ABCD Arq Bras Cir Dig

2024;37e1809 Review Article

https://doi.org/10.1590/0102-6720202400016e1809

AADVANCED
QUEDA DAMEGAESOPHAGUS
PRESSÃO PORTALTREATMENT:
APÓS DESVASCULARIZAÇÃO
WHICH TECHNIQUE
OFFERS THE BEST RESULTS?
ESOFAGOGÁSTRICA A SYSTEMATIC
E ESPLENECTOMIA REVIEW A VARIAÇÃO
INFLUENCIA
DO CALIBRE
TRATAMENTO DAS VARIZES
DO MEGAESÔFAGO E ASQUAL
AVANÇADO: TAXAS DEOFERECE
TÉCNICA RESSANGRAMENTO
MELHORES RESULTADOS? NA
UMA REVISÃO SISTEMÁTICA
ESQUISTOSSOMOSE NO SEGUIMENTO EM LONGO PRAZO?
Paulo Sérgio CHAIB1 , Gloria de Almeida TEDRUS1 , José Luís Braga de AQUINO1 ,
José Alexandre
Does the drop inMENDONÇA
1
portal pressure after esophagogastric devascularization and splenectomy
variation of variceal calibers and the rebleeding rates in schistosomiasis in late follow-up?

Walter de Biase SILVA-NETO1 , Claudemiro QUIRESE1 , Eduardo Guimarães Horneaux de MOURA2 ,

