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\begin{document}

\title{Successful Surgical Management of Symptomatic Antenatally Diagnosed
Congenital Pulmonary Airway Malformation: A Case Report}



\author[1]{Marah Mansour}%
\author[2]{Abdulmonem  Naksho}%
\author[3]{Yazan Abboud}%
\author[4]{Bayan Saqa}%
\author[5]{Mayas  Yousif}%
\affil[1]{Faculty of Medicine, Tartous University}%
\affil[2]{University of Westminster}%
\affil[3]{Cedars-Sinai Medical Center}%
\affil[4]{Damascus University}%
\affil[5]{Imam Zayn al-Abidin Hospital}%


\vspace{-1em}



  
  \date{May 28, 2024}


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\textbf{Successful Surgical Management of Symptomatic Antenatally
Diagnosed Congenital Pulmonary Airway Malformation: A Case Report}

\textbf{Key Clinical Message}

Routine obstetric ultrasound is important in detecting CPAM and it
should be followed up routinely throughout the pregnancy. Management of
CPAM is debatable and varies from conservative to invasive approaches.

\textbf{ABSTRACT}

Congenital pulmonary airway malformations (CPAM) are rare developmental
anomalies often diagnosed on prenatal ultrasound. This case presents a
30-year-old female who had a routine ultrasound in the 13th gestational
week which revealed cystic changes in the fetal left lung with
polyhydramnios. The delivered baby underwent an uneventful open left
lower lobectomy.

\textbf{Keywords:~} CPAM; Congenital lung lesions; Congenital pulmonary
airway malformations; Cystic adenomatoid malformation ; Case Report.

\textbf{Successful Surgical Management of Symptomatic Antenatally
Diagnosed Congenital Pulmonary Airway Malformation: A Case Report}

\textbf{Introduction}

Congenital pulmonary airway malformations (CPAM), characterized by an
abnormal airway pattern, are developmental malformations of the lower
respiratory tract that occur during lung branching morphogenesis. CPAM
has the potential to cause cystic and/or adenomatous pulmonary areas.
Despite being a rare condition with a prevalence of only 0.81/10,000
fetuses, CPAM is considered to be the most common congenital lung
abnormality, being responsible for 30--40\% of all malformations (1).
The pathogenesis and exact cellular mechanisms behind CPAM are not fully
understood yet; however, many potential genes were suspected to play a
part in the formation of lung cysts (2). Nowadays, with the advance of
prenatal ultrasound (90\% sensitivity and 77\% specificity for CPAM),
CPAM is often diagnosed antenatally, by detecting an echogenic mass
within the fetal lungs usually in the second trimester of gestation
(21--24 weeks), then followed up routinely during the course of
pregnancy (1). Magnetic resonance imaging (MRI) also plays an important
diagnostic role complementary to ultrasound since it is able to define
the type and extent of these lung lesions, thus guiding prenatal
intervention and postnatal care (2). CPAM can be classified into five
different types, each one of them vary in appearance and originate from
a different area in the lung (3). Prognosis, and therefore, management
usually depends on the classification and presence of symptoms and any
other fetal anomalies (3). We present a symptomatic case of CPAM with
the associated clinical, radiological and pathological details. The work
has been reported in line with the SCARE criteria (4).

\textbf{Case Report}

A 30-year-old pregnant female with an unremarkable surgical, medical,
drug or family history presented in the 13th week of gestation for a
routine Ultrasound (US) examination which revealed a cystic formation in
the left lung of the fetus associated with mild polyhydramnios. The
patient was asymptomatic and physical examination was unsignificant, By
monitoring, and during the 23rd and 26th weeks of gestation, the lesion
was stable and there was a slight increase in the amniotic liquid
(Fig.1). In the 39th week of gestation, the woman underwent caesarean
delivery and had a 3400 g weighing male who was admitted to the neonatal
intensive care unit by their parents and referred to the Department of
Pediatric Surgery complaining from severe respiratory distress worsening
during lactation, and it was decided to stay in the incubator for 24
hours. The chest radiography indicated the deviation of the mediastinal
structures toward the right-side Fig.2 (A). Chest radiography with oral
contrast excluded the presence of diaphragmatic hernia Fig.2 (B). At the
age of 14 days, a (computed tomography) CT scan of the chest with
contrast was performed and the diagnosis of CPAM was confirmed at the
expense of the lower left lobe (Fig.3). Except for the right axis
deviation, echocardiography did not indicate any cardiac abnormalities.
Upon completion of preoperative procedures, the 14-day-old patient
underwent an open left lower lobectomy via a left posterior thoracic
approach (Fig.4), and pathology studies of the resected part confirmed
the diagnosis of CPAM. The infant received post-operative care with
incubation for 3 days. On the third day after the operation, chest
radiography demonstrated the return of mediastinum to the midline, and a
compensatory hyperinflation of the residual lobes of the left lung.
Finally, the infant weaned off the respiratory support and was
discharged home at the age of 17 days without any complications. During
follow-up, the infant's condition was found to be satisfactory.

