Congenital Pulmonary Airway Malformation: Two Case

Reports
Cissy B Kartasasmita, Heda Melinda Nataprawira, Tri Wahyu, Rama
Yusdirwan, Evelyn Phangkawira
Department of Child Health, Universitas Padjadjaran, Hasan Sadikin Hospital
Department of Thoracic Surgery, Universitas Padjadjaran, Hasan Sadikin Hospital

Abstract
Congenital pulmonary airway malformation (CPAM) which was previously
named as congenital cystic adenomatoid malformation (CCAM) is the most
common type of congenital parenchymal lung malformation. The problem
in CPAM is late diagnosis, particularly in developing countries with limitted
resources. This anomaly can mimic as pneumothorax or congenital
diaphragma hernia. We presented two cases of this rare congenital
malformation in a two month-old girl and six month-old boy. A 2-month-old
female infant presented with respiratory failure, having mechanical
ventilation since admission and admitted to pediatric intensive care unit
(PICU). She was first diagnosed as having diaphragmatic hernia. After
thorax computed tomography (CT) performed which revealed multicystic
on the right lung, she underwent cystectomy during thoracotomy. She was
clinically improved and discharged in a good condition. The second 6
month-old male presented with respiratory distress and diagnosed as
pneumothorax and pneumonia. Thorax X-ray and CT revealed multiple
giant bulae on the left lung. He underwent lobectomy and discharged with
improved condition. Both infant have different type of CPAM which type II
and type I, respectively.
Key

words:

thoracotomy

Congenital

pulmonary

airway

malformation,

CCAM,

Congenital Pulmonary Airway Malformation: Two Case

Reports
Cissy B Kartasasmita, Heda Melinda Nataprawira, Tri Wahyu, Rama
Yusdirwan , Evelyn Phangkawira
Department of Child Health, Universitas Padjadjaran, Hasan Sadikin Hospital
Department of Thoracic Surgery, Universitas Padjadjaran, Hasan Sadikin Hospital

Introduction
Congenital pulmonary airway malformation (CPAM) previously named as
congenital

cystic

adenomatoid

malformation

(CCAM)

is

rare

developmental disorder that occurs due to excessive adenomatoid tissue

growth of terminal bronchi that suppress normal development of bronchi
and alveoli. CPAM is characterized by multicystic lesions due to
proliferation of the respiratory bronchiole. It occurs at the 6-8 th gestational
week. The etiology remains unknown, but a disturbed interaction between
mesodermal and ectodermal components of the lung during embryonic
development has been suggested. It is observed with an equal frequency
in both lungs and its incidence ranges between 1/10,000 and 1/35,000.
The lesions usually tend to involve a single lobe and especially the lower
lobe. The incidence of the disease does not vary according to race or
gender. Despite rare CPAM is a lifethreatening lung anomaly. Cysts which
grow and enlarge can compress the lungs, large vessels and esophagus.
As a result of this it can lead to mediastinal shift, hydrops and
polyhydramniosis in fetal life and life-threatening respiratory distress in
the postnatal period. We presented two case report of CPAM diagnosed
and managed in our hospital.
Case Presentation
Case 1. A 2-month-old female infant admitted to our hospital with
respiratory failure and had mechanical ventilation since admission. She
had been suffering from dyspnea since birth and got worse after. She was

born after an uneventful 40 weeks of gestation. Her birth body weight was
3800 grams and antenatal care reported no abnormalities. She had been
hospitalized 10 days at local hospital before referral.
She was admitted to our hopital with severe dyspnea 78/min, tachycardia
with heart rate 160 x/min, and bilateral ronchi and relatively decrease
sound over the right lung. After admission, a chest radiograph was taken
which showed a large rdiolucent area in the right lung. Bronchopneumonia
with bacterial infection and emphysematous lung was then considered.
Thoracal ultrasonogram revealed left lung herniation dan localized pleural
effusion. Thoracal CT Scan with contrast revealed the multiple hypodense
cystic lesions of various sizes, diameter 0,5 2 cm, mostly at superior,
medial and inferior lobes of right lung that caused the mediastinum
organs and left lung shoved to the left posterolateral e.c Congenital Cystic
Adenomatoid

Malformation

type

2.

She

was

then

intubated

and

transferred to pediatric intensive care unit. She underwent surgical

excision of the multiple cysts that was confined to the superior, medial
and inferior lobes of right lung. After the surgery, the rigid bronchoscopy
had done and it showed that left bronchial is smaller than the right
bronchial and the scope could not go further. The baby did very well
postoperatively. Histopathology demonstrated cystic structure without
malignancy proliferation and the conclusion was CPAM type II.

Figure 1. A. Chest X-ray showed emphysematous right lung (red arrow) and
infiltrat in the left lung. B. Chest CT-Scan demonstrated multiple hypodense
cystic lesions at right lung (yellow arrow) that caused the mediastinum organs
and left lung shoved to the left (blue arrow)

Figure 2. Post cystectomy chest X-ray shows obliterated cyst in the left lung
(red arrow) and mediastinum organs returned to the middle part of the
chest(yellow arrow)

Case 2. A 6-month-old male baby was admitted to our hospital suffering

from dyspnea since birth and got worse in this late 2 month. He was born
after an uneventful 38 weeks of gestation. Her birth body weight was
3000 grams and antenatal care reported no abnormalities. He had been
hospitalized 20 days at local hospital before referral.
She was admitted to our hopital with dyspnea 58/min, tachycardia with
heart rate 130 x/min, dehidration and bilateral ronchi and relatively
decrease sound over the upper left lung. After admission, a chest
radiograph was taken which showed multiple large bullae at left lung

Thoracal CT Scan with contrast revealed the multiple hypodense cystic

lesions of various sizes more than 2 cm, mostly at superior lobes of left
lung e.c CPAM. She was then underwent surgical excision lobectomy. The
baby did very well postoperatively.

