Published online: 2020-03-08

Review Article

Thoracoscopic Lobectomy for Congenital Pulmonary

Airway Malformation: Where Are We in 2019?
Arnaud Bonnard1

1 Robert Debré Children University Hospital, APHP, Paris University, Address for correspondence Arnaud Bonnard, MD, PhD, Robert
Paris, France Debré Children University Hospital, APHP, Paris University, 48
Boulevard Sérurier, 75019 Paris, France
Eur J Pediatr Surg (e-mail: [email protected]).

Abstract Thoracoscopic surgery for congenital pulmonary airway malformation (CPAM) is still a
matter of debate and used by approximately 50% of the surgeons in Europe. Several
questions need to be addressed about CPAM. The adequate treatment, the surgical
approach, and the follow-up are few of them. A review of recent articles published in
the literature over the past 5 years is done in trying to respond to these questions. A
multidisciplinary team is required to follow these patients since approximately 10 to

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15% will develop a chronic lung disease and asthma. In the case of conservative
Keywords management, computed tomography scan should be perform to monitor the evolu-
► CPAM tion of the CPAM. Minimally invasive surgery should be used whenever possible,
► thoracoscopic although the advantages of pulmonary function tests are not clearly defined, allowing a
► follow-up postoperative fast-track management.

Introduction training of surgeons, the minimally invasive approach appears

underused.
The state of play in congenital pulmonary airway malforma- Before making any judgment, we must ask: is there an
tion (CPAM) has been recently reported. advantage in using the thoracoscopic approach? Can a difficult
A survey on 181 European Paediatric Surgeons’ Association case, like bronchiectasis, be done with this approach? Is there
members (91% senior) from 48 countries was conducted com- any change in pre- or postoperative care to the time of
pleting an online questionnaire.1 Seventy five percent of minimally invasive surgery (MIS)? What follow-ups are need-
respondents operate on asymptomatic patients, 18% before ed for patients operated on by a thoracoscopic approach?
6 months of age, 62% between 6 and 12 months of age, and
20% after 12 months of age. Risk of infection, cancer, and
General Consideration
symptom development were the main reasons for justifying
resection in asymptomatic patients. Sixty-three percent pre- As reported above, less than half of the pediatric surgeons
ferred a thoracotomy while lobectomy was the preferred declared that they use the MIS approach for lobectomy. MIS for
procedure (58% respondents). The heterogeneity of the answers lobectomy has been demonstrated to have the same 30-day
shows that the debate remains open. These results have been readmission rate as the open approach.3 In univariate analysis,
confirmed in 2016 showing that utilization of the thoracoscopic it was associated with a lower rate of postoperative complica-
approach increased from 32.2% in 2008 to 48.2% in 2012 in the tion and a shorter hospital stay. However, these results did not
United States.2 A smaller age at surgery, the need for lobectomy, remain in multivariate analysis. The low percentage of pediatric
and difficult condition were naturally associated with a lower surgeons using the thoracoscopic approach might be related to
percentage of surgeon using the thoracoscopic approach. While three reasons.4 First, there is a low incidence of CPAM to get
attitudes are often linked to the surgical heritage and the adequate training and build thoracoscopic confidence for

received © Georg Thieme Verlag KG DOI https://doi.org/

January 7, 2020 Stuttgart · New York 10.1055/s-0040-1702221.
accepted ISSN 0939-7248.
January 10, 2020
Thoracoscopic Lobectomy for CPAM Bonnard

pediatric surgeons. Second, the anatomic approach to dissec- emia or hypercapnia and postoperative complications. Closed
tion is different compared with open surgery. Third, most of the monitoring during surgery should be done and near-infrared
time the sizes of the instruments are not appropriate for a small spectroscopy is certainly helpful. This has been addressed for
child, making the minimally invasive approach difficult for the esophageal atresia and diaphragmatic hernia repair,9 but it can
surgeons who like to use a mechanical stapler and do not have a be generalized for all thoracoscopic procedures.
5-mm stapler available. This is a part of the reasons that some
authors reported a combined approach.
What Instruments Should Be Used to Help
the Resection?
Complete Thoracoscopic Procedure or
The presence of a completely fused fissure is a challenge,
Combined Approach?
particularly during MIS resections. A comparison study
Some authors reported the need for a combined approach. This between patients who underwent a thoracoscopic lobectomy
is particularly useful especially at the beginning of the experi- with complete fissure, partially fused fissure, or totally fused
ence in MIS for CPAM. In video-assisted resection, an incision fissure found that blood loss was higher with a fused fissure.10
approximately 5 to 6 cm is performed and two 5-mm ports are A stapling device or a thermofusion device seems to be more
inserted for optics and lighting. Comparison between a com- appropriate in these difficult cases. A comparison between
plete MIS approach and a combined approach has shown that Ligasure (Covidien, Mansfield, Massachusetts, United States)
the mean operative time was longer in the complete-VATS and Enseal (Ethicon Endo-Surgery, Inc., Cincinnati, Ohio,
group (247–81.7 vs. 188.3–41.1 minutes, p ¼ 0.063), but this United States) devices showed that the Enseal device was
was not significant.5 Intraoperative bleeding has been associated with less air leakage leading the authors to advocate

