Treatment of Congenital Cystic Adenomatoid
Malformation: Should Lobectomy Always
Be Performed?
Hong Kwan Kim, MD, Yong Soo Choi, MD, Kwhanmien Kim, MD, Young Mog Shim, MD,
Gwan Woo Ku, MD, Kang-Mo Ahn, MD, Sang Il Lee, MD, and Jhingook Kim, MD
Departments of Thoracic Surgery and Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul,
South Korea
Background. Although parenchyma-saving resection
makes it possible to preserve the lung parenchyma, most
surgeons are reluctant to perform it for congenital cystic
adenomatoid malformation (CCAM) because it could
also result in recurrent pulmonary infection or residual
lesion. This study compared the early and late postoper-
ative outcomes according to the extent of resection in
CCAM patients to determine if the extent of resection
would influence the short- and long-term results.
Methods. Between 1995 and 2006, 45 patients underwent
surgical resection for CCAM. Ten patients received a seg-
mentectomy and 2 a wedge resection (the parenchyma-
saving group), and 32 received a lobectomy and 1 a
pneumonectomy (the lobectomy group). A retrospective
analysis was done to compare the early and late postop-
erative outcomes between two groups.
C ongenital cystic adenomatoid malformation (CCAM)
of the lung is a rare congenital abnormality [1].
Because patients with CCAM are susceptible to recurrent
pulmonary infection if they are left untreated, surgical
resection is the treatment of choice, and lobectomy is
considered a standard procedure [25]. However, when
more than one lobe is affected in CCAM patients, then
extensive resection such as pneumonectomy or bilobec-
tomy would be inevitable if lobectomy was attempted in
every case. Likewise, when multiple lobes are involved in
CCAM patients, lobectomy combined with either seg-
mentectomy or wedge resection makes it possible to
avoid a pneumonectomy. Furthermore, considering that
CCAM is usually diagnosed in neonates and infants who
have growth potential, parenchyma-saving resection
would be ideal as long as it is feasible and safe.
Patients undergoing this parenchyma-saving resec-
tion, however, are likely to have early postoperative
morbidities such as recurrent pulmonary infection or
prolonged air leakage, and in cases with a residual
lesion, malignant transformation as well as recurrent
infection could occur during follow-up. For this reason,
Accepted for publication Jan 14, 2008.
Address correspondence to Dr Jhingook Kim, Department of Thoracic
Surgery, Samsung Medical Center, 50 Ilwon-dong, Gangnam-gu, Seoul,
135-710, Korea; e-mail: jkimsmc@skku.edu.
2008 by The Society of Thoracic Surgeons
Published by Elsevier Inc
GENERAL THORACIC
Results. No significant differences were observed for
severity and duration of preoperative symptoms. No
in-hospital or late deaths occurred. There were no signif-
icant differences in the incidence of early postoperative
complications and late morbidities between the two
groups. No significant differences were observed be-
tween the two groups for hospital length of stay and
duration of chest tube placement.
Conclusions. The early and late outcomes were excel-
lent even after parenchyma-saving resection in patients
with CCAM. We suggest that parenchyma-saving resec-
tion can be safely performed in selected patients with a
well-confined CCAM lesion and thereby avoiding pneu-
monectomy in children.
(Ann Thorac Surg 2008;86:249 53)
2008 by The Society of Thoracic Surgeons
most surgeons have been reluctant to perform paren-
chyma-saving resection for CCAM and the optimal
extent of resection in CCAM patients has been contro-
versial [6, 7]. Despite these debates, we have attempted
to preserve the lung parenchyma, when this is both
feasible and safe, in selected patients with extensive
lesions at our institution. The objectives of this study
were to compare the early and late postoperative
outcomes according to the extent of resection in CCAM
patients and to determine if the extent of resection
would influence the short- and long-term results.
Patients and Methods
Between January 1995 and December 2006, 45 consecutive
patients with the diagnosis of CCAM underwent surgical
resection at our institution. Their medical records were
retrospectively reviewed to compare the clinical character-
istics and the early and late postoperative results between
the patients undergoing lobectomy and those undergoing
parenchyma-saving resection, which was defined as the
resection with an extent that is less than that of a lobectomy.
Also considered to be parenchyma-saving resection was a
segmentectomy of one lobe performed in conjunction with
a lobectomy of another lobe to avoid a pneumonectomy, as
well as multiple segmentectomies. The study was reviewed
0003-4975/08/$34.00
doi:10.1016/j.athoracsur.2008.01.036
 250 KIM ET AL Ann Thorac Surg
SURGERY FOR CYSTIC ADENOMATOID MALFORMATION 2008;86:249 53

