Retrospective Evaluation of Children with Congenital Pulmonary Airway Malformation: A Single Center Experience of 20 Years

Ortac R., Diniz G., Yildirim H. T., AKTAŞ S., Karaca I.

FETAL AND PEDIATRIC PATHOLOGY, vol.35, no.3, pp.143-148, 2016 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 35 Issue: 3
  • Publication Date: 2016
  • Doi Number: 10.3109/15513815.2016.1142033
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.143-148
  • Keywords: Congenital cystic adenomatoid malformation, congenital pulmonary airway malformation, a single center experience, OF-THE-LITERATURE, CYSTIC ADENOMATOID MALFORMATION, LUNG MALFORMATIONS, DIAGNOSIS, LESIONS
  • Dokuz Eylül University Affiliated: Yes


Objectives: Congenital pulmonary airway malformation (CPAM) is an uncommon congenital abnormality of the lungs that generally presents during prenatal period or early childhood. In this study, we aimed to evaluate clinical and pathologic findings of the children with CPAMs who were referred to our center between 1992 and 2011. Material and Methods: We reviewed 19 children with CPAM, who were diagnosed and treated at the Izmir Dr. Behcet Uz Children's Hospital between 1992 and 2011. All of them are alive and have been still followed up by our center. Results: The study population consisted of 9 boys (47.4%) and 10 girls (52.6%) with a mean age of 3.26 (1 month - 13years). Most newborns had respiratory distress, while recurrent pulmonary infections were detected in older children. Surgical treatment was performed on patients with subtypes I (n = 4; 21.1%), II (n = 8; 42.1%), III (n = 5; 26.3%), and IV (n = 2; 10.5%). In 13 cases (63.4%), lesions were located in the right lung and in almost all cases lesions were confined to one lobe. A one-month- old child with type I CPAM had multiple lesions involving two lobes and in only a newborn with type II CPAM, lesions were located bilaterally. There was no type 0 cases in this series. All cases were treated with lobectomy without any complication. Conclusion: In the present study, a realistic comprehensive picture of CPAM in a central children's hospital has been provided. In addition, we want to emphasize that complications and unnecessary medical treatment could be reduced with early surgery.