Seminars in Pediatric Surgery 23 (2014) 270–277

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Seminars in Pediatric Surgery

journal homepage: www.elsevier.com/locate/sempedsurg

Congenital lung malformations: Informing best practice

Robert Baird, MDCM, MSc, FRCSC, FACSa, Pramod S. Puligandla, MD, MSc, FRCSC, FACS, FAAPa,b,
Jean-Martin Laberge, MD, FRCSC, FACS, FAAPa,n
a
Department of Pediatric Surgery, McGill University, Montreal Children's Hospital, McGill University Health Center, Montreal, QC, Canada
b
Department of Pediatrics, McGill University, Montreal Children's Hospital, McGill University Health Center, Montreal, Quebec, Canada

a r t i c l e in f o abstract

The management of congenital lung malformations is controversial both in the prenatal and postnatal
Keywords: periods. This article attempts to inform best practice by reviewing the level of evidence with regard to
Congenital lung lesions prenatal diagnosis, prognosis, and management and postnatal management, including imaging, surgical
Congenital pulmonary airway indication, surgical approach, and risk of malignancy. We present a series of clinically relevant
malformations statements along those topics and analyze the evidence for each. In the end, we make a plea for an
Cystic adenomatoid malformation adequate description of the lesions, both before and after birth, which will allow future comparisons
Sequestration between management options and the initiation of prospective registries.
Pleuropulmonary blastoma
& 2014 Elsevier Inc. All rights reserved.

As with many surgically correctable congenital abnormalities,
the management of congenital lung malformations is based largely
on historical precedent and “best-guess” rationale. Within the last
decade, however, many publications have attempted to clarify the
prenatal and postnatal controversies surrounding best practice.
This review utilizes the well-validated grading of recommenda-
tions assessment, development and evaluation process (GRADE) to
critically evaluate the available literature. GRADE takes into
account the quality of available evidence to inform the strength
of generated recommendations and has emerged as the consensus
methodology to evaluate evidence.1
As described by the GRADE publications, the quality of the
evidence (QOE) will be expressed from high to very low, based on
limitations of study design and bias, heterogeneity of results,
indirectness of evidence (e.g., applying adult findings to pediatric
patients), imprecision of results, and the potential for publication
bias. Due to the absence of applicable randomized controlled trials
(RCTs) evaluating the care of patients with congenital lung lesions,
the approach to rating the available observational data has been
adapted from GRADE to exclude RCTs, as shown in Table 1.2
Recommendations are subsequently based on the quality of
evidence but also on the published magnitude of effect, the
balance between desirable and undesirable side effects, the
variability in patient and family values (e.g., cultural acceptability
of prenatal intervention), and cost-effectiveness. For the purposes
n
Correspondence to: Department of Pediatric Surgery, McGill University, Mon-
treal Children's Hospital, McGill University Health Center, 2300 Tupper St, Suite
C-820, Montreal, Quebec, Canada H3H 1P3.
E-mail address: jean-martin.laberge@muhc.mcgill.ca (J.-M. Laberge).
of this review, recommendations are not restricted solely to
questions surrounding therapeutic options but have also been
applied to questions pertaining to diagnosis and prognosis. All
recommendations have been characterized as Agree, Disagree, or
Neutral and qualified as being Weak or Strong, as demonstrated in
Figure 1. Thereafter, the QOE is reported in accordance with
GRADE convention.
Questions of nomenclature have been extensively reviewed in
previous publications.3 For the purposes of this review, we will
continue to use the term congenital cystic adenomatoid malfor-
mation (CCAM), which encompasses the newer term congenital
pulmonary airway malformation (CPAM), when histologic confir-
mation exists. Other common lung malformations include pulmo-
nary sequestration (PS), which may be intralobar (ILS) or
extralobar (ELS), and bronchial atresia. The term congenital lung
malformation (CLM) comprises all fetal lung pathologies without
relying on histologic diagnosis and will be used to refer to both
prenatal and postnatal lesions.
Diagnosis and management of lung malformations in utero
Those who interpret prenatal imaging should not attempt to
make a histological diagnosis. CCAM, PS, bronchial atresia, and
lobar overinflation all have overlapping features. The presence of a
systemic arterial supply to the abnormal lung is generally thought
to be associated with PS, but many examples of hybrid lesions,
with features of CCAM and sequestration, have been described.4,5
Furthermore, the absence of an obvious systemic arterial supply does
not eliminate the possibility of a PS. Therefore, the generic terms

