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Congenital lobar emphysema. Report of 17 cases

Monia Khemiri1, Faouzia Khaldi, Saayda Ben Becher

  • 1Service Médecine Infantile A, Hôpital d'enfants Tunis.

La Tunisie Medicale
|May 30, 2009
PubMed

Insights

Congenital lobar emphysema (CLE) is an uncommon cause of infant respiratory distress. This study found CLE to be the most frequent cystic lung malformation in children, with most cases successfully treated by lobectomy.

Area of Science:

  • Pediatric Pulmonology
  • Thoracic Surgery
  • Medical Imaging

Background:

  • Congenital lobar emphysema (CLE) is a rare congenital lung malformation.
  • It presents as a significant cause of respiratory distress in infants.
  • Understanding its frequency and clinical presentation is crucial for diagnosis and management.

Purpose of the Study:

  • To determine the frequency and clinical characteristics of Congenital lobar emphysema (CLE).
  • To analyze associated conditions and treatment outcomes for CLE.
  • To establish CLE as the most common cystic pulmonary malformation in the study cohort.

Main Methods:

  • Retrospective review of CLE cases managed between 1994 and 2004.
  • Analysis of patient demographics, age at diagnosis, and presenting symptoms.
  • Radiological assessment using Chest X-ray and CT scans; review of surgical outcomes.

Main Results:

  • Out of 31 cystic pulmonary malformations, 17 cases of CLE were identified.
  • The mean age at diagnosis was 4.5 months, with a male predominance (12 males, 5 females).
  • Common symptoms included respiratory distress and recurrent dyspnea; associated anomalies included bronchogenic cysts and congenital heart defects. All patients underwent lobectomy with an uneventful postoperative course in 16 cases.

Conclusions:

  • Congenital lobar emphysema (CLE) is an infrequent but significant cause of respiratory distress in neonates and infants.
  • CLE represents the most common type of cystic pulmonary malformation within this pediatric institution.
  • Surgical lobectomy is an effective treatment for CLE, with a high success rate.
Abstract

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