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The natural history of prenatally diagnosed congenital pulmonary airway malformations and bronchopulmonary sequestrations

  • Matilda Karlsson
    Correspondence
    Corresponding author at: Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden
    Affiliations
    Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden

    Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
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  • Peter Conner
    Affiliations
    Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden

    Center for Fetal Medicine Karolinska University Hospital, Stockholm, Sweden
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  • Henrik Ehren
    Affiliations
    Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden

    Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
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  • Catarina Bitkover
    Affiliations
    Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden

    Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
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  • Carmen Mesas Burgos
    Affiliations
    Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden

    Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden
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      Highlights

      • More than half (56%) of the prenatally diagnosed CPAM/BPS will reduce in size during the third trimester of gestation.
      • A quarter of the lesions (29%) will become undetectable late in gestation.
      • 19% diagnosed CPAM/BPS will develop symptoms after the neonatal period, and 81% will remain asymptomatic.
      • One third (31%) of the lung lesions in our institution needed surgical resection.
      • Large lesion size and mediastinal shifting correlate with a higher rate of surgical resection.

      Abstract

      Background

      The natural history of congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestrations (BPS) is not fully understood, and the management of the newborn with an asymptomatic lesion is a controversial issue. We aimed to study the natural history and outcome of CPAM/BPS at our institution with a policy of watchful waiting, and to investigate if any prognostic factors in the pre- and/or postnatal- period may predict the need for surgery.

      Material and methods

      A retrospective review study was conducted of children prenatally diagnosed with CPAM and/or BPS during the 18-year period, from 2002 to 2020. Data from the pre and postnatal period was collected and analysed.

      Results

      Sixty- six patients with prenatally observed lung lesions were entered in the study, with an overall survival rate of 94%. Fifty-six percent of the lesions decreased in size during gestation. Thirty-one percent had surgery and 69% could be managed conservatively with a median follow-up of 4 years. Nineteen percent developed symptoms after the neonatal period. Children with a presence of mediastinal shift on postnatal imaging (p = 0.003), with a high CVR (p = 0.005) and a large lesion size during gestation (p = 0.014) were significantly more likely to require surgery.

      Conclusion

      Prenatal regression is common among prenatally diagnosed CPAM/BPS and the majority of children that are asymptomatic beyond the neonatal period will remain asymptomatic throughout their childhood. Future analysis with a longer follow-up might give new insights in order to identify children at risk of developing symptoms.

      Level of evidence

      III

      Keywords

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