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Clinical spectrum of congenital cystic disease of the lung in children

Shin-ichi Takeda, Shinichiro Miyoshi, Masayoshi Inoue, Ken-ichi Omori, Meinoshin


Okumura, Hyung-Eun Yoon, Masato Minami and Hikaru Matsuda
Eur J Cardiothorac Surg 1999;15:11-17

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The European Journal of Cardio-thoracic Surgery is the official Journal of the European Association
for Cardio-thoracic Surgery and the European Society of Thoracic Surgeons. Copyright © 1999 by
European Association for Cardio-Thoracic Surgery. Published by Elsevier. All rights reserved. Print
ISSN: 1010-7940.

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European Journal of Cardio-thoracic Surgery 15 (1999) 11–17

Clinical spectrum of congenital cystic disease of the lung in children1

Shin-ichi Takeda*, Shinichiro Miyoshi, Masayoshi Inoue, Ken-ichi Omori, Meinoshin Okumura,
Hyung-Eun Yoon, Masato Minami, Hikaru Matsuda
First Department of Surgery, Osaka University Medical School, 2-2 Yamada Oka, Suita City, Osaka 565, Japan

Received 29 December 1997; received in revised form 28 September 1998; accepted 28 October 1998

Abstract

Objectives: Congenital cystic lesions of the lung are uncommon but share similar embryologic and clinical characteristics. The purpose
of this study is to review our institutional experience of congenital cystic lung disease, emphasizing the clinical spectrum of the disease
related to age, and present some cases with unusual clinical manifestations. Patients: Between 1962 and 1996, 26 patients (9 females and
17 males) under 15 years old underwent evaluation and surgical treatment for congenital cystic lung disease. Seven patients were under 1
year old, and 19 were in over 1 year old. There were 13 bronchogenic pulmonary cysts, 6 pulmonary sequestrations, 4 congenital cystic
adenomatoid malformations (CCAM), and 3 congenital lobar emphysemas. Results: All patients under 1 year old showed respiratory
distress with mediastinal shift but no episodes of infection. In contrast, 13 of the 19 patients over 1 year old had symptoms of recurrent
infection without respiratory distress. Five patients over 1 year old were entirely asymtomatic from birth. There were significant differences
(P , 0.05) in the frequencies of respiratory distress and infection between the two groups (x2-test). Lobectomy was performed in 21
patients, excision in 3 patients, segmentectomy in one patient, and exploration in one patient. There was no incident of postoperative
mortality or morbidity except for one patient with CCAM complicated by reexpansion lung edema. Twenty-one patients at long-term
follow-up from 2 to 30 years after surgery are doing well with no subsequent limitation of physical activities due to lung resection.
Conclusions: In patients under 1 year old, cystic lesions were discovered by respiratory distress; and in patients over 1 year old signs of
infection were the most important clinical features. Early recognition of these relatively rare congenital cystic lung lesions would lead to the
immediate, proper surgical intervention.  1999 Elsevier Science B.V. All rights reserved.

Keywords: Congenital cystic lung disease; Bronchopulmonary foregut malformation

1. Introduction bronchogenic pulmonary cysts are major four congenital


cystic lesions in the lung, but share similar embryologic
Congenital cystic lesion of the lung in children is uncom- and clinical characteristics [1,4]. These disease entities are
mon but potentially life-threatening, and warrants an urgent also named as bronchopulmonary foregut malformations.
diagnostic work-up [1–4]. These diseases may result from The first successful surgical removal was noted in 1933
compromised interaction between embryologic mesodermal by Reinhoff et al. [5] for a lung cyst in the right upper
and ectodermal lung components during development. Pul- lobe in a 3-year-old boy. With the improved safety of pedia-
monary sequestration, congenital cystic adenomatoid mal- tric anesthesia and the development of non-invasive diag-
formation (CCAM), congenital lobar emphysema, and nostic procedures, thoracic surgery for symptomatic cystic
lesions of the lung has advanced as well as the awareness of
these anomalies among pediatricians. This report retrospec-
tively reviews our institutional experience of congenital
* Corresponding author. Tel.: +81-6-879-3152; fax: +81-6-879-3159;
cystic lung disease, emphasizing the clinical spectrum of
e-mail: stakeda@surg1.med.osaka-u.ac.jp the disease related to age, and some case presentations
1
Presented in part at the Annual Assembly of the American Thoracic with unusual clinical manifestations.
Society, New Orleans, LA, USA, May 10–15, 1996.

