# Surgical Management of Congenital Lung Malformations in Children—A Single‐Center Analysis of 25 Years of Experience

## TL;DR

This paper analyzes 25 years of surgical treatments for congenital lung malformations in children, emphasizing the importance of early diagnosis and personalized care.

## Contribution

The study provides a detailed single-center retrospective analysis of surgical management and outcomes for various congenital lung malformations in pediatric patients.

## Key findings

- The study cohort included 37 patients with diverse congenital lung malformations, including CPAM and pulmonary sequestration.
- Early diagnosis and intervention were found to be critical for improving prognosis in children with CLMs.
- Collaborative, multidisciplinary management is essential for optimal outcomes in these patients.

## Abstract

Congenital lung malformations (CLMs) in pediatric patients encompass various structural abnormalities arising during fetal development, which can range from benign to life‐threatening. The most common types include congenital pulmonary airway malformation (CPAM) and bronchopulmonary sequestration (BPS).

This study aimed to retrospectively analyze patients treated surgically for CLMs, focusing on indications for surgery, surgical techniques, and outcomes.

Data were collected from the medical records of patients who underwent thoracoscopy (n = 140) or thoracotomy (n = 52) between 2000 and 2024. Among these, 50 patients were diagnosed with CLMs, who were taken for further analysis. Study group inclusion criteria were performing a CT/X‐ray imaging examination indicating the presence of a defect, surgery, and available pathology results. Exclusion criteria were incomplete data or lack of surgical procedure. Final study group included 37 patients who met inclusion criteria for further analysis. Detailed analysis encompassed demographics, clinical presentation, diagnostic methods, treatment, and follow‐up.

The cohort included patients diagnosed with CPAM type I (n = 12), CPAM type II (n = 7), pulmonary sequestration (n = 10), and other congenital malformations such as bronchogenic cyst (n = 2), congenital cystic pulmonary disease (n = 2), CPAM type IV—pleuropulmonary blastoma type I (PPB) (n = 1), juvenile emphysema (n = 2), and mediastinal cyst (n = 1). The average age at diagnosis was 37.61 months. The cohort consisted of 17 females and 20 males. The right lung was involved in 41.18% of cases, and the left lung in 58.82%. Symptoms at presentation included pneumonia (n = 9), respiratory failure (n = 8), emphysema (n = 3), and pneumothorax (n = 2). Fifteen patients were asymptomatic, and the diagnosis was incidental. Seven patients had other congenital diseases, such as heart defects. None of the patients other than the child with PPB were offered genetic diagnostics, albeit for DICER1 or KRAS mutations.

The study underscores the heterogeneity in age and clinical presentation at the time of CLM diagnosis, highlighting the importance of an individualized and tailored approach to management.

Early diagnosis and intervention are critical for improving prognosis in children with congenital lung malformations (CLM). Fetal diagnosis of CLM generally leads to favorable outcomes; however, there is limited evidence on the effectiveness of fetal therapy in improving outcomes for these anomalies. Collaborative management involving multiple specialties is essential for optimal outcomes.