2. Akuma and Okoye: Outcome of fetuses diagnosed with congenital cystic adenomatous malformation
190 Journal of Clinical Neonatology ¦ Volume 9 ¦ Issue 3 ¦ July-September 2020
35,000 live births.[2]
The widespread adoption of routine
ultrasound screening during pregnancy has resulted
in increased antenatal detection of affected fetuses.
Similarly, a better understanding of the natural antenatal
history of CCAM lesions has led to a more conservative
approach to management with a resultant decrease
in antenatal intervention and termination of affected
pregnancies.[3‑6]
In the postnatal period, affected infants can either be
asymptomatic or symptomatic, having varying degrees
of respiratory distress. It has been reported that only
about 17% of infants with antenatally diagnosed CCAM
are symptomatic at birth, while the vast majority
remain asymptomatic.[7]
There is a consensus of opinion
regarding the management of symptomatic CCAM
neonates who after radiological evaluations, will need
surgery. In contrast, the postnatal management of
asymptomatic neonates/infants remains controversial,
with some clinician advocating surgical resection
between 3 and 6 months of life,[8]
and others adopt a
more expectant (conservative) approach.[9]
This controversy in postnatal management of
asymptomatic infants exists mainly because of our
lack of knowledge regarding the natural postnatal
history of un‑resected CCAM lesions.[10]
In our
center, asymptomatic CCAM infants are managed
conservatively. The outcome of all pregnancies with a
fetal diagnosis of CCAM managed over a 12‑year period
is presented in this study. The symptom surveillance
of affected infants during the 1st
year of life was also
evaluated to help ascertain the safety of conservative
management approach in asymptomatic infants.
Methods
The case notes of mothers and infants with antenatal
diagnosis of CCAM born between January 1, 2004 and
December 31, 2015 were reviewed. Cases were identified
by querying various hospital databases, including the
fetal Medicine database, maternity database, the Neonatal
database, histopathology database, cardiothoracic surgery
database, and the regional congenital anomalies register.
Pertinent information on identified mother–infant dyad
including ethnicity, gestation at diagnosis, antenatal
progression/resolution of CCAM fetal lesion, antenatal
management, delivery details, neonatal outcome,
postnatal investigation, and management were extracted.
In addition, the clinical history of all surviving children
up to 1 year of age (ending December 31, 2016) was
also reviewed to assess symptom burden, hospital
readmission rates, and subsequent need for surgery
during infancy.
Data were subjected to simple descriptive statistics and
expressed as mean median, range, and percentages.
Antenatal and demographic differences between
symptomatic and asymptomatic infants were examined
using Mann–Whitney and Fisher’s exact test, and a
P < 0.05 was considered statistically significant. Ethical
approval was not required for this study, as this was a
service evaluation exercise.
Results
A total of 51 pregnancies with antenatal diagnosis of
CCAM were identified during the study period. One
pregnancy with an affected fetus who also had trisomy
21 ended in stillbirth. A further three pregnancies
were terminated on parental request. These four cases,
coupled with another five babies who had a postnatal
diagnosis of lung sequestration, were excluded, leaving
a final study population of 42 pregnancies/infants. The
study cohort was all singleton pregnancies that resulted
in live births [Figure 1].