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 R. Baird et al. / Seminars in Pediatric Surgery 23 (2014) 270–277 271

Table 1 been shown to involute or resolve later in pregnancy. Adzick,4

GRADE tool for grading the quality of evidence, adapted to reflect the lack of based on the Children's Hospital of Philadelphia experience with
randomized controlled trials.
more than 600 fetal lung lesions, reported a 68% rate of marked
Modify down Modify up Quality of evidence regression of PS before birth, compared to 15% regression of CCAM,
most of which remained detectable by postnatal CT scan. Cavoretto
Observational study (low rating) et al.,11 based on the King's College experience and a literature
Risk of bias Large effect High þþþþ review, reported a 50% antenatal regression of PS and a similar
 1 Serious þ 1 Large
 2 Very serious þ 2 Very large
regression rate for non-hydropic echogenic “CAM,” with 63% of
Inconsistency Dose response Moderate þþþ these latter resolutions being confirmed on postnatal CT scan or
 1 Serious þ 1 Evidence of graduated MRI. There is currently insufficient evidence to accurately predict
 2 Very serious response the behavior of a CLM in utero. True resolution of a lesion (no
Indirectness All plausible confounders Low þþ
evidence on postnatal CT scan), typically seen with microcystic
 1 Serious þ 1 would reduce effect
 2 Very serious þ 1 Would suggest a spurious lesions, is uncommon and may indicate that the prenatal lesion
effect if none seen was a localized bronchial obstruction with secondary fluid
Imprecision Very low þ retention.11
 1 Serious
 2 Very serious
Publication bias Statement 3
 1 Likely
 2 Very likely Fetal hydrops associated with CLM is associated with a high
mortality rate. Fetal intervention or preterm delivery is indicated.
Adapted with permission from Guyatt et al.2
Recommendation: Strongly agree; QOE: Poor–very poor.
Hydrops associated with CLMs has been described in 5–30% of
“congenital lung lesion” or “congenital lung malformation” (CLM)
cases, depending on the referral pattern of the reporting institu-
should be used in the prenatal period, and malformations should be
tion.4,11 Survival without treatment after the onset of hydrops is
described as macrocystic, microcystic (echogenic), or mixed lesions
the exception, rather than the rule, with scattered case reports
based on their sonographic appearance.3,6 Furthermore, features
throughout the literature.10 Indeed, Adzick et al.12 in 1998 reported
including the size, volume, location, appearance, and the presence
25 patients that developed hydrops in association with a CLM with
of a systemic arterial supply, mediastinal shift, pleural effusion, ascites,
no survivors. Importantly, subsequent series investigating CLMs
or other signs of hydrops should be given to complete the ultrasound
with hydrops have demonstrated an improved fetal survival rate
report. In instances when the diagnosis is unclear, fetal MRI may be
with a combination of prenatal intervention and/or preterm
useful and has been used routinely in some centers.4,7
delivery. This body of literature is comprised of multiple case
series and comparisons to historical controls.6,8,10,13,14 Despite the
Statement 1 lack of contemporary comparative or randomized studies, the
recommendation to provide antenatal care is reasonably based
Most congenital lung malformations have a favorable progno- on the extremely poor prognosis without intervention.
sis, with the majority of neonates being asymptomatic at birth. Multiple options exist for antenatal care including invasive and
Recommendation: Strongly agree; QOE: Moderate. non-invasive strategies (i.e., maternal steroids—see Statement 5).
While a recent review has summarized much of the embryol- Currently, no recommendations can be made regarding the
ogy and pathophysiology of congenital lung lesions,8 several areas supremacy of one invasive option versus another. Factors that
of controversy still exist, including the prognosis associated with a should inform the choice of intervention include the gestational
prenatally diagnosed lung lesion. An early report demonstrating age of the patient, the position of the fetus and placenta, the
that 40% were lethal in utero9 led some centers to recommend presence/absence of macrocysts, the presence/absence of a high-
pregnancy termination simply because of mediastinal shift or flow vascular component as well as individual and institutional
polyhydramnios. However, most authors now agree that the expertise. Finally, the values of parents and caregivers must be
majority of pregnancies with an affected fetus have an excellent accounted for during prenatal therapy decision-making, given the
outcome. After eliminating rare cases of therapeutic abortions (usually uncertainty surrounding outcomes. In instances when fetal lung
indicated because of concomitant pathology), both cystic lesions and maturity is felt to provide a reasonable chance of postnatal
pulmonary sequestrations have a survival rate 495%, as consistently survival before and after definitive care of the CLM, preterm
demonstrated throughout multiple case series.4,10,11 The risk of pub- delivery remains one of the treatment options, although conclu-
lication bias for this epidemiological information is small, and the QOE sive evidence regarding this strategy is scant. In instances when
is modified upwards by the large number of cumulative patients. fetal demise appears likely prior to postnatal viability, available
therapeutic options include repeated cyst aspiration and thoraco-
Statement 2 amniotic shunting for primarily cystic lesions as well as for pleural
effusion secondary to PS. A systematic review has supported the
Prenatal regression may be more common in pulmonary efficacy of this approach, but only for fetuses with evidence of
sequestration (PS) than in cystic lung lesions/CPAMs. Recommen- hydrops.15 More recent publications have advocated this
dation: Neutral; QOE: Poor. approach for large lesions prior to the development of hydrops.11,14
The natural history of prenatal lung lesions remains difficult to Fetal lobectomy has been employed successfully for microcystic
predict. While many will undergo a proliferative phase during the lesions in fetal centers of excellence,4 while resection on placental
second trimester, a significant number of prenatal lesions have circulation or on ECMO (EXIT-to-resection and EXIT-to-ECMO) has

