[The extralobar sequestration of the fetal lung]

The extralobar sequestration is a rare pulmonary malformation. An accurate antenatal evaluation is required for a timely therapy and subsequently a good outcome. Here an unusual case of extralobar pulmonary sequestration in a male human fetus is reported. Antenatal ultrasound at 28th week of gestation has revealed a fetal hydrothorax in coexistence with pulmonary hypoplasia and an isolated pulmonary structure. Authors summarise their postnatal findings with special reference to the pathogenesis of an accessory lung. The aim of this report is to define the association of clinical, gross, and histological features of this rare congential malformation in order to improve the antenatal diagnosis. This case indicates that an extralobar pulmonary sequester is not connected to the tracheobronchial tree, and that the arterial as well as the venous blood supply is realised by aberrant systemic vessels. Moreover, histologically revealed dilatations of the normally differentiated terminal airways within the sequester suggest that hyperechogenity can not be a reliable diagnostic criterion. For the accurate assessment of a pulmonary sequestration a detailed antenatal evaluation of both, the arterial and the venous blood supply is essential.