Table 1. Operative Techniques patients and a pneumonectomy was performed in 1; a

segmentectomy was performed in 10 patients and
Operative Procedures No.
wedge resection was performed in 2. Among the 10
Lobectomy group 33 patients who underwent a segmentectomy, a single
Lobectomy 32 segmentectomy was performed in 6 patients, multiple
Pneumonectomy 1 segmentectomies were done in 3, and a lobectomy of
Parenchyma-saving group 12 one lobe in conjunction with a segmentectomy of
Single segmentectomy 6 another lobe was done in 1. The operative techniques
Multiple segmentectomies 3 are summarized in Table 1. The study population was
Lobectomy segmentectomy 1 divided into two groups by the extent of resection; that
Wedge resection 2 is, 33 patients were in the lobectomy group (lobectomy
or pneumonectomy), and 12 were in the parenchyma-
GENERAL THORACIC

saving group (segmentectomy or wedge resection).

and approved by the Institutional Review Board of Sam-
sung Medical Center.
Six patients (13.3%) were diagnosed with CCAM pre-
natally by fetal ultrasonography and their median age at
the time of operation was 1.2 years. All patients under-
went computed tomographic (CT) scanning of the chest
to evaluate the extent of their CCAM. Surgical resection
for CCAM was recommended when patients had symp-
toms such as respiratory difficulty or recurrent pulmo-
nary infection. Even in asymptomatic patients, surgical
resection was also indicated when the extent of lesion
was estimated to involve more than half of a lobe, as seen
on their CT scans. When patients were diagnosed prena-
tally or during the neonatal period, whether to proceed
with surgical resection depended on the presence of
symptoms; operations were postponed until the age of
6 months if patients showed no symptoms, but were
urgently performed, regardless of the age at presenta-
tion, if patients showed significant symptoms.
A lobectomy was the procedure of choice for the
treatment of CCAM. A segmentectomy or wedge re-
section was chosen when this was considered feasible
owing to the well-confined pathology. When more than
one lobe was affected, then a segmentectomy of one
lobe combined with a lobectomy of another lobe or
multiple segmentectomies were performed to avoid a
pneumonectomy. A lobectomy was performed in 32
A histopathologic examination was performed on all
specimens and was reviewed by a pathologist. The
lesions were histologically classified using the criteria
suggested by Stocker: type I (macrocystic, multiple large
cysts); type II (polymicrocystic, variable numbers of small
cysts); type III (homogeneous echogenic mass). Twenty
patients had type I CCAM, 24 had type II, and 1 had
mixed type I and II. No patients had type III.
All patients were regularly followed up postopera-
tively. Telephone interviews were conducted with pa-
tients who were lost to follow-up to obtain the late
postoperative outcomes. Descriptive statistics were used
to describe patient characteristics and outcomes. The
normally distributed continuous data were expressed as
means standard deviations. Categoric data were ex-
pressed as counts and proportions. Student t tests and 2
or Fisher exact tests were used to compare the continu-
ous and categoric variables, respectively. Significance
was accepted at values of p 0.05.
Results
Patient Profile
The clinical characteristics of the study population are
presented in Table 2. Twelve patients (26.7%) underwent
a parenchyma-saving resection. The lobectomy group
consisted of 22 boys and 11 girls (mean age, 13.7 14.6

Table 2. Clinical Characteristics of the Study Population

All Patients Lobectomy Parenchyma-Saving p Value

Patient, totals 45 33 12
Age at operation, mean SD y 11.2 13.3 13.7 14.6 4.3 4.5 0.002
Gender, No. 0.728
Male 29 22 7
Female 16 11 5
Pre-op symptoms, No. (%) 29 (64) 21 (64) 8 (67) 1.0
Symptom duration, median mon 3.0 4.0 2.0 0.303
Symptom duration 0.24
12 months 34 23 11
12 months 11 10 1
CCAM complications, No. (%)
Pneumonia 17 (38) 15 (45) 2 (17) 0.096
Lung abscess 5 (11) 5 (15) 0 (0) 0.303

CCAM congenital cystic adenomatoid malformation; SD standard deviation.