Fabricio Ferreira COELHO3 , Paulo HERMAN3

RESUMO
ABSTRACT-– Racional:
BACKGROUND: O tratamento de escolha para
Advanced megaesophagus pacientes
predisposes com
to risks hipertensãoinfections
of malnutrition portal
Central Message
esquistossomótica
and cancer, in addition com sangramento
to having a significantde varizes
impact é a ofdesconexão
on quality ázigo-portal
life. There is currently mais
no consensus
esplenectomia
in the literature (DAPE) associada
regarding à terapia
the best surgical endoscópica.
option Porém,
for advanced estudos mostram
megaesophagus, although aumento
there is a In the world literature there is still no consensus
predilection forvarizes
esophagectomy, on the best surgical option for definitive
do calibre das em algunsdespite this surgery
pacientes durantebeing associated with
o seguimento significant
em longo morbidity
prazo. and
Objetivo:
mortality. Other surgical procedures, such as esophageal mucosectomy and Heller cardiomyotomy, treatment of advanced megaesophagus. Subtotal
Avaliar o impacto da DAPE e tratamento endoscópico pós-operatório no comportamento esophagectomy is still suggested as the main
have been proposed with good results. AIMS: To conduct a systematic review and meta-analysis of
das
the varizes esofágicas
literature e recidiva
on the surgical hemorrágica,
treatment of advanced demegaesophagus.
pacientes esquistossomóticos.
METHODS: Databases Métodos:
used treatment option for advanced megaesophagus
Foram
included estudados 36 pacientes
PubMed, Latin American com seguimento
and Caribbean superior
Health a cinco
Sciences anos,(Lilacs),
Literature distribuídos
Embase em
and in elective cases; however, the procedure presents
dois grupos:
Medical queda
Literature da pressão
Analysis portalSystem
and Retrieval abaixoOnline
de 30% e acimaasde
(MedLine), 30%
well comparados
as reference comTwo
research. o significant morbidity and mortality rates.
reviewers
calibre dasselected the articles no
varizes esofágicas independently.
pós-operatórioRESULTS: A total
precoce of 14além
e tardio articles
do were
índicechosen, which
de recidiva With the purpose of offering a less morbid
included 1,862 patients. The studies were divided into two groups: laparoscopic cardiomyotomy treatment for these patients, who are likely to be
hemorrágica. Resultados
with fundoplication (213 patients) and major surgeries (1,649 patients). The studies yielded mostly already weakened by this disease, some authors
esofágicas que, durante
good or excellent o seguimento
results regarding aumentaram
late outcomes in bothdegroups.
calibreHowever,
e foramtherecontroladas com
was significant propose performing Heller cardiomyotomy
morbidity associated with the major surgeries group. CONCLUSIONS: Laparoscopic Heller myotomy Evolução
which isdo calibre das
generally varizes nosatisfactory.
considered período pré e pós-
ocan
comportamento
be performed ondo calibre
patients das
with varizes megaesophagus,
advanced no pós-operatóriowith precoce nem
lower rates tardio nem and
of complications os operatório precoce e tardio
mortality
índices decompared
recidiva to major surgeries,
hemorrágica. with reservations regarding late outcomes results.
Conclusão
HEADGINGS: Esophageal Achalasia. Digestive System Surgical Procedures. Esophagectomy. Myotomy. Perspectives
Treatment Outcome. Systematic Review. Mensagem central
operatórios precoces ou tardios. A comparação entre a queda de pressão do portal e as Our systematic review with meta-analysis allows
Aus desconexão
to concludeázigo-portal
that patients e with
esplenectomia
advanced
RESUMO – RACIONAL:
DESCRITORES: O megaesôfago
Esquistossomose avançadoportal.
mansoni. Hipertensão predispõe riscos
Cirurgia. clínicos
Pressão deporta.
na veia desnutrição, infecções
Varizes esofágicas apresenta importante
megaesophagus can impacto
be safelyna treated
diminuição
with
e neoplasias, além de impacto significativo na qualidade de vida. Não há um consenso atual
e gástricas. precoce do calibre
laparoscopic Hellerdas cardiomyotomy
varizes esofágicas with
na
na literatura ante a melhor opção de seu tratamento cirúrgico, embora haja predileção pela esquistossomose;
fundoplication. Thisentretanto,
surgical parece
modality,quewhich
a
esofagectomia, cirurgia de significativa morbimortalidade associada. Outras modalidades cirúrgicas associação
encompassescomaaless
terapia endoscópica
complex abdominal é asurgery
maior
têm sido propostas, com bons resultados, como a mucosectomia esofágica e a cardiomiotomia responsável
procedure,pelo controle
yields da recidivaresolution
high symptom hemorrágica.
rates,
laparoscópica
ABSTRACT à Heller. OBJETIVOS:
- Background: Realizarofuma
The treatment revisão
choice forsistemática com metanálise
patients with da literatura
schistosomiasis with low complication rates, low mortality rates and
acerca do tratamento cirúrgico do megaesôfago avançado. MÉTODOS: As bases de dados utilizadas
previous episode of varices is bleeding esophagogastric devascularization and splenectomy
foram PubMed, Lilacs, Embase e MedLine, além de pesquisas de referências relacionadas. Os artigos
satisfactory results. However, caveats should be
(EGDS) in association with made regarding the late long-term outcome.
foram selecionados por doispostoperative endoscopic therapy.
revisores independentemente. However,Foram
RESULTADOS: studies have shown
selecionados 14 Perspectiva
varices
artigos, recurrence
que incluem especially after Os
1.862 pacientes. long-term follow-up.
estudos foram Aim:
divididos em Todoisassess
grupos:the impact on
cardiomiotomia Este estudo avaliou o impacto tardio no índice
laparoscópica
behavior com fundoplicatura
of esophageal varices (213
and pacientes) e cirurgias deafter
bleeding recurrence grande porte (1.649 pacientes).
post-operative endoscopic Os de ressangramento de pacientes submetidos ao
estudos analisados
treatment evidenciam
of patients submittedquetoambos
EGDS. os Methods:
grupos apresentaram resultados
Thirty-six patients semelhantes
submitted to quanto
EGDS tratamento cirúrgico e endoscópico. A queda na
ao desfecho tardio, considerado majoritariamente bom ou excelente, no entanto, houve significativa
morbimortalidade associada ao grupo de cirurgias maiores. CONCLUSÕES: A cardiomiotomia
portal pressurecom
drop, more or less pode
than 30%, and compared with the behavior ofcom
esophageal variação do calibre das varizes quando comparado
laparoscópica fundoplicatura ser realizada no megaesôfago avançado, taxas de
varices and thee rate
complicações of bleeding
mortalidade recurrence.
reduzidas frente Results
às cirurgias de grande porte, porém, com ressalvas o seu diâmetro no pré e pós-operatório precoce e
quanto ao desfecho tardio a longo prazo. tardio. A comparação entre a queda de pressão
late post-operative varices caliber when compared the pre-operative data was observed portal e as taxas de ressangramento, também
DESCRITORES: AcalásiainEsofágica.
despite an increase diameterProcedimentos
during follow-up Cirúrgicos
that was docontrolled
Sistema Digestório. Esofagectomia.
by endoscopic therapy.
Miotomia. Resultado do Tratamento. Revisão Sistemática.
Conclusion evidenciar se apenas a terapia endoscópica, ou
variceal calibers when comparing pre-operative and early or late post-operative diameters. operações menos complexas poderão controlar o
The comparison between the portal pressure drop and the rebleeding rates was also not sangramento das varizes.

HEADINGS: Schistosomiasis mansoni. Portal hypertension. Surgery. Portal pressure.

Esophageal and gastric varices.

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From 1Pontifícia
Trabalho realizado Universidade
no 1Serviço deCatólica de Campinas,
Cirurgia Geral Postgraduate
e Aparelho Program of de
Digestivo, Departamento Health Sciences,
Clínica Campinas
Cirúrgica, Faculdade(SP), Brazil. Universidade Federal de Goiás, Goiânia, GO,
de Medicina,
Brasil; 2Serviço de Endoscopia, Hospital das Clínicas e Departamento de Gastroenterologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil; 3Serviço de
How to
Cirurgia docite thisHospital
Fígado, article:das
Chaib PS, Tedrus
Clínicas GA, de-Aquino
e Departamento JLB, Mendonça
de Gastroenterologia, JA. Advanced
Faculdade megaesophagus
de Medicina, Universidadetreatment: which
de São Paulo, São technique offers the best results? A
Paulo, SP, Brasil
systematic review. ABCD Arq Bras Cir Dig. 2024:37e1809. https://doi.org/10.1590/0102-6720202400016e1809.
Como citar esse artigo: de Biase Silva-Neto WB, Quirese C, De Moura EGH, Coelho FF, Herman P. A queda da pressão portal após desvascularização esofagogástrica e esplenectomia

