JOURNAL OF LAPAROENDOSCOPIC & ADVANCED SURGICAL TECHNIQUES

Volume 26, Number 7, 2016

Mary Ann Liebert, Inc.
DOI: 10.1089/lap.2016.0085

Full Report: Pediatric

Contemporary Management of Achalasia

by Pediatric Surgeons:
A Survey of the International Pediatric Endosurgical Group
Joanna L. Gould, MD, Rebecca M. Rentea, MD, and Shawn D. St. Peter, MD

Abstract

Introduction: Achalasia is a rare neurodegenerative disorder of the esophagus. Surgical repair consists of esophagomyotomy, often in conjunction with an antireflux procedure. We sought to determine practice patterns in
surgical treatment of pediatric achalasia.
Methods: Data regarding preferences were collected as part of a comprehensive online-based survey sent to
members of the International Pediatric Endosurgery Group (IPEG) completed by 191 surgeons of which 141
performed esophagomyotomies for achalasia.
Results: Procedures performed per surgeon were 12 (n = 21, 15%); 35 (n = 49, 34%); 610 (n = 39, 28%); 1120
(n = 21, 15%); >20 (n = 11, 8%). Most approached the operation laparoscopically (n = 127, 90%). Workup
before esophageal myotomy consisted of a diagnostic esophagram (n = 133, 94%) or manometry (n = 102, 73%).
Only 60% of surgeons (n = 84) required an EGD. No preference observed in division location of the phrenoesophageal ligament for mobilization of the esophagus. There was a predominant preference for hook cautery
(n = 82, 58%) over harmonic shears (n = 30, 21%), heated sealing device LigaSure (n = 18, 13%), and other
devices (n = 11, 8%) for muscle division. Intraoperatively, 57% (n = 80) had endoscopy and 50% (n = 71) had
postoperative esophagram before initiation of enteral feeding. For antireflux procedure, Thal/Dor approach was
performed most frequently (n = 111, 79%) followed by the Toupet (n = 18, 13%) and Nissen (n = 4, 3%) and none
(n = 7, 5%). Diet restrictions were provided in 76% (n = 107) of postoperative patients.
Conclusion: Given the infrequency of achalasia in children, there are a range of treatment plans among pediatric
surgeons. We have identified current practices as a first step in developing more standard treatment pathways.
esophageal myotomy (Heller myotomy) with or without an
antireflux procedure and most recently peroral endoscopic
myotomy (POEM).
Given the rarity of achalasia in the pediatric population as
well as the variety of technical approaches and management
strategies in diagnosis and treatment, we sought to determine
the current practice patterns through a survey of the International Pediatric Endosurgery Group (IPEG) membership.

Introduction

chalasia is a rare neurodegenerative disorder that is

incompletely understood, but thought to be secondary to
an imbalance between defective inhibitory innervation combined with spared excitatory function leading to esophageal
motor abnormalities and lower esophageal sphincter (LES)
dysfunction. This leads to the characteristic failure of LES
relaxation and absence of esophageal peristalsis.13 There is
an annual incidence of 1/100,000 cases with less than 5% of
these being in children (0.11 cases per 100,000 pediatric patients).4 Given the relative infrequency of pediatric achalasia,
there are little data that guide management in children, with
current treatments aimed at symptom palliation.
Following diagnosis, treatment options are similar to those
offered in adults. Nonoperative management includes botulinum toxin injection, oral calcium channel blockers, and
pneumatic dilations (PD). Surgical treatment consists of an

Materials and Methods

The survey was conducted between October and November

2014 on behalf of the IPEG Research Committee using an
online provider for Web-based surveys (SurveyMonkey
[www.SurveyMonkey.com]). All IPEG members (n = 650)
were contacted by e-mail and invited to complete an anonymous questionnaire that included personal background and
management of achalasia. The invitation was sent out by

Department of Surgery, Childrens Mercy Hospital, Kansas City, Missouri.

e-mail with an embedded link to connect to the survey. Two

reminder messages were sent during a 4-week period. Trainee/
fellows were excluded from the study. A completion of all
items was not mandatory if the question did not apply to the
respondents practice.
Data were collected on demographics, specialty board
certification, experience as measured by year in practice and
number of cases performed if done for achalasia, preoperative workup required, operative approach, muscle division
specifics, preference on type and need for wrap, as well as
postoperative management.
Statistical analysis

Answers were anonymously collected, converted into a

database with Microsoft (Redmond, WA) Office Excel
(Version 2015), and analyzed using descriptive statistics.
Each response was calculated as a percentage to account for
the fallout rate per operation.
Results
Demographics