1010-7940/99/$ - see front matter  1999 Elsevier Science B.V. All rights reserved.
PII: S10 10-7940(98)002 62-0
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12 S. Takeda et al. / European Journal of Cardio-thoracic Surgery 15 (1999) 11–17

2. Patients and methods due to a ballooning of the cyst (Fig. 1); emergency left lower
lobectomy was performed. In this case, communication
Between 1962 and 1996, 368 patients under 15 years old between the airway and cyst might have caused the
with thoracic surgical conditions underwent evaluation and check-valve phenomenon resulting in ballooning of the
surgical treatment in the Department of Surgery of the tumor.
Osaka University Medical School. The hospital records of There were 2 patients with CCAM and 3 with CLE under
these patients were reviewed. Chest wall deformity includ- 1 year old. Patient #2 was referred to our hospital because of
ing funnel chest and pigeon chest was the most common respiratory distress with apparent mediastinal shift on chest
condition, observed in 136 (37.0%) patients, and mediast- X-ray film (Fig. 2A). This case was categorized in an early
inal tumor was noted in 117 (31.8%) patients. Congenital series, and emergency right middle lobectomy was per-
cystic disease of the lung was seen in 26 patients (7.1%). Of formed with a diagnosis of bronchogenic cyst. A histopatho-
these 26 patients, 9 were females and 17 were males, ran- logical diagnosis of CCAM (Stocker type I) was obtained,
ging in age from birth to 14 years old. Seven patients were and the patient needed mechanical ventilation 2 days after
under 1 year old, and 19 were over 1 year old. There were 13 surgery because of reexpansion due to pulmonary edema of
bronchogenic pulmonary cysts (1 , 1 year, 12 . 1 year), 6 the right lower lobe. Patient #3 had severe respiratory dis-
pulmonary sequestrations (1 , 1 year, 5 . 1 year), 4 con- tress and required mechanical ventilation from birth, and
genital cystic adenomatoid malformations (CCAM) (2 , 1 emergency left upper lobectomy was performed after a
year, 2 . 1 year), and 3 congenital lobar emphysemas diagnosis of CCAM, with symptomatic improvement. His-
(3 , 1 year, 0 . 1 year). One patient with CCAM also tology showed a Stocker type III lesion of CCAM. All 3
had a lesion of pulmonary sequestration. We compared clin- patients with CLE, under 1 year of age, were seen in our
ical manifestations between the patients under 1 year old early series. Patient #4 exhibited stridor from birth and chest
and those over 1 year of age. Diagnostic studies included X-ray and angiography revealed situs inverusus (Fig. 2B).
standard chest X-rays and computed tomography (CT) Thoracotomy revealed a grossly overdistended left middle
scans, aided by selective use of angiography. Magnetic lobe, but operative resection was abandoned since the upper
resonance imaging (MRI) was performed to confirm the and lower lobes were extremely hypoplastic. This patient
diagnosis and to better characterize the cystic content in therefore showed little symptomatic improvement after the
recent series. Surgical therapy was mainly indicated with exploratory thoracotomy. However, gradual resolution with
based on the symptom of respiratory distress and episodes symptomatic improvement was observed during somatic
of infection in addition to the clinical diagnosis of congeni- growth. A marked mediastinal shift with hyperaeration of
tal cystic disease of the lung. the affected lobe was identified in Patients #5 and #6 with
significant respiratory distress. These patients were treated
by upper segmentectomy and right lobectomy with com-
3. Results plete cure of the symptom. Examination of the resected
lobes revealed marked emphysema with overinflation of
Table 1 shows the clinical profiles of the patients with the alveoli and destruction of alveolar septa.
congenital cystic disease of the lung under 1 year of age, There were 2 extralobar and 4 intralobar pulmonary
including bronchogenic pulmonary cyst (BC), congenital sequestration (PS) cases in the present pediatric population.
cystic adenomatoid malformation (CCAM), congenital One patient with PS under 1 year of age who underwent
lobar emphysema (CLE) and pulmonary sequestration (PS). surgical intervention at our institution had an extremely rare
Two patients with BC under 1 year old had symptoms condition. This patient (#7) was a 6-month-old female who
with respiratory distress. Patient #1 had the symptom of had showed stridor and cough since she was 4 months old.
respiratory distress associated with acute mediastinal shift Her right thorax was occupied by anomalous lung and dex-

