Prenatal growth characteristics and pre/postnatal management of bronchopulmonary sequestrations

J Pediatr Surg. 2018 Feb;53(2):265-269. doi: 10.1016/j.jpedsurg.2017.11.020. Epub 2017 Nov 16.

Abstract

Purpose: The prenatal natural history of intralobar and extralobar bronchopulmonary sequestrations (BPSs), including lesion growth patterns and need for prenatal intervention, have not been fully characterized. We review our series of BPSs to determine their natural history and outcomes in the context of the need for prenatal intervention.

Methods: A retrospective review of the pre/postnatal course of 103 fetuses with an intralobar (n=44) or extralobar BPS (n=59) managed at a single institution between 2008 and 2015 was performed. Outcomes included prenatal lesion growth trajectory, presence of hydrops, need for prenatal intervention, survival, and postnatal surgical management.

Results: Most extralobar (71%) and intralobar BPSs (94%) decreased in size or became isoechoic from initial to final evaluation. Peak lesion size occurred at 26-28weeks gestation. Eight fetuses developed hydrothorax, four of which (all extralobar BPSs) also developed hydrops. All four hydropic fetuses received maternal betamethasone, and three hydropic fetuses underwent thoracentesis and/or thoracoamniotic shunt placement with subsequent hydrops resolution. All fetuses survived. Forty-one intralobar (93%) and 35 extralobar BPSs (59%) were resected after birth.

Conclusions: BPSs tend to decrease in size after 26-28weeks gestation and rarely require fetal intervention. Lesions resulting in hydrothorax ± hydrops can be effectively managed with maternal steroids and/or drainage of the hydrothorax.

Level of evidence: IV.

Keywords: Bronchopulmonary sequestration; Hydrops; Hydrothorax; Laser coagulation; Thoracentesis; Thoracoamniotic shunt.

MeSH terms

  • Bronchopulmonary Sequestration / diagnosis
  • Bronchopulmonary Sequestration / embryology*
  • Bronchopulmonary Sequestration / mortality
  • Bronchopulmonary Sequestration / therapy*
  • Female
  • Fetal Therapies / methods*
  • Follow-Up Studies
  • Humans
  • Infant, Newborn
  • Male
  • Perinatal Care / methods*
  • Pregnancy
  • Prenatal Diagnosis
  • Retrospective Studies
  • Treatment Outcome