Strongly posive Weakly posive Neutral Weakly negave Strongly negave

Do it Maybe Do it Unsure Probably shouldn’t Don’t

Fig. 1. GRADE recommendations, indicating the direction and strength. (Adapted with permission from Guyatt et al.2)

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272 R. Baird et al. / Seminars in Pediatric Surgery 23 (2014) 270–277
been described in rare instances.16,17 Additional options include
radiofrequency or laser ablation, as well as percutaneous
ultrasound-guided sclerotherapy.3 Ruano et al.'s18 experience and
review suggests that laser ablation of the feeding vessel (as is
possible in PS) is superior to intraparenchymal laser for micro-
cystic lung lesions with hydrops. These options will likely continue
to be primarily represented as single-center case series for the
foreseeable future. Publication bias and the heterogeneity of
patients and results undermine the generalizability of published
outcomes. The recent review by Whitlox et al. evaluating all forms
of prenatal intervention for CLMs illustrates the danger of pub-
lication bias: the summary postnatal survival rate of patients with
PS and effusions after intervention is 92%, with CCAM without
hydrops 87%, and CCAM with hydrops is 69%. These numbers likely
reflect a “rose-colored” reality by failing to account for unpub-
lished poor outcomes.13
Statement 4
The CVR has emerged as a useful prognostic tool and appears
reproducible and widely accepted. Recommendation: Strongly
agree; QOE: Moderate.
Since many congenital lung lesions appear to regress in utero
while others progress to hydrops, a prognostic tool that allows
closer monitoring of fetuses at risk was developed.19 The CCAM
volume ratio (CVR) measures the volume of the lung lesion,
divided by the head circumference to normalize for gestational
age. In the initial reports, a CVR 4 1.6 in echogenic lesions was
associated with an increased risk of hydrops. This finding has been
validated by more recent case series,20 although others have
proposed a threshold CVR 42.0.21 These publications have corre-
lated CVR with the development of hydrops and the need for
intervention as opposed to mortality, and favorable test character-
istics have been consistently reported. Recent evidence suggests
that the CVR can also be used to determine which fetuses require
delivery at a high-risk center.22
Statement 5
Prenatal maternal betamethasone administration is indicated
to decrease the size of large microcystic CLMs and reverse hydrops.
Recommendation: Strongly agree; QOE: Poor.
Maternal betamethasone administration has been demon-
strated to induce regression of CLMs and reverse fetal hydrops.
This initial observation was made in second trimester patients
with fetal hydrops who were not candidates for fetal interven-
tion.23 Several subsequent case series have demonstrated an
improved survival rate and decreased CVR when steroids were
administered.24 Although a randomized trial was initiated com-
paring steroids with placebo (NCT00670956), the demonstrated
efficacy of the drug with subsequent case series has threatened the
study equipoise and led to the withdrawal of the investigation. It is
unlikely that higher level evidence will become available to further
characterize the magnitude of benefit of maternal steroids and the
risk of publication bias is high (that patients treated with steroids
with a negative outcome are not reflected in the literature).
Steroids may act at multiple levels, but the mechanism likely
involves decreasing the production of lung fluid and increasing
reabsorption within the CLM, thereby mimicking the third trimes-
ter changes that naturally occur.25 Most authors feel that the
indication for steroid administration is in the context of a micro-