\textbf{Discussion}

CPAM, once referred to as congenital cystic adenomatoid malformation
(CCAM), is an uncommon, developmental lung anomaly of unknown cause,
presenting as cystic changes to the terminal bronchioles (5). There are
a few differential diagnoses to CPAM that include bronchogenic cysts
(BC), congenital lobar emphysema (CLE), and bronchopulmonary
sequestration (BPS) (1). Antenatally, CPAM can cause some complications
in the fetus, the most important of which is Hydrops Fetalis due to the
compressing of the mass on other structures, which usually requires an
emergency cesarean section (3). At birth, 70\% of CPAM are asymptomatic.
Among antenatally diagnosed patients, 70\% of antenatally diagnosed CPAM
are asymptomatic; while the other 30\% cause neonatal respiratory
distress, with 10\% have severe respiratory distress requiring assisted
ventilation (1). In some cases, CPAM remains unrecognized until
adolescence or later life when complications, such as recurrent
pulmonary infections, pneumothorax and lung abscess, start to happen
(5). In our case, there was symptomatic, antenatally diagnosed CPAM,
associated with Hydrops Fetalis antenatally (requiring caesarean
delivery), and severe respiratory distress postnatally (requiring
neonatal intensive care unit). Therefore, left lower lobectomy was
decided, and open surgery was chosen over thoracoscopy due limited
resources (lack of equipment and expert hands). The management of CPAM
is highly debatable, and varies from administering maternal
betamethasone prenatally (to decrease the size of large CPAMs and
reverse hydrops) to minimally invasive or even open surgical resection
postnatally, with the most controversial area remains the postnatal
management of asymptomatic cases. Prophylactic lung resection in
asymptomatic patients is sometimes done, and has advantages that include
reduced risk of infection, malignancy and decreased complication rate,
while disadvantages include mortality and morbidity associated with
operation and cancer risk despite excision. Due to the low incidence and
wide spectrum of manifestation of CPAM, it is not surprising that the
quality of the evidence on management styles is generally poor (6).
David et al (2016) proposed prenatal Fig.5 (A) and postnatal Fig.5 (B)
management algorithms (2). A meta-analysis by Naser and Bass (2012),
suggested that thoracoscopic resection is a safe and feasible
alternative to open resection of congenital lung lesions in experienced
hands, and no differences between thoracotomy versus thoracoscopy for
management of CPAMs were observed in terms of overall complications and
the duration of surgery. However, days with chest tube in place and
length of hospitalization period were longer after open surgery (7).

\textbf{Conclusion}

CPAM, previously known as CCAM, is the most common congenital lung
lesion. CPAM is classified into five different types, varying in origin,
and appearance. Routine obstetric US is particularly important in
detecting CPAM at around the second trimester of gestation and is
followed up routinely through the term of the pregnancy. Management of
CPAM is debatable and varies from conservative to invasive approaches.

\textbf{References}

\begin{enumerate}
\tightlist
\item
  Leblanc C, Baron M, Desselas E, et al. Congenital pulmonary airway
  malformations: state-of-the-art review for pediatrician's use. Eur J
  Pediatr. 2017;176(12):1559-1571. doi:10.1007/s00431-017-3032-7.
\item
  David M, Lamas-Pinheiro R, Henriques-Coelho T. Prenatal and Postnatal
  Management of Congenital Pulmonary Airway Malformation. Neonatology.
  2016;110(2):101-115. doi:10.1159/000440894.
\item
  Phillips P. Congenital Pulmonary Airway Malformation: A Case Study and
  Case Comparison. J Diagn Med Sonogr. 2016;32(5):294-298.
\item
  Agha RA, Franchi T, Sohrab C, Mathew G, Kirwan A, Thomas A, et al. The
  SCARE 2020 guideline: updating consensus Surgical Case Report (SCARE)
  guidelines. International Journal of Surgery. 2020; 84(1):226-30
\item
  Enuh HA, Arsura EL, Cohen Z, et al. A fatal case of congenital
  pulmonary airway malformation with aspergillosis in an adult. Int Med
  Case Rep J. 2014;7:53-56. Published 2014 Mar 20.
  doi:10.2147/IMCRJ.S54850.
\item
  Baird R, Puligandla PS, Laberge JM. Congenital lung malformations:
  informing best practice. Semin Pediatr Surg.
  2014;23(5):270277.doi:10.1053/j.sempedsurg.2014.09.007.
\item
  Nasr A, Bass J. Thoracoscopic vs open resection of congenital lung
  lesions: a meta-analysis. J Pediatr Surg. 2012;47(5):857-861.
  doi:10.1016/j.jpedsurg.2012.01.036.
\end{enumerate}

\textbf{Figure Legends}

Fig.1: Antenatal sonography monitoring (a) in the
23\textsuperscript{rd}week, there were well-defined cystic structures in
the left lung accompanied by mild polyhydramnios (b) in the
26\textsuperscript{th}week, a 20\selectlanguage{ngerman}×40 cm anechoic multicystic lesion was
occupying the left side of the thoracic cavity with moderate
polyhydramnios.

Fig.2: (A) postnatal plain trunk X-ray with mild diffuse opacity on the
left side that suggests a large cystic lesion and mediastinal shifting
to the right. (B) plain trunk X-ray with oral contrast assures the
absence of the diaphragmatic hernia.

Fig.3: CT scan of the chest with contrast suspects of 20×40 cm CPAM in
the left lower lobe consisting of enhanced solid mass in the posterior
part associated with a multicystic structure located anteriorly with
compensatory hyperinflation of the left upper lobe, which causes the
lung herniation to the right side.

Fig.4: Open left lower lobectomy via a left posterior thoracic approach.

Fig5: (A) Prenatal management algorithms of CPAM by David et al (2016).

ECHO = Echocardiogram. *~ Multiple courses of betamethasone may be an
option. (B) Postnatal management algorithms of CPAM by David et al
(2016).

ECHO = Echocardiogram; PPHN = persistent pulmonary hypertension of the
newborn; ECMO = extracorporeal membrane oxygenation.\selectlanguage{english}
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