Table 1
Clinical profiles of the patients (under 1 year old)

Case Age/sex Disease Symptom (I MS D) Treatment, remarks

1 3 months/F BC + (− + +) Emergency LLL


2 5 months/M CCAM I + (− + +) RML, reexpansion lung edema
3 3 days/M CCAM III + (− + +) Emergency LUL
4 7 months/F CLE + (− + +) Exploration
5 3 months/F CLE + (− + +) Segmentectomy
6 7 months/M CLE + (− + +) RUL
7 7 months/F EPS + (− + +) Excision, celiac artery blood supply

I, infection; MS, mediastinal shift; D, dyspnea; RUL, right upper lobectomy; RML, right middle lobectomy; LUL, left upper lobectomy; LLL, left lower
lobectomy; BC, bronchogenic pulmonary cyst; CCAM, congenital cystic adenomatoid malformation; CLE, congenital lobar emphysema; EPS, extralobar
pulmonary sequestration.

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S. Takeda et al. / European Journal of Cardio-thoracic Surgery 15 (1999) 11–17 13

genital cystic disease of the lung over 1 year of age. Ten of


12 patients with BC over 1 year of age had a history of
repeated infection and cough, but none showed respiratory
distress. In Patient #11, the bronchogenic cyst was discov-
ered by an episode of infection after surgical correction of
the funnel chest. Two patients were entirely asymptomatic
and found in the annual check-up at school. In 11 of the 12
patients, surgery was recommended and performed as soon
as the malformation was found. In Patient #10, surgical
treatment was arranged after the finding that the cyst
increased in size 1 year after the initial check-up. Nine
patients underwent lobectomy, and in one patient excision
of the cyst located in the periphery was performed. All
patients are doing well after surgery without infectious
signs; however, Patient #15 (Fig. 4), even though asympto-
matic, required chemotherapy for tuberculosis since a his-
Fig. 1. Patient #1 had a symptom of respiratory distress associated with tologically distinctive caseous lesion was found on the cyst
acute mediastinal shift due to ballooning of the cyst; emergent left lower
wall.
lobectomy was performed.
The symptom of CCAM in patients over 1 year old was
trotated heart instead of the normal right lung. (Fig. 3, left). silent. The cystic lesion of a 2-year-old male with CCAM
Several investigative procedures disclosed an abnormal (#20) was discovered by the examination for recurrent pul-
lung that communicated with esophagus (Fig. 3, right) and monary infection following repair of funnel chest. A 14-
had a systemic arterial supply from the abdominal aorta year-old female (#21) with CCAM and extralobar pulmon-
without right pulmonary artery. Resection of the abnormal ary sequestration was asymptomatic from birth. The
lung was performed without complication; the patient gra- patient’s hyperlucent area in the right lower lung field was
dually recovered and is developing well 8 years after sur- identified in the annual check-up at school. After a diagnosis
gery. This anomaly seemed to be classifiable as extralobar of lung tuberculosis, chemotherapy was initiated without
sequestration without normal left lung, i.e. as one feature of any improvement of the abnormal shadow. Chest CT
lung bud foregut anomaly. revealed a multicystic area in the right lower lobe, and
Table 2 shows clinical profiles of the patients with con- operative lobectomy was done after the diagnosis of cystic

Table 2
Clinical profiles of the patients (over 1 year old)

Case Age/sex Disease Symptom (I MS D) Treatment, remarks

8 8 years/M BC − (− − −) RLL
9 8 years/M BC + (+ − −) RMLL
10a 9 years/M BC − (− − −) LUL
11 8 years/M BC + (+ − −) RUL
12 8 years/M BC + (+ − −) RUL
13 7 years/M BC + (+ − −) RLL
14 8 years/M BC + (+ − −) RLL
15 2 years/F BC + (+ − −) RUL
16 5 years/M BC + (+ − −) Excision
17 10 years/M BC + (+ − −) RLL, funnel chest
18 6 years/F BC + (+ − −) RLL
19 7 years/M BC + (+ − −) RLL
20 2 years/M CCAM I − (− − −) LLL, funnel chest
21b 14 years/F CCAM I, EPS − (− − −) RLL, systemic artery blood supply
22 13 years/M IPS + (+ − −) LUL, intercostal artery blood supply
23 5 years/M IPS + (+ − −) RLL, intercostal artery blood supply
24 13 years/M IPS + (+ − −) RLL, bronchial artery blood supply
25 5 years/F IPS + (+ − −) LLL, intercostal artery blood supply
26 7 years/M EPS − (− − −) Excision, systemic artery blood supply