cystic CLM. In fact, steroid administration has rendered fetal
lobectomy for microcystic CLM obsolete in centers that previously
offered it.26 It is unclear whether macrocytic lesions respond to
maternal steroids,27 and a trigger based on an absolute CVR before
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hydrops or “rate of change” of CVR has not been established at
present.
Postnatal management
The classification of congenital lung malformations has been
extensively documented in previous publications and will not be
revisited here.3,6 It must be emphasized, however, that only a
histologic examination can definitively support a diagnosis. Diag-
noses based on imaging alone (whether antenatal or postnatal
without pathologic confirmation) remain speculative, complicat-
ing the interpretation of much of the pre-existing literature.
Furthermore, CT scan remains the gold standard to confirm the
presence or absence of a CLM. Postnatal MRI may be useful for
extralobar PS but has limited capability to accurately assess
parenchymal lung lesions28; simple chest radiographs have long
been shown to be inadequate.29
Statement 1
CLMs present after birth frequently regress and disappear.
Recommendation: Strongly disagree; QOE: Poor.
Most authors who describe the postnatal resolution of CCAM/
CPAM fail to provide the necessary postnatal imaging that defin-
itively documents the presence followed by the disappearance of a
CLM. Others include densities visible on postnatal CT scan that
may represent areas of fluid retention not typical of CCAM.30 There
is currently no conclusive publication that demonstrates the
resolution of a typical cystic (type 1 or 2) CLM confirmed on
postnatal CT scan. There is insufficient evidence to completely
deny the possibility of postnatal resolution of a true cystic lung
lesion as opposed to a localized area of hyperlucency, however
watchful waiting based on the presumption of eventual resolution
appears unjustified. On the other hand, extralobar PS is known to
spontaneously regress and rarely becomes symptomatic, hence
simple observation seems warranted.4,29
Statement 2
Asymptomatic cystic congenital lung malformations should be
resected prior to the onset of symptoms. Recommendation: Weakly
agree; QOE: Moderate.
While the decision-making for children with symptomatic
CLMs is reasonably straightforward, there is ongoing debate
regarding the need for and the timing of surgery in children with
asymptomatic lesions. Tables 2 and 3 provide a summary of these
arguments with relevant evidence for each claim. For those who
support surgical intervention, the safety of pulmonary resection in
infants and children is no longer a source of debate as the
outcomes are generally good. Complication rates after surgery
range between 6% and 9% and are mostly related to prolonged air
leak.31 Mortality is a very rare occurrence in experienced hands
and is thus not a valid outcome measure. The vast majority of
these infants are also extubated immediately following the proce-
dure, and this may be facilitated by the use of regional/epidural
anesthesia.32,33 Pulmonary function, as reported in two small
prospective studies, has also been demonstrated to be within
norms for age after long-term follow-up ( 45 years).34,35 Further-
more, a recent study comparing infants who underwent resection
to those who were observed showed no difference in lung function
at 6 and 12 months of age, despite the fact that all surgical
candidates were symptomatic and that 5/13 of these patients
required neonatal respiratory support.36
Supporters for prophylactic lung resection in asymptomatic
patients with CLMs and ILS also justify their position based on the
 R. Baird et al. / Seminars in Pediatric Surgery 23 (2014) 270–277 273