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reported to be less (45.2–56.1 vs. 150.7–153.2 g, p ¼ 0.047), this device.11
and hospital stay shorter (5.8–2.0 vs. 9.0–2.4 days, p ¼ 0.0043) A 5-mm stapling device is available now which allows
in the complete MIS approach group than in the combined one. controlling safely in the bronchus or major pulmonary
The same technic has been also reported using an Alexis vessel.4 Appropriate use is necessary and the authors recom-
wound retractor which can help in performing the procedure.6 mend not taking too much tissue enclosed in the jaws of the
The median operative time was reported to be fairly long stapler to avoid an inadequate seal. All such devices might
(199.5 minutes, range: 112–507 minutes) and the conversion provide help and improvement in thoracoscopic lobectomy
to open surgery rate was 37.5%. Although it is a good approach, but the number of patients operated on will remains the only
this could be the best way to use in a nonexperienced team as a variable for the pediatric surgeon to improve the outcome. A
transition between an open approach and a complete MIS learning curve of 50 patients to obtain stable postoperative
approach. results has been reported but the 110th case was the turning
point for stable outcomes with a short operation time.12

What Kind of Ventilation Is Required during

Thoracoscopic Lobectomy? Postoperative Management
The one-lung ventilation has been typically employed for a After lobectomy, two major concerns need to be addressed:
thoracoscopic procedure since it offers a better surgical expo- the use of a chest tube and the postoperative analgesia. Chest
sure and an easier pulmonary resection for the surgeon. tubes can induce pain and immobilization, increase risk of
However, this technique is linked to a greater risk of perioper- infection, and impair postoperative ventilation, which can
ative hypoxemia, consequence of the ventilation/perfusion lead to atelectasis and pulmonary infection. The decision to
mismatch, and positioning errors of the bronchial blockers, not drain after lobectomy, whatever MIS or open, should be
making this technic sometimes a real challenge for the anes- reserved for simple cases. An easy lobectomy, or sequestrec-
thesiologist. For this reason, there is an increasing use of tomy, without previous history of infection for a cystic lung
double-lung ventilation. Low tidal volumes and higher respi- disease is a possibility. In a series of 246 thoracoscopic
ratory rates are applied, which combined with a CO2 insuffla- lobectomies without postoperative drainage, 82% developed
tion pressure improve the working space. Theoretically, subcutaneous emphysema, but no pneumothorax.13 A large
double-lung ventilation is more challenging for the surgeon majority of these patients can present subcutaneous emphy-
and is accompanied by a consequent rise of the PaCO2 that is sema which can resolved spontaneously.
tolerable and acceptable up to 55 mm Hg.7 Improvement in postoperative analgesia and postoperative
A randomized trial comparing lung-protective ventilation pain management now allows using a fast-track pathway in
(LPV) using a control ventilation with positive end-expiratory thoracoscopic lobectomy. The patient can be discharged home
pressure to a single-lung ventilation (SLV) found LPV showed a the day after the surgery without impacting the rate of com-
lower rate of complications compared with the SLV group (25.5 plications.14 Starting a program of thoracoscopic resection for
vs. 9.1; odds ratio: 0.29; 95% confidence interval: 0.10–0.88; CPAM requires the participation of a multidisciplinary team
p ¼ 0.02).8 Complications were defined by infection, pleural including anesthesiology, surgery, pediatrics, physiotherapy,
effusion, or atelectasis. Nevertheless, an experienced anesthe- and pneumology staff. Morbidity and mortality are very low,
sia team is required for a thoracoscopic procedure. This might and a sufficient number of patients are needed to offer a safe
help to avoid any preoperative complications such as hypox- and quick recovery on the children.

