Spectrum of pulmonary sequestration: Association with anomalous pulmonary venous drainage in infants

Abstract

Pulmonary sequestration is a spectrum of related lesions, each of which may be absent or present: (1) bronchial sequestration of pulmonary parenchyma; (2) arterial supply from systemic circulation; (3) anomalous pulmonary venous drainage to the right atrium; (4) communications between bronchus and esophagus; (5) defects of diaphragm; (6) gross lung anomalies, such as horseshoe lungs or hypoplasia. Any combination of these primary lesions can occur in an individual patient. Diagnosis should be directed towards each component of the spectrum. Of special importance is the venous connection, as anomalous pulmonary venous drainage can involve not only the sequestered segment but the entire ipsilateral lung, making surgical therapy far more complex. Treatment of choice is surgical resection, associated, if needed, with rerouting of the pulmonary venous return. Classification of sequestration of the lung as intra- and extralobar is of secondary importance: these 2 groups do not represent lesions of different embryological significance.

Congenital intralobar pulmonary sequestration with cystic adenomatoid malformation

“…ILSs, although rare in the neonate, are more common overall than extralobar sequestrations, comprising 75% of all report cases of pulmonary sequestrations. 3 Pulmonary sequestrations are complex malformations, many of which do not easily fit into current nomenclature easily. 4 There are six features that must be evaluated to characterize a particular lesion.…”

Concurrent Aortic Valvular Disease and Pulmonary Sequestration: Clinical Implications

“…Whereas the anomalous drainage is usually from the sequestrum itself, the entire ipsilateral lung can be involved. 10 In one case, 11 drainage from the sequestrum to the left atrium led to left-to-left shunting. This resulted in an initial misdiagnosis of residual aortic stenosis after aortic valvuloplasty.…”

Recurrence of right lower lobe pneumonia 3 years after the first episode in an otherwise healthy 13-year-old girl

“…Others may present as a single display respiratory distress or feeding difficulties shortly after birth [8][9][10]. In slightly older children, respiratory symptoms, occasionally with congestive cardiac failure, may occur [8,[10][11][12]. The intralobar variety is also located in 60% of homogeneous opacity or a well-defined triangular shaped or a cystic mass, or, less commonly, a cystic mass eventually with air fluid levels, caused by a bronchial communication, in 26% of cases [12,13].…”