstruction 7.0 2.2 1.1
Modified from Vermeulen BD, Siersema PD. Esophageal stenting in clinical
practice: an overview. Curr Treat Options Gastroenterol. 2018;16:260–273.
Benign
Dysphagia
(n = 232)
Esophageal
Leakage
(n = 599)
TABLE 4 Common Early and Late Complications
After Esophageal Stent Placement
Early Complications
(<2 weeks), 10%–20%
Retrosternal pain
Minor hemorrhage
Aspiration
Gastroesophageal reflux
disease
Migration
Occlusion
Airway erosion/compression
Vascular erosion
Modified from Vermeulen BD, Siersema PD. Esophageal stenting in clinical
practice: an overview. Curr Treat Options Gastroenterol. 2018;16:260–273.
the use of stents equipped with a distal reflux valve, especially in
patients with GEJ lesions. Other measures to prevent acid reflux and
aspiration of gastric contents include maintaining an upright (or
semi-upright) position at all times and a daily proton pump inhibitor.
Stent occlusion secondary to tumor in-growth is most commonly
seen with uncovered or partially covered stents but can now be
successfully managed with a stent-in-stent technique (Fig. 6). This
chapter focuses on the major complications of stent migration and
vascular and aerodigestive erosions.
Late Complications
(>2weeks), 15%–37%
Migration
Stent fracture
Obstruction/tumor ingrowth
Erosion
Airway erosion/compression
Vascular erosion
Tissue Erosion
Stent erosion into neighboring mediastinal structures such as the
big vessels and airway is rare but highly lethal when it occurs. Tissue
necrosis resulting from high radial pressure exerted on the esophageal wall is often compounded by poor tissue perfusion and impaired
wound healing secondary to malnutrition or radiation exposure.
Some studies have shown that a history of radiation to the chest or
mediastinum increases the risk of tissue erosion from ES. Similarly,
dual stenting of the esophagus and trachea is also associated with an
increased risk of tissue erosion.
Vascular Erosion
Vascular erosion is rare but highly lethal. Although aortoesophageal
fistula is perhaps the most common, vascular erosion involving the
retroesophageal subclavian and common carotid arteries have been
reported. One must always have a high degree of suspicion as to its
possibility. For example, an abrupt sentinel hematemesis associated
with retrosternal chest pain in a patient with a history of esophageal
stent placement should prompt immediate evaluation and intervention before a potential catastrophic exsanguination. Emergent management of an aortoesophageal fistula involves prompt control of
the potentially fatal hemorrhage, followed by timely vascular repair
(Fig. 7). Concurrent activation of the massive transfusion protocol
and airway protection may be necessary. In such an emergent setting,
endoscopic balloons can often be used to control the bleed, and some
surgeons have even successfully used Sengstaken-Blakemore tubes
for this purpose. Temporary intravascular occlusion and emergent
surgery are also options. Once control is obtained, it can be followed
with endovascular repair of the aortic defect. After the patient is
stabilized, attention can then be turned to the esophageal defect.
After removal of the offending stent, a small defect may be primarily repaired with muscle interposition. An esophagectomy may be
necessary for larger defects. Alternatively, in moribund patients, a
conservative approach involving diversion of esophageal contents
and palliative care may be more appropriate.
ESOPHAGUS
AB
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71
A
C
B
FIG. 6 (A) Endoscopic view of a fractured self-expanding metal stent
(SEMS) with associated ingrowth of esophageal mucosa. (B) Interval
placement of an SEMS within the lumen of the preexisting, fractured
SEMS to facilitate removal. (C) Successful extraction of both fractured
SEMS (top) and intact SEMS (bottom).
FIG. 7 Thoracic endovascular aortic repair of aortic defect after an aortoesophageal fistula. (A) Circled area shows contrast medium leaking from the
aortic defect. (B) Arch aortogram showing the endovascular stent graft. (From Liang H, Chen C, Liu W, Yu F. Definitive treatment for aortoesophageal fistula by
endovascular stent graft. Indian J Surg. 2016;78:151–154.)
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Aerodigestive Erosion
Tracheoesophageal erosions have been reported in 4% to 8% of
patients after ES (see Fig. 4). Unlike the dramatic presentation seen
with vascular erosion, patients often present with nonspecific symptoms such as dyspnea and frequent pulmonary infections. Although a
chest CT scan may help identify the lesion of interest, bronchoscopic
and endoscopic evaluations are often necessary to characterize the
extent and size of the defect. The management of tracheoesophageal
fistulas depends on the patient’s hemodynamic status and on the
underlying pathology that led to esophageal stricture. In a patient
who presents in extremis, securing the airway must be the priority.