A total of 207 individuals completed the online survey

(32%). Of these, 191 commented on performance of esophagomyotomy. More broadly, 202 described themselves as pediatric surgeons and the remainder general surgeons. One-fourth
(n = 50) of the participants had more than 20 years of experience, and 30% (n = 60) had between 10 and 20 years of experience. Nearly 74% (n = 149) of the participants had performed
over 50 pyloromyotomies.
Preoperative management

Of the 191 surgeons who answered, 141 (74%) perform

esophagomyotomies for achalasia. The number of procedures
performed per surgeon was as follows: 12 (n = 21, 15%);
35 (n = 49, 34%); 610 (n = 39, 28%); 1120 (n = 21, 15%);
>20 (n = 11, 8%). The majority of responders approach the
operation laparoscopically (n = 127, 90%) while robotic and
open approaches were used with equal frequencies at 5%
(n = 7) each. None of the surgeons used POEM. Workup
before esophageal myotomy most frequently consisted of
a diagnostic esophagram (n = 133, 94%) or manometry
(n = 102, 73%). Only 60% of surgeons (n = 84) required an
EGD and few requested Trypanosoma cruzi studies.
Operative management

No preference between circumferential, isolated anterior, or

anterior and lateral division of the phrenoesophageal ligament
for mobilization of the esophagus was demonstrated; 32%
(n = 45) mobilize circumferentially, while 36% (n = 51) mobilize anterior and laterally. The remaining 32% (n = 45) only
divide the attachments between the diaphragm/crus and
esophagus anteriorly. Placement of anchoring sutures between
the crus and esophagus after myotomy was performed equally
(n = 68, 48%).
Muscle division was preferentially performed with hook
cautery (n = 82, 58%) over harmonic shears (n = 30, 21%), heat
sealing device LigaSure (Covidien-Medtronic, Minneapolis,
MN) (n = 18, 13%), and other devices (n = 11, 8%) for muscle
division. Of the 11 surgeons that choose other instruments, five
surgeons (3%) used scissors, two (1%) used a scalpel, and the

GOULD ET AL.

remaining used other forms of electric devices. Standard recommended myotomy length is 5 cm on the esophagus with
2 cm extension onto the gastric wall. In this review, 33% (n = 47)
of surgeons surveyed determine length based on endoscopic
findings of patency. To determine patency endoscopically, 57%
(n = 80) of those surveyed believed it was necessary to conduct
intraoperative endoscopy. Those who use a specific length range
from 2 to 12 cm with 56 cm being the mean. Some of the
variation stems from clinical judgment based on the childs size.
Failure of surgical intervention is most commonly secondary to gastroesophageal reflux disorder and recurrent dysphagia. Secondary to this, it is common to perform an antireflux
procedure in conjunction with Heller myotomy. Five percent
(n = 7) of surgeons surveyed did not perform any type of fundoplication to prevent postoperative gastroesophageal reflux.
In the remaining, fundoplication accomplished by the Thal/Dor
approach was performed most frequently (n = 111, 79%) followed by the Toupet (n = 18, 13%) and Nissen (n = 4, 3%). Fifty
percent of surgeons (n = 70) ordered postoperative esophagram
before initiation of enteral feeds, and dietary restrictions were
provided for 76% (n = 107) of postoperative patients.
Discussion

Achalasia is a well-known disorder of esophageal motility

with an evolving treatment algorithm for pediatric patients.
Nearly all data and recommendations are based on adult
studies and outcomes highlighting the question of degree of
correlation between pediatric and adult management. In light
of the difference between pediatric and adult recommendations, this survey aimed to identify the preferred surgical
strategy of the IPEG members, a group that was established
as a venue to showcase advanced endoscopic techniques and
the application of new developments.
Similar to adult populations, laparoscopic Heller myotomy
(LHM) with or without an antireflux procedure is increasingly the treatment of choice for children.5 This is the first
survey to examine variability in pediatric surgical management globally and demonstrates that the accepted standard of
care is relatively consistent across the ages. The vast majority
of the participants from all continents worked in academic
institutions and had performed between 3 and 10 or more
LHM in their career. The preferred surgical technique among
IPEG members is LHM with Thal/Dor fundoplication.
One of the main controversies in achalasia management
continues to center around LHM versus PD. While many
therapies for achalasia exist in the adult population, mechanical dilation has historically been the gold standard upon
which other therapies were compared. Short-term results are
proven to be effective; however, long-term results are less
promising. Currently, LHM has evolved as the preferred
procedure for adults with esophageal achalasia, while medical treatments and/or endoscopic esophageal PDs are usually
reserved for those older than 45 years and those with high
surgical risk.6 Previous studies have demonstrated the superior long-term results of myotomy; however, this has been
challenged by one large multicenter randomized trial demonstrating lack of LHM superiority to PD.7 Notably, this study did
not define failure when repeat interventions were required. In
the pediatric population, a systematic review demonstrated that
adequate comparative data are lacking to determine ideal
treatment for pediatric achalasia.5 Additionally across the

