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Indian Pediatr 2018;55:883-884 |
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Clinical
Characteristics and Associated Congenital Lesions with
Tracheomalacia in Infants
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D Vijayasekaran, S Balasubramanian, Somu Sivabalan
and K Vindhiya
From the Departments of Pediatrics and Pulmonology,
Kanchi Kamakoti CHILDS Trust Hospital, Chennai, India.
Correspondence to: Dr D Vijayasekaran, Consultant
Pulmonologist, Kanchi Kamakoti CHILDS Trust Hospital,12-A, Nageshwara
Road, Nungambakkam, Chennai 600 034, India.
Email: [email protected]
Received: April 22, 2017;
Initial review: October 03, 2017;
Accepted: July 31, 2018.
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Objective: To describe clinical presentation, bronchoscopy findings
and associated anomalies in cases of congenital tracheomalacia in
infants (age <1y). Methods: Hospital record review of 88 infants
(mean age 8 mo, 57 males) diagnosed as having tracheomalacia by flexible
bronchoscopy between 2012 and 2015. Results: The predominant
features were wheeze (57.9%), stridor (42.1%), cough (38.6%), pneumonia
(29.5%) and collapse (12.5%). On bronchoscopy, malacia was observed in
lower half of trachea in 51 (57.9%) infants. Synchronized airway lesions
observed were laryngomalacia (30.7%) and bronchomalacia (3.4%). 15 (17%)
infants had associated congenital heart disease and 21 (23.8%) required
care in intensive care unit. Conclusion: Wheeze,stridor and cough
are the main symptoms in tracheomalacia. Laryngomalacia and congenital
heart diseases are the most common other anomalies associated in these
infants.
Keywords: Congenital heart disease, Bronchomalacia, Flexible
bronchoscopy,Outcome.
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A irway malacia is a structural anomaly that
predisposes children to recurrent respiratory problems. Congenital
airway malacias include tracheomalacia, bronchomalacia, and
tracheobronchomalacia [1]. Tracheomalacia is due to the developmental
defect in the cartilage of the tracheal wall. Since the walls of the
trachea are softer than normal, partial collapse of trachea results
during expiration, and gets exaggerated during activity [2]. Depending
on the site affected, tracheomalacia may present with a wide spectrum of
clinical problems from noisy breathing to acute severe wheeze. Children
present with respiratory compromise, and require intensive care and
ventilator support. Morbidity increases if tracheomalacia is associated
with other anomalies. This study was undertaken to describe the clinical
presentation, characteristics and associated anomalies of infants with
tracheomalacia.
Methods
Records of infants who were diagnosed as congenital
tracheomalacia based on the flexible bronchoscopy between 2012 and 2015
at Kanchi Kamakoti CHILDS Trust Hospital, were reviewed. All the
bronchoscopy procedures were video recorded with mutual verification by
a fellow doctor trained in pediatric pulmonology. Except in two infants,
all the bronchoscopy procedures were done under local anesthesia [3].
The observation of the anterior tracheal wall collapsing and reducing
>50% of lumen diameter against the posterior wall was the basis of
diagnosis of tracheomalacia. In addition to the bronchoscopic details,
the spectrum of clinical presentation, associated radiographic and
echocardio-gram findings were recorded.
Results
Of the 88 infants (mean age 8 mo, 57 boys) with
tracheomalacia diagnosed during the study period, 16 (18.2%) were born
preterm, out of which five had received surfactant and ventilation for
respiratory distress syndrome. Nineteen (21.6%) of infants were aged
below 3 months, 20 (22.7%) were in the age group 4-6 months, 16 (18.2%)
were aged between 7 and 9 months, and 33 (37.5%) were aged 10 months and
above. The predominant complaints were wheeze in 51 (57.9%), stridor in
37 (42.1), and cough in 34 (38.6%) infants. All infants with
tracheomalacia had recurrent respiratory problems requiring repeated
nebulizations. Radiographic features included pneumonia (consolidation)
in 26 (29.5%), collapse in 11 (12.5%), mild cardiomegaly in 2 (2.3%),
hypoplasia of lung in 1 (1.2%); 48 (54.5%) had a normal chest X-ray.
Bronchoscopy demonstrated malacia of the upper half of trachea in 37
(42.1%) and lower half in 51 (57.9%). Synchronized airway lesions were
observed in 30 (34.1%) infants, which included laryngomalacia in 27
(30.7%) and bronchomalacia in 3 (3.4%). Congenital heart diseases were
noted in 15 (17.0 %), which included atrial septal defect (4),
ventricular septal defect (3), patent ductus artoriodus (3) pulmonary
hypertension (2), partial anomalous pulmonary venous connection (1),
single ventricle (1) and Fallot’s tetralogy (1). Twenty-one (23.8%) of
infants with tracheomalacia required PICU care.
Discussion
In this hospital record review, we documented that
synchronized airway lesions and congenital heart diseases are found
commonly in infants with tracheomalacia. The actual incidence of
tracheomalacia cannot be arrived at as it was a hospital-based study.
Earlier studies report on estimated incidence of at least 1 in 2,100
children [4]. Though some centers use general anesthesia and rigid
bronchoscopes, we used flexible bronchoscopy under topical anesthesia.
As tracheomalacia is a dynamic condition, flexible bronchoscopy
performed under topical anesthesia is the preferred technique [5].
A recent study in infants with moderate and severe
laryngomalacia, flexible bronchoscopy demonstrated synchronized lower
airway anomalies in 48%, of which tracheomalacia was the most common
[6]. Parenchymal lung lesions are also associated with
tracheomalacia, and combined therapy to control pulmonary infection
along with airway clearance (remove the backlog of secretions) may
improve the outcome [7]. A high proportion of congenital heart disease
in children with tracheonalacia has also been reported earlier [8-10],
and it may be worthwhile screening all such children with
echocardiography.
In our series, about one-fourth infants required ICU
care for severe wheeze. In airway malacias, due to the defective
cartilage, the contour of airways is maintained by the bronchial smooth
muscle tone. When these children are treated with beta-agonists, their
wheeze may worsen due to a further reduction in the muscle tone [11].
Thus any infant presenting with acute wheeze requiring a hospitalization
whose response to treatment is inadequate or worsening with standard
protocol warrants bronchoscopy to rule out underlying congenital airway
anomalies.
The main limitations of this study are: retrospective
data, referral bias, small sample size, and lack of follow-up data and
surgical outcomes.
We conclude that infants with tracheomalacia have a
high frequency of associated anomalies of airway and cardiovascular
system.
Contributors: DV, KV: designing the study and
analyzing the data. DV, SBS: drafted the manuscript; DV, SS: were
involved in review of literature and manuscript preparation. The final
manuscript was approved by all the authors.
Funding: None; Competing Interest: None
stated.
What This Study Adds?
• A high proportion of hospitalized infants with
tracheomalacia have an associated cardiac anomaly.
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