REVIEW

International Journal of Surgery 9 (2011) 204e208

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International Journal of Surgery

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Review

Oesophagectomy in the management of end-stage achalasia e Case reports

and a review of the literature
Julia M. Howard, Laura Ryan, Kheng T. Lim, John V. Reynolds*
Department of Surgery, St. James’s Hospital, Dublin 8, Ireland

a r t i c l e i n f o a b s t r a c t

Article history: Achalasia is an oesophageal motility disorder characterised by aperistalsis and failure of relaxation of
Received 24 July 2010 a hypertensive lower oesophageal sphincter. Treatment intent targets the sphincter, and either Heller’s
Received in revised form myotomy or pneumatic dilatation successfully relieves dysphagia in the majority of cases. End-stage
4 November 2010
achalasia, typified by a massively dilated and tortuous oesophagus, may occur in patients previously
Accepted 17 November 2010
Available online 25 November 2010
treated but where further dilatation or myotomy fails to relieve dysphagia or prevent nutritional dete-
rioration, and oesophagectomy may be the only option. We describe two patients with end-stage
achalasia and nutritional failure despite exhaustive conventional therapy including pneumatic dilatation
Keywords:
Achalasia
and surgical myotomy. Both patients were successfully managed with transhiatal oesophagectomy and
Oesophagectomy cervical gastro-esophageal anastomosis, with excellent symptomatic control and improved quality of life.
Mega-oesophagus These cases are discussed and the literature reviewed.
Ó 2010 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.

1. Introduction therapy is inadequate, progressive dilatation and increasing tortu-

Achalasia is a primary motility disorder of the oesophagus
characterised by loss of peristalsis in the oesophageal body,
impaired relaxation of the lower oesophageal sphincter (LOS)
during swallowing, and increased resting pressure of the LOS.1It
results from loss of ganglion cells in the myenteric plexus, but the
precise aetiology remains unknown.1 Common symptoms include
dysphagia, regurgitation and weight loss, and occasionally respi-
ratory symptoms due to aspiration of oesophageal content.2
Achalasia is a rare disease, with an estimated annual incidence
of 0.5 cases per 100,000 people, and a prevalence of 8 cases per
100,000 people per year.3 No treatment is available that will restore
oesophageal peristalsis and normalise LOS relaxation. Therefore,
treatment aims to palliate rather than cure, through reducing the
pressure gradient across the LOS to facilitate and improve gravita-
tional oesophageal emptying.4
The two most commonly used treatment modalities for acha-
lasia are pneumatic dilatation (PD) and surgical myotomy.4,5
Achalasia represents a wide spectrum of disease severity, and
while some patients achieve excellent long-term symptom control
from a single PD, other patients will go on to require multiple
interventions to control symptoms. In the absence of therapy, or if
* Corresponding author. Tel.: þ353 1 608 2189; fax: þ353 1 454 6534.
E-mail addresses: drjuliahoward@gmail.com (J.M. Howard), lauraryan1982@
yahoo.co.uk (L. Ryan), ktianlim@rcsi.ie (K.T. Lim), reynoljv@tcd.ie (J.V. Reynolds).
osity of the oesophagus may occur, resulting in end-stage disease.6
End stage disease, characterised by a markedly dilated and tortuous
“burned- out” oesophagus and recurrent obstructive symptoms,
may require oesophageal resection in order to restore gastro-
intestinal function, reverse nutritional deficits and reduce the risk
of aspiration pneumonia.6e8
In this report, we aimed to examine our own experience in the
management of patients with end-stage achalasia and to review
the literature available on the use of oesophagectomy in the
management of end-stage achalasia.
2. Cases
In this Unit, 70 patients have been managed with achalasia
between 1998 and 2007.9The following two cases (3.5%) underwent
oesophagectomy for end-stage achalasia.
2.1. Case 1
A 57 year old female who had a 39 year history of achalasia
was referred to our unit with severe dysphagia, weight loss and
recurrent aspiration pneumonia. She had undergone a trans-
thoracic Heller’s myotomy at the time of her diagnosis, with
good symptom control for 25 years. Following symptom recur-
rence she was managed with three pneumatic dilatations using
the Rigiflex balloon (3e3.5 cm), all of which provided only
temporary control of symptoms. At this time, she was referred

