Documente Academic
Documente Profesional
Documente Cultură
URRENT
C
OPINION
Purpose of review
In recent years, several studies on the treatment and follow-up of achalasia have been published. This
review aims at highlighting interesting publications from the recent years.
Recent findings
Treatment of achalasia aims at relieving functional obstruction at the level of the esophagogastric junction.
Several treatment options such as pneumodilation and laparoscopic Heller myotomy (LHM) are available
for this purpose. A large prospective randomized trial comparing pneumodilation and LHM demonstrated
comparable success rates and quality of life for the two treatment options. Long-term data demonstrate
comparable treatment success rates, when redilation in case of recurrent symptoms after pneumodilation is
accepted. The most important risk factor for treatment failure is the manometric subtype, with a worse
outcome for type I and type III compared with type II achalasia. Recently, peroral endoscopic myotomy
(POEM) has been described with high success rates. Comparative studies with longer follow-up are
awaited. A prospective study assessing the risk of esophageal carcinoma in patients with achalasia
showed a 28-fold increased risk to develop carcinoma.
Summary
Either LHM or pneumodilation have high comparable short-term clinical success rates. Based on the
increased risk to develop esophageal carcinoma, a screening program may be indicated. POEM is a new
interesting treatment but longer follow-up data are awaited.
Keywords
achalasia, high-resolution manometry, laparoscopic Heller myotomy, peroral endoscopic myotomy,
pneumatic dilation
INTRODUCTION
INITIAL TREATMENT
www.co-gastroenterology.com
Copyright Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Esophagus
KEY POINTS
The characteristic feature of achalasia is the loss of
enteric neurons in the myenteric plexus, leading to
severely disturbed esophageal motility and typical
symptoms such as dysphagia, regurgitation and
weight loss.
Due to a lack in pathophysiological insight, treatment is
currently confined to mechanical disruption of the lower
esophageal sphincter, aimed at relieving functional
obstruction at the esophagogastric junction.
Pneumatic dilation and laparoscopic Heller myotomy
are the most commonly used treatment modalities and
have comparable success rates.
Treatment success depends on the manometric subtype
defined on pretreatment high-resolution manometry.
Patients with achalasia should be monitored on a
regular basis to prevent possible complications such as
sigmoid like esophagus and esophageal squamous
cell carcinoma.
were diagnosed by an esophagogram with watersoluble contrast. The most important risk factor
for esophageal perforation was age (age >60 years,
hazard ratio 3.4 compared with 60 years), a finding
consistent with that of previous studies. In a large
European study, the risk for perforation was also
significantly higher if the first dilation was performed with a 35 mm balloon rather than a
30 mm balloon (31 vs. 4%) [6 ,8 ]. Although perforation is a potentially life-threatening complication, as evidenced by the death of one patient
who had perforation further complicated bymediastinal hemorrhage, most of the patients who experienced perforation in this study (69%) were treated
successfully with conservative (nonoperative) treatment (i.e. antibiotics and nothing by mouth). Four
of 16 (25%) patients needed drainage of pleural
effusion, but none required surgery to close the
perforation. Notably, the long-term outcome after
esophageal perforation was reported to be excellent
in 69% of patients after a follow-up of 84 months,
which is comparable to treatment success reported
for patients without perforation [7 ].
Recently, Katzka and Castell [8 ] performed a
meta-analysis of 29 studies evaluating pneumatic
dilation regarding efficacy, rate of perforation and
dilation technique. Pooled results of studies with
the currently used Rigiflex balloons showed an 88%
1-year efficacy, gradually declining with time to 70
and 29% after 5 and 10 years, respectively, with a
combined perforation rate of 2%. Efficacy and durability of clinical success were increased by applying
repeated or graded dilations rather than just a single
dilation. In case of recurrent symptoms, redilation
leads to a further increase of the success rate to
8193% after 610 years [3,4]. Based on these numbers, one can conclude that pneumodilation has a
high rate of success when a graded distension protocol and redilation in case of recurrent symptoms are
used. Nevertheless, a clinician should always be
aware of the risk of perforation, and of the need to
evaluate and treat possible perforations promptly. It
is important to stress that early diagnosis of esophageal perforation is crucial, either by assessment of
pain evoked by ingestion of water 12 h after the
procedure, or by routinely performing a postdilation
radiograph of the esophagus using water-soluble
contrast.