Table 1: Study cohort demographics
Characteristics (features) n (%)
Gender
Male 21 (50)
Female 21 (50)
Ethnicity
White 27 (64.2)
Asian 9 (21.4)
Black 2 (4.8)
Others 2 (4.8)
Not known 2 (4.8)
Site of CCAM lesion
Left 20 (47.6)
Right 22 (52.4)
Size of CCAM lesion (cm)
<2 9 (21.4)
2‑5 14 (33.3)
>5 6 (14.3)
Missing data 13 (31.0)
Cyst appearance
Macrocystic 13 (31.0)
Microcsytic 19 (45.2)
Solid 1 (2.4)
Mixed 1 (2.4)
Missing data 8 (19.0)
Mode of delivery
Vaginal 31 (73.8)
Cesarean section 11 (26.2)
Other imaging in
pregnancy
Fetal MRI 6 (14.3)
None 36 (85.7)
CCAM – Congenital cystic adenomatous malformation;
MRI – Magnetic resonance imaging
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Journal of Clinical Neonatology ¦ Volume 9 ¦ Issue 3 ¦ July-September 2020
hydrops and pulmonary hypoplasia were limited only
to fetuses who became symptomatic after birth – A
statistically significant difference. Mediastinal shift and
polyhydramnios also occurred more in fetuses who
became symptomatic at birth than those who were
asymptomatic. The former involved: 10/13 (77%) versus
12/29 (41.4%) of cases and the latter 4/13 (30.8%)
versus 3/29 (10.3%) of symptomatic and asymptomatic
cases, respectively. However, neither of these differences
was statistically significant.
In the majority of cases 34/42 (81%), fetal CCAM
lesion remained either static in size or resolved (partial
or complete) during pregnancy. This contrasts with the
few cases, 3/42 (7.1%) where the CCAM lesion actually
increased in size during the antenatal period. Fetal
CCAM lesions were more likely to undergo partial or
complete resolution during pregnancy in fetuses who
became asymptomatic after birth than those who were
symptomatic: 13/29 (44.8%) and 7/29 (24.1%) versus
3/13 (23.1%) and 2/13 (15.4%), respectively. In contrast,
fetal CCAM lesions were more likely to either remain
static or increase in size during pregnancy in fetuses who
became symptomatic after birth than those who were
asymptomatic: 3/13 (23.1%) and 2/13 (15.4%) versus
6/29 (20.7%) and 1/29 (3.4%), respectively. However,
none of these observed differences in fetal CCAM
behavior was statistically significant. Two fetuses had an
antenatal intervention, and both were symptomatic after
birth.
Figure 1: Study Overview Flow Chart. CCAM - Congenital cystic adenomatous malformation. NICU - Neonatal Intensive Care Unit. Neo - Neonatology.
CTS – Cardiothoracic Surgery. CT – Computed Tomography. *CT scan done at 9 months and Surgery at 10 months of age. **CT scan done 10 months
and Surgery at 12 months of age. ***CT scan done at 2.5 months of age but Surgery not done
Demographics
Table 1 summarizes the demographics of the study cohort,
which shows a majority of Caucasian infants with no sex
predilection. The right lung was marginally more affected.
Where data were available, the majority of the lesions
23/42 (54.7%) were small in size, measuring <5 cm in the
widest diameter. In appearance, most of the fetal lesions,
19/42 (45.2%), were classified microcystic (Stocker
type 2); 13/42 (26%) were macrocytic (Stocker type 1);
1/42 (2.4%) were of solid (Stocker type 3) and mixed
variety each.[11]
This data was missing in 8/42 (19%) of
cases. Fetal magnetic resonance imaging was the only
other imaging used for antenatal evaluation in this cohort
and was done in 6/42 (14.3%) of cases. Majority of the
women delivered vaginally 31/42 (73.8%), while the
remainder 11/42 (26.2%) had cesarean section.
Antenatal course
Table 2: summarizes the progress of the fetal lesions
during pregnancy and associated antenatal complications.
The mean gestation at diagnosis of CCAM was
21 + 4 weeks (17 + 4 weeks – 31 + 3 weeks). CCAM
was an isolated finding in 16/42 (38.1%) of the cohort,
but in 26/42 (61.9%), there was one or more associated
complications. Isolated CCAM occurred more in fetuses
who were asymptomatic after birth 14/29 (48.3%) than
those who were symptomatic: 2/13 (15.4%); (P = 0.084).
