Skip to main content

Advertisement

SpringerLink
Log in

Predicting Survival of Congenital Diaphragmatic Hernia on the First Day of Life

  • Original Scientific Report
  • Published:
World Journal of Surgery Aims and scope Submit manuscript

Abstract

Background

This study aimed to determine perinatal risk factors for 30-day mortality of congenital diaphragmatic hernia (CDH) patients and develop a prognostic index to predict 30-day mortality of CDH patients. Identifying risk factors that can prognosticate outcome is critical to obtain the best management practices for patients.

Methods

A retrospective study was performed for patients who were diagnosed with CDH from November 2000 to August 2016. A total of 10 prenatal risk factors and 14 postnatal risk factors were analyzed. All postnatal variables were measured within 24 h after birth.

Results

A total of 95 CDH patients were enrolled in this study, including 61 males and 34 females with mean gestational age of 38.86 ± 1.51 weeks. The overall 30-day survival rate was 63.2%. Multivariate analysis revealed that five factors (polyhydramnios, gestational age at diagnosis <25 weeks, observed-to-expected lung-to-head ratio ≤45, best oxygenation index in 24 h  >11, and severity of tricuspid regurgitation ≥ mild) were independent predictors of 30-day mortality of CDH. Using these five factors, a perinatal prognostic index for 30-day mortality was developed. Four predictive models (poor, bad, good, and excellent) of the perinatal prognostic index were constructed, and external validation was performed.

Conclusions

Awareness of risk factors is very important for predicting prognosis and managing patients. Five independent perinatal risk factors were identified in this study. A perinatal prognostic index was developed for 30-day mortality for patients with CDH. This index may be used to help manage CDH patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Losty PD (2014) Congenital diaphragmatic hernia: where and what is the evidence? Semin Pediatr Surg 23:278–282. https://doi.org/10.1053/j.sempedsurg.2014.09.008

    Article  Google Scholar 

  2. Bohn D (2002) Congenital diaphragmatic hernia. Am J Respir Crit Care Med 166:911–915. https://doi.org/10.1164/rccm.200204-304CC

    Article  PubMed  Google Scholar 

  3. Downard CD, Jaksic T, Garza JJ et al (2003) Analysis of an improved survival rate for congenital diaphragmatic hernia. J Pediatr Surg 38:729–732. https://doi.org/10.1016/jpsu.2003.50194

    Article  PubMed  Google Scholar 

  4. Beresford MW, Shaw NJ (2000) Outcome of congenital diaphragmatic hernia. Pediatr Pulmonol 30:249–256

    Article  CAS  Google Scholar 

  5. Congenital Diaphragmatic Hernia Study Group (2001) Estimating disease severity of congenital diaphragmatic hernia in the first 5 minutes of life. J Pediatr Surg 36:141–145

    Article  Google Scholar 

  6. Ontario Congenital Anomalies Study Group (2004) Apparent truth about congenital diaphragmatic hernia: a population-based database is needed to establish benchmarking for clinical outcomes for CDH. J Pediatr Surg 39:661–665

    Article  Google Scholar 

  7. Javid PJ, Jaksic T, Skarsgard ED et al (2004) Survival rate in congenital diaphragmatic hernia: the experience of the Canadian Neonatal Network. J Pediatr Surg 39:657–660

    Article  PubMed  Google Scholar 

  8. Bahlmann F, Merz E, Hallermann C et al (1999) Congenital diaphragmatic hernia: ultrasonic measurement of fetal lungs to predict pulmonary hypoplasia. Ultrasound Obstet Gynecol 14:162–168. https://doi.org/10.1046/j.1469-0705.1999.14030162.x

    Article  CAS  PubMed  Google Scholar 

  9. Harrison MR, Adzick NS, Nakayama DK et al (1985) Fetal diaphragmatic hernia: fetal but fixable. Semin Perinatol 9:103–112

    CAS  PubMed  Google Scholar 

  10. Trachsel D, Selvadurai H, Bohn D et al (2005) Long-term pulmonary morbidity in survivors of congenital diaphragmatic hernia. Pediatr Pulmonol 39:433–439. https://doi.org/10.1002/ppul.20193

