title: 5, 4, 3, 2, 1: embryologic variants of pentalogy of Cantrell.
abstract: BACKGROUND
  The purpose of this study was to evaluate our experience with pentalogy of Cantrell and the various embryologic variants.
  
  
  METHODS
  Patient charts and diagnostic imaging studies of all fetuses evaluated at Texas Children's Fetal Center for pentalogy of Cantrell between April 2004 and June 2014 were reviewed retrospectively. Data collected from patient charts included demographic information, clinical presentation, fetal and postnatal imaging findings, operative treatment, pathologic evaluation, and outcomes.
  
  
  RESULTS
  There were 10 patients who presented with embryologic variants of pentalogy of Cantrell over a 6-y period. Two cases displayed the full range of embryologic defects observed, and eight cases exhibited variants of the classic pentalogy. Sternal and pericardial defects were each present in 40% of patients. Additional anomalies present included pulmonary hypoplasia, pulmonary artery stenosis, and chromosomal abnormalities. Four patients presented with diaphragmatic defects but no defect in the pericardium, and one patient presented with a defective pericardium but no associated diaphragmatic defect, suggesting highly specific losses of somatic mesoderm during embryologic development. One patient was lost to follow-up, and a second patient underwent termination of pregnancy. Five of the remaining eight patients survived, one of which had the full range of embryologic defects and now attends preschool but requires speech and occupational therapy. The remaining surviving patients have developed without serious sequelae.
  
  
  CONCLUSIONS
  This report highlights the spectrum of anomalies observed in the pentalogy of Cantrell and demonstrates that these fetuses can survive but with substantial morbidity.
authors.affiliation: Texas Children's Fetal Center, Texas Children's Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
authors.forename: Bhavika
authors.initials: B
authors.lastname: Kaul
authors.affiliation: Texas Children's Fetal Center, Texas Children's Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
authors.forename: Fariha
authors.initials: F
authors.lastname: Sheikh
authors.affiliation: Texas Children's Fetal Center, Texas Children's Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
authors.forename: Irving J
authors.initials: IJ
authors.lastname: Zamora
authors.affiliation: Texas Children's Fetal Center, Texas Children's Hospital, Houston, Texas; Department of Radiology, Baylor College of Medicine, Houston, Texas.
authors.forename: Amy R
authors.initials: AR
authors.lastname: Mehollin-Ray
authors.affiliation: Texas Children's Fetal Center, Texas Children's Hospital, Houston, Texas; Department of Radiology, Baylor College of Medicine, Houston, Texas.
authors.forename: Christopher I
authors.initials: CI
authors.lastname: Cassady
authors.affiliation: Texas Children's Fetal Center, Texas Children's Hospital, Houston, Texas; Division of Cardiology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
authors.forename: Nancy A
authors.initials: NA
authors.lastname: Ayres
authors.affiliation: Texas Children's Fetal Center, Texas Children's Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
authors.forename: Darrell L
authors.initials: DL
authors.lastname: Cass
authors.affiliation: Texas Children's Fetal Center, Texas Children's Hospital, Houston, Texas; Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas. Electronic address: oolutoye@bcm.edu.
authors.forename: Oluyinka O
authors.initials: OO
authors.lastname: Olutoye
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000196:embryology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D058502:Pentalogy of Cantrell / Q000175:diagnosis / Q000196:embryology* / Q000601:surgery; D010496:Pericardium / Q000002:abnormalities / Q000196:embryology; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies; D013249:Sternum / Q000002:abnormalities / Q000196:embryology
keywords: Bifid sternum; Congenital anomalies; Congenital diaphragmatic hernia; Pentalogy of Cantrell