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Prenatal growth characteristics and pre/postnatal management of bronchopulmonary sequestrations

John S Riley1, John W Urwin1, Edward R Oliver1

  • 1The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Journal of Pediatric Surgery
|December 13, 2017
PubMed

Insights

Bronchopulmonary sequestrations (BPSs) typically shrink after 26-28 weeks gestation and rarely need prenatal intervention. Fetal hydrothorax or hydrops can be managed with maternal steroids or drainage procedures.

Area of Science:

  • Perinatology
  • Fetal Surgery
  • Pediatric Surgery

Background:

  • Bronchopulmonary sequestration (BPS) is a rare congenital lung malformation.
  • The prenatal natural history, including lesion growth and the need for intervention, remains incompletely understood.

Purpose of the Study:

  • To characterize the prenatal natural history of intralobar and extralobar BPS.
  • To evaluate lesion growth patterns and the necessity for prenatal intervention in BPS.

Main Methods:

  • Retrospective review of 103 fetuses with intralobar or extralobar BPS (2008-2015).
  • Analysis of prenatal lesion growth, hydrops development, interventions, survival, and postnatal surgical outcomes.

Main Results:

  • Most BPS lesions decreased in size or became isoechoic prenatally, with peak size around 26-28 weeks gestation.
  • Eight fetuses developed hydrothorax, four with hydrops, successfully treated with maternal betamethasone and/or pleural drainage.
  • All fetuses survived, with most undergoing postnatal resection.

Conclusions:

  • BPS lesions generally exhibit a regressing natural history post-mid-gestation.
  • Fetal intervention for BPS is uncommon; hydrothorax/hydrops can be managed effectively with steroids and/or drainage.
Abstract

Keywords:
Bronchopulmonary sequestrationHydropsHydrothoraxLaser coagulationThoracentesisThoracoamniotic shunt

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