    Article  PubMed  Google Scholar 

  11. Laudy JA, Van Gucht M, Van Dooren MF et al (2003) Congenital diaphragmatic hernia: an evaluation of the prognostic value of the lung-to-head ratio and other prenatal parameters. Prenat Diagn 23:634–639. https://doi.org/10.1002/pd.654

    Article  CAS  PubMed  Google Scholar 

  12. 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 30:67–71. https://doi.org/10.1002/uog.4052

    Article  CAS  Google Scholar 

  13. Balassy C, Kasprian G, Brugger PC et al (2010) Assessment of lung development in isolated congenital diaphragmatic hernia using signal intensity ratios on fetal MR imaging. Eur Radiol 20:829–837. https://doi.org/10.1007/s00330-009-1633-x

    Article  PubMed  Google Scholar 

  14. Quintero RA, Quintero LF, Chmait R et al (2011) The quantitative lung index (QLI): a gestational age-independent sonographic predictor of fetal lung growth. Am J Obstet Gynecol 205(544):e541–548. https://doi.org/10.1016/j.ajog.2011.07.031

    Article  Google Scholar 

  15. Ruttenstock E, Wright N, Barrena S et al (2015) Best oxygenation index on day 1: a reliable marker for outcome and survival in infants with congenital diaphragmatic hernia. Eur J Pediatr Surg 25:3–8. https://doi.org/10.1055/s-0034-1393960

    Article  PubMed  Google Scholar 

  16. Suda K, Bigras JL, Bohn D et al (2000) Echocardiographic predictors of outcome in newborns with congenital diaphragmatic hernia. Pediatrics 105:1106–1109

    Article  CAS  PubMed  Google Scholar 

  17. Thebaud B, Azancot A, de Lagausie P et al (1997) Congenital diaphragmatic hernia: antenatal prognostic factors. Does cardiac ventricular disproportion in utero predict outcome and pulmonary hypoplasia? Intensive Care Med 23:10062–10069

    CAS  PubMed  Google Scholar 

  18. Brindle ME, Cook EF, Tibboel D et al (2014) A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns. Pediatrics 134:e413–419. https://doi.org/10.1542/peds.2013-3367

    Article  PubMed  Google Scholar 

  19. Sharma V, Berkelhamer S, Lakshminrusimha S (2015) Persistent pulmonary hypertension of the newborn. Matern Health Neonatol Perinatol 1:14. https://doi.org/10.1186/s40748-015-0015-4

    Article  PubMed  PubMed Central  Google Scholar 

  20. Fumino S, Shimotake T, Kume Y et al (2005) A clinical analysis of prognostic parameters of survival in children with congenital diaphragmatic hernia. Eur J Pediatr Surg 15:399–403. https://doi.org/10.1055/s-2005-872925

    Article  CAS  PubMed  Google Scholar 

  21. Hedrick HL, Danzer E, Merchant A et al (2007) Liver position and lung-to-head ratio for prediction of extracorporeal membrane oxygenation and survival in isolated left congenital diaphragmatic hernia. Am J Obstet Gynecol 197(422):e421–424. https://doi.org/10.1016/j.ajog.2007.07.001

    Article  Google Scholar 

  22. Albanese CT, Lopoo J, Goldstein RB et al (1998) Fetal liver position and perinatal outcome for congenital diaphragmatic hernia. Prenat Diagn 18:1138–1142

    Article  CAS  PubMed  Google Scholar 

  23. Jani J, Keller RL, Benachi A et al (2006) Prenatal prediction of survival in isolated left-sided diaphragmatic hernia. Ultrasound Obstet Gynecol 27:18–22. https://doi.org/10.1002/uog.2688

    Article  CAS  Google Scholar 

  24. Stolar CJ, Dillon PW (2012) Congenital Diaphragmatic Hernia and Eventration. In: Coran AG, Scott Adzick N, Krummel TM et al (eds) Pediatric Surgery, 7th edn. Mosby, Philadelphia, pp 809–824

    Chapter  Google Scholar 

  25. Schultz CM, DiGeronimo RJ, Yoder BA et al (2007) Congenital diaphragmatic hernia: a simplified postnatal predictor of outcome. J Pediatr Surg 42:510–516. https://doi.org/10.1016/j.jpedsurg.2006.10.043

    Article  PubMed  Google Scholar 

  26. Alfaraj MA, Shah PS, Bohn D et al (2011) Congenital diaphragmatic hernia: lung-to-head ratio and lung volume for prediction of outcome. Am J Obstet Gynecol 205(43):e41–48. https://doi.org/10.1016/j.ajog.2011.02.050