The common complications of CCAM during pregnancy
were: mediastinal shift 22/42 (52.4%), polyhydramnios
7/42 (16.9%), nonimmune hydrops 4/42 (9.5%) and
pulmonary hypoplasia 3/42 (7.1%). Nonimmune
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192 Journal of Clinical Neonatology ¦ Volume 9 ¦ Issue 3 ¦ July-September 2020
Postnatal course
Table 2 and Figure 1 summarizes the differences
in clinical features between the symptomatic and
asymptomatic infants after birth and their progress during
the neonatal period and infancy. Symptomatic infants
were born at a lower mean gestation and also weighed
less than their asymptomatic counterparts: 34.6 weeks
and 2401.3 g versus 39.1 weeks 3293.2 g, respectively.
Both differences were statistically significant.
Conversely, asymptomatic infants were born in better
condition than symptomatic infants with correspondingly
better mean Apgar scores at 1 min and 5 min,
respectively, 8.2 and 9.7 versus 7.4 and 8.8, respectively.
However, only the difference in the mean Apgar scores
at 5 min was statistically significant (P = 0.004).
All the symptomatic infants needed admission to the
neonatal intensive care unit (NICU) in contrast to
a single asymptomatic infant (1/29; 3.4%) admitted
to NICU because of prematurity. The remaining
asymptomatic babies were managed successfully in
the postnatal ward area after birth. The mean length of
hospital stay was significantly more for symptomatic
infants than asymptomatic infants: 10.6 days versus
2.1 days; P = 0.001.
A chest radiograph was done in all the babies soon
after birth. This initial chest X‑ray was significantly
more likely to be reported “abnormal” in symptomatic
than asymptomatic babies: 8/13 (61.5%) versus
10/29 (34.5%); P = 0.006. In contrast, chest computed
tomography (CT) scan was rarely done during the initial
hospital stay after birth (neonatal period); 7/42 (16.7%).
All such “early” chest CT scans were “abnormal” and
done exclusively in symptomatic infants who also had
abnormal initial chest X‑ray report.
Three more babies 3/42 (7.1%) had CT chest scans
later in infancy. One of these babies was symptomatic
at birth and had a “normal” initial chest X‑ray report
but abnormal chest CT scan at 9 months of age. The
other two babies were asymptomatic at birth. One of
these two had an “abnormal” initial chest X‑ray report
and the other a “normal” report. The former had chest
CT scan at 2.5 months of age, which was suggestive
of a small area of lobar emphysema and the latter had
chest CT scan at 10 months of age, which was reported
“abnormal – CCAM.”
All the seven symptomatic babies who had
abnormal “early” chest CT reports underwent
surgery (lobectomy) in the neonatal period. Only
1/7 (14.3%) of these operated symptomatic babies
died postsurgery. Two other babies in the cohort
also had surgery during infancy – 10 months and
12 months, respectively. Both of these babies who
Table 2: Antenatal characteristics of congenital cystic adenomatous malformation lesions and postnatal
outcomes ‑ comparison between symptomatic and asymptomatic infants
Symptomatic infants (n=13/42) Asymptomatic infants (n=29/42) P
Associated antenatal complications (%)
No associated complications 2/13 (15.4) 14/29 (48.3) 0.084
One/more associated antenatal complications 11/13 (84.6) 15/29 (40) 0.084
Mediastinal shift 10/13 (77) 12/29 (41.4) 0.314
Polyhydramnios 4/13 (30.8) 3/29 (10.3) 0.075
Hydrops (nonimmune) 4/13 (30.8) None 0.006
Pulmonary hypoplasia 3/13 (23.1) None 0.025
Antenatal progress (%)
Complete resolution 2/13 (15.4) 7/29 (24.1) 0.695
Partial resolution 3/13 (23.1) 13/29 (44.8) 0.303
CCAM lesion static 3/13 (23.1) 6/29 (20.7) 1.00
Increase in size 2/13 (15.4) 1/29 (3.4) 0.222
Missing data 3/13 (23.1) 2/29 (6.9) 0.302
Antenatal intervention 2/13 (15.4) None 0.091
Postnatal outcomes
Gestation at birth (weeks), mean (range) 34.6 (29‑40) 39.1 (30‑42) <0.001
Birth weight (g) 2401.3 (1290‑5090) 3293.2 (2220‑4510) 0.008
Apgar at 1 min 7.4 (2‑9) 8.2 (7‑10) 0.263
Apgar at 5 min 8.8 (3‑10) 9.7 (9‑10) 0.004
Abnormality on initial chest X‑ray (%) 11/13 (84.6) 10/29 (34.5) 0.006
Surgical intervention (%) 8/13 (61.5) 1/29 (3.4) <0.000
Length of stay (days) 10.6 (1‑28) 2.1 (1‑7) 0.001
CCAM – Congenital cystic adenomatous malformation
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had “delayed” chest CT scans at 9 months and
10 months, respectively, survived surgery. There were
overall 4/42 (9.5%) deaths in the study cohort – One
postsurgery and three from the complications of
prematurity. All the babies who demised were
symptomatic at birth.