One should maintain spontaneous ventilation if possible, but the
patient should be promptly intubated if there is an inability to ventilate. A variety of intubation methods can be used if ventilation is
difficult including contralateral mainstem intubation, use of a double
FIG. 8 Endoscopic view of an esophageal clip that stabilizes the proximal
aspect of an esophageal stent.
FIG. 9 Endoscopic view of the Apollo Overstitch device used for suture
fixation of the proximal aspect of an esophageal stent to the esophageal
mucosa.
FIG. 10 Comparison of the Apollo Overstitch device appearance at the distal end of the dual channel endoscope (left) and the Apollo Overstitch Sx device
appearance at the distal end of the single channel endoscope (right).
ESOPHAGUS
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73
lumen tube, employing a bronchial blocker, or sedating the patient to
ventilate. In some cases, tracheal stenting and esophageal diversion
may be needed, and definitive management may be delayed. Stable
patients with good functional status and with good esophageal tissue
integrity can undergo either a primary repair with muscle interposition or an esophagectomy. Otherwise, tracheal stenting, esophageal
diversion, and palliative care should be considered.
Stent Migration
Stent migration is perhaps the most common complication of esophageal stricture, occurring in 11% to 33% of cases. Over the past 2
decades, various interventions aimed at reducing the frequency of
migrations, such as anchoring the stent to the esophageal wall, have
Axios
(Boston Scientific,
Natick, MA, United
States)
Spaxus
(Taewoong
Medical, Gimposi,
South Korea)
Nagi
(Taewoong
Medical, Gimposi,
South Korea)
been introduced. Endoclips are used to secure the proximal stent
end to the esophageal wall, but a high migration rate persists (Fig. 8).
Endoscopic suturing using Apollo OverStitch and Apollo OverStitch
Sx devices (Figs. 9 and 10) has also shown some benefit in small studies; however, high-quality evidence is not available. More recently,
a specialized over-the-scope clip (OTSC) has become available for
stent fixation with promising results. Small studies indicate reduced
migration rates that, however, still exceed 15%. Additionally, off-label use of lumen-apposing metal stent (LAMS) has been studied for
management of short-segment benign gastrointestinal strictures.
Their unique dumbbell-shaped design helps to fix the stent in place
(Fig. 11). LAMS is an alternative to traditional options; however, data
are limited and future studies are needed to measure their efficacy.
The best treatment of migration is probably prevention. A study
Aixstent
(Leufen Medical,
Berlin, Germany)
Hanarostent Plumber
(M.I Tech, Pyeongtaek-si,
South Korea)
Stents
Availability in
United States
Flange diameter
(mm)
Length (mm)
Lumen diameter
(mm)
Delivery catheter
(Fr)
Material
Deployment
through-the-scope
Delivery in single
step
FIG. 11 List of currently available LAMS with representative images. From Sharma P, McCarty TR, Chhoda A, et al. Alternative uses of lumen apposing metal stents.
World J Gastroenterol. 2020;26(21):2715–2728.
Yes
21, 24, 29
10
10, 15, 20
10.8
Silicone-covered
Nitinol-braided stent
Yes
Yes
No
23, 25, 31
20
8, 10, 16
10
Silicone-covered
Nitinol-stent
Yes
No
No
20, 25, 31
10, 20, 30
10, 12, 14, 16
9, 10
Silicone-covered
Nitinol-stent
Yes
No
No
25
30
10, 15
10
Silicone-covered
Nitinol-stent
Yes
No
No
22 to 28
10, 30
10, 12, 14, 16
10.5
Silicone-covered
Nitinol-stent
Yes
No
74 MANAGEMENT OF ESOPHAGEAL PERFORATION
S u g g e S t e d R e a d i n g S
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TABLE 5 Rates of Complication Based on Stent Indwelling Time in the Management of Esophageal Leaks
and Perforations
Anastomotic Leak Perforation
<2 Weeks >2 Weeks P Value <4 Weeks >4 Weeks P Value
Number 29 16 96 21
Migration 4 (14%) 7 (44%) 0.04 9 (9%) 9 (43%) 0.0007
Dysphagia 5 (17%) 8 (50%) 0.04 4 (4%) 6 (29%) 0.0022
Hemorrhage 0 1 (6%) 0.4 0 2 (10%) 0.03
Stent fracture 3 (10%) 6 (38%) 0.05 5 (5%) 7 (33%) 0.001
Airway
compromise
Data from Freeman RK, Ascioti AJ, Dake M, Mahidhara RS. An assessment of the optimal time for removal of esophageal stents used in the treatment of an
esophageal anastomotic leak or perforation. Ann Thorac Surg. 2015;100:422–428.