    Article  Google Scholar 

  27. Ruano R, Takashi E, da Silva MM et al (2013) Quantitative lung index, contralateral lung area, or lung-to-head ratio to predict the neonatal outcome in isolated congenital diaphragmatic hernia? J Ultrasound Med 32:413–417

    Article  PubMed  Google Scholar 

  28. Brindle ME, Cook EF, Tibboel D et al (2014) A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns. Pediatrics 134:e413–419. https://doi.org/10.1542/peds.2013-3367

    Article  PubMed  Google Scholar 

  29. Khmour AY, Konduri GG, Sato TT et al (2014) Role of admission gas exchange measurement in predicting congenital diaphragmatic hernia survival in the era of gentle ventilation. J Pediatr Surg 49:1197–1201. https://doi.org/10.1016/j.jpedsurg.2014.03.011

    Article  PubMed  Google Scholar 

  30. Guibaud L, Filiatrault D, Garel L et al (1996) Fetal congenital diaphragmatic hernia: accuracy of sonography in the diagnosis and prediction of the outcome after birth. AJR Am J Roentgenol 166:1195–1202. https://doi.org/10.2214/ajr.166.5.8615269

    Article  CAS  PubMed  Google Scholar 

  31. Metkus AP, Filly RA, Stringer MD et al (1996) Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg 31:148–151 discussion 151-142

    Article  CAS  Google Scholar 

  32. Jani J, Peralta CF, Van Schoubroeck D et al (2006) Relationship between lung-to-head ratio and lung volume in normal fetuses and fetuses with diaphragmatic hernia. Ultrasound Obstet Gynecol 27:545–550. https://doi.org/10.1002/uog.2735

    Article  CAS  PubMed  Google Scholar 

  33. Zoghbi WA, Enriquez-Sarano M, Foster E et al (2003) Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography. J Am Soc Echocardiogr 16:777–802. https://doi.org/10.1016/s0894-7317(03)00335-3

    Article  Google Scholar 

  34. Rottier R, Tibboel D (2005) Fetal lung and diaphragm development in congenital diaphragmatic hernia. Semin Perinatol 29:86–93

    Article  PubMed  Google Scholar 

  35. Crawford DC, Wright VM, Drake DP et al (1989) Fetal diaphragmatic hernia: the value of fetal echocardiography in the prediction of postnatal outcome. Br J Obstet Gynaecol 96:705–710

    Article  CAS  PubMed  Google Scholar 

  36. Shaw KS, Filiatrault D, Yazbeck S et al (1994) Improved survival for congenital diaphragmatic hernia, based on prenatal ultrasound diagnosis and referral to a combined obstetric-pediatric surgical center. J Pediatr Surg 29:1268–1269

    Article  CAS  PubMed  Google Scholar 

  37. Wilson JM, Fauza DO, Lund DP et al (1994) Antenatal diagnosis of isolated congenital diaphragmatic hernia is not an indicator of outcome. J Pediatr Surg 29:815–819

    Article  CAS  PubMed  Google Scholar 

  38. Ruano R, Takashi E, da Silva MM et al (2012) Prediction and probability of neonatal outcome in isolated congenital diaphragmatic hernia using multiple ultrasound parameters. Ultrasound Obstet Gynecol 39:42–49. https://doi.org/10.1002/uog.10095

    Article  CAS  PubMed  Google Scholar 

  39. Adzick NS, Vacanti JP, Lillehei CW et al (1989) Fetal diaphragmatic hernia: ultrasound diagnosis and clinical outcome in 38 cases. J Pediatr Surg 24:654–657 discussion 657–658

    Article  CAS  PubMed  Google Scholar 

  40. Sharland GK, Lockhart SM, Heward AJ et al (1992) Prognosis in fetal diaphragmatic hernia. Am J Obstet Gynecol 166:9–13

    Article  CAS  PubMed  Google Scholar 

  41. Bouchghoul H, Senat MV, Storme L et al (2015) Congenital diaphragmatic hernia: does gestational age at diagnosis matter when evaluating morbidity and mortality? Am J Obstet Gynecol 213:535 e531-537. https://doi.org/10.1016/j.ajog.2015.06.012