/10.1590/0102-672020210001e1581
Correspondence: Financial source: None
Paulo Sérgio Chaib. Conflicts of interests: None
Correspondência:
Email: [email protected]; [email protected] Received: 04/10/2023
Walter De Biase da Silva Neto
Accepted: 03/14/2024
E-mail: [email protected]; Recebido para publicação: 17/09/2020
Editorial Support: National Council for Scientific
[email protected] andpublicação:
Aceito para Technological Development (CNPq).
14/12/2020

ABCD
ABCD Arq
Arq Bras
Bras CirCir
DigDig 2024;37e1809
2021;34(2):e1581 1/4
1/9
REVIEW ARTICLE

INTRODUCTION The eligibility criteria included:

A
Participant type (P): patients diagnosed with advanced
chalasia is an inflammatory neurodegenerative megaesophagus.
disorder of the esophagus, which, through the Types of intervention (I and C): esophagectomy, esophageal
destruction of neurons in the myenteric plexus mucosectomy, Serra-Doria surgery, Heller cardiomyotomy.
of the distal esophagus, prevents relaxation of the lower The types of intervention were not applicable to control patients.
esophageal sphincter (LES) and incoordination of esophageal The survey included a review of non-comparative studies.
peristalsis24,35,38. It is defined as a denervation esophagopathy Types of outcomes (O): surgical outcomes considering
with broad-spectrum dysmotility 13 hampering emptying morbidity, mortality, complications, length of stay, late results,
and dilation of the esophagus, clinically characterized as effectiveness, quality of life.
megaesophagus16. The aim of our work was to search for the most current
Terminal achalasia occurs in around 10–15% of all patients forms of surgical treatment for advanced megaesophagus,
with the disease22 and is characterized by advanced megaesophagus and, hence it was decided to include only articles published
(grades III and IV — Resende/Mascarenhas classification), with in the last ten years. Furthermore, as this is an uncommon
dolichomegaesophagus (“sigmoid-esophagus”), significant disease, the included articles had to have a sample of patients
tortuosity, esophageal diameter above 6 cm. It occurs due to greater than or equal to eight cases, submitted to previous
failure of previous treatments9,24. These patients present conditions treatments or not.
with severe symptoms, which directly impact their quality of
life. Furthermore, they commonly present life-threatening Inclusion criteria
complications, such as malnutrition, immunodeficiency, repetitive • Studies that included patients with advanced achalasia
bronchoaspiration and a high risk of developing sepsis and and/or advanced megaesophagus of any etiology (grades
neoplasms9,24,38. III and IV, sigmoid esophagus, terminal achalasia),
In the world literature, there is still no consensus undergoing any type of definitive surgical treatment.
on the best surgical option for definitive treatment of • Studies with patients aged ≥18 years.
advanced megaesophagus. Subtotal esophagectomy is • Studies with a patient sample greater than or equal
still suggested as the main treatment option for advanced to eight cases.
megaesophagus in elective cases; however, this procedure • Cohort studies, cross-sectional studies, case series,
presents significant morbidity (19 to 69%) and mortality randomized or non-randomized clinical trials.
rates (0 to 9%)1,23,34. • Studies evaluated and selected by two independent
Alternative techniques such as esophageal mucosectomy, reviewers.
developed by Aquino et al. 3, present significantly better • Studies written in English, Portuguese or Spanish.
results when compared to esophagectomy in the treatment • Articles published from 2012 onwards.
of terminal achalasia. On the other hand, it involves carrying
out a major abdominal surgery with all the risks inherent to Exclusion criteria
such procedure5,6. • Studies with patients without a diagnosis of advanced
With the purpose of achieving a less morbid treatment achalasia/advanced megaesophagus.
for these patients, who may already be weakened by this • Studies with patients diagnosed with advanced achalasia/
disease, some authors propose performing laparoscopic megaesophagus undergoing definitive non-surgical
Heller cardiomyotomy, with results generally considered treatments.
satisfactory. However, the accumulated risk of long-term • Case reports, correspondence, animal models, literature
neoplasia, regurgitation and bronchoaspiration is questioned reviews, systematic reviews or meta-analyses.
when keeping the esophagus in situ, an inert pouch, and • Studies without full text.
impaired emptying12,21.
The present study is justified based on the need to Selection of articles
provide a better understanding of the different types of surgical A search using a predefined strategy was carried out
treatments for advanced megaesophagus, considering the risks in electronic databases by two reviewers independently.
and postoperative morbidity and mortality, as well as results, Any disagreement between reviewers was settled by
effectiveness, and late outcomes. A more incisive guide to consensus after discussion with a third researcher. The
allow the surgeon’s selection of the best surgical treatment articles were screened according to previously established
for advanced megaesophagus is required. inclusion/exclusion criteria. When similar articles from the
Our work aimed to carry out a systematic review with meta- same institution were found, the article with a larger patients’
analysis on the surgical treatment of advanced megaesophagus, sample was selected.
with view at describing the main modalities currently in use Two separate reviews were carried out, one qualitative
and whose scope involves the comparative assessment of and one quantitative (meta-analysis). The latter compared the
such modalities’ morbidity, mortality, complications rates and following outcomes: morbidity/complications, mortality and
outcomes and late results. late outcomes considered good or excellent.

