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Extralobar pulmonary sequestration: analysis of 15 cases.

J T Stocker, K Kagan-Hallet

    American Journal of Clinical Pathology
    |December 1, 1979
    PubMed
    Summary

    Extralobar pulmonary sequestration is a rare congenital lung malformation. Early diagnosis in newborns is critical, as most infants with this condition diagnosed at birth did not survive.

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    Area of Science:

    • Pediatric Surgery
    • Neonatal Medicine
    • Thoracic Pathology

    Background:

    • Extralobar pulmonary sequestration (EPS) is a congenital lung malformation.
    • It involves lung tissue with an abnormal systemic arterial supply and venous drainage.
    • This condition can lead to significant respiratory distress and mortality in neonates.

    Purpose of the Study:

    • To analyze the clinical presentation and outcomes of patients with extralobar pulmonary sequestration.
    • To identify associated congenital anomalies and their impact on survival.
    • To describe the pathological findings in affected infants.

    Main Methods:

    • Retrospective review of 15 patients diagnosed with extralobar pulmonary sequestration.
    • Analysis of clinical data, including age at diagnosis, associated anomalies, and outcomes.
    • Pathological examination of resected specimens and autopsy findings.

    Main Results:

    • Twelve of 15 patients (80%) were diagnosed on the first day of life, and all died.
    • EPS occurred more frequently in males (11 cases).
    • Associated anomalies were common (10/15 patients), including diaphragmatic hernia and bronchogenic cysts. Four infants presented with polyhydramnios and/or edema. Lymphatic dilatation was noted in four cases.

    Conclusions:

    • Extralobar pulmonary sequestration diagnosed neonatally has a very high mortality rate.
    • The presence of associated congenital anomalies significantly impacts prognosis.
    • Early recognition and management strategies are crucial for improving outcomes in affected infants.

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