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Congenital diaphragmatic hernia sacs: prenatal imaging and associated postnatal outcomes

  • Edward R. OliverEmail author
  • Suzanne E. DeBari
  • Samantha E. Adams
  • Ryne A. Didier
  • Steven C. Horii
  • Teresa Victoria
  • Holly L. Hedrick
  • N. Scott Adzick
  • Lori J. Howell
  • Julie S. Moldenhauer
  • Beverly G. Coleman
Original Article
  • 73 Downloads

Abstract

Background

The presence of a hernia sac in congenital diaphragmatic hernia (CDH) has been reported to be associated with higher lung volumes and better postnatal outcomes.

Objective

To compare prenatal imaging (ultrasound and MRI) prognostic measurements and postnatal outcomes of CDH with and without hernia sac.

Materials and methods

We performed database searches from January 2008 to March 2017 for surgically proven cases of CDH with and without hernia sac. All children had a detailed ultrasound (US) examination and most had an MRI examination. We reviewed the medical records of children enrolled in our Pulmonary Hypoplasia Program.

Results

Of 200 cases of unilateral CDH, 46 (23%) had hernia sacs. Cases of CDH with hernia sac had a higher mean lung-to-head ratio (LHR; 1.61 vs. 1.17; P<0.01), a higher mean observed/expected LHR (0.49 vs. 0.37; P<0.01), and on MRI a higher mean observed/expected total lung volume (0.53 vs. 0.41; P<0.01). Based on a smooth interface between lung and herniated contents, hernia sac or eventration was prospectively questioned by US and MRI in 45.7% and 38.6% of cases, respectively. Postnatally, hernia sac is associated with shorter median periods of admission to the neonatal intensive care unit (45.0 days vs. 61.5 days, P=0.03); mechanical ventilation (15.5 days vs. 23.5 days, P=0.04); extracorporeal membrane oxygenation (251 h vs. 434 h, P=0.04); decreased rates of patch repair (39.0% vs. 69.2%, P<0.01); and pulmonary hypertension (56.1% vs. 75.4%, P=0.03).

Conclusion

Hernia sac is associated with statistically higher prenatal prognostic measurements and improved postnatal outcomes. Recognition of a sharp interface between lung and herniated contents may allow for improved prenatal diagnosis; however, delivery and management should still occur at experienced quaternary neonatal centers.

Keywords

Congenital diaphragmatic hernia Hernia sac Lung-to-head ratio Magnetic resonance imaging Neonates Prenatal ultrasound Total lung volume 

Notes

Acknowledgments

We thank Mr. Michael J. Kallan for his assistance with the statistical analyses in this study. Preliminary data were presented at the International Fetal Medicine and Surgery Society Annual Meeting on Oct. 9, 2017.