Table 2
A summary of the purported advantages for the resection of the asymptomatic CLM.

Purported advantages Supporting evidence

Pre-existing malignancy Up to 5% of all “CCAMs” have been shown to harbor cancer in a retrospective series71
Malignant transformation Approximately 1% of CCAMs harbor elements of BAC in a retrospective series and review72
Risk of infection One series demonstrated a 43% of infection at 2 years (n ¼ 21)43; another recent series had 15% 44

Decreased complication rate Higher conversion rate for MIS cases after infection66,67
Decreased length of stay and complications31,39
Compensatory lung growth Normal PFTs after lobectomy after age 5 years (n ¼ 14)34
Superior radionuclide results in children operated on o 1 year (n ¼ 93)40
Spontaneous pneumothorax Rare case reports, impossible to calculate incidence29

risk of developing infection or symptoms during the period of
observation. The risk of infection for congenital lung lesions has
been estimated to range between 10% and 30% within the first year
of life.29,37 The development of symptoms in previously asympto-
matic patients is an additional concern that has been reported to
occur by 7 months of age in a clinically significant proportion of
patients.31,38 The significance of operating on symptomatic
patients should not be underestimated. In several studies, symp-
tomatic patients had significantly more intra- and postoperative
complications and longer hospitalizations compared to infants
who were asymptomatic at the time of operation.31,33,39
Some authors have also advocated for early surgical resection
to take advantage of compensatory lung growth. While still a
source of debate and contention,29,35 compensatory lung growth is
thought to be responsible for the normal pulmonary function
results in children receiving surgical resection early in infancy.34,40
Indeed, both of these studies demonstrated normal residual
volume to total lung capacity ratios (o30%) and reduced emphy-
sematous changes on radionuclide imaging in a majority of
patients, respectively, findings that would not have occurred if
increases in lung volume were due to alveolar distension alone.
Other reasons why surgery has been advocated include the
uncertainty in the diagnosis and presence/absence of malignancy
based on radiology alone (CPAM type I/IV versus pleuropulmonary
blastoma, see below),41,42 the discordance between the presump-
tive radiological and final pathological diagnosis,32 the risk of
pneumothorax or rapid cyst enlargement that may cause adverse
cardio-respiratory events, and the basic contention that these
lesions do not represent variants of normal and thus should be
removed 29,32,41,43 (Figure 2).
Despite the above arguments, the need for surgery is not a
universally held opinion. Recently, Ng et al.44 retrospectively
reviewed 74 infants with an antenatal diagnosis of CLM who were
managed expectantly, 72 of whom proceeded to live delivery.
Although the authors report only a 5% rate of “symptoms requiring
surgical resection” after a median follow-up of 5 years, only 53% of
asymptomatic lesions were consistent with CCAM on postnatal CT
imaging, with the other patients having either a normal CT (7%), a
PS (29%), or CLE (7%), lesions that many authors would also have
observed 29 (Figure 3 and Table 4). Furthermore, follow-up data in
this study appears to have been obtained from hospital records
rather than by direct contact with patients, which leaves doubt
regarding the true proportion of patients who remained asympto-
matic. Another study advocating the safety of expectant
management of asymptomatic CCAMs relied on regional databases
and hospital coding to exclude the possibility that complications
resulting in surgery would have been missed given a “relatively
static population” in their geographical region.45
Another argument often mentioned against prophylactic resection
of cystic lung lesions is the ineffectiveness of preventing the
subsequent development of malignancy. Papagiannopoulos et al.46
reported a neonate presenting with a pneumothorax associated with
a large CLM that underwent lobectomy who subsequently presented
with pleuropulmonary blastoma (PPB) at the age of 2.5 years. Seven
additional cases of malignancy AFTER lung resections were subse-
quently identified, with most patients having undergone a formal
lobectomy. Unfortunately, details of histology are lacking, but the
short duration between the time of operation and the subsequent
diagnosis of cancer (mean ¼ 21 months) suggests the possibility that
cancer existed at the time of original resection. These cases likely
represent primary PPBs with initial inadequate treatment as opposed
to subsequent malignant transformation of a CCAM.41 Indeed,
malignant transformation, as observed with delayed presentations
of bronchioloalveolar carcinoma (BAC), an entity distinct from PPB,
occurs later and has been linked to chronic inflammation and
mucinous cell precursors found in type 1 CCAMs.29,47,48 In addition,
PPB is known to occasionally present as synchronous or metachro-
nous bilateral disease. An excellent overview of PPB exists for
physicians and families alike (http://www.ppbregistry.org/).
There is currently insufficient evidence to definitively recommend
prophylactic surgery for cystic CLMs.31 While clinical evidence of
compensatory lung growth is indeterminate, the evaluation of the
published epidemiology of risks with either surgery or watchful
waiting suggests that the long-term risks of malignancy and infection
outweigh the short-term increased risk incurred with surgery.
Indeed, surgery on symptomatic patients is associated with signifi-
cantly higher complication rates that can be avoided with elective
surgery in asymptomatic patients. In the end, it is clear that the
debate regarding the indications for surgery and its timing compared
to simple observation will continue until long-term outcome studies
involving large numbers of patients can be completed.49–51
Recent studies44,45 provide us with a preliminary experience of
expectant management, but more stringent long-term follow-up
into adulthood will be necessary to fully assess the risk of delayed
complications such as infection, pneumothorax, and BAC. Further-
more, risks related to repeated radiological studies including CT
scans will have to be considered.52 Nonetheless, based on the
current uncertainty regarding the natural history of these lesions,