&&
&
&
&
Pneumodilation
Pneumodilation disrupts the LES by forceful
inflation of an air-filled balloon. Briefly, a noncompliant Rigiflex balloon (Boston Scientific, Nanterre,
France) is inserted over an endoscopically placed
guidewire, and positioned at the level of the LES.
Under fluoroscopic guidance, the balloon is inflated
until the waist caused by the impression of the
esophaogastric junction is completely obliterated.
Usually, a graded distension protocol with increasing balloon sizes (30, 35 and 40 mm) is used, leading
to success rates of 7080%. Treatment success further increases to more than 90% when redilation is
allowed in case of recurrent symptoms [35]. Pneumatic dilation should preferentially start with the
smallest Rigiflex balloon (30 mm) to lower the risk of
esophageal perforation [6 ]. Perforation is the most
serious complication of pneumatic dilation, and
therefore one of the major drawbacks for clinicians.
In a recent retrospective study by Vanuytsel et al.
[7 ], pneumodilation was complicated by transmural esophageal perforation in 16 of 830 (1.9%) procedures [or 16 of 372 (4.3%) patients]. Perforations
&&
&
390
www.co-gastroenterology.com
Copyright Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Complications include conversion to an open procedure (2%) and mucosal perforation, on average
observed in 6.9% of patients [9,10]. However, most
perforations are noticed during surgery and immediately closed, such that these perforations cause
clinical problems only in approximately 0.7% of
patients [10].
A Heller myotomy is usually combined with
an antireflux procedure, lowering the incidence
of gastroesophageal reflux disease after treatment
from 32 to 8.8% [10]. The type of fundoplication
used differs among centers. In a study comparing
Dor (anterior 1808) and Nissen (3608) fundoplication, efficacy in preventing abnormal reflux was
similar, but dysphagia was more frequently observed
after Nissen fundoplication (2.8 vs. 15%, P < 0.001)
[11], arguing against the use of a total fundoplication.
Success rates for LHM reported in retrospective,
single-center studies are high, which resulted in
increasing enthusiasm for the surgical approach.
In a recent meta-analysis including 3086 patients
by Campos et al. [10], success rates were as high
as 89% (range 77100%) after a mean follow-up of
35 months. However, similar to pneumatic dilation,
treatment success rates for LHM decline over time
to 60%, as demonstrated in several studies with a
follow-up of 610 years [1214]. In patients with
recurrent symptoms following LHM, both pneumodilation and redo myotomy lead to acceptable
treatment success rates, ranging from 50 to 67%
for pneumodilation and from 58 to 87% for redo
myotomy [1517].
&&
&&
&&
www.co-gastroenterology.com
391
Copyright Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Esophagus
FOLLOW-UP
Follow-up of patients with achalasia is not only
important to ensure optimal symptom control,
but also to prevent possible complications such as
esophageal decompensation [5,9]. Furthermore,
patients with achalasia have an increased risk to
develop dysplasia and eventually even esophageal
squamous carcinoma [26,27]. Current guidelines do
not provide guidance on follow-up of patients with
achalasia [28,29]. However, several studies have
proposed that regular follow-up should be performed to decide on retreatment, preferably based
on the results of a functional test such as a timed
barium esophagogram [3,30].
Retreatment
As discussed above, treatment success gradually
decreases in patients with longstanding disease
(10 year) to 4060% [4,18,31,32]. Importantly,
however, additional treatment, both after initial
pneumodilation or LHM, has satisfactory results
with success rates of 6080% and improvement of
esophageal emptying [4,9,12,16]. The decision to
392
www.co-gastroenterology.com
retreat patients may be a clinical challenge, especially as symptoms and functional data (e.g. stasis on a
barium swallow, LES pressure measurements) do not
always match. Furthermore, patients often learn to
tolerate a certain level of symptoms or adapt their
diet accordingly. However, timely detection of
patients in need for additional treatment may be
important to avoid long-term complications such as
esophageal decompensation.
