title: Predictive value of fetal lung volume in prenatally diagnosed skeletal dysplasia.
abstract: OBJECTIVE
  Pulmonary hypoplasia is a major cause of death in lethal skeletal dysplasias. We hypothesize that in fetuses with prenatally diagnosed skeletal dysplasia, comparison of observed-to-expected (O/E) lung volume will help predict lethality.
  
  
  METHODS
  We conducted a retrospective chart review of patients referred for evaluation of suspected fetal skeletal anomalies. Twenty-three pregnancies were identified with confirmed fetal diagnosis of skeletal dysplasia for which fetal magnetic resonance imaging (MRI) was performed between 21 and 38 weeks of gestation and ultrasound biometry data were available. Femur length to abdominal circumference ratio (FL/AC) and O/E lung volumes were calculated. The association between O/E lung volume, FL/AC, and lethality was measured using logistic regression.
  
  
  RESULTS
  Lethality was significantly associated with O/E lung volume (p = 0.002) and FL/AC (p = 0.0476). Analysis with receiver-operating characteristic curves suggested that O/E lung volume of 47.9% or FL/AC of 0.124 could be useful clinical cutoffs in the prediction of lethality.
  
  
  CONCLUSIONS
  In fetuses with skeletal dysplasia, fetal MRI-derived O/E lung volume was predictive of lethality. When evaluating a fetal skeletal dysplasia, fetal MRI may be considered in cases for which ultrasound-based lethality prediction is ambiguous or uncertain in order to provide families with the most complete and accurate information.
authors.affiliation: Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: K Nicole
authors.initials: KN
authors.lastname: Weaver
authors.forename: Jodie
authors.initials: J
authors.lastname: Johnson
authors.forename: Beth
authors.initials: B
authors.lastname: Kline-Fath
authors.forename: Xue
authors.initials: X
authors.lastname: Zhang
authors.forename: Foong-Yen
authors.initials: FY
authors.lastname: Lim
authors.forename: Brad
authors.initials: B
authors.lastname: Tinkle
authors.forename: Howard M
authors.initials: HM
authors.lastname: Saal
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Hopkin
mesh_terms: D001848:Bone Diseases, Developmental / Q000473:pathology*; D005260:Female; D005315:Fetal Diseases / Q000473:pathology*; D006801:Humans; D008168:Lung / Q000473:pathology*; D008297:Male; D009929:Organ Size; D011237:Predictive Value of Tests; D011247:Pregnancy; D011256:Pregnancy Outcome; D012189:Retrospective Studies