1743-9191/$ e see front matter Ó 2010 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.ijsu.2010.11.010
 REVIEW
J.M. Howard et al. / International Journal of Surgery 9 (2011) 204e208
to our specialist oesophageal surgery unit for further manage-
ment. At the time of referral her BMI was 22, and she required
naso-gastric feeding due to hypo-albuminemia (19 g/L) and
nutritional failure.
Initial investigations included a timed barium swallow, a CT
thorax and an endoscopy. The barium study revealed a markedly
dilated oesophagus with food debris, a tight oesophago-gastric
junction and markedly delayed emptying of the oesophagus.
Endoscopy confirmed a fluid-filled mega-oesophagus. The CT scan
demonstrated the mega-oesophagus and signs of aspiration
pneumonitis (Fig. 1). At this centre we performed a single pneu-
matic dilatation with a Rigiflex balloon (3.5 cm). This failed, and we
elected to do a laparoscopic abdominal Heller’s myotomy with
a 6 cm oesophageal myotomy with gastric extension for 3 cm.
Manometry was performed and demonstrated aperistalsis of
the oesophageal body. The patient failed to obtain even temporary
symptom control post-operatively, and so decision was made to
perform oesophageal resection.
A transhiatal oesophagectomy was carried out via abdominal
midline and left neck incisions with blunt mobilisation of the
mega-oesophagus, gastric pull-up and a cervical anastomosis.
Despite moderate peri-oesophageal adhesions, there were no
significant intra-operative difficulties, and a transhiatal approach
was safely performed. The approximate intra-operative blood loss
was 450 ml.
Routine post-oesophagectomy care was provided, including
naso-gastric tube drainage, epidural and patient controlled anal-
gesia (PCA), and enteral feeding via a jejunostomy tube placed
intra-operatively. She was discharged home twenty days post-
operatively.
Histopathology and immunostaining of the resected specimen
was consistent with achalasia, revealing marked hypertrophy of the
inner circular layer of the muscularis propria, fibrosis in the muscle
layer of the distal oesophagus, and an absence of ganglion cells in
either Auerbachs or Meissner’s plexus.
She completed the Eckardt achalasia patient satisfaction and
asymptomatic outcome score10 at her 12 month post-operative
review. BMI had increased to 27. She had a Total Symptom Score
(TSS) of 2, equating to good symptomatic outcome.
Fig. 1. Coronal view from the CT Thorax from patient 1, showing dilated oesophagus
with food and fluid stasis.
205
2.2. Case 2
A 65 year old female with a fifteen year history of achalasia was
referred to our unit with refractory disease. She had undergone
a laparoscopic Heller’s myotomy at the time of diagnosis, with
symptom recurrence after four years. Following on from this, the
patient underwent six pneumatic dilatation’s and re-do myotomy
with only temporary symptom control. Re-do myotomy had been
performed one year prior to referral, without success, and the
patient complained of progressive severe dysphagia, regurgitation,
and nutritional failure. BMI at time of referral was 19.5. Diagnostic
work-up was performed. OGD showed a grossly dilated and aper-
istaltic oesophagus. Timed barium swallow demonstrated a grossly
dilated oesophagus and tight narrowing at the LOS, with markedly
delayed oesophageal emptying, consistent with advanced achalasia
(Fig. 2). At this centre we performed a single PD but this failed to
provide any symptom relief. Manometry demonstrated aperistalsis
of the oesophageal body and incomplete relaxation of the oeso-
phago-gastric junction.
A transhiatal oesophagectomy was performed. The approximate
intra-operative blood loss was 510 ml. There were no post-opera-
tive complications, and she was discharged home on day 14 post-
operatively.
The resected specimen revealed a dilated oesophagus with
a diverticulum (Fig. 3). Histopathological examination and immu-
nostaining demonstrated a marked reduction in myenteric and
submucosal plexi, consistent with achalasia.
At 9 month follow-up, she was completely asymptomatic, with
an Eckardt Total Symptom Score10 of 0, and reported being satis-
fied with her outcome, and willingness to undergo the procedure
again if given her time back. She had re-gained weight with a BMI
of 24.5.
Fig. 2. Barium meal image from patient 2, demonstrating a grossly dilated lower
oesophagus with beaking of the gastro-oesophageal junction, consistent with
advanced achalasia.
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206 J.M. Howard et al. / International Journal of Surgery 9 (2011) 204e208
Fig. 3. Transhiatal oesophagectomy specimen from patient 2, with a diverticulum at
the left lower end of the oesophagus.
2.3. Literature search strategy and selection criteria
References for the literature review were identified by searches
of Pub Med, MEDLINE and Current Contents using the search terms
“achalasia”, “end-stage achalasia”, and “oesophagectomy”. Refer-
ences from identified articles were investigated for relevance.
Abstracts and reports from meetings were not included. Only
papers published in English between 1970 and May 2009 were
included. The papers are shown in Table 1.
3. Discussion
End stage achalasia occurs in less than 5% of all achalasia
patients, and may be characterised both clinically and radiologi-
cally. Radiological features include a tortuous (sigmoid) and