On discharge from hospital, 6/38 (15.8%) of eligible infants
did not have follow‑up. The majority of the remaining
eligible infants (23/38; 60.5%) had follow‑up with a single
disciple – Neonatology (18/23; 78.3%), cardiothoracic
surgery (4/23; 17.4%), and respiratory physician (1/23;
4.3%). However, the remaining eligible infants (9/38;
23.7%) had follow‑up with either two or more disciplines
postdischarge from hospital. A number of infants were lost
to follow‑up during infancy, but the median duration of
follow‑up was 10 months. Majority of the babies followed
up (34/38; 89.5%) remained asymptomatic during infancy.
However, 4/38 (10.5%) babies became symptomatic
during follow‑up. The most common symptoms reported
were wheezes and recurrent chest infection. Only one child
needed hospital re‑admission for chest infection during the
follow‑up period.
Discussion
Improving antenatal surveillance of congenital
anomalies in recent years has led to a steady increase
in the antenatal detection of CCAM.[10,12]
Most lesions
appear during the second trimester and are detected
during the routine morphology (anomaly) scan or shortly
thereafter.[13]
This is congruent with our finding where the
median time of detection was 21 weeks and 4 days. Once
identified, it is important to undertake serial sonographic
monitoring of the fetal lesion and surveillance for
associated complications, which can impact postnatal
outcomes. The majority of the fetal lesions will either
regress to some degree or remains static in size during
pregnancy.[14]
Only in a minority of cases do the CCAM
lesion increases in size or disappears completely during
fetal life.[15]
Mediastinal deviation, polyhydramnios,
nonimmune hydrops, and pulmonary hypoplasia are
some of the known complications of antenatal CCAM.[16]
All four complications were encountered in our cohort
to a varying degree.
The behavior of the fetal CCAM lesion and presence
of associated complications during pregnancy can
be used in combination to predict the postnatal
condition of the baby. Such prognostic ability could
potentially assist clinicians who undertake antenatal
counseling of at‑risk pregnancies. Nonimmune hydrops
is a recognized poor outcome predictor for antenatal
CCAM.[1,6,11‑13,15,17,18]
In our cohort, pulmonary hypoplasia
and nonimmune hydrops were limited to fetuses
who became symptomatic after birth. Similarly, fetal
surgical intervention is another reported marker for
the poorer outcome.[16]
In our cohort, thoracentesis and
thoraco‑amniotic shunt were done on two fetuses who
developed severe nonimmune hydrops, and both were
symptomatic after birth.
Most affected fetuses, however, remained asymptomatic
after birth.[7,12]
Our experience is in agreement with
published literature, given that 69% of our cohort were
asymptomatic in the neonatal period. These asymptomatic
infants were born at a more mature gestation, were
heavier in weight and had significantly better 5 min
Apgar scores. Reassuringly, the asymptomatic infants
were safely managed alongside their mothers without
need for NICU admission if there were no other health
concerns. These findings could also help clinicians
when making a decision regarding the appropriate place
for the delivery of affected pregnancies. Most of the
mothers delivered vaginally, and their asymptomatic
babies stayed in the hospital for a short period of time.
