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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.
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.
1010-7940/99/$ - see front matter 1999 Elsevier Science B.V. All rights reserved.
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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)
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.
Table 2
Clinical profiles of the patients (over 1 year old)
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.
4. Discussion
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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