Table 3
A summary of the purported disadvantages for the resection of the asymptomatic CLM.

Purported disadvantages Supporting evidence

Operative mortality Systematic review of operations for asymptomatic patients, one death (n ¼ 436)31
Operative morbidity 5% Rate of morbidity after operations for asymptomatic patients, primarily prolonged air leaks31
Cancer despite excision Eight discrete events although previous resections unclear and short interval between excision and cancer diagnosis46

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274 R. Baird et al. / Seminars in Pediatric Surgery 23 (2014) 270–277

Fig. 2. RESECT. A fetus was diagnosed with a large mixed solid/cystic lung lesion
filling 80% of the thoracic cavity at 19 weeks gestation. Despite severe mediastinal
shift and inversion of the diaphragm, hydrops did not develop and the lesion
gradually regressed after 24 weeks gestation. The baby was asymptomatic at birth.
A mild mediastinal shift on initial chest radiograph disappeared by day 6 (A).
Although the baby remained asymptomatic at 3 weeks of age (B and C), the fear of
sudden enlargement with respiratory distress led to an early elective lobectomy at
5 weeks of age. She was discharged 4 days after surgery. Microscopic examination
revealed a type 2 CCAM. In retrospect, such a lesion was indistinguishable from a
type 1 PPB.41 (Adapted with permission from Laberge et al.29)
Fig. 3. OBSERVE. A fetus with an echogenic left lower chest mass with evidence of
a systemic arterial supply was followed up uneventfully until birth, with apparent
resolution of the mass in the third trimester. Chest radiograph at 6 days of age was
normal (A). CT scan at 6 months (B and C) clearly showed an extralobar
sequestration (non-aerated), with systemic arterial supply and venous return.
The child is asymptomatic, with no significant shunting.

