Pediatric emergency medicine trisk 4095 4095
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present in more than one lobe of the lung in up to 3% of cases. If a CPAM lesion
also receives systemic blood supply, which is a characteristic of BPS, it is termed
a hybrid lesion . The tissue within a CPAM does not function in normal gas
exchange but is connected with the tracheobronchial tree; therefore, these lesions
can lead to air trapping and recalcitrant pulmonary infections. Rarely, patients
may develop malignant degeneration within the lesion (pleuropulmonary
blastoma, rhabdomyosarcoma).
FIGURE 124.7 Congenital pulmonary airway malformation in a 12-month-old girl with
recurrent episodes of left-sided pneumonia of the lower lobe.
BPSs arise from an accessory bronchopulmonary bud of the foregut.
Histologically, they consist of pulmonary tissue; however, they are not connected
with the normal bronchial tree or pulmonary vessels (and hence, the pulmonary
tissue is “sequestered”). Occasionally, sequestrations have a connection with the
esophagus or stomach, because of their foregut derivation. They have a systemic
rather than pulmonary blood supply. A sequestration is described as intralobar if
it is contained within the normal pleura, or extralobar if it has its own pleural