Database
The databases searched electronically were PubMed,
METHODS Medical Literature Analysis and Retrieval System Online
(MedLine), Latin American and Caribbean Health Sciences
Our systematic review was conducted in accordance with Literature (Lilacs) and Embase. The structured search
the recommendations and following the Preferred Reporting strategy involved the following terms: (esophageal achalasia)
Items for Systematic Reviews and Meta-Analyses (PRISMA)25 OR (achalasia) OR (end-stage achalasia) OR (megaesophagus)
method checklist. After a prepared question, the Patient or OR (advanced megaesophagus) OR (sigmoid-esophagus) AND
Problem, Intervention, Control or Comparison, Outcomes (surgery) OR (minimally invasive surgery) OR (laparoscopic
(PICO) strategy was used in order to identify the outcome of myotomy) OR (laparoscopic heller myotomy) OR (laparoscopic
advanced megaesophagus surgical treatment. cardiomyotomy) OR (serra-doria surgery) OR (esophagectomy)

2/9 ABCD Arq Bras Cir Dig 2024;37e1809

 ADVANCED MEGAESOPHAGUS TREATMENT: WHICH TECHNIQUE OFFERS THE BEST RESULTS? A SYSTEMATIC REVIEW

OR (esophageal resection) OR (mucosectomy) OR (esophageal RESULTS

mucosectomy) AND (groups) OR (trial) OR (surgery) OR
(randomly) OR (randomized) OR (clinical trial) OR (comparative The total number of articles assessed was 969 and the total
study) OR (controlled clinical trial) OR (randomized controlled number of articles selected for the work which met the pre-
trial) AND (surgery outcomes) OR (outcomes) OR (morbidity) established inclusion/exclusion criteria was 14, totaling 1,862
OR (mortality) OR (follow-up) OR (quality of life). patients.
The search for references of relevant articles and abstracts The database screening involved 958 articles. Out of
published in conference proceedings was also considered in these, after excluding duplicate articles and those that were
the review. The last survey was carried out in June 2022. The not relevant to the work, 84 articles were selected for full
survey results are reported in Table 1. text reading. Finally, eight articles were selected for the work.
The remaining articles were excluded because they did not
Bias risk analysis methodology in non-randomized present a scope relevant to the work or because data were
studies missing in connection with the objective of this study.
Non-randomized studies were subjected to the risk Within the data search carried out, some abstracts published
of bias analysis using the ROBINS-I platform (Risk Of in conference proceedings were identified and reviewed.
Bias In Non-randomized Studies — of Interventions); the A reference search for relevant articles was also carried out.
same methodology was used to assess the risk of bias of A total of 11 pertinent articles were found and, after application
a randomized study32. of the exclusion criteria, six articles were finally selected.
The papers were separated into two large groups: patients
Statistical analysis undergoing cardiomyotomy (six articles; n=213) and
Statistical analysis was carried out through the development patients undergoing major surgeries (nine articles; n=1,649),
of a meta-analysis using the Cochrane Review Manager (RevMan) and this group included the following surgeries: esophagectomy,
software (https://training.cochrane.org/online-learning/core- subtotal esophagectomy, transhiatal esophagectomy, minimally
software/revman), organized in forest plot and funnel plot invasive esophagectomy, esophageal mucosectomy, Serra-Doria
graphs. Statistical significance was considered at p<0.05 and esophagocardioplasty. The major surgeries mentioned above
confidence interval at 95% (95%CI)15. The heterogeneity of the were considered as such because they necessarily involved a
studies was assessed using the I test². digestive anastomosis.
The article by Tassi et al.33 was allocated to both groups as
it encompasses patients studied using both surgical modalities.
Some studies within the group of major surgeries presented
results involving more than one surgical technique2,11,20.
Table 1 - Search results. Of the 14 studies selected for the work, one18 was not
Articles found Selected articles eligible for meta-analysis due to missing data. Hence, the
Data base
n n meta-analysis included a total of 13 articles and 686 patients.
PubMed 127 2
MEDLINE 260 2 Preferred Reporting Items for Systematic Reviews
Lilacs 247 3 and Meta-Analyses (PRISMA) flowchart
Others 11 6 The selection and inclusion of articles is shown in the
Total 969 14 PRISMA flowchart (Figure 1).

Figure 1 - Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) flowchart25.

