title: Are all pulmonary hypoplasias the same? A comparison of pulmonary outcomes in neonates with congenital diaphragmatic hernia, omphalocele and congenital lung malformation.
abstract: BACKGROUND
  Patients with congenital diaphragmatic hernias (CDH), omphaloceles, and congenital lung malformations (CLM) may have pulmonary hypoplasia and experience respiratory insufficiency. We hypothesize that given equivalent lung volumes, the degree of respiratory insufficiency will be comparable regardless of the etiology.
  
  
  METHODS
  Records of all fetuses with CDH, omphalocele, and CLM between January 2000 and June 2013 were reviewed. MRI-based observed-to-expected total fetal lung volumes (O/E-TFLV) were calculated. An analysis of outcomes in patients with O/E-TFLV between 40% and 60%, the most inclusive range, was performed.
  
  
  RESULTS
  285 patients were evaluated (161, CDH; 24, omphalocele; 100, CLM). Fetuses with CDH had the smallest mean O/E-TFLV. CDH patients were intubated for longer and had a higher incidence of pulmonary hypertension. Fifty-six patients with the three diagnoses had an O/E-TFLV of 40%-60%. The need for ECMO, supplemental oxygen at 30days of life, and 6-month mortality were similar among groups. CDH patients had a significantly longer duration of intubation and higher incidence of pulmonary hypertension than the other two diagnoses.
  
  
  CONCLUSIONS
  Given equivalent lung volumes (40%-60% of expected), CDH patients require more pulmonary support initially than omphalocele and CLM patients. In addition to lung volumes, disease-specific factors, such as pulmonary hypertension in CDH, also contribute to pulmonary morbidity and overall outcome.
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX.
authors.forename: Adesola C
authors.initials: AC
authors.lastname: Akinkuotu
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX.
authors.forename: Fariha
authors.initials: F
authors.lastname: Sheikh
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX; Department of Pediatrics, Baylor College of Medicine, Houston, TX; Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX.
authors.forename: Darrell L
authors.initials: DL
authors.lastname: Cass
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX.
authors.forename: Irving J
authors.initials: IJ
authors.lastname: Zamora
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX.
authors.forename: Timothy C
authors.initials: TC
authors.lastname: Lee
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Department of Radiology, Baylor College of Medicine, Houston, TX.
authors.forename: Christopher I
authors.initials: CI
authors.lastname: Cassady
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Department of Radiology, Baylor College of Medicine, Houston, TX.
authors.forename: Amy R
authors.initials: AR
authors.lastname: Mehollin-Ray
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Department of Radiology, Baylor College of Medicine, Houston, TX.
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Williams
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX.
authors.forename: Rodrigo
authors.initials: R
authors.lastname: Ruano
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Department of Pediatrics, Baylor College of Medicine, Houston, TX.
authors.forename: Stephen E
authors.initials: SE
authors.lastname: Welty
authors.affiliation: Texas Children's Fetal Center, Baylor College of Medicine, Houston, TX; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX; Department of Pediatrics, Baylor College of Medicine, Houston, TX; Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX. Electronic address: oolutoye@bcm.edu.
authors.forename: Oluyinka O
authors.initials: OO
authors.lastname: Olutoye
mesh_terms: D015199:Extracorporeal Membrane Oxygenation; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D006554:Hernia, Umbilical / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D065630:Hernias, Diaphragmatic, Congenital / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008176:Lung Volume Measurements; D008279:Magnetic Resonance Imaging; D011379:Prognosis; D012138:Respiratory Therapy; D012189:Retrospective Studies
keywords: CDH; Fetus; Lung malformation; Lung volumes; Omphalocele