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  • Title: Intralobar pulmonary sequestration associated with a congenital pulmonary airway malformation type II.
    Author: Couluris M, Schnapf BM, Gilbert-Barness E.
    Journal: Fetal Pediatr Pathol; 2007; 26(5-6):207-12. PubMed ID: 18363152.
    Abstract:
    Intralobar pulmonary sequestration (ILPS) is a rare congenital malformation of the lung. It is described as a segment of lung parenchyma with normal pleura and systemic blood supply and it has poor communication with the tracheobronchial tree. Patients usually present in later childhood or adulthood with a history of recurrent pneumonias. The malformation is rarely associated with other congenital anomalies such as a congenital pulmonary airway malformation (CPAM). A CPAM is a congenital cystic lesion of the lung that presents usually in the newborn period as respiratory distress. We describe the case of a 2-month-old female who presented to the local emergency room with the initial diagnosis of pneumonia, surgical diagnosis of ILPS, and a final pathological diagnosis of ILPS with an associated CPAM.
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