I, infection; MS, mediastinal shift; D, dyspnea; RUL, right upper lobectomy; RMLL, right middle and lower lobectomy; RLL, right lower lobectomy; LUL,
left upper lobectomy; LLL, left lower lobectomy; BC, bronchogenic pulmonary cyst; CCAM, congenital cystic adenomatoid malformation; CLE, congenital
lobar emphysema; EPS, extralobar pulmonary sequestration; IPS, intralobar pulmonary sequestration.
a
Surgery was arranged after the finding that the cyst show increased in size 1 year after the initial check-up.
b
In this case, pulmonary sequestration was found during surgery for CCAM.

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14 S. Takeda et al. / European Journal of Cardio-thoracic Surgery 15 (1999) 11–17

tration. The postoperative courses were uneventful in all


patients.
To summarize the cases we experienced, all the patients
under 1 year of age were symptomatic, and the major cause
of respiratory distress was mediastinal shift due to the cystic
lesions. In contrast, 26% of the patients over 1 year old with
cystic lung disease were asymptomatic as shown in Table 3.
The major clinical manifestation was infection, seen in 74%
of the patients. In most of the early part of the series, chest
X-ray was the only diagnostic test performed. Lobectomy
was performed in 21 patients, segmentectomy was done in
one patient, and excision was done in 3 patients. There was
no postoperative mortality or morbidity except for one
patient with CCAM who was complicated by reexpansion
lung edema requiring mechanical ventilation. Twenty-one
patients at long-term follow-up ranging from 2 to 30 years
after surgery are doing well with no subsequent limitation of
physical activities due to lung resection.

4. Discussion

During the development of the embryo, separation of the


trachea and the esophagus occurs, and migration of the early
lung bud takes place. The lung tissue shows further differ-
entiation thereafter into airway epithelium and alveolar
cells. In this developmental stage, numerous abnormalities
can take place [1–3]. Bronchopulmonary foregut malforma-
tions are combinations of these forms of disordered lung
growth such as dysplasia, hypoplasia, or hyperplasia invol-
Fig. 2. Patient #2 was referred to our hospital because of respiratory dis- ving one or more structural components of the lung. In
tress with apparent mediastinal shift on chest X ray film (A, upper panel). contrast, pneumatocele and unilateral hyperlucent lung
Right middle lobectomy was performed to find histologically CCAM with [7], even though they mimic congenital cystic diseases,
Stocker type I. Patient #4 with CLE exhibited stridor from birth, and chest are regarded as being of acquired origin in their pathogen-
X-ray (B, lower panel) revealed situs inverusus. Thoracotomy found an
esis, and were excluded in the current series.
overinflated left middle lobe.
Bronchogenic pulmonary cysts (BS) were the most com-
lung disease. The pathological finding was consistent with mon lesion in our series, and are examples of anomalies
CCAM as described by Kwittken et al. [6] and was classi- occurring early in lung development. Bronchogenic cysts
fied into Stocker I. In this case, extralobar sequestration was might properly be called lung bud cysts, since they probably
incidentally found, which was excised simultaneously. originated from embryonic bud tissue before the bronchi
Mediastinal shift with respiratory distress was not noted in were formed [8,9]. They may be lined with squamous
patients with late onset of CCAM (#20 and #21) as we epithelium rather than respiratory epithelium, presumably
experienced. due to the recurrent infection. In the current series, we used
Four of 6 patients (66.7%) with PS over 1 year of age, the term of bronchogenic cyst for the congenital cystic mass
including the case #21 had symptoms of recurrent infection. in the lung parenchyma. Pure mediastinal bronchogenic
Intralobar sequestration (cases #22, #23, #24 and #25) had cysts, which can be treated by simple excision, were
been diagnosed by the angiographic demonstration of the excluded from this study. Only one case was treated with
anomalous blood supplies and confirmed at thoracotomy. excision, and the other cases required lobectomy. One
Patient #24 had a systemic blood supply from a bronchial patient at the age of 3 months required emergency lobect-
artery communicating with a pulmonary artery. In Patients omy because of acute onset of expansion of the cyst asso-
#20 and #26 with extralobar sequestrations, the lesions were ciated with respiratory distress. We have not experienced
found unexpectedly during thoracotomies for other dis- complications such as tension pneumothorax or empyema
eases; CCAM for Patient #20 and thymic hyperplasia for due to rupture of the cyst.
Patient #26. Four patents with intralobar sequestrations CCAM probably results from a cessation of bronchial
were treated by lobectomy, and removal of the sequestrated maturation and concomitant overgrowth of mesenchymal
lung was performed for 2 patients with extralobar seques- elements, which produce the adenomatoid appearance of

