title: Severe pulmonary hypoplasia associated with giant cervical teratomas.
abstract: BACKGROUND
  The use of the ex utero intrapartum treatment (EXIT) procedure has salvaged many fetuses with giant neck masses. Despite an adequate airway, a subset of these patients die from an inability to achieve adequate gas exchange.
  
  
  METHODS
  We reviewed our experience with the EXIT procedure from 1996 to 2004. The EXIT was used to deliver 23 fetuses with giant neck masses.
  
  
  RESULTS
  Three fetuses with giant cervical teratomas died of severe pulmonary hypoplasia. On postmortem, these patients had severe airway distortion by the mass. The carina was retracted superiorly to the first or second rib resulting in compression of the lungs in the apices of the chest and pulmonary hypoplasia. Hypoplasia was reflected in the lung weights of 24 vs 38 g and 17 vs 34 g for age-matched normal lung.
  
  
  CONCLUSIONS
  Unsuspected obstructive fetal neck masses can be fatal because of an inability to secure an airway. Prenatal ultrasonography can identify fetuses at risk, allowing the fetus to be salvaged using the EXIT procedure. Despite obtaining airway control, a subset of these patients will die because of pulmonary hypoplasia. When counseling patients with large cervical masses it is important to discuss potential pulmonary hypoplasia in these patients.
authors.affiliation: Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
authors.forename: Kenneth W
authors.initials: KW
authors.lastname: Liechty
authors.forename: Holly L
authors.initials: HL
authors.lastname: Hedrick
authors.forename: Ann M
authors.initials: AM
authors.lastname: Hubbard
authors.forename: Mark P
authors.initials: MP
authors.lastname: Johnson
authors.forename: R Douglas
authors.initials: RD
authors.lastname: Wilson
authors.forename: Eduardo D
authors.initials: ED
authors.lastname: Ruchelli
authors.forename: Lori J
authors.initials: LJ
authors.lastname: Howell
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Crombleholme
authors.forename: Alan W
authors.initials: AW
authors.lastname: Flake
authors.forename: N Scott
authors.initials: NS
authors.lastname: Adzick
mesh_terms: D000402:Airway Obstruction / Q000209:etiology* / Q000628:therapy; D001344:Autopsy; D036861:Delivery, Obstetric; D017809:Fatal Outcome; D006801:Humans; D007231:Infant, Newborn; D008171:Lung Diseases / Q000151:congenital / Q000209:etiology*; D009333:Neck / Q000473:pathology*; D011659:Pulmonary Gas Exchange; D012189:Retrospective Studies; D013724:Teratoma / Q000150:complications* / Q000151:congenital; D016896:Treatment Outcome