Figure 2. Chest X-ray show multiple large bullae at left lung

Figure 3. Macroscopic appearance of the cyst

DISCUSSION
The incidence just cited can be compared with the incidence reported for
congenital cystic adenomatoid malformation (CCAM), reported to occur in
1 in 10,000 to 1 in 25,000 births (see later); this malformation is the most
common of the ones involving lung development and comprises about
25% of the congenital parenchymal malformations. Yet, others quote an
incidence of pulmonary sequestration at 0.15% to 1.7% in the general
population, without providing a reference. Stocker found 13 cases of
extralobar sequestrations in a series of 47,000 autopsies performe on
stillborn and liveborn infants. Considering that most patients with

sequestration do not die, it is unlikely that the incidence could be higher

than 1 in 4000 births.
In the past, many classifications of congenital lung malformations have
been proposed, although none has gained universal acceptance. These
have

been

based

on

anatomic

localization,

histopathologic

type,

pathogenesis, or extent of lung involvement. Stocker described in 1977

three distinct histological subtypes which later were expanded to five in
2002. Each type was related with specific symptoms and radiographic
findings. Type 1 is present in 50-70 % of cases and has the best prognosis
(2). Type 2 has a poor prognosis and associates frequently other structural
and chromosomal abnormalities (2). A more useful classification seems to
be the one introduced by Adzick et al (4-5) using prenatal ultrasound
examination. This classification simply differentiates antenatally lung
lesions into macrocystic and microcystic.

Clinical presentation is variable. About 10% become symptomatic during

fetal life. The large mass inside the lung can restrict lung growth, can
produce

mediastinal

shift,

cardiovascular

compromise

and

cava

obstruction leading to non-immune hydrops fetalis (HF). 60% of patients

become symptomatic within one month of life, another 10% between one
and six months and 15 % by adolescence. The key symptom in neonatal
period is the respiratory distress. Outside of the neonatal period recurrent
pulmonary infections in the affected lung with poor
response to medical treatment is the commune presentation mode.
Postnatal the diagnostic is made on X-ray showing multiple air filled
spaces (macrocystic) or a solid area (microcystic). It is essential to take
the X-ray with an in situ nasogastric tube in order to avoid mistaken with a
diaphragmatic hernia (2). Computed tomography (CT) is the most utile
technique for diagnostic and pre-therapeutic evaluation. It show large air

filled, fluid filled cysts or containing air fluid levels (type 1); solid mass
with multiple small cysts (type3).
Surgical resection of CPAM lesions is recommended when patient had
symptoms such as respiratory difficulty or recurrent pulmonary infection.
Even in asymptomatic patient, it was indicated when the extent of lesion
involve more than half of a lobe. It also avoid malignant transformation of
the lesion. Excision of the CCAM is accomplished by lobectomy or
segmentectomy and in certain cases even pneumonectomy. The usual
intervention is lobectomy via thoracotomy or thoracoscopical approach.
Lobectomy

is

segmentectomy

preferred
and

because

longterm

of

potential

complications

early

after

air-leak

after

pneumonectomy.

Thoracoscopy provides several potential advantages over thoracotomy;

lower pain, better postoperative pulmonary mechanics. Moreover up to
30%

of

neonates

develop

scoliosis

after

thoracotomy.

Anesthetic

management includes tracheal intubation and ventilation, central venous

line, isoflurane/oxygen analgesia, muscle relaxation. For a better surgical
access, protection of the normal lung and reduced blood loss one-lung
ventilation (OLV) should be used. Early complications include sepsis, air
leaks with pneumothorax, bronchopleural fistula, wound infections. Later
complications and sequelae are incomplete excision, asthma, pneumonia.
Prognosis depends of several factors such as: cyst volume, histological
type (type I has the best prognosis), the presence of complications during
fetal life, the presence and severity of early respiratory distress. Neonates
tolerate well lobectomy and the remaining lung usually evolves and
expands into the remaining cavity replacing the lost pulmonary tissue.

Reference
1. McDonough RJ. Congenital pulmonary airway malformation: A case report and
review of the literature. Resp Care. 2012;57(2):302-6.
2. Stocker JT. Congenital and developmental diseases in pulmonary pathology. In:
Hammer SP, editor. Pulmonary pathology. New York:Springer-Verlag;1994:155-90.
3. Kim HK, Choi YS, Kim K, Shim YM, Ku GW, Ahn KM, et al. Treatment of congenital
cystic adenomatoid malformation: Should lobectomy always be performed? Ann
Thorac Surg. 2008;86:249-53.
4. Nadeem M, Elnazir B, Greally P. Congenital pulmonary malformation in children.
2012;10:1-7.