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S. Takeda et al. / European Journal of Cardio-thoracic Surgery 15 (1999) 11–17 15

Fig. 3. Patient #7 was a 6-month-old female who had had stridor and cough since she was 4 months old. Her right thorax was occupied with anomalous lung
and dextrotated heart instead of the right normal lung (A, left). Several investigative procedures disclosed an abnormal lung that communicated with the
esophagus (A, right), absence of right pulmonary artery and had a systemic arterial supply from the abdominal aorta. This anomaly was classified as
extralobar sequestration without normal left lung, or total lung ectoplasia.

the anomaly in the early stage of development [1,3,4,10]. number may cause hyperinflation, as proposed by Hislop et
Histologically, cartilage is absent, reflecting the bronchial al. [19]. It is necessary for pediatricians to evaluate asso-
maldevelopment. There often exist a small bronchial com- ciated anomalies because 14% of the cases of CLE have
munication which leads to infection and overinflation of the coexistent congenital heart disease [20]. More importantly,
cystic disease. Stocker and associates [11,12] outlined the surgeons must differentiate acute reversible lobar emphy-
classification of these lesions based on the clinical presenta- sema from the serious irreversible lesions [18]. In our early
tion and pathologic pictures related to the lung maturity. series, we decided not to resect the hyperinflated middle
According to the report by Bailey et al. [4], 7 out of 16 lobe of Patient #4 because the other lobes seemed hypoplas-
cases with CCAM had associated lesions, in which pulmon- tic; however the patient’s condition eventually improved
ary sequestration was the most common seen in 6 out of 17 gradually thereafter. Retrospectively, emphysematous
patients with CCAM. CCAM usually affects only one lobe, lesions of this case seemed to be reversible lobar emphy-
and when multilobar, remains unilateral. All lobes are sema. It is similar to the cases reported by Kennedy et al.
involved with equal frequency [13]. In cases with bilobar [21] who evaluated the patients with CLE who were treated
or bilateral lesions, prognosis is known to be poor because
of pulmonary hypoplasia of the residual lung [4,14,15]. Table 3
With the recent advent of perinatal ultrasonography [16], Clinical symptoms and signs in cystic lung disease in children (comparison
it has become possible to diagnose CCAM in utero; in between the patients under and over 1-year-old)
other words, raising the possibility of elective surgery ,1-year-old .1-year-old P-valuea
after birth or fetal surgery for treatment of CCAM [17].
Symptomatic 7 (100%) 14 (74%)
Congenital lobar emphysema (CLE) is a marked pulmon- Asymptomatic 0 (0%) 5 (26%) NS
ary hyperinflation state that resembles all of the clinical Infection (+) 0 (0%) 14 (74%)
features of obstructive emphysema [1,4,18,19]. One possi- Infection (−) 7 (100%) 5 (26%) ,0.05
ble etiology is that cartilaginous defect weakens the Mediastinal shift or dyspnea
Present 7 (100%) 0 (0%)
bronchus, which may cause collapse on expiration followed
Absent 0 (0%) 19 (100%) ,0.01
by air-block hyperinflation [4,18]. Another mechanism is
a 2
that overgrowth of the alveoli, i.e. an increase in alveolar x -analysis.