1 (3%) 2 (13%) 0.3 3 (3%) 2 (10%) 0.2
by Freeman et al. evaluating the impact of stent indwelling time
in 162 patients treated with ES showed a significant reduction in
stent-related complications in patients who had a shorter indwelling
time (Table 5). In patients with anastomotic leaks, a 30% (P
reduction in stent migration rates was noted with a stent duration
of fewer than 14 days. Patients with acute perforation experienced
a 34% reduction in stent migration when the indwelling time was
fewer than 28 days.
= 0.04)
CONCLUSION
ES remains a mainstay in the palliation of advanced malignant
strictures. Although its indications have greatly expanded to include
various benign esophageal diseases, complications associated with
this procedure mandate thorough preprocedural evaluation of the
patients as well as an individualized therapeutic approach.
Management of
Esophageal Perforation
Juan A. Muñoz-Largacha, MD, and James M. Donahue, MD
INTRODUCTION
Despite advances in diagnostic imaging and early recognition,
esophageal perforation continues to be a life-threatening condition
with high morbidity and mortality rates. Reported mortality ranges
between 10% and 25% when diagnosis and treatment are initiated
within 24 hours and can be as high as 40% to 60% when diagnosis
and treatment are delayed. Other patient-related factors such as
comorbidities, functional condition, presence of sepsis and multiorgan failure, and location and cause of perforation can also influence
mortality in this group of patients.
Anderson M, Sippey M. Endoscopic stent placement: indications and success
rates. Annals of Laparoscopic and Endoscopic Surgery. 2019;4:55.
Jacobson BC, Hirota W, Baron TH, Leighton JA, Faigel DO. The role of
endoscopy in the assessment and treatment of esophageal cancer.
Standards of Practice Committee. American Society for Gastrointestinal
Endoscopy. Gastrointest Endosc. 2003;57(7):817.
Kim KY, Tsauo J, Song H-Y, Kim PH, Park J-H. Self-expandable metallic
stent placement for the palliation of esophageal cancer. J Korean Med Sci.
2017;32:1062–1071.
Knyrim K, Wagner HJ, Bethge N, Keymling M, Vakil N. A controlled trial of
an expansile metal stent for palliation of esophageal obstruction due to
inoperable cancer. N Engl J Med. 1993;329:1302–1307.
Sharma P, Kozarek R. Role of esophageal stents in benign and malig-
nant diseases. Practice Parameters Committee of American College of
Gastroenterology. Am J Gastroenterol. 2010;105(2):258.
Vermeulen BD, Siersema PD. Esophageal stenting in clinical practice: an
overview. Curr Treat Options Gastroenterol. 2018;16:260–273.
Iatrogenic injuries resulting from endoscopic procedures (diagnostic, biopsy, dilation, sclerotherapy, laser therapy, photodynamic
therapy, stent placement, transesophageal echocardiogram, esophageal ultrasound) are the most common cause of esophageal perforation, accounting for 60% to 70% of cases. Spontaneous rupture, or
Boerhaave’s esophagus, is the second most common cause of esophageal perforation (15%–30%). Other, less frequent causes include
trauma (penetrating or blunt), foreign body or caustic ingestion, cervical, nonesophageal surgery (thyroidectomy, mediastinoscopy, spine
surgery), and malignancy of the esophagus, lung, or other mediastinal
structures. Esophageal perforation has also been described in association with infectious processes in immunocompromised patients
(candida, herpes, syphilis, and tuberculosis), in the setting of severe
gastroesophageal reflux, and following Mallory-Weiss tears (Table 1).