European Journal of Pediatric Surgery

 Thoracoscopic Lobectomy for CPAM Bonnard

How Does Thoracoscopy Impact Pulmonary tional age of 36.2 weeks was reported.20 Fourteen (66,7%)
Function? patients were intubated for respiratory distress at a median
age of 7.5 days. The CPAM volume ratio (CVR) measured
Pulmonary resection via conventional open thoracotomy prenatally was 1.4  1.0. Thirty percent developed pulmonary
may be performed using a muscle sparing technique, but hypertension postoperatively and 25% required venovenous or
the concern is that the open technique risks subsequent venoarterial extracorporeal membrane oxygenation (ECMO)
scoliosis or winging of the scapula. Does the thoracoscopic for a median of 10 days. Among symptomatic neonates, univar-
(MIS) approach really matter and is it better over an open iate analysis revealed a significant correlation between the
technic regarding the lung function test? length of hospitalization and the gestational age at birth
In a preliminary comparative study between 15 patients (p ¼ 0.001) as well as birthweight (p ¼ 0.002). There was no
who underwent thoracoscopic lobectomy and a control group, association between the length of hospitalization and other
those who underwent thoracoscopic lobectomy had a normal patient characteristics, including hydrops status, CVR, ECMO
lung function 5 years after the operation.15 A recent paper with utilization, and diagnosis. With a median follow-up of 35.5
a 7-year follow-up compared the open and the MIS approach months (interquartile range: 19.0–80.8), all children were free
on the results of pulmonary function tests.16 The mean age at of supplemental oxygen. Eleven (55%) children presented
operation was similar (20.0  13.3 and 16.6  8.5 months, with chronic morbidity, with half of them requiring inhaled
range: 1 month to 6 years) respectively between the open bronchodilators to treat ongoing asthma and chronic lung
and the thoracoscopic group. The thoracoscopic group showed disease. Both the maximum CVR and duration of mechanical
a better performance in forced vital capacity (FVC; 98.9 vs. ventilation were significant predictors of respiratory morbidity
84.3% predicted, p ¼ 0.03), forced expiratory volume in 1 sec- (p ¼ 0.02 and 0.04, respectively).

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ond (FEV1; 88.5 vs. 76.1% predicted, p ¼ 0.04), and alveolar- Obviously, patients presented with early symptom onset
volume-adjusted diffusion capacity of carbon monoxide (106.4 and even those requiring prenatal intervention for hydrops
vs. 91.4% predicted, p ¼ 0.03). The FEV1-to-FVC ratio, total lung will be at risk for developing chronic lung disease. However,
capacity, and residual volume showed no statistical difference. follow-up should not be concentrated on this specific popula-
This difference may be related to less muscle disruption with tion. In our experience and from nonpublished data, approxi-
the MIS approach. Pleural fusion and postoperative adhesion mately 10% of the patients operated on for asymptomatic
between the remaining lobe and the thoracic cavity could also pulmonary lesion will develop chronic lung disease requiring
have a role in these results. This has been confirmed very treatment.
recently in an another paper published on patients enrolled in a Regarding neurodevelopment in the same population of
prospective cohort study and stratified in two groups between patients needing a surgery at birth or even a prenatal inter-
those operated on for complications and those treated with a vention, a study has also been recently reported.21 In 45
conservative management.17 Children with congenital lung patients, 28.9% were treated prenatally with either a surgical
malformation may be at risk for reduced lung function and resection or an ex utero intrapartum treatment (EXIT) proce-
exercise tolerance, especially those who required surgery. The dure and 71.1% were operated on within the first month of life
major bias of this study is that patients were mainly operated for respiratory distress related to the malformation. The mean
by an open approach (20 out of 29 patients), which reinforces age of the study group at the time of the neurologic assessment
the idea that MIS should be used when possible for these was 19.3  10.3 months (range: 5–42 months). Eleven percent
patients. Moreover, MIS has been reported to be possible and were diagnosed with a hearing loss, one patient who required
safe and this can be performed even if the patient presented ECMO presented with seizure related to a subdural hemor-
with preoperative pulmonary infection or abscess, but more rhage, and ischemic lesion, neuromuscular hypotonia (35.6%),
experience on it would be needed.18 hypertonia (4.4%), and delayed motor coordination (11%) were
The recent improvement in postoperative care, especially also found. About 64% patients presented with neurologic or
in postoperative analgesia, combined with the thoracoscopic neurosensitive symptoms. This is probably due to a relatively
approach may help in reducing the hospital stay and follow- aggressive attitude, especially prenatally with four fetal lung
ing a fast-track pathway for resection of CPAM.14 This has resections and nine EXIT procedures, which are certainly a
never been reported with thoracotomy. major cause of sequelae. The superiority of steroid treatment
Furthermore, the MIS approach has been shown to be over fetal surgery on survival rate has been previously noted in
reproducible for trainees provided they are properly men- the year 2012.22
tored.19 Encouraging a culture of safety and raising awareness Patients were also evaluated on their developmental evolu-
of error traps in pediatric thoracoscopy to minimize potential tion at 5 months of age, with regular follow-up visits at 12, 24,
harm and improve quality of care is important. 36, and 42 months. Bayley Scales of Infant Development-third
edition (BSID-III) was used, providing a composite score for
cognitive, language, and motor outcomes.23 The normalized
What Is the Impact of CPAM Resection Early
population mean and standard deviation of each composite
in Life?
score was 100  15. The results demonstrated that those chil-
Does very early CPAM resection impact chronic pulmonary dren with high-risk CPAM are at low risk for neurodevelop-
disease? A retrospective study on 21 neonates treated surgically mental delays during the first 3 years of life with most of them
for congenital lung malformation at birth at a median gesta- presenting functions within the average range. Nevertheless,