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 R. Baird et al. / Seminars in Pediatric Surgery 23 (2014) 270–277 275

Table 4 Table 6
Management guidelines for congenital lung malformations. (Adapted with permis- Summary of recommendations for CLMs in the postnatal period.
sion from Laberge et al.29)
Statement Recommendation Quality of
Operate All bronchogenic cysts evidence
All aerated sequestrations (intralobar)
All lesions with visible cysts on CT scan Asymptomatic cystic congenital lung Weakly agree Moderate
Observe Asymptomatic CLE malformations should be resected prior
ELS without significant shunting to the onset of symptoms.
Unclear Segmental hyperlucency Formal lobectomy is superior to Weakly agree Very poor
segmentectomy in the treatment of
CLE¼ congenital lobar emphysema; ELS ¼ extralobar sequestration. unilobar CLMs.
Thoracoscopy is a safe and feasible option Neutral Poor
in selected children with CLMs,
informed consent must include detailed discussions that describe particularly those without previous
both surgical and conservative/observation strategies and the infection.
current state of evidence. CLMs present after birth frequently Strongly disagree Poor
regress and disappear.

Statement 4

While open thoracotomy should be considered the standard

Formal lobectomy is superior to segmentectomy in the treat-
approach for any child undergoing a lung resection, the emergence
ment of unilobar CLMs. Recommendation: Weakly agree; QOE:
of advanced minimally invasive techniques has led to the increas-
Very poor.
ing use of thoracoscopy. In addition to obvious cosmetic advan-
Formal lobectomy has been the traditional standard for the
tages, advocates report that the magnification provided by
resection of parenchymal CLMs. However, lung-sparing strategies
thoracoscopy allows for significantly improved discrimination
such as segmentectomy have been advocated for these lesions,
between the normal and diseased lung, as well as improved
even by thoracoscopy.53,54 These techniques have an intuitive
visualization of fissures and vascular structures.
appeal in the young, growing child. However, the risks and
Since the first publication by Albanese et al.59 in 2003, several
consequences of incomplete resection may only be identified
reports have described the feasibility and safety of thoracoscopic
because of postoperative complications, after final pathology
lung resections, including a meta-analysis.54,60 Neonatal thoraco-
review or on postoperative CT scanning.29,55 This approach may
scopic lobectomy has also been shown to be associated with
be more applicable for smaller, well-defined segmental lesions and
minimal morbidity. Indeed, Rothenberg et al.61 described their
in children with bilateral or multilobar disease.54,56 Many authors
experience in 75 asymptomatic patients weighing less than 10 kg
still recommend lobectomy for the majority of parenchymal CLMs
who had prenatally diagnosed congenital lung lesions, a much
to prevent postoperative air leaks, residual disease, and late
more challenging subset of patients due to their smaller size.
malignancy.43,57,58 The systematic review by Stanton et al.31 dem-
These results have been supported by others groups around the
onstrated a 15% rate of residual disease after segmental resection
world with conversion rates generally ranging from 0% to 14%62–64
as opposed to 0% with lobectomy. Thus, while there are a small
but as high as 70% in one series when lung-sparing surgery was
number of cases of residual disease in the literature as a whole and
performed.65 Prior infection has been shown to increase the
publication bias is likely, there is adequate evidence to recommend
complication and conversion rate.66,67 Patients with congenital
a formal lobectomy for typical cases of intralobar CLM, excepting
lobar emphysema/overinflation represent another subset of
multilobar or extremely localized disease.
patients where a thoracoscopic approach may be more

Statement 5

Thoracoscopy is a safe and feasible option in selected children

with CLMs, particularly those without previous infection. Recom-
mendation: Neutral; QOE: Poor.

Table 5
Summary of recommendations for CLMs in the prenatal period.