Multiple studies have assessed risk factors for the
need for retreatment after therapy. Vaezi et al. [33]
have demonstrated that 90% of patients with esophageal stasis need additional treatment within a year,
even if they have few or no symptoms shortly after
initial treatment. The data from the European achalasia trial confirm that monitoring esophageal emptying after treatment is a helpful tool for predicting
recurrence [6 ]. Therefore, 24 weeks after treatment, a timed barium esophagogram should be
performed, and this can be repeated during follow-up visits. In case esophageal stasis recurs,
additional treatment may be indicated.
&&
Copyright Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
CONCLUSION
Due to a lack of understanding of the pathogenesis
of achalasia, initial treatment still focuses on
mechanical disruption of the LES, rather than on
restoring esophageal motility. As demonstrated
in a recent prospective, randomized trial, a graded
pneumodilation protocol with the possibility
for redilation in case of recurrent symptoms is as
successful as LHM, and leads to comparable drop
in LES pressure, reduction in esophageal stasis
and improved quality of life. Ideally, the choice
of treatment should be based on risk factors such
as achalasia subtype and age in order to generate
an individualized therapeutic strategy. Follow-up
of the patient with achalasia should aim at
relieving recurrent symptoms and preventing
possible complications. POEM has generated great
enthusiasm among endoscopists, but longer
follow-up data and randomized comparative trials
are required before widespread acceptance of this
new approach.
Acknowledgements
None.
Conflicts of interest
GEB is supported by a grant (Odysseus program,
G.0905.07) of the Flemish Fonds Wetenschappelijk
Onderzoek (FWO).
There are no conflicts of interest.
www.co-gastroenterology.com
393
Copyright Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Esophagus
26. Leeuwenburgh I, Scholten P, Alderliesten J, et al. Long-term esophageal
cancer risk in patients with primary achalasia: a prospective study. Am J
Gastroenterol 2010; 105:21442149.
27. Eckardt AJ, Eckardt VF. Cancer surveillance in achalasia: better late than
never? [editorial]. Am J Gastroenterol 2010; 105:21502152.
28. Spechler SJ. AGA technical review on treatment of patients with dysphagia
caused by benign disorders of the distal esophagus. Gastroenterology 1999;
117:233254.
29. Vaezi MF, Richter JE. Diagnosis and management of achalasia. American
College of Gastroenterology Practice Parameter Committee. Am J Gastroenterol 1999; 94:34063412.
30. Gerson LB. Pneumatic dilation or myotomy for achalasia? Gastroenterology
2007; 132:811813.
31. West RL, Hirsch DP, Bartelsman JF, et al. Long term results of pneumatic
dilation in achalasia followed for more than 5 years. Am J Gastroenterol 2002;
97:13461351.
32. Eckardt VF, Aignherr C, Bernhard G. Predictors of outcome in patients with
achalasia treated by pneumatic dilation. Gastroenterology 1992; 103:1732
1738.
394
www.co-gastroenterology.com
33. Vaezi MF, Baker ME, Achkar E, Richter JE. Timed barium oesophagram: better
predictor of long term success after pneumatic dilation in achalasia than
symptom assessment. Gut 2002; 50:765770.
34. Zaninotto G, Rizzetto C, Zambon P, et al. Long-term outcome and risk of
oesophageal cancer after surgery for achalasia. Br J Surg 2008; 95:1488
1494.
35. Rohof WO, Bergman JJ, Bartelsman JF, et al. Screening for dysplasia in
idiopathic achalasia using lugol staining [abstract]. Gastroenterology 2011;
140 (Suppl 1):S227.
36. Dawsey SM, Fleischer DE, Wang GQ, et al. Mucosal iodine staining improves
endoscopic visualization of squamous dysplasia and squamous cell carcinoma of the esophagus in Linxian. China Cancer 1998; 83:220231.
37. Brossard E, Ollyo JB, Fontolliet CH, et al. Achalasia and squamous cell
carcinoma of the esophagus: is an endoscopic surveillance justified? [abstract]. Gastroenterology 1992; 102 (suppl):A4.
38. Chino O, Kijima H, Shimada H, et al. Clinicopathological studies of esophageal carcinoma in achalasia: analyses of carcinogenesis using histological
and immunohistochemical procedures. Anticancer Res 2000; 20:3717
3722.
Copyright Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.