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16 S. Takeda et al. / European Journal of Cardio-thoracic Surgery 15 (1999) 11–17

having communication with gastrointestinal tract as we


experienced in Patient #7. Considering the 3 distinctive
features: (1) absence of normal right lung, (2) abnormal
lung tissue communicating with the esophagus, and (3) sys-
temic blood supply into the lesion, this case was classified
into lung ectoplasia according to Schechter et al. [27].
Reviewing recent reports [2,4,15], more than 50% cases
with congenital cystic lesions of the lung were seen in
infancy before 6 months of age. Age distribution in the
current series was a little different. However, the main
symptom in infancy with these anomalies was the same.
Therefore we concluded that the symptoms of these patients
depended on the onset of age, regardless of the type of
disease. In patients under 1 year of age, cystic lesions
were discovered by respiratory distress; and sign of infec-
tion was a core clinical feature in patients over 1 year of age.
Since newborns and infants have a weak chest wall and
mediastinum structure, the intrapulmonary expanding
mass may cause tracheal bronchial compression result in
respiratory distress. Presumably the patients who survive
Fig. 4. Patient #15, even though asymptomatic, required chemotherapy with minimum or slight respiratory distress, however,
since the histologically distinctive caseous lesion (tuberculosis) was
finally end suffering from recurrent infection.
found on the cyst wall.
As to the diagnostic modality, chest X-ray was once the
most cost and time efficient method of diagnosing surgical
conservatively and found functional improvement of the lesion, and was the only diagnostic study in our early series
affected lobe. [9]. Nowadays, congenital cystic lung disease in children
Pulmonary sequestration (PS) probably occurs very early can be readily diagnosed with current imaging modalities,
in the embryonic development before the pulmonary and such as CT, MRI, and ultrasonography and digital subtrac-
aortic circulation become separate; and Pryce [22] clearly tion angiography. Color Doppler sonography has recently
described the lesion as a distinct clinical entity. Sequestra- made it possible to identify the feeding vessels non-inva-
tion could represent a separate mini-lung bud, which keeps a sively [28]. Bronchoscopy was of minimal help in determin-
systemic artery and drains into either venous system ing the nature of the problem; it only helped to rule out other
depending on its intimacy with normal lung bud to which lesions under considerations, such as distal and aspirated
it is adjacent [1,2,15,23]. Extralobar sequestration is usually foreign bodies [23]. The appropriate diagnostic test would
asymptomatic, and is found incidentally in the posterior part allow for efficient treatment, avoiding complications. The
of the costphrenic angle. An intralobar pulmonary seques- plain chest X-ray serves as the starting point for diagnostic
tration is usually located in the posterior basilar region of evaluation and sometimes, all modalities are needed. In
the lower lobe [15,23]. Children or young adults with recur- addition, asymptomatic cystic lesions were sometimes
rent lower lobe pneumonia should be suspected of having found during the examination or follow-up for another
intralobar sequestration. An intralobar pulmonary seques- anomaly as we experienced in 2 patients.
tration may present rarely in the neonatal period with Infants and children tolerate lobectomy extremely well
respiratory distress secondary to the congestive heart failure with compensatory lung growth [29] so that total lung
[24]. Ligation of the aberrant systemic artery supply without volume and gas exchange capacity return toward normal
resection of the sequestrated lung tissue has been performed during somatic maturation. In an experimental study,
in asymptomatic or hemodynamically unstable patients when immature canines were raised to maturity after right
[25]. However, we recommend surgical resection because pneumonectomy (55% of lung resection), compensatory
there was the possibility of microscopic infection in the lung growth returned the gas exchange and exercise perfor-
sequestrated lung with late occurrence of symptoms even mance to normal [30,31]. There was no incidence of post-
in patients without any apparent symptom at time of sur- operative mortality or morbidity except for one patient with
gery. Gerle et al. [26] collected 13 rare cases of pulmonary CCAM who was complicated by reexpansion lung edema
sequestration having communication with the gastrointest- requiring mechanical ventilation in the current series. Most
inal tract; and then proposed to describe this disease entity of the cases with cystic lung disease, prognosis is good after
as ‘congenital bronchopulmonary foregut malformation’ lung resection. Rarely, in patients with bilateral lesions such
that includes extralobar and intralobar sequestrations. This as CCAM, outcome after bilateral resection of the lung was
disease entity seems to share a common embryogenesis. On poor because of pulmonary hypoplasia [2,15]. The surgical
the other hand, controversy still exists over the sequestration resection of the involved lung results in good relief of symp-

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S. Takeda et al. / European Journal of Cardio-thoracic Surgery 15 (1999) 11–17 17

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Clinical spectrum of congenital cystic disease of the lung in children
Shin-ichi Takeda, Shinichiro Miyoshi, Masayoshi Inoue, Ken-ichi Omori, Meinoshin
Okumura, Hyung-Eun Yoon, Masato Minami and Hikaru Matsuda
Eur J Cardiothorac Surg 1999;15:11-17
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