Ann Thorac Surg
2008;86:249 53
Table 3. Early and Late Postoperative Outcomes
All Patients
Patient, total 45
Early outcomes
Early morbidity, No. (%) 3 (7)
Prolonged air leak, No. (%) 2 (4)
LOS, mean SD days 7.6 4.2
CT duration, mean SD days 5.0 2.4
Late outcomes
Follow-up, mean SD mon 63.8 40.7
Late morbidity, No (%) 3 (7)
Residual lesion, No. (%)a 1 (2)
a
Postoperative computed tomography scans were done in 16 patients: 8 in the lobectomy group and 8 the parenchyma-saving group.
CT chest tube placement; LOS length of stay;
years). The parenchyma-saving group consisted of 7 boys
and 5 girls (mean age, 4.3 4.5 years). Before their
operations, 21 patients (63.6%) in the lobectomy group
and 8 (66.6%) in the parenchyma-saving group had
symptoms such as pneumonia. The median duration of
preoperative symptoms was 4.0 months for the patients
who underwent a lobectomy compared with 2.0 months
for those who underwent a parenchyma-saving resec-
tion. Five patients in the lobectomy group showed a lung
abscess that was related to CCAM, but no patients in the
parenchyma-saving group showed a lung abscess. No
significant differences were observed between the lobec-
tomy group and the parenchyma-saving group for sever-
ity of symptoms, the preoperative duration of symptoms,
or the complications related to CCAM.
Early Postoperative Outcomes
There were no in-hospital deaths. Among the 33 patients
who underwent a lobectomy or pneumonectomy for
CCAM, 2 (6.1%) had early postoperative complications,
including wound infection in 1 and a prolonged air leak
in 1. Except for a patient who had a prolonged air leak
postoperatively, none of the patients who underwent
parenchyma-saving resection experienced early postop-
erative morbidities. The incidence of early postoperative
complications in the two groups was not significantly
different. The mean hospital length of stay was 7.6 4.3
days in the lobectomy group and 7.8 4.0 days in the
parenchyma-saving group (p 0.903). The mean dura-
tion of postoperative chest tube placement was 4.9 1.9
days in the lobectomy group and 5.3 3.7 days in
parenchyma-saving group (p 0.784).
Late Follow-Up Outcomes
Follow-up was completed for all the patients, with a
mean duration of 63.8 months (range, 9.5 to 149.3
months). There were no significant differences in the
follow-up duration between the two groups. No late
deaths occurred during follow-up.
In the lobectomy group, two patients (6.1%) had late
morbidities: 1 patient had spontaneous pneumothorax
and mycoplasma pneumonia developed in 1 patient at 10
KIM ET AL 251
SURGERY FOR CYSTIC ADENOMATOID MALFORMATION
Lobectomy Parenchyma-Saving p Value
33 12
2 (6) 1 (8) 1.0
1 (3) 1 (8) 1.0
7.6 4.3 7.8 4.0 0.903
4.9 1.9 5.3 3.7 0.784
63.8 40.1 63.8 44.0 0.995
2 (6) 1 (8) 1.0
GENERAL THORACIC
0 (0) 1 (8) 1.0
SD standard deviation.
months after lobectomy; this patient recovered after
antibiotic treatment. Among the parenchyma-saving
group, only one late complication (8.3%) occurred during
follow-up. Bacterial pneumonia developed 5 months
postoperatively in the patient who had undergone mul-
tiple segmentectomies. There were no significant differ-
ences in the incidence of late morbidities between the
two groups.
Eight patients (24.2%) in the lobectomy group and 8
(66.7%) in the parenchyma-saving group underwent
postoperative CT scans during follow-up. No residual
lesions were found in the lobectomy group, but 1 patient
in the parenchyma-saving group showed a residual le-
sion. This patient had undergone a left lower lobe basal
segmentectomy and he has been followed up for the
residual lesion without any symptoms.
None of the patients who received operations during
childhood reported growth retardation on their follow-
up. The data for the early and late postoperative out-
comes after surgical treatment for CCAM are summa-
rized in Table 3.
Comment
Congenital cystic adenomatoid malformation is a rare
congenital anomaly of the lung. Patients with CCAM
usually present with respiratory difficulty, and recurrent
pulmonary infection is often seen in neonates and infants
[2, 8]. It is reasonable that surgical resection is considered
the treatment of choice for patients with symptoms due
to CCAM, but whether to perform surgical resection even
for asymptomatic patients remains controversial [5,
9 12]. Many authors have agreed that early surgical
resection should be performed before complications re-
lated to CCAM develop, whereas others have adopted a
more conservative observational approach, suggesting
that such risks might be insignificant compared with the
morbidity that can occur after surgical resection [9 15].
Aziz and colleagues [11] revealed that only 10% of
asymptomatic CCAM lesions developed complications
when left untreated, and they suggested that a nonop-
 252 KIM ET AL
SURGERY FOR CYSTIC ADENOMATOID MALFORMATION
erative approach might be a reasonable alternative for
children with asymptomatic CCAM.
Most authors have nevertheless advocated early elec-
tive operation to avoid the potential risks of untreated
CCAM such as recurrent pulmonary infection or malig-
nant transformation [7, 9, 1519]. The incidence of post-
operative complications is also assumed to be lower after
early elective operation for CCAM than after an urgent
intervention for CCAM complications [5, 20]. Moreover,
it has been known that resection of a compressive lung
lesion enables the remaining lung to induce its compen-
satory growth [5]. To summarize, the accumulated haz-
GENERAL THORACIC
ards of pulmonary infection and the risks of malignancy
later in life outweigh the risks of early and elective
surgery [7]. It therefore stands to reason that we have