Compliance with ethical standards

Conflicts of interest

None

References

  1. 1.
    Langham M, Kays D, Ledbetter D et al (1996) Congenital diaphragmatic hernia. Epidemiology and outcome. Clin Perinatol 23:671–687CrossRefGoogle Scholar
  2. 2.
    Beresford MW, Shaw NJ (2000) Outcome of congenital diaphragmatic hernia. Pediatr Pulmonol 30:249–256CrossRefGoogle Scholar
  3. 3.
    Mah VK, Zamakhshary M, Mah DY et al (2009) Absolute vs relative improvements in congenital diaphragmatic hernia survival: what happened to “hidden mortality.” J Pediatr Surg 44:877–882CrossRefGoogle Scholar
  4. 4.
    Brownlee EM, Howatson AG, Davis CF, Sabharwal AJ (2009) The hidden mortality of congenital diaphragmatic hernia: a 20-year review. J Pediatr Surg 44:317–320CrossRefGoogle Scholar
  5. 5.
    Peralta CFA, Cavoretto P, Csapo B et al (2005) Assessment of lung area in normal fetuses at 12-32 weeks. Ultrasound Obstet Gynecol 26:718–724CrossRefGoogle Scholar
  6. 6.
    Jani J, Keller RL, Benachi A et al (2006) Prenatal prediction of survival in isolated left-sided diaphragmatic hernia. Ultrasound Obstet Gynecol 27:18–22CrossRefGoogle Scholar
  7. 7.
    Jani J, Nicolaides KH, Keller RL et al (2007) Observed to expected lung area to head circumference ratio in the prediction of survival in fetuses with isolated diaphragmatic hernia. Ultrasound Obstet Gynecol 30:67–71CrossRefGoogle Scholar
  8. 8.
    Jani J, Cannie M, Sonigo P et al (2008) Value of prenatal magnetic resonance imaging in the prediction of postnatal outcome in fetuses with diaphragmatic hernia. Ultrasound Obstet Gynecol 32:793–799CrossRefGoogle Scholar
  9. 9.
    Spaggiari E, Stirnemann J, Bernard JP et al (2013) Prognostic value of a hernia sac in congenital diaphragmatic hernia. Ultrasound Obstet Gynecol 41:286–290CrossRefGoogle Scholar
  10. 10.
    Zamora IJ, Cass DL, Lee TC et al (2013) The presence of a hernia sac in congenital diaphragmatic hernia is associated with better fetal lung growth and outcomes. J Pediatr Surg 48:1165–1171CrossRefGoogle Scholar
  11. 11.
    Victoria T, Jaramillo D, Roberts TPL et al (2014) Fetal magnetic resonance imaging: jumping from 1.5 to 3 tesla (preliminary experience). Pediatr Radiol 44:376–386CrossRefGoogle Scholar
  12. 12.
    Victoria T, Johnson AM, Edgar JC et al (2016) Comparison between 1.5-T and 3-T MRI for fetal imaging: Is there an advantage to imaging with a higher field strength? AJR Am J Roentgenol 206:195–201CrossRefGoogle Scholar
  13. 13.
    Metkus AP, Filly RA, Stringer MD et al (1996) Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg 31:148–151CrossRefGoogle Scholar
  14. 14.
    DeKoninck P, Gratacos E, Van Mieghem T et al (2011) Results of fetal endoscopic tracheal occlusion for congenital diaphragmatic hernia and the set up of the randomized controlled TOTAL trial. Early Hum Dev 87:619–624CrossRefGoogle Scholar
  15. 15.
    Mehollin-Ray AR, Cassady CI, Cass DL, Olutoye OO (2012) Fetal MR imaging of congenital diaphragmatic hernia. Radiographics 32:1067–1084CrossRefGoogle Scholar
  16. 16.
    Rypens F, Metens T, Rocourt N et al (2001) Fetal lung volume: estimation at MR imaging-initial results. Radiology 219:236–241CrossRefGoogle Scholar
  17. 17.
    Ward VL, Nishino M, Hatabu H et al (2006) Fetal lung volume measurements: determination with MR imaging — effect of various factors. Radiology 240:187–193CrossRefGoogle Scholar
  18. 18.
    Grizelj R, Bojanić K, Vuković J et al (2017) Hernia sac presence portends better survivability of isolated congenital diaphragmatic hernia with “liver-up.” Am J Perinatol 34:515–519CrossRefGoogle Scholar
  19. 19.
    Zamora IJ, Mehollin-Ray AR, Sheikh F et al (2015) Predictive value of MRI findings for the identification of a hernia sac in fetuses with congenital diaphragmatic hernia. AJR Am J Roentgenol 205:1121–1125CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Edward R. Oliver
    • 1
    • 2
    • 3
    Email author
  • Suzanne E. DeBari
    • 2
    • 3
  • Samantha E. Adams
    • 2
    • 4
  • Ryne A. Didier
    • 1
    • 2
    • 3
  • Steven C. Horii
    • 1
    • 2
    • 5
  • Teresa Victoria
    • 1
    • 2
    • 3
  • Holly L. Hedrick
    • 1
    • 2
    • 4
  • N. Scott Adzick
    • 1
    • 2
    • 4
  • Lori J. Howell
    • 2
    • 4
  • Julie S. Moldenhauer
    • 1
    • 2
    • 4
  • Beverly G. Coleman
    • 1
    • 2
    • 3
  1. 1.Perelman School of MedicineUniversity of PennsylvaniaPhiladelphiaUSA
  2. 2.Center for Fetal Diagnosis and TreatmentChildren’s Hospital of PhiladelphiaPhiladelphiaUSA
  3. 3.Department of RadiologyChildren’s Hospital of PhiladelphiaPhiladelphiaUSA
  4. 4.Department of SurgeryChildren’s Hospital of PhiladelphiaPhiladelphiaUSA
  5. 5.Department of RadiologyHospital of the University of PennsylvaniaPhiladelphiaUSA

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