massively dilated oesophagus, usually greater than 6 cm in diam-
eter. Clinically, patients present with severe dysphagia, regurgita-
tion, and nutritional failure.6 The management of patients with
end-stage disease is challenging, and many have already under-
gone multiple failed therapeutic procedures. Unfortunately, treat-
ment options are limited, and oesophageal resection may be
required in selected patients.
All patients who present with symptom recurrence following
therapy for achalasia must be carefully evaluated with repeat
endoscopy, manometry and contrast study in order to determine
the cause of treatment failure, which most commonly results
from inadequate or healed myotomy, the development of reflux
oesophagitis and stricture, obstruction from the fundoplication
wrap, carcinoma, or development of a para-esophageal hernia.11,12
Patients who have only undergone pneumatic dilatation should
be offered surgical myotomy.13 Patients with failed myotomy
should be offered re-do myotomy, or oesophagectomy in those
patients with a highly tortuous and dilated sigmoid oesophagus.11
Patients with failed re-do myotomy should be offered oesopha-
gectomy if symptoms are severe and quality of life is
affected.11,14e17
Devaney et al. reported on the largest series to date, of 93
patients who underwent oesophagectomy for end-stage acha-
lasia.11 They suggest the use of oesophagectomy only in those
patients who have a tortuous, sigmoid shaped mega-oesophagus,
stating that the tortuosity will interfere with oesophageal
emptying even after adequate myotomy. Guidelines for selection of
patients who may benefit from oesophagectomy are described in
Table 2. In contrast, if the patient has a massively dilated but
relatively straight oesophagus, Heller’s myotomy can provide good
symptom relief, and should be performed in the first instance.11
Both of our patients have obtained excellent symptomatic relief
of dysphagia, report excellent symptom control, as well as
improved quality of life and satisfaction with their outcome. A
review of the literature shows similar results (Table 1), with good
symptom control reported in 75e100% of patients. However,
oesophagectomy is not without risk, and every patient must be
fully informed of all associated risks. Reported mortality rates of
5e10% are described, while morbidity rates of up to 50% have been
reported, and anastomotic leak in 10e20% of patients. Patients
must also be informed of longer-term complications. Anastomotic
stricture has been reported in up to 50% of patients, depending on
length of post-operative follow-up.11 Dumping syndrome, reported
in up to 20% of patients,16 tends to be self-limiting and may be
managed medically if necessary, and vagal-sparing oesophagec-
tomy may reduce this risk.18
Oesophageal resection is often more technically difficult in the
patient with achalasia, compared with resection for malignancy,
due to a combination of factors. The anatomy is altered in mega-
oesophagus, with oesophageal deviation most commonly into the
right chest, making pneumothorax and need for tube thoracostomy
more common in these patients. Secondly, hypertrophy of the
oesophageal musculature can result in a richer blood supply,
making haemostasis more difficult, especially during mediastinal
dissection. Thirdly, mobilisation of the cervical oesophagus can also
be more difficult, especially in those patients who have oesopha-
geal dilatation extending more proximally to the level of the
thoracic inlet, and extra care must be paid to avoiding recurrent
laryngeal nerve injury. Finally, previous instrumentation and
surgery can cause the development of scarring and adhesions with
adjacent aorta and left lung, making transhiatal mobilisation more
difficult.11
We routinely perform transhiatal oesophagectomy with gastric
pull-up and cervical anastomosis for benign oesophageal disease.
Several surgical approaches have been described for oesophageal
resection in achalasia, including transthoracic,7,19,20 tran-
shiatal6,8,11,16,21 and more recently, laparoscopic transhiatal.17,21,22
For reconstruction, most surgeons advocate cervical oesophago-
gastric anastomosis,6,7,11,15e17,21,22 although colonic interposi-
tion19,20 and jejunal bypass8 have been described.
In the hands of an experienced surgeon, the transhiatal
approach is safe and effective in patients with end-stage achalasia,
and reduces the morbidity risk associated with thoracotomy.
However, due to additional technical difficulties associated with
resection as described above, mediastinal dissection and haemo-
stasis is more challenging, and conversion to a transthoracic
procedure may be required. For these reasons, some authors
advocate the transthoracic approach.15,19 Although still a relatively
new procedure, a laparoscopic transhiatal approach can provide
much improved visualisation during mediastinal dissection, with
improved morbidity and mortality.23
In conclusion, these two cases, combined with results from
several larger studies highlight the usual albeit rare scenario of
end-stage achalasia and the therapeutic benefit of oesophagec-
tomy, resulting in long-term resolution of the disabling obstructive
symptoms, nutritional failure, and poor quality of life.
Table 1
Table demonstrating results of literature review on the use of oesophagectomy in the management of end-stage achalasia.

