Revisiting the Histopathologic Spectrum of Congenital Pulmonary Developmental Disorders

Abstract

A retrospective analysis of lobectomy, pneumonectomy, cystectomy specimens (10 years) and stillbirth/neonatal autopsies (5 years) was carried out to analyze the histologic spectrum of congenital pulmonary developmental disorders/malformations. The autopsy data was analyzed to identify the lesions that are more diffuse and not amenable to surgical management. A total of 166 cases of pulmonary developmental disorders/ malformation were found. Out of 2,155 stillbirth/neonatal autopsies, there were 105 cases of pulmonary hypoplasia, 2 cases of congenital pulmonary lymphangiectasis, 2 cases of extralobar sequestration, and 3 cases of congenital pulmonary airway malformation (CPAM). Among the surgical specimens, there were 21 cases of CPAM, 11 cases of intralobar sequestration, 5 cases of congenital lobar emphysema and 17 cases of bronchogenic cyst. All these malformations have been described in reference to the latest updated classification of developmental disorders/malformations.

Keywords :

• pulmonary developmental disorder
• pulmonary hypoplasia
• congenital pulmonary airway malformation (CPAM)
• intralobar sequestration (ILS)
• congenital pulmonary lymphangiectasis (CPL)
• congenital lobar emphysema (CLE)
• autopsy