title: Microcystic congenital pulmonary airway malformation with hydrops fetalis: steroids vs open fetal resection.
abstract: OBJECTIVE
  Congenital pulmonary airway malformations (CPAM) are rare lesions often diagnosed during routine prenatal ultrasound. The presence of hydrops fetalis is an indicator of poor prognosis. Here we present a retrospective review of fetuses undergoing either open fetal surgery or steroids for predominantly microcystic CPAM with hydrops fetalis.
  
  
  METHODS
  A retrospective review of patients undergoing open fetal surgery or steroids for CPAM at our institution was performed. The primary outcome was survival.
  
  
  RESULTS
  A retrospective review of all patients referred to our institution with the diagnosis of CPAM was performed. Fetuses with predominantly microcystic CPAM and the presence of hydrops fetalis treated with steroid or surgery were included. Thirteen patients were treated with steroids, and 11 patients underwent open fetal surgery. In the steroid group 12 (92%) of 13 fetuses survived to delivery versus 9 (82%) of 11 in the open fetal surgery group. Only 5 (56%) of 9 of the patients in the open fetal surgery group survived to neonatal discharge compared to 10 (83%) of 12 in the steroid group.
  
  
  CONCLUSIONS
  In the present retrospective study, improved survival was seen in fetuses with hydrops fetalis and predominantly microcystic CPAM treated with steroids when compared with open fetal surgery. Steroids should be considered for first-line therapy in these cases.
authors.affiliation: Department of Surgery, University of California at San Francisco, San Francisco, CA 94143-0570, USA.
authors.forename: Kenneth C
authors.initials: KC
authors.lastname: Loh
authors.forename: Eric
authors.initials: E
authors.lastname: Jelin
authors.forename: Shinjiro
authors.initials: S
authors.lastname: Hirose
authors.forename: Vickie
authors.initials: V
authors.lastname: Feldstein
authors.forename: Ruth
authors.initials: R
authors.lastname: Goldstein
authors.forename: Hanmin
authors.initials: H
authors.lastname: Lee
mesh_terms: D001623:Betamethasone / Q000627:therapeutic use*; D005260:Female; D005333:Fetus / Q000601:surgery*; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D015160:Hydrops Fetalis / Q000188:drug therapy* / Q000601:surgery*; D008168:Lung / Q000002:abnormalities* / Q000601:surgery*; D011247:Pregnancy; D012189:Retrospective Studies
chemical_list: D005938:Glucocorticoids; D001623:Betamethasone