Reference Study Time from dx Access Reconstruction Morbidity & mortality Mean Outcome Conclusion
size to surgery follow-up
Devaney11 93 Not given Transhiatal:87 pts, Gastric with cervical Mortality: 2%; Major morbidity: 3.2 yrs 95% asymptomatic. Transhiatal oesophagectomy is
(conversion to anastomosis :91 pts, 30%; (Anastomotic Leak: 10%); 96% would undergo a safe and effective therapy in
transthoracic in 6 pts) Colonic interposition: 2 pts Anastomotic stricture: 50% same again selected patients with
(prior gastric surgery) end-stage disease
Miller15 37 13.2 yrs Transhiatal:9 pts, Gastric:31 pts, Colon Mortality: 5.4%; Morbidity: 6.3 yrs Excellent outcome: 74%, Recommend transthoracic
Transthoracic :28 pts interposition: 6 pts 32.4%; (Anastomotic good outcome: 17%. Increased oesophagectomy for improved
leak: 5.4%) risk of bleeding in transhiatal visualisation and haemostasis,
approach. Surgical conduit rather than transhiatal approach
did not affect patient outcome

J.M. Howard et al. / International Journal of Surgery 9 (2011) 204e208

Banbury6 32 13 yrs Transhiatal :21 pts, Gastric. Cervical/thoracic Mortality: 0%; Anastomotic 3.5 yrs 100% on normal diet. 87% felt Oesophagectomy with gastric
Transthoracic:11 pts anastomosis leak: 13% improved, 7% felt the same, pull-up provides durable and
7% felt worse effective relief of dysphagia
in the majority of pts
Orringer16 26 Not reported Transhiatal (2 pts Gastric, cervical Mortality: 0%; Morbidity: 19%; 96% tolerate normal Oesophagectomy provides a
required conversion anastomosis Anastomotic leak: 4%; unrestricted diet reliable and effective treatment
to transthoracic) Anastomotic stricture:38%; for end stage achalasia
Dumping syndrome: 19%

REVIEW
Peters20 19 Not reported Left Thoraco-abdominal Colon interposition Mortality: 0%; Morbidity: 21% 93% felt “cured” with Oesophagectomy is a safe and
excellent quality of life effective therapy in end-stage
achalasia
Tank21 15 6.5 yrs Transhiatal Gastric, cervical Mortality: 5%; Morbidity: 1 yr 100% had normal swallow Oesophagectomy can be
anastomosis 50%; (Anastomotic leak: 10%) performed with acceptable
mortality in patients with
achalasia
Palanivelu17 11 13 mo. Laparoscopic transhiatal Gastric, cervical Mortality: 9%; Anastomotic 18.5 yrs Symptomatic improvement Laparoscopic oesophagectomy
anastomosis leak: 18% in 82% is a safe and effective procedure
in specialised centres
Hsu19 9 17 yrs Left thoraco-abdominal Colonic interposition Mortality: 0%; 6 yrs Good patient satisfaction. Limited distal oesophagectomy
Major morbidity: 22% 75% tolerating normal diet with short-colon interposition
is a safe and feasible alternative
for patients with end stage
achalasia
Glatz7 8 14 yrs Right thoraco-abdominal Gastric. Thoracic 0% 6 yrs 100% on a normal diet Oesophagectomy is a safe and
anastomosis appropriate treatment option
from managing end-stage
achalasia
Lewandowski8 7 10.1 yrs Transhiatal Jejenal bypass in 0% Not reported 100% had normal swallow Jejunal bypass offers a safe and
6 patients. and no dysphagia effective method of managing
Transhiatal mega-oesophagus
oesophagectomy with
colonic interposition
in 1 patient
Schuchert22 6 19 yrs Laparoscopic transhiatal Gastric, cervical Mortality: 0%; Morbidity: 2 yrs Not described Laparoscopic oesophagectomy
anastomosis 50%; (Anastomotic leak 17%) is a safe and effective procedure
in specialised centres

207
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208 J.M. Howard et al. / International Journal of Surgery 9 (2011) 204e208

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Clinical Eckhardt10 grading score of 6e12 (“Poor Response”) (3):854e8.
Severe dysphagia or regurgitation 12. Ellis Jr FH, Gibb SP. Reoperation after esophagomyotomy for achalasia of the
esophagus. Am J Surg 1975;129(4):407e12.
Nutritional failure
13. Dobrucali A, Erzin Y, Tuncer M, Dirican A. Long-term results of graded pneu-
Poor quality of life
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Radiological & Massively dilated oesophagus (>6 cm)
geal achalasia. World J Gastroenterol 2004;10(22):3322e7.
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