Abstract

Background: Congenital Lobar emphysema (CLE) is one of the rarest cystic lesions in the thoracic cavity among children. It is often misdiagnosed and managed incorrectly. We are reporting our institutional experience and recommendations regarding the prevention of common errors in the management of this condition. Methods: This study was conducted at the Paediatric Surgery Department, The Children’s Hospital & the University of Child Health Sciences Lahore from January 2015 to December 2018. All the patients who underwent thoracotomy for congenital lobar emphysema at our center were included in the study and their charts were reviewed. Results: A total of 19 patients had been treated at our center in this duration. The mean age of patients was 2.85 ± 2.11 months. Thirteen of them (68%) were male and the most common presenting complaint (94.7%) was respiratory distress. Chest X-ray was done in all of the patients while a CT scan was needed in 11 patients (57.9%). The most involved lobe was left upper (n=15). Lobectomy was done in all patients. Post-operative ventilation was required in 2 patients (10.5%) and pneumonia developed in 2 patients (10.5%). The most significant complication was wound infection (9/19, p=<0.01). Conclusion: Our results about perioperative management are consistent, however, we received cases with misdiagnosis. We have proposed recommendations to address this issue.

Highlights

  • Congenital Lobar Emphysema (CLE) is one of the rare anomalies of the lungs, having a prevalence of 1 in 20,000 to 1 in 30,000.[1]. It was first reported by Nelson in 1932 and was named by Robertson and James in 1951.[2]. Its antenatal prevalence is not known yet

  • There has been an alternate theory of hyperalveolosis, according to which the increased number of alveoli leads to hyperinflation of the affected lobe.[4]

  • The mediastinum is shifted to the contralateral side, which leads to bilateral functional compromise and eventually leading to respiratory distress, cyanosis, and repeated pneumonia, which may be the typical feature in this disease

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Summary

Introduction

Congenital Lobar Emphysema (CLE) is one of the rare anomalies of the lungs, having a prevalence of 1 in 20,000 to 1 in 30,000.[1] It was first reported by Nelson in 1932 and was named by Robertson and James in 1951.[2] Its antenatal prevalence is not known yet. CLE is defined as hyperinflation of lobes of the lungs which allows airflow on inspiration only. Due to hyperinflation of the affected lobe, it gives a typical appearance on chest X-ray (CXR) including the presence of lung markings and ipsilateral atelectasis. Congenital Lobar emphysema (CLE) is one of the rarest cystic lesions in the thoracic cavity among children. It is often misdiagnosed and managed incorrectly. We are reporting our institutional experience and recommendations regarding the prevention of common errors in the management of this condition

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