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.

Congenital pulmonary lymphangiectasis

“…A very recent report on the histopathological spectrum of congenital pulmonary developmental disorders stated that out of 2,155 stillbirth/neonatal autopsies there were 105 cases of pulmonary hypoplasia, two cases of CPL, two cases of extralobar sequestration, and three cases of congenital pulmonary airway malformation [32]. This data would suggest that approximately one in a thousand either stillborns or neonatal deaths is to some degree attributable to CPL, and is not consistent with significantly higher estimates reported in a review paper by Bellini et al [2].…”

Linfangiectasia pulmonar congénita unilateral en un neonato prematuro

“…The incidence of CPL is difficult to estimate since there are only publications of case reports and small series of cases. Autopsy studies suggest that approximately 0.5-1% of newborns who died in the neonatal period had CPL [6][7][8] . Generally, in the CPL both lungs are affected and have a poor prognosis with a mortality rate of 50-98% 8,9 .…”

“…Autopsy studies suggest that approximately 0.5-1% of newborns who died in the neonatal period had CPL [6][7][8] . Generally, in the CPL both lungs are affected and have a poor prognosis with a mortality rate of 50-98% 8,9 . Unilateral presentation is extremely rare, only few cases have been reported, it has a better prognosis and sometimes it has a spontaneous resolution [10][11][12] .…”

Congenital cystic lung disease: prenatal ultrasound and postnatal multidetector computer tomography evaluation. Correlation with surgical and pathological data

“…CPAMs make up almost 30-40 % of all congenital lung lesions, and are characterised by an excessive overgrowth of the terminal respiratory bronchioles and lung structures with alveolar growth suppression [2]. Genes such as HOXB5, Fgf7, and platelet-derived growth factor-B (PDGF-B) have been implicated in the pathogenesis of CPAM [3][4][5].…”

“…Associated malformations involving other organs may be present. PS is the second most common lesion after CPAM, characterised by a portion of lung parenchyma that does not connect with the tracheobronchial system and is therefore not ventilated whereas a systemic arterial supply is always present [2]. Based on the age of onset, PS may be distinguished in intralobar and extralobar; the latter may be associated with other congenital anomalies [1,8,9].…”