    Article  Google Scholar 

  42. Quintero RA, Kontopoulos EV, Quintero LF et al (2013) The observed vs. expected lung-to-head ratio does not correct for the effect of gestational age on the lung-to-head ratio. J Matern Fetal Neonatal Med 26:552–557. https://doi.org/10.3109/14767058.2012.736000

    Article  PubMed  Google Scholar 

  43. Trachsel D, McCrindle BW, Nakagawa S et al (2005) Oxygenation index predicts outcome in children with acute hypoxemic respiratory failure. Am J Respir Crit Care Med 172:206–211. https://doi.org/10.1164/rccm.200405-625OC

    Article  PubMed  Google Scholar 

  44. Sinha CK, Islam S, Patel S et al (2009) Congenital diaphragmatic hernia: prognostic indices in the fetal endoluminal tracheal occlusion era. J Pediatr Surg 44:312–316. https://doi.org/10.1016/j.jpedsurg.2008.10.078

    Article  PubMed  Google Scholar 

  45. Basiewicz-Slaczka E, Woloszczuk-Gebicka B, Yaqoub S et al (2015) The Value of the oxygenation index in the prediction of postnatal outcome in neonates with congenital diaphragmatic hernia. Preliminary Report. Dev Period Med 19:283–288

    PubMed  Google Scholar 

  46. Musewe NN, Smallhorn JF, Benson LN et al (1987) Validation of Doppler-derived pulmonary arterial pressure in patients with ductus arteriosus under different hemodynamic states. Circulation 76:1081–1091

    Article  CAS  PubMed  Google Scholar 

  47. Reisner SA, Azzam Z, Halmann M et al (1994) Septal/free wall curvature ratio: a noninvasive index of pulmonary arterial pressure. J Am Soc Echocardiogr 7:27–35

    Article  CAS  PubMed  Google Scholar 

  48. Mourani PM, Sontag MK, Younoszai A et al (2008) Clinical utility of echocardiography for the diagnosis and management of pulmonary vascular disease in young children with chronic lung disease. Pediatrics 121:317–325. https://doi.org/10.1542/peds.2007-1583

    Article  PubMed  PubMed Central  Google Scholar 

  49. Wynn J, Krishnan U, Aspelund G et al (2013) Outcomes of congenital diaphragmatic hernia in the modern era of management. J Pediatr 163(114–119):e111. https://doi.org/10.1016/j.jpeds.2012.12.036

    Article  Google Scholar 

  50. Group UCET (1996) UK collaborative randomised trial of neonatal extracorporeal membrane oxygenation. Lancet 348:75–82. https://doi.org/10.1016/S0140-6736(96)04100-1

    Article  Google Scholar 

  51. ELSO (2017) Extracorporeal Life Support: The Red Book: the ELSO Red Book. 5th Ed, Extracorporeal Life Support Organization

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Surgery, Korea University College of Medicine, Seoul, Korea

    Chaeyoun Oh

  2. Department of Pediatric Surgery, Seoul National University College of Medicine, Children’s Hospital, 101, Daehang-ro, Yeongeon-dong, Jongro-Gu, Seoul, 03080, Korea

    Joong Kee Youn, Ji-Won Han, Hee-Byum Yang, Hyun-Young Kim & Sung-Eun Jung

  3. Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Sanghoon Lee & Jeong-Meen Seo

  4. Department of Pediatric Surgery, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Korea

    In Geol Ho

  5. Department of Pediatric Surgery, Pusan National University Children’s Hospital, Yangsan, Korea

    Soo-Hong Kim & Yong Hoon Cho

  6. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea

    Seung Han Shin

Authors
  1. Chaeyoun Oh
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Joong Kee Youn
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ji-Won Han
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Hee-Byum Yang
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Sanghoon Lee
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Jeong-Meen Seo
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. In Geol Ho
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Soo-Hong Kim
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Yong Hoon Cho
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Seung Han Shin
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Hyun-Young Kim
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Sung-Eun Jung
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hyun-Young Kim.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Oh, C., Youn, J.K., Han, JW. et al. Predicting Survival of Congenital Diaphragmatic Hernia on the First Day of Life. World J Surg 43, 282–290 (2019). https://doi.org/10.1007/s00268-018-4780-x

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00268-018-4780-x

Search

Navigation