Chest radiograph is mandatory in affected babies and
should be done irrespective of symptomatology.[8]
This initial investigation is more likely to be reported
“abnormal” in symptomatic babies, a pattern observed
in our study. The commonly reported “abnormal
findings” included: areas of cystic lucency within a
lobe with intersposed normal lungs, localized or diffuse
opacification, nonspecific linear air space shadowing,
mediastinal deviation and features suggestive of either
localized or generalized lung hypo‑/or hyper‑inflation.[19]
However, despite this mandatory recommendation, the
utility of chest radiograph in the evaluation of CCAM
has been questioned in previous reports because of its
low specificity and sensitivity.[18]
Chest CT is a better imaging tool to delineate
abnormal lung lesions, including CCAM.[20]
Chest
CT (early and late) was only done on a handful of
our symptomatic infants. We are therefore unable to
calculate the sensitivity and specificity of chest X‑ray
as the initial evaluation test in our cohort because of
this practice. However, chest CT scan in two babies
(one symptomatic and asymptomatic) who had “normal”
chest X‑ray reports during infancy were “abnormal” and
confirmatory of CCAM. Similarly, there were a number
of both symptomatic and asymptomatic babies whose
initial chest X‑ray was reported “abnormal” but did not
have chest CT scan. One of such baby’s lung lesion
was later diagnosed as lobar emphysema on a chest CT
scan done in infancy. Given this observation, we would
suggest that chest CT scan should be done in all affected
babies after the initial chest X‑ray; irrespective of the
chest radiography report. This should help confirm the
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6. Akuma and Okoye: Outcome of fetuses diagnosed with congenital cystic adenomatous malformation
194 Journal of Clinical Neonatology ¦ Volume 9 ¦ Issue 3 ¦ July-September 2020
diagnosis of CCAM or other related lung malformations,
a recommendation that is in consonance with more
recent guidelines.[21]
There is a consensus of opinion regarding the
management of symptomatic neonates who have
CCAM.[22]
They would usually have surgery in the
immediate newborn period after the radiological
evaluation of the lesion. The majority of the symptomatic
babies in our cohort were managed in similar fashion
and surgery, when indicated, was performed in the
neonatal period. The observed postsurgery complications
were generally minor and included: air leak and pleural
effusion. Surgery was also performed in one of our
symptomatic babies after abnormal CT scan evaluation
during infancy, without associated complications. There
was a single death postsurgery performed in the neonatal
period in our cohort.
The postnatal management of asymptomatic
neonates/infants who had fetal CCAM remains
controversial, with clinicians segregating into two
broad opinions.[16]
One group advocates a “conservative
approach” consisting of radiological evaluation after
delivery and symptom surveillance thereafter with the
provision of surgery if symptoms develop or there is an
increase in the size of the lesion.[9]
The second group,
however, adopt a more proactive approach offering surgery
during infancy after early radiological evaluation.[8]
The
pros and cons of both management strategy are well
documented in the literature.[10,23]
This controversy in
postnatal management of asymptomatic infants exists
mainly because of our lack of knowledge regarding the
natural postnatal history of un‑resected CCAM lesions.[10]
Moreover, there has been no prospective study comparing
the outcomes of asymptomatic infants managed using the
two management strategies.
