Krishna Mohan Gulla1, Man Singh Parihar1, Kana Ram Jat1, Sandeep Agarwala2, Rakesh Lodha3, S K Kabra1. 1. Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India. 2. Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India. 3. Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India. Correspondence to: Dr Rakesh Lodha, Professor, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110 029, India. rakesh_lodha@hotmail.com.
Abstract
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.
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.