ABCD Arq Bras Cir Dig 2024;37e1809 3/9

REVIEW ARTICLE

Qualitative results: systematic review Quantitative results: meta-analysis

The results were summarized in tables, as explained The meta-analysis was carried out based on a systematic
below. The surgeries were divided into two large groups, correlation between morbidity/complications and mortality and
named “cardiomyotomy” and “major surgeries” (Tables 2A, late outcomes considered good or excellent, for both groups.
2B, 3A, 3B). The objective items of study and comparison in In this way, four forest plot graphs were generated, two for the
this work were the following: study design, type of surgical cardiomyotomy group and two for the major surgery group
treatment performed, number of patients, average age, (Figures 2, 3, 4, 5). Analysis of the risk of bias of the selected
gender, definition and classification of advanced achalasia/ studies was carried out based on the ROBINS-I platform, as
megaesophagus, general complications and morbidity, shown in Table 4. A correlation was made between the relative
mortality, time of hospitalization, average follow-up time risk (RR) generated from the meta-analyses for the outcomes
and late results. assessed. Table 5 demonstrates such comparative analysis.

Table 2A - Systematic review of studies of cardiomyotomy with fundoplication for advanced megaesophagus.
No. of Average Gender Classification of
Study Study design Treatment carried out
patients age (years) (M/F) achalasia
Laparoscopic Heller
Panchanatheeswaran Retrospective M50%
cardiomyotomy + antireflux 8 39.5 “Sigmoid esophagus”
et al.26 cohort F50%
procedure
Laparoscopic Heller
Retrospective M6
Pantanali et al.27 cardiomyotomy + Pain 11 56 >10 cm (diameter)
cohort F5
fundoplication
Retrospective Laparoscopic Heller-Dor
Simić et al.31 10 51 - “Sigmoid esophagus”
cohort cardiomyotomy
III: >6 cm, IV: >3
Laparoscopic Heller 10
Retrospective III: 61 III: M0M3 esophageal curves and
Rosemurgy et al.29 cardiomyotomy + anterior III: 3
cohort IV 56 IV: F4M3 >6 cm
fundoplication IV: 7
(diameter)
142 grade III: >6cm (diameter)
Retrospective Laparoscopic Heller-Dor
Costantini et al.7 III: 87 46 - grade IV: “sigmoid-shaped
cohort cardiomyotomy
IV: 55 esophagus”
Laparoscopic Heller-Dor “Pull- CLH:
Retrospective
Tassi et al.33 down” cardiomyotomy (CLH) x CLH: 32 CLH: 57 M34.37% “End-stage achalasia”
cohort
Esophagectomy (E) F65.62%
M: male; F: female.

Table 2B - Systematic review of cardiomyotomy studies with fundoplication for advanced megaesophagus.
Average
Length of
Study Complications/morbidity Mortality follow-up Late results
stay (days)
time
Morbidity 0%
Panchanatheeswaran
1 iatrogenic intraoperative None 4.25 19.5 months 100% Excellent or Good (50–50%)
et al.26
complication
Pantanali et al. 27 Morbidity 0% None 1 31.5 months 72.8% Excellent or Good
Morbidity 0%
1 mucosal perforation
Simić et al.31 None 2 28 months 94.4% resolution of dysphagia
1 trocar bleeding
1 wound infection
III:
33% Excellent
Intraoperative: 0
III: 4 66% Good
Rosemurgy et al.29 Postoperative period: None 27 months
IV: 3 IV:
1 (atelectasia)
25% Excellent
75% Good
89.5% Good outcome
Morbidity 4.7% III: 90.8%
22 mucosal perforations IV 76.4%
Costantini et al. 7
0.1% (AMI) - 62 months
1 splenic injury Failure:
2 Trocar bleeding III 9.2%
IV 23.6%
CLH: 12.5%
1 mucous fistula
There were CLH:
1 mucous membrane CLH: 68
Tassi et al.33 none in both CLH: 6 46.87% Excellent
1 hyper-dysphagia months
groups 34.37% Good
1 hyper competent
fundoplication
CLH: Laparoscopic Heller-Dor “Pull-down” cardiomyotomy; AMI: acute myocardial infarction.

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 ADVANCED MEGAESOPHAGUS TREATMENT: WHICH TECHNIQUE OFFERS THE BEST RESULTS? A SYSTEMATIC REVIEW

Table 3A - Systematic review of major surgery studies for advanced megaesophagus.

No. of Average age Gender Classification of
Study Study design Treatment carried out
patients (years) (M/F) achalasia
Retrospective M49.01%
Molena et al.18 Esophagectomy 963 54.6 -
cohort F50.99%
“sink trap
Felix et al.10 Case series Transhiatal esophagectomy 11 44 M8 F3
megaesophagus”
40
Retrospective Transhiatal esophagectomy (THE) Advanced
Oliveira et al.20 THE: 23 - -
cohort x Mucosectomy (ME) megaesophagus
ME: 17
Retrospective Serra-Doria M14 Grades III and IV
Aquino et al.4 19 63 a 78
cohort esophagocardioplasty F5 (Rezende Classification)
229
Retrospective Esophageal mucosectomy (ME) x M70.3% Advanced
Aquino et al.2 ME: 115 15-76 years
cohort Transhiatal esophagectomy (THE) F29.7% megaesophagus
THE: 114
Transhiatal VLP esophagectomy M59.5% Advanced
Crema et al.8 Cohort 136 59.3
with vagus nerve preservation F40.45% megaesophagus
open: M8
30
Randomized Open transhiatal esophagectomy open: 47.2 F7 Grades III and IV
Fontan et al.11 open: 15
clinical trial vs. VLP VLP: 44.1 VLP: M11 (Rezende Classification)
VLP: 15
F14
Retrospective M51.8%
Torres-Landa et al.34 Esophagectomy (E) 209 56 -
cohort F48.2%
Laparoscopic Heller-Dor “Pull- E:
Retrospective
Tassi et al.33 down” cardiomyotomy (CLH) x E: 12 E: 59 M62.5% “End-stage achalasia”
cohort
Esophagectomy (E) F37.5%
M: male; F: female; VLP: videolaparoscopic.