Most clinicians in our center gravitate toward the
conservative strategy in managing asymptomatic infants
who had fetal CCAM. Emphasis is given to symptom
surveillance during infancy. A chest CT scan is performed
if the diagnosis is in doubt, and the duration of follow‑up
is at the discretion of the attending physician, although
usually guided by future development of significant
symptoms. Most of our babies remained asymptomatic
during follow‑up and those who became symptomatic
experienced only a mild degree of symptoms. However,
there was nonuniformity of personnel overseeing the
follow‑up with significant drop out from the follow‑up
program even before the end of infancy. A recent
UK‑based survey of 20 pediatric surgical units completed
by 72 consultants in pediatric surgery (51% response rate)
highlighted similar ongoing variability in the provision of
care for asymptomatic infants who have CCAM as found
in our study.[24]
Given this problem, it is imperative that
future surveillance programs should be more robust
with defined criteria and end‑point. We suggest that
these babies should be followed up either by a pediatric
respiratory consultant or a consultant pediatrician with
interest in respiratory diseases who could provide this
service up until transition into adulthood. Similarly,
developing an International registry to collect outcomes
on these asymptomatic infants will help us gain better
knowledge regarding the natural history of this condition
and hopefully resolve this management debate either
way.[25]
Limitations of the study
We acknowledge that this report is based on the
experiences from a single perinatal center in a
region of the United Kingdom, and the duration
of symptom surveillance was limited to infancy.
However, this follow‑up time frame was chosen
to help evaluate the “safety” of the conservative
approach to managing asymptomatic infants who have
CCAM given that published reports suggest that most
such infants become symptomatic by a median age
of 6.9 months (range 2.5–10 months).[7]
Moreover,
proponents of elective surgery in asymptomatic infants
recommend doing lobectomy between 3 and 6 months
of age.[8]
The follow‑up period in our study, therefore,
covered both periods and so deemed reasonable. We,
however, cannot guarantee that with a longer period
of surveillance, these infants would not become
symptomatic, needing further radiological investigation
and surgery.
Second, the issues raised in our report are relevant to
many perinatal centers around the world and, therefore,
valuable. We continue to lack scientific evidence to
inform postnatal management of infants with antenatal
diagnosis of CCAM, especially those who are
asymptomatic at birth. There is, therefore, an urgent
need to undertake a large multi‑center, multi‑national,
prospective, longitudinal study of infants with antenatal
diagnosis of CCAM. Such a study should help provide
information on the natural history of this condition,
thereby assisting in the development of best practice
guidelines. Such evidence‑based guidelines should
help clarify who is best placed to oversee symptom
surveillance and the duration of follow‑up.
Conclusion
Our study has shown that when there is antenatal
detection of CCAM, most of such pregnancies have only
minor complications and are delivered vaginally. The
behavior of the fetal lesion, coupled with the presence
of complications, can help prognosticate the baby’s
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Journal of Clinical Neonatology ¦ Volume 9 ¦ Issue 3 ¦ July-September 2020
condition postdelivery. Such knowledge can be useful
during antenatal counseling and when planning the
optimum place of delivery.
Chest CT scan should be performed on all affected
infants to help confirm the diagnosis and to delineate
the CCAM lesion postnatally. The debate regarding the
management of asymptomatic infants in the postnatal
period will continue for the foreseeable future since
we still lack scientific evidence to inform postnatal
management in this sub‑group of affected infants. In the
interim, conservative management strategy, employing
symptom surveillance remains a tenable approach for
asymptomatic infants provided this is overseen by
appropriately skilled clinicians who can offer long‑term
follow‑up till transition into adulthood.
Finally, there is an urgent need to establish an
international registry to collate information regarding
infants who have CCAM to help gain knowledge
regarding the natural history of this condition. Such
evidence should eventually facilitate the development
of a robust guideline on how best to manage this rare
congenital malformation.
Acknowledgment
We would like to thank Dr. Kamini Yadav,
Dr. D K Vasudev, Dr. Manjiri Khare, Mr. Giles Peek,
Dr. Judith Budd and Dr. Helen Porter, for their help with
case identification.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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malformation (CCAM): Remove or not, how can we progress?
Paediatr Respir Rev 2013;14:168.