Statement Recommendation Quality of

evidence

Most congenital lung malformations have a Strongly agree Moderate

favorable prognosis, with the majority of
neonates being asymptomatic at birth.
The CVR has emerged as a useful prognostic Strongly agree Moderate
tool and appears reproducible and widely
accepted.
Prenatal maternal betamethasone Strongly agree Poor
administration is indicated to decrease the
size of large microcystic CLMs and reverse
hydrops.
Fetal hydrops associated with CLM is associated Strongly agree Poor–very Fig. 4. Management controversial. A left posterior thoracic echogenic mass was
with a high mortality rate. Fetal intervention poor detected in utero at 19 weeks gestation. It was unchanged at 24 weeks but was
or preterm delivery is indicated. barely visible on sonogram at the end of gestation. The child was asymptomatic at
Prenatal regression may be more common in Neutral Poor birth and CXR showed a minimal lucency in the left lower lobe. A small area of lung
pulmonary sequestration (PS) than in cystic hyperlucency without cystic changes persisted on CT scan at 7 months. The
lung lesions/CPAMs. resected specimen was consistent with type 2 CCAM. The child was discharged
home 2 days after surgery. (Adapted with permission from Laberge et al.29)

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276 R. Baird et al. / Seminars in Pediatric Surgery 23 (2014) 270–277
challenging.68 When large bullae are present, cauterizing the
lesion to shrink it may facilitate thoracoscopic lobectomy (Rothen-
berg S., personal communication, Jan 2014). From a technical
standpoint, there have also been cases of instrument malfunction
or thermal injury from vascular sealing devices.62
The apparent safety and advantages of thoracoscopic lung
resection are clouded by publication bias, with experienced
surgeons reporting good outcomes, while serious complications
or death in low-volume centers may go unreported. Furthermore,
in the above-cited meta-analysis, two of the six series included
were exclusively addressing bronchogenic cysts, which is very
different from lung resections.60 Like any complex minimally
invasive procedure, a learning curve has been documented with
thoracoscopic lobectomies. Importantly, cases performed while
surmounting a learning curve can result in excessively prolonged
case durations and the potential for increased acidosis and
decreased cerebral perfusion.64,69 Thus, while thoracoscopic resec-
tion indeed appears safe and feasible based on the experience of
experts, there is currently insufficient evidence to endorse its
widespread adoption outside the practice of individuals and
institutions with suitable expertise and technical skills.
Conclusion
We have attempted to review the literature on CLMs in search
of evidence to guide best practice. We have used a series of
statements representing several important aspects of prenatal
and postnatal diagnosis and the management of these lesions
(summarized in Tables 5 and 6). Not surprisingly, given the low
incidence and wide spectrum of manifestation of CLMs, we have
found that the quality of the evidence is generally poor.
The most controversial area remains the postnatal manage-
ment of asymptomatic CLMs, which are usually discovered by
routine fetal ultrasound examination. While the evidence weakly
favors prophylactic resection, it has become obvious that not all
CLMs “are created equal.” Mediastinal bronchogenic cysts, small
extralobar PS, and lesions (or rather, “images”) that disappear
before birth are often confused in reports dealing with postnatal
CLMs. Similarly, small subsegmental areas of pulmonary hyper-
lucency are incorrectly equated with large lobar multicystic lung
malformations. While CLM is the best term for prenatal lung
lesions, we advocate postnatal discrimination based on adequate
imaging studies, pending histologic confirmation. In this way,
mediastinal bronchogenic cysts and extralobar PS (intrathoracic,
intradiaphragmatic, or subdiaphragmatic) would stop being con-
fused with cystic lung lesions; intraparenchymal lesions would be
separated into those that are clearly cystic and those with only
hyperlucency/overinflation, with occasional mixed types (Figures
2–4). The presence or absence of a systemic arterial supply would
complete the description. Only with these distinguishing features
would a prospective registry evaluating the short- and long-term
complications of surgery or observation be meaningful. An attempt
at such a registry was described in 2006 as the Long Term
Outcome Study (LoTOS) initiative, although it has not been
reported since and is not registered on ClinicalTrials.gov or
www.clinicaltrialsregister.eu.70 While others have called for a
multicenter prospective study,6,36 unification of case definitions
is required as a first step. Until then, “Best Practice” will likely
remain “Best Judgment” of individual surgeons and centers.
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