PEDIATRIC ACHALASIA MANAGEMENT SURVEY

literature PD, which requires intubation, is often only performed

on school aged children. Need for reintervention, similar to the
adult literature, is vague with a range of 25%90%5 with the
period of symptom-free time dropping significantly in those
children requiring subsequent treatments.2
Antireflux procedure following LHM is another source of
contention. In a previous comprehensive review, comparative data on type of fundoplication were summarized. Nissen
fundoplication (360) was deemed inappropriate secondary
to high progressive esophageal stasis and functional obstruction rates believed to be created by complete wrap.8 Dor
fundoplication (180 anterior) results in abnormal pH seen in
5.710% of patients up to 1 year postoperatively.8 Advocates
state that the anterior wrap provides a degree of protection
over the myotomy from any subclinical intraoperative injuries. Conversely, those who advocate for a Toupet (270
posterior) describe its ability to hold the myotomy open
without actually covering an anterior myotomy.8 While the
majority of IPEG members performed an antireflux operation
at the time of myotomy, 6% did not perform an antireflux
procedure. The authors who report LHM without antireflux
procedure report a high rate of symptom relief and low incidence of postoperative complications, despite the lack of
esophageal motility restoration to normal.9
One potential topic for discussion is the apparent lack of
POEM in the pediatric population. It has the combined benefits
of an endoscopic procedure with the long-term efficacy of a
surgical myotomy.10 Before a 2015 study done by Caldaro
et al., the procedure had only been done in adults with proven
safety and efficacy.10 Emerging data have shown that it has
shorter operative times, lower complication rates, faster time to
feeding, longer myotomy, and more rapid discharge.10 A
single-center experience of 26 patients with an age range of 6
17 years demonstrated at 2-year treatment success with a 20%
development of reflux.11 Given its recent introduction with
children, there may be potential for a shift in standard treatment
once more experienced endoscopists are available and trained
to provide comparable or improved results for achalasia.10
Another notable difference is the absence of endoscopic biopsy in preoperative workup. For adults, biopsy is recommended
to exclude malignancy before surgical intervention. None of the
respondents endorsed biopsy before operative intervention.
However, 1.5% of those polled endorsed obtaining Trypanosoma cruzi studies, as secondary achalasia leading to malnutrition can often be the first symptom of digestive pathology in a
patient with Chagas disease.
An inherent aspect of being a pediatric surgeon is facing a
wide variety of rare conditions. Surgical groups have grown
to allow for coverage of the busy call schedules, which creates the scenario of having a diluted experience with already
uncommon procedures. If only six cases were used as the
threshold for stabilizing on the learning curve, half of the
respondents do not have a mature experience. This suggests
we should consider concentrating the experience among
fewer surgeons at each institution. This lack of individual
experience likely explains the fact that despite surveying a
minimally invasive organization, 5% of respondents still
perform an open myotomy.
Despite offering a general picture of the current practice of
achalasia among IPEG members, we are aware of limitations of
our study. There could have been a bias in selecting the surgeons as not all members responded. In addition, we can only

assume that the participants provided honest answers. Surveys

in general are susceptible to recall bias. We also did not ask
about long-term follow-up on this cohort of patients or need for
reintervention. Finally, certain information such as recurrence
rates could have a potential bias in patient selection.
Conclusion

Laparoscopic achalasia repair is currently being performed

by 90% of all participating IPEG members. The preferred
surgical technique among IPEG members is LHM with Thal/
Dor fundoplication. The fact that limited information exists
on symptom recurrence or age-specific recommendations,
method of achalasia diagnosis, procedure techniques, and
follow-up suggests the focus of future studies.
Disclosure Statement

No competing financial interests exist.

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Address correspondence to:

Shawn D. St. Peter, MD
Department of Surgery
Childrens Mercy Hospital
2401 Gillham Road
Kansas City, MO 64108
E-mail: sspeter@cmh.edu