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© 2020 Journal of Clinical Neonatology | Published by Wolters Kluwer - Medknow
Objective: Although much is known about the antenatal course of congenital
cystic adenomatous malformation (CCAM), the postnatal course is less well
documented. The vast majority of infants remain asymptomatic at birth, with the
controversy surrounding the postnatal management of such infants. We reviewed
the outcome of fetuses diagnosed with CCAM in our center and evaluated their
symptom burden during the 1st
year of life. Methods: A retrospective review
of maternal and infant medical records of all cases with antenatal diagnosis
of CCAM managed in a regional perinatal center over 12 years, and infant
outcome at 1 year of age is presented. Results: Forty‑two eligible singleton
pregnancies/infants were identified. Thirteen babies (30.9%) were symptomatic
and 29/42 (69.1%) asymptomatic at birth. 7/13 (53.9%) symptomatic infants had
associated antenatal complications. The fetal lesions were more likely to remain
static or reduce in size during pregnancy in asymptomatic (26/29; 89.6%) than
symptomatic babies (8/13; 61.6%). All babies had chest radiographs after birth,
but computed tomography (CT) scan was done in only a few symptomatic babies;
7/42 (16.7%) during the neonatal period and a further 3/42 (7.1%) during infancy.
The majority (8/13; 61%) of the symptomatic babies had surgical intervention
compared to only 1/29 (3.4%) asymptomatic babies who had surgery. However,
most babies remained symptom‑free during infancy. Death within the cohort
was limited to babies who were symptomatic (4/13; 31%). Conclusion: Most
pregnancies diagnosed with CCAM remain uncomplicated. The behavior of the
prenatal lesion could help predict the postnatal outcome. Our experience highlights
that CCAM persistence cannot be excluded from chest radiographs; hence, the
need for chest CT scans. Symptom surveillance should help guide the need for
surgery in asymptomatic infants. However, only skilled clinicians who can offer
long‑term follow‑up, until the transition into adulthood should oversee surveillance
to ensure safety.
Keywords: Congenital cystic adenomatous malformation, fetal surgery, foetus,
symptom surveillance
The Outcome of Fetuses Diagnosed with Congenital Cystic
Adenomatous Malformation of the Lungs: Experience of a Regional
Neonatal Center (2004–2016)
Akuma Oti Akuma, Ayevbekpen Grace Okoye1
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DOI:
10.4103/jcn.JCN_1_20
Address for correspondence: Dr. Akuma Oti Akuma,
Neonatal Intensive Care Unit, Corniche Hospital, P. O. Box 378,
Abu Dhabi, United Arab Emirates.
E‑mail: akumaochibuzo@aol.com
Original Article
Introduction
Congenital cystic adenomatous malformation (CCAM)
is a congenital bronchopulmonary malformation
characterized by a lack of normal alveoli and
excess proliferation/cystic dilatation of the terminal
bronchioles.[1]
It usually occurs sporadically with a
reported incidence that varies from 1 in 10,000 to 1 in
Neonatal Intensive Care Unit,
Corniche Hospital,
Abu Dhabi, United Arab
Emirates,
1
Neonatal Intensive Care
Unit, Luton and Dunstable
University Hospital NHS
Trust, Luton, England, UK
How to cite this article: Akuma AO, Okoye AG. The outcome of fetuses
diagnosed with congenital cystic adenomatous malformation of the lungs:
Experience of a regional neonatal center (2004–2016). J Clin Neonatol
2020;9:189-95.
Abstract
Submitted: 01-Jan-2020
Revised: 11-May-2020
Accepted: 17-Apr-2020
Published: 07-Aug-2020
This is an open access article distributed under the terms of the Creative Commons
Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak,
and build upon the work non‑commercially, as long as the author is credited and the new
creations are licensed under the identical terms.
For reprints contact: reprints@medknow.com
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![Akuma and Okoye: Outcome of fetuses diagnosed with congenital cystic adenomatous malformation
190 Journal of Clinical Neonatology ¦ Volume 9 ¦ Issue 3 ¦ July-September 2020
35,000 live births.[2]
The widespread adoption of routine
ultrasound screening during pregnancy has resulted
in increased antenatal detection of affected fetuses.
