Article Text

CONGENITAL PULMONARY MALFORMATIONS: CLINICAL, IMAGING FINDINGS AND OUTCOME
  1. L Sfaihi Ben Mansour1,
  2. A Bouraoui1,
  3. B Maalej1,
  4. H Aloulou1,
  5. A Mahfoudh2,
  6. D Stoppalyonnet4,
  7. M R Barbouch3,
  8. A Saad5,
  9. Th Kammoun1,
  10. M Hachicha1
  1. 1Pediatric Departement, CHU Hedi Chaker, Sfax, Tunisia
  2. 2Urgence and Reanimation Pediatric Departement, CHU Hedi Chaker, Sfax, Tunisia
  3. 3Laboratoire Immunologie, Institut Pasteur, Tunis, Tunisia
  4. 4Institut Curie, Paris, France
  5. 5Laboratoire Cytogenetique, CHU Farhat Hached Sousse, Sousse, Tunisia

Abstract

Background Congenital malformations of the lung are rare and vary widely in their presentation and severity. The evaluation of affected patients frequently requires multiple imaging modalities to diagnose the anomaly and plan surgical correction.

Materials and Methods From January 1987 to March 2008, a total of 18 patients from birth to 5 years of age (median 5.76 months) with congenital pulmonary malformation were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs, computer tomography (CT), magnetic resonance imaging (MRI) were analysed to confirm the diagnosis of congenital pulmonary malformations.

Results During 21 years, 18 patients were diagnosed with congenital malformations of the lung, which included bronchogenic cyst (1 case), cystic adenomatoid malformation (3 cases), congenital lobar emphysema (8 cases), pulmonary sequestration (1 case), pulmonary agenesis (2 cases) and pulmonary hypoplasia (2 cases). One patient had three simultaneous abnormalities (pulmonary sequestration, bronchogenic cyst and cystic adenomatoid malformation). Common clinical presentations were respiratory distress (2 cases), respiratory infections (5 cases) and dyspnoea (11 cases). Diagnostic modalities included chest radiography (18 cases), CT scan (14 cases), MRI scan (1 case) and bronchoscopy (3 cases). Thoracotomy with excision of the lesion by lobectomy or pneumonectomy resulted in the survival of 14 patients (77%). Four deaths (23%) were due to pulmonary sequestration (1 case), nosocomial infection (1 case) and chronic respiratory insufficiency (2 cases).

Conclusion These data demonstrate that congenital bronchopulmonary malformations can usually be diagnosed by plain chest x ray films. CT may occasionally be necessary. All lesions, including symptomatic lesions in neonates, can be managed surgically soon after diagnosis.

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