DIAGNOSIS
A prompt diagnosis of esophageal perforation is crucial to improve
outcomes and survival, and a high level of suspicion should be
TABLE 1 Causes of Esophageal Perforation
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Iatrogenic Endoscopic procedures (diagnostic, biopsy,
dilation, stent placement)
Transesophageal echocardiography
Esophageal ultrasonography
Spontaneous Barogenic-Boerhaave syndrome
Traumatic Blunt
Penetrating
Surgical intervention: cervical intervention or
mediastinal surgery (i.e., thyroidectomy)
Ingestion Caustic
Foreign body
Neoplastic Primary esophageal malignancy
Mediastinal malignancy with esophageal
involvement and erosion
Infectious Candida, herpes, syphilis, tuberculosis
ESOPHAGUS
FIG. 1 Esophagram with water-soluble contrast. Evidence of esophageal
perforation and contrast extravasation in the right posterolateral aspect of
the mid-esophagus (red arrows).
75
maintained to achieve this goal. A detailed history and physical
examination are of utmost importance and will help determine the
most likely cause of perforation. Commonly, patients will report
recent esophageal instrumentation or forceful vomiting. Initial complaints often involve a vague description of pain localized to the neck,
chest, shoulder, or epigastrium. As the process progresses, patients
may experience dyspnea, dysphagia, or odynophagia. On physical
examination, patients may remain hemodynamically stable or present with signs of septic shock or mediastinitis such as tachycardia
or hypotension. Laboratory values generally reveal a leukocytosis.
Commonly, a chest x-ray is performed as part of the initial assessment of these patients, which may reveal presence of pneumomediastinum, pleural effusion, pneumothorax, subcutaneous emphysema,
or an abnormal cardiomediastinal contour. When esophageal perforation is on the differential diagnosis, further imaging studies such as
contrast esophagram and computed tomography (CT) are performed
for better evaluation of the extent of the injury, location, and degree
of mediastinal and pleural contamination.
Esophagram
An esophagram is a noninvasive diagnostic tool that can help determine the presence and location of a perforation. Either water-soluble
contrast (Gastrografin) or diluted barium can be used. Gastrografin
is generally preferred over barium because of the lower risk of mediastinitis. However, the sensitivity of Gastrografin has been reported
between 60% and 70%, so negative results do not completely exclude
perforation, especially in the cervical esophagus, because of the rapid
transit of the contrast. In addition, barium has been shown to have
better resolution for differentiating between contained and noncontained perforations. In practice, a Gastrografin esophagram is often
obtained first, and if inconclusive, repeat images with diluted barium
can be obtained as necessary for improved sensitivity and specificity.
If Gastrografin demonstrates free perforation into the pleural space
or abdominal cavity, the procedure is terminated. Figure 1 shows
evidence of esophageal perforation on a Gastrografin esophagram.
Computed Tomography
CT is readily available and provides important supportive information
related to an esophageal perforation, such as differentiating between a
contained versus noncontained perforation. A contained perforation
is characterized by no or minimal extravasation of contrast at the site
of perforation without evidence of pleural or peritoneal contamination, or by contrast drainage back into the esophagus. In addition, CT
allows for evaluation of the location of injury as well as the severity of
mediastinal, pleural, or peritoneal contamination if present. Figure 2
shows evidence of esophageal perforation on chest CT.
Computed Tomography Esophagography
CT esophagography is a more recently available option with the
potential to improve workflow and expedite the diagnosis of esophageal perforations by reducing the need for two radiologic examinations. Multiple reports over the past decade have shown its utility for
the evaluation of postoperative leaks after esophagectomy; however,
its use in the setting of acute esophageal perforations must be clarified. This technique is generally readily available, is less cumbersome
for the patient, and does not require a fluoroscopy suite, an additional technician, and a supervising radiologist. One of the main limitations of a fluoroscopic esophagram is that critically ill patients may
not be able to swallow the contrast or stand and change positions,
which are requisite parts of the examination. In CT esophagography,
noncontrast images are obtained first. For patients who cannot swallow, this allows for repositioning of a nasogastric tube (NGT) into
the proximal esophagus to allow for contrast administration. These
precontrast images are also useful to identify any radiopaque structures (i.e., staples, calcifications) that could be confounded with the
contrast in subsequent images. Then, oral contrast is either ingested
or administered via the NGT, and supine images are obtained. The
reported sensitivity and specificity of this technique are comparable to those for fluoroscopic esophagography, with an equal, if not
superior, accuracy for the diagnosis of acute esophageal perforations.