European Journal of Pediatric Surgery

Thoracoscopic Lobectomy for CPAM Bonnard

presenting below-average scores suggests a prolonged follow- tive, single-centre, randomised controlled trial. Br J Anaesth
up is justified in this population. 2019;122(05):692–701
9 Costerus S, Vlot J, van Rosmalen J, Wijnen R, Weber F. Effects of
neonatal thoracoscopic surgery on tissue oxygenation: a pilot
Conclusion study on (neuro-) monitoring and outcomes. Eur J Pediatr Surg
2019;29(02):166–172
The management of CPAM is a matter of debate. No random- 10 Murakami H, Koga H. Lane G, Hirayama S, Suzuki K, Yamataka A.
ized control trial exists to address the different questions of Does fissure status affect the outcome of thoracoscopic pulmo-
either aggressive or conservative management, timing of nary lobectomy? Pediatr Surg Int 2019;36(01):57–61
11 Koga H, Suzuki K, Nishimura K, et al. Comparison of the value of
surgery, follow-up, and the best surgical approach. This
tissue-sealing devices for thoracoscopic pulmonary lobectomy in
review suggests that about one-quarter of children will
small children: a first report. Pediatr Surg Int 2014;30(09):
develop symptoms, mainly infectious, which will need sur- 937–940
gical resection. In the case of conservative management, a 12 Park S, Kim ER, Hwang Y, et al. Serial improvement of quality
long-term follow-up is mandated, but the frequency of metrics in pediatric thoracoscopic lobectomy for congenital lung
computed tomography scan use is at the discretion of the malformation: an analysis of learning curve. Surg Endosc 2017;31
(10):3932–3938
surgeons. A multidisciplinary team would be better to follow
13 Cheng K, Yuan M, Xu C, Yang G, Liu M. A chest tube may not
these children after surgery since approximately 10 to 15% necessary in children thoracoscopic lobectomy. Medicine (Balti-
will exhibit a chronic lung disease and various degree of more) 2019;98(26):e15857
asthma. Minimally invasive techniques should be employed 14 Clermidi P, Bellon M, Skhiri A, et al. Fast track pediatric thoracic
where possible; however, these can be challenging cases, and surgery: toward day-case surgery? J Pediatr Surg 2017;52(11):
mentoring is a best way to avoid pitfalls and severe 1800–1805

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15 Lau CT, Wong KKY, Tam P. Medium term pulmonary function test
complications.
after thoracoscopic lobectomy for congenital pulmonary airway
malformation: a comparative study with normal control.
Conflict of Interest J Laparoendosc Adv Surg Tech A 2018;28(05):595–598
None declared. 16 Lau CT, Wong KKY. Long-term pulmonary function after lobecto-
my for congenital pulmonary airway malformation: is thoraco-
scopic approach really better than open? J Pediatr Surg 2018;53
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European Journal of Pediatric Surgery