Similarly, a better understanding of the natural antenatal
history of CCAM lesions has led to a more conservative
approach to management with a resultant decrease
in antenatal intervention and termination of affected
pregnancies.[3‑6]
In the postnatal period, affected infants can either be
asymptomatic or symptomatic, having varying degrees
of respiratory distress. It has been reported that only
about 17% of infants with antenatally diagnosed CCAM
are symptomatic at birth, while the vast majority
remain asymptomatic.[7]
There is a consensus of opinion
regarding the management of symptomatic CCAM
neonates who after radiological evaluations, will need
surgery. In contrast, the postnatal management of
asymptomatic neonates/infants remains controversial,
with some clinician advocating surgical resection
between 3 and 6 months of life,[8]
and others adopt a
more expectant (conservative) approach.[9]
This controversy in postnatal management of
asymptomatic infants exists mainly because of our
lack of knowledge regarding the natural postnatal
history of un‑resected CCAM lesions.[10]
In our
center, asymptomatic CCAM infants are managed
conservatively. The outcome of all pregnancies with a
fetal diagnosis of CCAM managed over a 12‑year period
is presented in this study. The symptom surveillance
of affected infants during the 1st
year of life was also
evaluated to help ascertain the safety of conservative
management approach in asymptomatic infants.
Methods
The case notes of mothers and infants with antenatal
diagnosis of CCAM born between January 1, 2004 and
December 31, 2015 were reviewed. Cases were identified
by querying various hospital databases, including the
fetal Medicine database, maternity database, the Neonatal
database, histopathology database, cardiothoracic surgery
database, and the regional congenital anomalies register.
Pertinent information on identified mother–infant dyad
including ethnicity, gestation at diagnosis, antenatal
progression/resolution of CCAM fetal lesion, antenatal
management, delivery details, neonatal outcome,
postnatal investigation, and management were extracted.
In addition, the clinical history of all surviving children
up to 1 year of age (ending December 31, 2016) was
also reviewed to assess symptom burden, hospital
readmission rates, and subsequent need for surgery
during infancy.
Data were subjected to simple descriptive statistics and
expressed as mean median, range, and percentages.
Antenatal and demographic differences between
symptomatic and asymptomatic infants were examined
using Mann–Whitney and Fisher’s exact test, and a
P < 0.05 was considered statistically significant. Ethical
approval was not required for this study, as this was a
service evaluation exercise.
Results
A total of 51 pregnancies with antenatal diagnosis of
CCAM were identified during the study period. One
pregnancy with an affected fetus who also had trisomy
21 ended in stillbirth. A further three pregnancies
were terminated on parental request. These four cases,
coupled with another five babies who had a postnatal
diagnosis of lung sequestration, were excluded, leaving
a final study population of 42 pregnancies/infants. The
study cohort was all singleton pregnancies that resulted
in live births [Figure 1].
Table 1: Study cohort demographics
Characteristics (features) n (%)
Gender
Male 21 (50)
Female 21 (50)
Ethnicity
White 27 (64.2)
Asian 9 (21.4)
Black 2 (4.8)
Others 2 (4.8)
Not known 2 (4.8)
Site of CCAM lesion
Left 20 (47.6)
Right 22 (52.4)
Size of CCAM lesion (cm)
<2 9 (21.4)
2‑5 14 (33.3)
>5 6 (14.3)
Missing data 13 (31.0)
Cyst appearance
Macrocystic 13 (31.0)
Microcsytic 19 (45.2)
Solid 1 (2.4)
Mixed 1 (2.4)
Missing data 8 (19.0)
Mode of delivery
Vaginal 31 (73.8)
Cesarean section 11 (26.2)
Other imaging in
pregnancy
Fetal MRI 6 (14.3)
None 36 (85.7)
CCAM – Congenital cystic adenomatous malformation;
MRI – Magnetic resonance imaging
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