Table 3B - Systematic review of studies of major surgeries for advanced megaesophagus.

Study Complications/morbidity Mortality Length of stay (days) Average follow-up time Late results
Morbidity 43.5%
Readmission 2.2%
Reoperation 6.7%
Torres-Landa et al.34 None 10 1 month Not assessed
Sepsis 9.5%
Pneumonia 12.4%
Blood transfusion 20.5%
E: 43.75%
E:
3 anastomosis fistulas There were
37.5%
Tassi et al.33 1 pyloroplasty fistula none in both E: 23 E: 61 months
excellent
1 pleural empyema groups
25% good
1 acute respiratory failure

Complications/ Excellent or good

morbidity - late outcome - Risk Ratio
cardiomyotomy cardiomyotomy Risk Ratio
Study or Subgroup
M-H, M-H, Random, 95%CI
Events Total Events Total Weight Random, Year
95%CI
Panchanatheeswaran 0.06
0 8 8 8 3.8 2013
et al.26 [0.00–0.87]
0.06
Pantanali et al.27 0 11 8 11 3.7 2013
[0.00–0.91]
0.05
Simic et al.31 0 10 9 10 3.8 2015
[0.00–0.80]
0.06
Costantini et al.7 7 142 121 142 52.9 2018
[0.03–0.12]
0.05
Rosemurgy et al.29 0 10 10 10 3.8 2018
[0.00–0.72]
0.15
Tassi et al.33 4 32 26 32 32.1 2022 Favours Favours
[0.06–0.39]
[Complications/morbidity [Excellent or good late
0.08 – cardiomyotomy] outcome – cardiomyotomy]
Total (95%CI) 213 213 100.0
[0.05–0,13]
Total events 11 182
Heterogeneity: Tau2 = 0.00; χ2 = 3.13, df = 5 (p=0.68); I2 = 0%
Test for overall effect: Z = 9.46 (p<0.00001)

Figure 2 - Comparative meta-analysis between morbidity/complications x good or excellent late outcome in cardiomyotomy –
Forest plot15.

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REVIEW ARTICLE

Complications/ Excellent or good

Risk Ratio Risk Ratio
Study or Subgroup mortality late outcome
M-H, Random, 95%CI
Events Total Events Total Weight M-H, Random, 95%CI Year
Pantanali et al.27 0 11 8 11 16.6 0.06 [0.00–0.91] 2013
Panchanatheeswaran
0 8 8 8 17.1 0.06 [0.00–0.87] 2013
et al.26
Simic et al.31 0 10 9 10 16.8 0.05 [0.00–0.80] 2015
Costantini et al.7 0 142 121 142 16.2 0.00 [0.00–0.07] 2018
Rosemurgy et al.29 0 10 10 10 16.9 0.05 [0.00–0.72] 2018
Tassi et al.33 0 32 26 32 16.4 0.02 [0.00–0.30] 2022

Total (95%CI) 213 213 100.0 0.03 [0.01–0.09] Favours Favours

Total events 0 182 [Complications/ [Excellent or good
Heterogeneity: Tau2 = 0.00; χ2 = 4.01, df = 5 (p=0.55); I2 = 0% mortality] late outcome]
Test for overall effect: Z = 6.16 (p<0.00001)
Figure 3 - Comparative meta-analysis between mortality x good or excellent late outcome in cardiomyotomy — Forest plot15.

Complications/ Excellent or good

Risk Ratio
morbidity late outcome Risk Ratio
Study or Subgroup
M-H, Random, M-H, Random, 95%CI
Events Total Events Total Weight Year
95%CI
Felix et al.10 0 11 11 11 3.6 0.04 [0.00–0.66] 2015
Oliveira et al.20 21 40 35 40 16.8 0.60 [0.44–0.82] 2015
Aquino et al.4 5 19 13 19 13.1 0.38 [0.17–0.87] 2016
Aquino et al.2 124 229 95 114 17.6 0.65 [0.56–0.75] 2017
Crema et al.8 19 136 132 136 16.2 0.14 [0.09–0.22] 2018
Fontan et al.11 12 30 30 30 16.2 0.41 [0.27–0.63] 2018
Torres-Landa et al.34 91 209 0 209 3.5 183.00 [11.44–2928.56] 2021
Tassi et al.33 5 12 7 12 13.0 0.71 [0.31–1.63] 2022

Total (95%CI) 686 571 100 0.49 [0.27–0.86] Favours Favours

Total events 277 323 [Complications/ [Excellent or good
Heterogeneity: Tau2 = 0.49; χ2 = 78.22, df = 7 (p=0.00001); p=91% morbidity] late outcome]
Test for overall effect: Z = 2.46 (p<0.01)
Figure 4 - Comparative meta-analysis between morbidity/complications x good or excellent late outcome in major surgeries –
Forest plot15.

