Discussion:
Congenital lesion of the lung in children is uncommon but potentially
life-threatening, and warrants an urgent diagnostic
work-up.7,8,9,10 These lesions may involve the lung
parenchyma, bronchi, arterial supply, and venous drainage. They may
present with respiratory symptoms at birth or may be detected
incidentally either before or after birth. These lesions show close
relationship in terms of embryology and clinical presentation. Despite
the availability of the much-improved diagnostic modality the awareness
of these anomalies among pediatricians is still lacking. The main reason
behind the misdiagnosis of these cases is provisional diagnosis of
Tuberculosis based on respiratory symptoms and a lesion on X-ray chest.
In the parts of the world where Tuberculosis forms a prominent
differential diagnosis of all chronic chest conditions, labelling these
congenital lesions as tuberculosis is not surprising. These children are
empirically prescribed ATT and in case of no response to ATT, the
children are referred to higher centre for further management and hence
these cases land up with us. Some cases of CLE and macro cystic variety
of CCAM are misdiagnosed as pneumothorax and ICD insertion is done. But
physicians soon realize that they are dealing with something else and
they refer these cases, which form a major chunk of our study group.
These mistakes do take place because of lack of awareness on part of
physicians who do not suspect these conditions and hence do not look for
them. Evidence for starting ATT or putting ICD is based on clinical
examination, history and Chest X ray findings only. Clinical profile and
Chest X ray are not enough to diagnose these lesions because of
significant overlap of symptoms and X ray features. The gold standard
for diagnosis of congenital lesion of lung in children is contrast
enhanced computed tomography (CECT) chest. CCAMs presents as cystic or
solid lung lesions confined to a part of lung only. They account for
25% of total congenital lesions and most commonly present with
respiratory distress in newborns. Most of these lesions are now
routinely diagnosed on prenatal
ultrsonography.11,12,13,14 CCAM probably results from
a cessation of bronchial maturation and concomitant overgrowth of
mesenchymal elements, which produce the adenomatoid appearance of the
anomaly in the early stage of development.15 CCAMs
communicate with the bronchial tree at birth and therefore typically
contain air soon after birth.13 The imaging appearance
is determined by the size and number of cysts (figure 1). Lesions are
typically solitary with no lobar predilection. Because of the risks of
recurrent infection and malignant potential, resection remains the
current treatment.13,14 Congenital lobar emphysema
(CLE) is a marked pulmonary hyperinflation state that resembles all of
the clinical features of obstructive
emphysema.7,10,16,17 Congenital lobar emphysema
usually involves a single lobe and presents with respiratory distress in
the neonate.13,18Findings of air trapping with lobar
hyperinflation, mediastinal shift away from the involved lung, and
compression of the ipsilateral and contralateral lung. CT will show an
expanded lobe with attenuated vascular structures (figure2). It
is necessary for pediatricians to evaluate associated anomalies because
14% of the cases of CLE have coexistent congenital heart
disease.19 Bronchopulmonary sequestrations (BPSs) make
up10–30% of congenital cystic lung lesions. Pulmonary sequestration
develops from abnormal budding of foregut and is characterized by
non-functioning lung parenchyma that does not communicates with the
tracheobronchial tree and have an anomalous systemic arterial supply.
Bronchogenic cysts are the most common cystic lesion of the mediastinum.
They usually present with recurrent infection or airway compression
leading to wheezing, atelectasis, and air trapping and often present
later in childhood than other congenital lung lesions. Detailed x ray
and CECT chest findings of common congenital lesions of lung in children
are shown in table 2.
In our study group 13 (48.1 %) cases were misdiagnosed by primary care
providers and were unnecessarily subjected to either ATT or ICD which
resulted in higher morbidity in these cases either due to delay in
diagnosis and management or because of the treatment per se that is ICD
which complicates the surgery. Point we would like to highlight by this
study is that sooner or later all congenital lesions of lung will need
surgical intervention whether symptomatic or not. Therefore, they need
to be diagnosed correctly before starting any treatment whether ATT or
ICD. Chest X ray is not sufficient enough for making a diagnosis, CECT
chest should be done in all cases of congenital lesions of lung
irrespective of the age and symptoms of presentation. Although the
treatment of cystic lung lesions is quite straight forward, a strong
index of suspicion is essential in diagnosing this uncommon entity. This
entails prompt referral to a pediatric surgical centre thereby obviating
unnecessary prolonged and needless medical management.
The author would like to declare that there is no conflicts of interest
and this research received no specific grant from any funding agency in
the public, commercial or not-for-profit sectors.