title: Fetal MRI-calculated total lung volumes in the prediction of short-term outcome in giant omphalocele: preliminary findings.
abstract: OBJECTIVE
  To determine the value of fetal MRI-calculated total lung volumes (TLV) in the prediction of short-term outcome in patients with giant omphalocele (GO).
  
  
  METHODS
  We reviewed all cases of GO undergoing fetal MRI after 21 weeks' gestation and receiving postnatal care at our institution between 2003 and 2010. Observed/expected (O/E) TLV was calculated using age-matched TLV normograms [Radiology 2001;219:236-241]. Postnatal outcomes were stratified based on O/E TLV above or below 50% of expected.
  
  
  RESULTS
  Seventeen GO cases fulfilled the entry criteria. The mean age at fetal MRI evaluation was 25.8 ± 4.8 weeks' gestation. The mean GO TLV (21.0 ± 13.2) was lower than age-matched population norms (p < 0.001), resulting in a mean O/E TLV of 52.3 ± 16.8%. The mean gestational age at delivery was 36.8 ± 1.6 weeks. Overall survival was 94%. Fourteen (88%) infants underwent staged reduction, and 2 underwent silver sulfadiazine treatment and delayed repair. Infants with ≤50% of predicted O/E TLV (n = 11, 65%) had lower Apgar scores at birth (p = 0.03), prolonged ventilatory support (p = 0.004), delayed oral intake (p = 0.03), and longer hospitalization (p = 0.03) compared to patients with ≥50% of expected O/E TLV. Two infants (both O/E TLV <50%) required tracheostomy placement.
  
  
  CONCLUSIONS
  In the assessment of GO fetuses, MRI-based O/E TLV of <50% was predictive of increased postnatal morbidity.
authors.affiliation: The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadelphia, Pa. 19104, USA.
authors.forename: Enrico
authors.initials: E
authors.lastname: Danzer
authors.forename: Teresa
authors.initials: T
authors.lastname: Victoria
authors.forename: Michael W
authors.initials: MW
authors.lastname: Bebbington
authors.forename: Jennifer
authors.initials: J
authors.lastname: Siegle
authors.forename: Natalie E
authors.initials: NE
authors.lastname: Rintoul
authors.forename: Mark P
authors.initials: MP
authors.lastname: Johnson
authors.forename: Alan W
authors.initials: AW
authors.lastname: Flake
authors.forename: N Scott
authors.initials: NS
authors.lastname: Adzick
authors.forename: Holly L
authors.initials: HL
authors.lastname: Hedrick
mesh_terms: D058109:Airway Management; D005260:Female; D005315:Fetal Diseases / Q000473:pathology*; D005865:Gestational Age; D006554:Hernia, Umbilical / Q000150:complications / Q000196:embryology* / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D015931:Intensive Care, Neonatal; D008168:Lung / Q000196:embryology* / Q000473:pathology; D008176:Lung Volume Measurements / Q000379:methods; D008279:Magnetic Resonance Imaging; D008297:Male; D009017:Morbidity; D011247:Pregnancy; D011296:Prenatal Diagnosis; D011379:Prognosis; D012131:Respiratory Insufficiency / Q000453:epidemiology / Q000209:etiology / Q000628:therapy