performed urgent operations for CCAM patients when
they have symptoms such as respiratory difficulty or
recurrent pulmonary infection. In the same context, sur-
gical resection was recommended even for the asymp-
tomatic CCAM patients when the extent of the lesion was
considered significant enough to result in complications
related to CCAM.
Lobectomy is the procedure of choice for patients with
CCAM, especially when the lesion is confined to a single
lobe. Infants and children tolerate lobectomy well, with
compensatory lung growth, so that the total lung volume
and gas exchange capacity return toward normal during
somatic maturation [12, 21]. However, when multiple
lobes are affected in patients with CCAM, then extensive
resection such as pneumonectomy or bilobectomy seems
to be unavoidable if we stick to the principle of lobec-
tomy. A parenchyma-saving procedure in these circum-
stances makes it possible to avoid performing extensive
resection beyond a simple lobectomy procedure. Theo-
retically, as long as parenchyma-saving resection is fea-
sible and safe, preserving the normal pulmonary paren-
chyma would be ideal in patients, such as neonates and
infants, who have growth potential.
A more conservative policy of segmentectomy seems to
have been followed by some investigators [17, 18]. Dav-
enport and coworkers [7] advocated a conservative ap-
proach of performing lobectomy of the most involved
lobe and performing segmentectomy of the remaining
cysts when the lesion involved adjacent lobes in CCAM
patients. Yet it should be noted that parenchyma-saving
resection can be complicated by a prolonged air leak in
the early postoperative period, and in patients with a
residual lesion, malignant transformation as well as re-
current infection could develop during follow-up, which
would necessitate a repeat operation [6, 18, 22]. For this
reason, the optimal extent of resection in CCAM patients
has been controversial.
Despite these potential disadvantages of parenchyma-
saving resection, we have attempted to preserve the lung
parenchyma whenever possible in selected cases. A seg-
mentectomy or wedge resection was performed when it
was considered feasible owing to the well-confined pa-
thology. When more than one lobe was affected, then a
segmentectomy in conjunction with lobectomy, or mul-
tiple segmentectomies, were performed to avoid pneu-
Ann Thorac Surg
2008;86:249 53
monectomy. In contrast with other researchers previous
concerns, our data showed excellent early and late out-
comes. Of the 12 patients who underwent parenchyma-
saving resection, only one incidence of a prolonged air leak
occurred; during follow-up, only one patient was found to
have a residual lesion, and this required no further treat-
ment. The differences in the hospital length of stay or the
duration of postoperative chest tube placement between
the lobectomy group and the parenchyma-saving group
were not significant.
In fact, it is difficult to explain the reason why the
outcomes of parenchyma-saving resection were rela-
tively good in our series. Possibly, we decided to preserve
the pulmonary parenchyma in highly selected patients
who had well-confined lesion and this might have led to
our excellent results. This means, in other words, per-
forming parenchyma-saving resection might not be de-
sirable in patients with diffuse, poorly confined lesions,
even though it is highly likely to perform a pneumonec-
tomy in these cases due to multiple lobes involvement.
Lobectomy is obviously the standard procedure for pa-
tients with CCAM, but parenchyma-saving resection
should be taken into consideration for selected cases in
which multiple lobes are involved and the procedure
appears to be feasible due to the well-confined lesion.
Our study has several limitations. Because our data
were retrospectively collected and parenchyma-saving
resection was performed in only selected cases, it seems
difficult to ascertain whether lobectomy should be always
performed in CCAM patients. This might be inappropri-
ate if we tried to determine which procedure would be
optimal regarding the extent of resection in CCAM
patients based solely on this series. Had we performed a
prospective, randomized controlled trial, it would have
been helpful to determine if parenchyma-saving resec-
tion could be justified even in patients with localized
CCAM.
In addition, the study population was rather small and
thus it does not seem that the power of our suggestions is
so strong as to be conclusive. Despite these limitations,
however, we can firmly suggest that parenchyma-saving
resection is one of the surgical options in highly selected
cases of CCAM, especially when multiple lobes are
involved and so performing a pneumonectomy is
inevitable.
In summary, although a lobectomy was the procedure
of choice at our institution for the treatment of CCAM,
parenchyma-saving resection was performed in selected
patients to avoid an extensive resection. We compared
the early and late postoperative outcomes according to
the extent of resection in CCAM patients and we tried to
determine if the extent of resection would influence both
the short- and long-term results. The early and late
outcomes were excellent even after parenchyma-saving
resection, in contrast to the previous concerns raised by
the past reports. We suggest that parenchyma-saving
resection can be safely performed in selected patients
with a well-confined CCAM lesion, and so this avoids
performing pneumonectomy in children, a procedure
with potentially devastating outcomes.
Ann Thorac Surg
2008;86:249 53
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