Complications/ Excellent or good

Risk Ratio
mortality late outcome Risk Ratio
Study or Subgroup
M-H, Random, M-H, Random, 95%CI
Events Total Events Total Weight Year
95%CI
Felix et al.10 0 11 11 11 2.9 0.04 [0.00–0.66] 2015
Oliveira et al.20 0 40 35 40 2.8 0.01 [0.00–0.22] 2015
Aquino et al.4 0 19 13 19 2.8 0.04 [0.00–0.58] 2016
Aquino et al.2 11 229 95 114 62.5 0.06 [0.03–0.10] 2017
Crema et al.8 2 136 132 136 11.2 0.02 [0.00–0.06] 2018
Fontan et al.11 2 30 30 30 15.0 0.08 [0.02–0.27] 2018
Torres-Landa et al.34 0 209 0 209 Not estimable 2021
Tassi et al.33 0 12 7 12 2.8 0.07 [0.00–1.05] 2022

Favours Favours
Total (95%CI) 686 571 100 0.05 [0.03–0.08] [Complications/ [Excellent or
Total events 15 323 mortality] good late outcome]
Heterogeneity: Tau2 = 0.00; χ2 = 5.80, df = 6 (p=0.45); I2 = 0%
Test for overall effect: Z = 12.79 (p<0.00001)
Figure 5 - Comparative meta-analysis between mortality x good or excellent late outcome in major surgeries – Forest plot15.

DISCUSSION very impressive. Although most studies involved less than 12

patients, even in studies with a greater number of patients,
From the review of the data gathered in this work, we such as those by Costantini et al.7 (142 patients), and Tassi
can detect some significant aspects of the surgical treatment et al.33 (32 patients), these numbers reached rates of 89.5 and
of advanced megaesophagus. The systematic review and 81.24% respectively, in a late assessment with more than 60
meta-analysis carried out allow for sufficient data to be months follow-up.
provided for an in-depth analysis of the two large treatment Complications and morbidity in the major surgery group
groups evaluated. were significantly higher than in the cardiomyotomy group. In
The late results of the cardiomyotomy group were considered most studies, between 40 and 50% of patients underwent this
satisfactory by the authors (good or excellent) and are indeed form of treatment; in one series it reached 69.2%.

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Table 4 - Risk of bias in the studies included in the meta-analysis32.

Risk of bias domains
Study
D1 D2 D3 D4 D5 D6 D7 Overall
Rosemurgy et al.29
Fontan et al.11
Pantanali et al.27
Torres-Landa et al.34
Aquino et al.4
Oliveira et al.20
Crema et al.8
Aquino et al.2
Felix et al.10
Panchanatheeswaran et al.26
Simic et al.31
Molena et al.18
Costantini et al.7
Tassi et al.33
Domains: Judgment
D1: Bias due to confounding.
Critical
D2: Bias due to selection of participants.
D3: Bias in classification of interventions.
Serious
D4: Bias due to deviations from intended interventions.
D5: Bias due to missing data.
Moderate
D6: Bias in measurement of outcomes.
D7: Bias in selection of the reported result. Low

