title: Prenatal diagnosis and management of mainstem bronchial atresia.
abstract: The prenatal diagnosis, natural history and management of mainstem bronchial atresia have not been described previously. We report two cases of prenatally diagnosed proximal bronchial atresia. The first patient presented at 18 weeks with sonographic and MRI findings consistent with bronchial atresia with fetal hydrops. The mother developed the mirror syndrome and labor was induced. A non-viable fetus was delivered at 25 weeks. The second patient presented at 16 weeks gestation with evidence of an intrathoracic mass that was subsequently prenatally diagnosed as a right mainstem bronchial atresia. The right lung increased rapidly in size and was associated with the onset of fetal hydrops. At 24 weeks, fetal pneumonectomy was performed but the fetus expired intraoperatively due to cardiovascular collapse. Post-mortem findings in both cases confirmed the presence of an atretic mainstem bronchus with massive enlargement of the lung. Bronchial atresia involving the mainstem bronchus is associated with a poor prognosis.
authors.affiliation: Center for Fetal Diagnosis and Treatment, The Chhildren's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
authors.forename: Sundeep G
authors.initials: SG
authors.lastname: Keswani
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Crombleholme
authors.forename: Bruce R
authors.initials: BR
authors.lastname: Pawel
authors.forename: Mark P
authors.initials: MP
authors.lastname: Johnson
authors.forename: Alan W
authors.initials: AW
authors.lastname: Flake
authors.forename: Holly L
authors.initials: HL
authors.lastname: Hedrick
authors.forename: Lori J
authors.initials: LJ
authors.lastname: Howell
authors.forename: R Douglas
authors.initials: RD
authors.lastname: Wilson
authors.forename: George H
authors.initials: GH
authors.lastname: Davis
authors.forename: N Scott
authors.initials: NS
authors.lastname: Adzick
mesh_terms: D001980:Bronchi / Q000002:abnormalities* / Q000196:embryology* / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D015160:Hydrops Fetalis / Q000150:complications; D008279:Magnetic Resonance Imaging; D011013:Pneumonectomy; D011296:Prenatal Diagnosis; D015619:Respiratory System Abnormalities / Q000150:complications / Q000175:diagnosis* / Q000196:embryology / Q000473:pathology / Q000601:surgery*