In practice, a fluoroscopic esophagram is often obtained following a
negative CT esophagram before enteral nutrition is implemented.
Endoscopy
Endoscopy is another helpful diagnostic and potentially therapeutic
tool for management of esophageal perforations. Its use should be
considered in patients with high suspicion for perforation who could
not undergo a swallow examination or underwent a nondiagnostic
study. It allows for direct visualization and careful inspection of
mucosal integrity and viability as well as characterization of the
76 MANAGEMENT OF ESOPHAGEAL PERFORATION
AB
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FIG. 2 Chest CT with contrast. (A) Patient with spontaneous distal esophageal perforation with pneumomediastinum and periesophageal fluid with con-
trast extravasation (red arrow) and bilateral small pleural effusions (asterisks). (B) Large periesophageal fluid collection (red arrow) with associated pneumo-
mediastinum and small right pleural effusion (asterisk).
anatomic location of the injury and identification of other pathologic
changes or underlying diseases of the esophagus such as strictures or
neoplasms. Careful inspection with minimal air insufflation should
be performed because this could potentially increase the size of the
perforation or cause a tension pneumothorax through a free perforation into the pleural space. As discussed later, endoscopy can also be
used for therapeutic purposes, as a stent may be placed in the same
setting as the diagnostic procedure.
Assessing Severity of Illness and Prognosis
Several illness severity scoring systems, such as the APACHE system, have been applied to patients with esophageal perforation with
various degrees of accuracy. The Pittsburgh Severity Score (PSS)
is a scoring system specially devised for esophageal perforation
patients that was first described by Abbas et al. in 2009. The authors
attempted to identify clinical factors that could predict survival and
potentially identify those patients amenable to nonoperative management. The score includes 10 clinical variables that were considered by the authors to be potentially important indicators of injury
severity and patient outcomes, which are assigned a value from 1 to 3
(Table 2). In their report, all survivors had PSS ≤3.3, and all patients
who died had a PSS ≥6.5. Lower PSS scores were also associated
with successful nonoperative management, shorter length of stay,
and decreased morbidity. Similar findings have been found in other,
independent cohorts. The PSS is a useful tool in terms of estimating
prognosis for patients with esophageal perforation, but its validation
with the use of prospective studies and potential improvement with
more rigorous analysis of clinical data are still needed to definitively
guide management.
MANAGEMENT
The management of patients with confirmed esophageal perforation
after appropriate diagnostic workup can be determined by clinical
and imaging characteristics. In a select group of patients with contained perforations, a nonoperative approach may be appropriate.
For patients with free perforations, operative management with open
primary repair of the perforation has been the traditionally preferred
approach, particularly when the repair can be undertaken within 24
hours of the perforation. Recent evidence has shown that a less invasive approach consisting of endoscopic stenting and thoracoscopic
mediastinal and pleural drainage produces encouraging results. In
TABLE 2 Pittsburgh Severity Score
Variables Points Assigned
• Age>75years
1
• Tachycardia(>100bpm)
• Leukocytosis(>10.000WBC/mL)
• Pleuraleffusiononimaging*
• Fever(>38.5°C)
• Noncontainedperforation
†
2
• Respiratorydistress(>O2 require-
ments, RR >30, need for mechanical
ventilation)
• Timetodiagnosis>24hours
• Hypotension
3
• Malignancy
*Chest x-ray, esophagram, or chest CT.
†
Esophagram or chest CT.
bpm, Beats per minute; RR, respiratory rate; WBC, white blood cells.
the most dire situations, esophageal resection and exclusion may
be necessary. Regardless of the therapeutic approach chosen, initial
management of patients with esophageal perforation should include
close monitoring in a surgical critical care unit. Patients should be
maintained nil per os (NPO), appropriately resuscitated with intravenous fluid, and started on broad-spectrum antibiotics to include
fungal coverage. Adequate intravenous analgesia and a proton pump
inhibitor (PPI) should be started, and early total parenteral nutritional support can be considered if a prolonged course is expected.
Nonoperative Management
Criteria for nonoperative management were initially described by
Cameron et al. in 1979 and further modified by Altorjay. These
included early diagnosis, contained perforations or perforations
draining back into the esophagus, no significant mediastinal contamination, absence of sepsis or multiorgan failure, and perforation
not associated with malignancy or obstruction of the esophagus. If
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