Table 5 - Relative risk between the cardiomyotomy and major

surgery groups compared to the comparative analysis 0.08 and that of mortality was 0.03. This risk was considerably
of morbidity/complications and mortality x good or lower than the RR of complications and mortality in relation
excellent late outcomes. to the favorable late outcome in the major surgery group, 0.49
and 0.05, respectively.
Morbidity/
Groups
complications
Mortality This allows us to conclude that both modalities show
Cardiomyotomy 0.08 0.03 good general surgical results; however, patients undergoing
Major surgeries 0.49 0.05 cardiomyotomy have lower risks of developing complications
and/or mortality, compared to patients undergoing major
surgeries, as already assessed in the systematic review of this
study. Furthermore, there are other considerable underlying
Late results in this group, unlike the case of the cardiomyotomy factors in this framework, such as shorter hospital stays, reduced
group, were assessed heterogeneously. In general, they were hospital costs and lower demand for treatment complexity —
also considered mostly satisfactory in all the series. when compared to major surgeries.
From the comparative meta-analysis between complications/ An important caveat must be made regarding the term
morbidity and good or excellent late outcomes in the cardiomyotomy “definitive treatment”, since most studies present a short
group, it was concluded that there is a low impact of morbidity/ to medium-term follow-up period. There are still questions
complications in relation to cardiomyotomy with fundoplication regarding relapses and/or progression of the disease in this
for patients with advanced megaesophagus. The RR was 0.08 treatment modality.
(p<0.00001, 95%CI 0.05–0.13). The data found are in accordance with the world literature.
In the comparative analysis between mortality and good Meta-analysis by Niño-Ramírez et al.19 involving 5,492 patients
or excellent late outcomes in the cardiomyotomy group, the undergoing laparoscopic Heller cardiomyotomy revealed a
RR for this outcome was 0.03 (p<0.00001, 95%CI 0.01–0.09), 4.9% rate of adverse events, most of them associated with
that is, there is also a considerably low impact of the outcome perforation of the esophageal mucosa. The 30-day mortality
in this analysis. rate in this group of patients was 0.09%.
When evaluating the comparative review between morbidity/ The systematic review with meta-analysis by Orlandini
complications and good or excellent late outcomes in the group et al.22 evaluated 350 patients who underwent surgical Heller
of major surgeries, there is a relatively low impact of morbidity/ cardiomyotomy for advanced sigmoid megaesophagus with
complications compared to good or excellent late outcomes for the following late results rates: complication, 8.0%; mortality,
major surgeries, with a RR of 0.49 (p=0.01, 95%CI 0.27–0.86). 0.8%; retreatment requirement, 12.8%; and 76.2% probability
The comparative analysis between mortality and good or of results considered good or excellent after this surgical
excellent late outcomes in the group of major surgeries also procedure. It was concluded that this surgical modality is
shows that there is a low impact of mortality compared to the acceptable as definitive treatment for patients with advanced/
late outcome, with a RR of 0.05 (p<0.00001, 95%CI 0.03–0.08). sigmoid megaesophagus, because it avoids esophagectomy, has
When comparing these two groups, it can be concluded low morbidity and mortality rates and low rates of retreatment
that they both present similar results from their treatments, requirements22.
with a low impact on morbidity and mortality and a tendency In a similar review, Herbella and Patti14, in an assessment
to favorable late outcomes. The RR of complications in relation of 122 patients in eight studies, found an average of 79% good or
to a favorable late outcome in the cardiomyotomy group was excellent late results without any associated mortality, in patients

ABCD Arq Bras Cir Dig 2024;37e1809 7/9

REVIEW ARTICLE

with advanced megaesophagus undergoing Heller cardiomyotomy. This is probably due to the low frequency of the disease in
They concluded that laparoscopic Heller cardiomyotomy question. Furthermore, there is a heterogeneity of the studies
is a viable option as a definitive treatment for advanced discriminated. Also, different modalities of evaluation and
megaesophagus, with relief of dysphagia in a significant number classification of terminal achalasia/advanced megaesophagus,
of patients, the possibility of use in more fragile patients, in different periods of evaluation of late results and different
addition to preventing or hindering the possible indication of modalities of evaluation of outcomes used such as questionnaires,
esophagectomy in the future14. classifications (Brandt, Eckardt), evaluation of the dysphagia
Panchanatheeswaran et al.26 concluded that this surgical symptom and levels of personal satisfaction.
modality should be considered the first therapy line for patients with Given the findings of this review study, randomized clinical
sigmoid megaesophagus. They also suggest that esophagectomy trials are required to confirm them. It is not possible to determine
should be reserved for cases of cardiomyotomy failure26. the best profile of patients with advanced megaesophagus
Costantini et al.7, who included in their work 1,001 patients indicated for major surgery; however, it is estimated that they
with all-grade achalasia who underwent Heller-Dor laparoscopic constitute a small portion of this patients’ population.
surgical cardiomyotomy, concluded that there is a high probability
of dysphagia relief in around 80% of these patients even 20 years
after the procedure. Furthermore, they concluded that surgical
complications are rare and that recurrences can be treated in CONCLUSIONS
most cases endoscopically, through dilation, besides obtaining
acceptable rates of late reflux. On the other hand, they claim This systematic review with meta-analysis allows us to
that the main predictors of unsatisfactory late results are the conclude that patients with advanced megaesophagus can be
manometric pattern of achalasia, type III, the presence of safely treated with laparoscopic Heller cardiomyotomy with
sigmoid esophagus (2.5 odds ratio) and a high chest pain score7. fundoplication. This surgical modality, which encompasses
The recurrence of symptoms after esophageal cardiomyotomy less complex abdominal surgery, presents high symptom
requires thorough evaluation, as pointed out by Orlandini et al.21 resolution rates, low complication rates, low mortality rates
and Tustumi et al.37. The rationale for classifying the condition as and satisfactory results. Caveats must be considered regarding
“persistent”, “early recurrence” and “late recurrence” is suggested, the late long-term outcome.
which should help guiding the diagnosis and treatment of those Even so, the present study indicates a favorable indication
patients. Clinical history data and exams such as the esophagram for the challenging surgical treatment of this complex
and upper gastrointestinal endoscopy (UGE) are essential in a disease. This fact can certainly guide the surgeon in his/her
logical approach that can encompass diagnoses ranging from decision making.
incomplete myotomy and very tight or migrated antireflux valves
to neoplasia or even disease progression (megaesophagus).
With reservations about the individuality of conduct in each
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