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Published on: March 6, 2019
Congenital Lung Malformations: Experience From a Tertiary Care Center in India
Krishna Mohan Gulla1, Man Singh Parihar1, Kana Ram Jat1
1Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Insights
Congenital lung malformations (CLM) are common in Indian children, with lung hypoplasia being most frequent. Early diagnosis and management significantly reduce hospitalizations and improve outcomes for these rare conditions.
Area of Science:
- Pediatric Pulmonology
- Medical Genetics
- Thoracic Surgery
Background:
- Limited data exists on congenital lung malformations (CLM) and their clinical progression in developing nations.
- This study addresses the data gap by analyzing CLM cases from an Indian tertiary care center.
Purpose of the Study:
- To investigate the prevalence and clinical course of congenital lung malformations in children within an Indian tertiary care setting.
- To identify common CLM types, diagnostic delays, and the impact of management on patient outcomes.
Main Methods:
- A 10-year retrospective chart review was conducted.
- Data from 48 children diagnosed with CLM at a pediatric chest clinic were analyzed.
Main Results:
- Congenital lung hypoplasia/agenesis was the most common CLM (50%), followed by cystic pulmonary airway malformation (19%).
- Median age at diagnosis was 24 months, with delayed detection and surgical intervention noted.
- Associated congenital heart disease was present in 27% of cases. Management, including surgery, significantly reduced hospitalization rates.
Conclusions:
- Lung hypoplasia is the predominant CLM in this population, with poor antenatal detection rates.
- Delayed diagnosis and surgical intervention are significant challenges.
- Regular follow-up and appropriate management are crucial for reducing morbidity in children with CLM.
Background:
There are limited data on congenital lung malformations (CLM) and their clinical course from developing countries.
Methods:
A 10-year retrospective chart review of records of children with CLM attending pediatric chest clinic at an Indian tertiary care center was conducted.
Results:
Among the 48 children (24 boys) included in the review, the malformations included congenital lung ypoplasia/agenesis in 24 (50%), cystic pulmonary airway malformation in 9 (19%), bronchogenic/foregut cyst in 8 (18%), and congenital lobar emphysema in 4 (9%). Median (IQR) age at symptom onset and diagnosis were 1.5 (0.4,9.5) and 24 (3,62) months, respectively. Median (IQR) weight for age for age z-score at presentation was -2.4 (-1.4,-3.4). More than a third (37.5%) children underwent surgical removal of resectable lesions at median (IQR) age of 14 (6,42) months. 14 (27%) children had associated congenital heart disease. Median duration of follow-up was 13 months. In children with lung hypoplasia, median (IQR) number of hospitalizations in follow-up were significantly less than that prior to diagnosis 0 (0,0) vs 1(0,2) (P=0.001). Median (IQR) numbers of hospitalizations in follow up were significantly less than that of prior to surgical resection 0 (0,0) vs 1(1,1) (P=0.016) in children with CPAM.
Conclusion:
Lung hypoplasia was the most common congenital lung malformation in our setup. Detection of malformation during antenatal period was poor. Age of diagnosis and surgical intervention is often delayed. Regular follow up and definitive and/or supportive management decreased the morbidity.
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