eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Neonatology

Congenital Diaphragmatic Hernia: Follow-up

Author: Robin H Steinhorn, MD, Raymond and Hazel Speck Berry Professor of Pediatrics, Division Head of Neonatology, Associate Chair of Pediatrics, Northwestern University School of Medicine
Contributor Information and Disclosures

Updated: Jul 7, 2009

Follow-up

Further Inpatient Care

  • Pulmonary care in congenital diaphragmatic hernia (CDH)
    • Severely affected infants have chronic lung disease. These infants may require prolonged therapy with supplemental oxygen and diuretics, an approach similar to that for bronchopulmonary dysplasia. The use of steroids, particularly high doses for prolonged periods, is controversial and may hinder appropriate lung and brain development.
    • Late pulmonary hypertension has been successfully treated with low-dose inhaled nitric oxide. This therapy can be delivered via nasal cannula following extubation. In this setting, the delivered dose is diluted because of entrainment of room air. In a recent report, the median duration of treatment using inhaled nitric oxide delivered via nasal cannula was 17 days.
  • Neurologic evaluation
    • Following recovery, a neurologist or developmental pediatrician should perform an examination that includes an evaluation for CNS injury using head CT scanning.
    • The incidence of hearing loss appears to be particularly high in patients with congenital diaphragmatic hernia (approximately 40% of infants). An automated hearing test should be performed prior to discharge.
  • Gastroesophageal reflux
    • The incidence of significant gastroesophageal reflux is very high in patients who survive congenital diaphragmatic hernia, and studies document an incidence of 45-85%.
    • The need for a diaphragmatic patch may be a significant predictor of gastroesophageal reflux. Severe reflux may result in chronic aspiration and is, therefore, aggressively treated.
    • Although most infants can be medically treated with H2-blockers or proton pump inhibitors in combination with a motility agent such as metoclopramide, surgical intervention is sometimes required.

Further Outpatient Care

  • Growth assessment: Failure to thrive is common, and, in some studies, more than 50% of patients are below the 25th percentile for height and weight during the first year of life. In one study, one third of infants required gastrostomy tube placement to improve caloric intake. The need for supplemental oxygen at the time of discharge is a significant predictor for subsequent growth failure. Possible causes include increased caloric requirements due to chronic lung disease, oral aversion after prolonged intubation, poor oral feeding due to neurologic delays, and gastroesophageal reflux.
  • Developmental follow-up11
    • Because of the risk for CNS insult and sensorineural hearing loss, infants should be closely monitored for the first 3 years of life, preferably in a specialty follow-up clinic. These risks are particularly high in infants who are discharged home on supplemental oxygen. Reassess hearing at age 6 months (and later if indicated) because late sensorineural hearing loss occurs in approximately 40% of patients.
    • Even if a child has no major neurodevelopmental delays, he or she should be evaluated prior to entering school to determine if any subtle deficits may predispose the child to learning disabilities.

Transfer

  • Transfer to an extracorporeal membrane oxygenation (ECMO) center
    • Guidelines for ECMO consultation are available from the ELSO.
    • Baseline criteria for ECMO consideration include evaluation for risk factors because of the invasive nature of the therapy and need for heparinization. Although criteria are center-specific, infants should generally be older than 34 weeks' gestation, have a weight greater than 2000 g, have no major intracranial hemorrhage on cranial sonography, have been on mechanical ventilator support for fewer than 10-14 days, and have no evidence for lethal congenital anomalies or inoperable cardiac disease.
    • Timing is always difficult, but referral and transfer should occur prior to refractory hypoxia. Early consultation and discussion with the ECMO center is strongly recommended.

Prognosis

  • Pulmonary recovery: Overall reported survival varies among institutions. Remember that a single institution's results may look better than those provided by population-based studies because of case-selection biases. When all resources, including ECMO, are provided, reported survival rates range from 40-90%. The ELSO registry reports the ECMO survival rate at 52%.
  • Long-term morbidity: As noted, survivors are at risk for significant long-term morbidity, including chronic lung disease, growth failure, gastroesophageal reflux, hearing loss, and neurodevelopmental delay. The risk appears to be highest in infants with severe lung disease (need for supplemental oxygen), need for patch closure of the diaphragm, and need for gastrostomy tube feeding.

Miscellaneous

Special Concerns

  • Prenatal diagnosis12
    • Using ultrasonography, congenital diaphragmatic hernia (CDH) may be prenatally diagnosed as early as the second trimester. Suggestive findings include polyhydramnios, an absent or intrathoracic stomach bubble, and mediastinal and cardiac shift. A detailed examination (level II ultrasonography) is typically necessary.
    • Prenatal diagnosis allows for chromosomal analysis and screening for other anomalies prior to the infant's birth. In addition, it allows the mother time to make important decisions about the pregnancy, including delivery in a facility with a neonatal ICU (NICU) that offers advanced respiratory support for the newborn infant.
 


More on Congenital Diaphragmatic Hernia

Overview: Congenital Diaphragmatic Hernia
Differential Diagnoses & Workup: Congenital Diaphragmatic Hernia
Treatment & Medication: Congenital Diaphragmatic Hernia
Follow-up: Congenital Diaphragmatic Hernia
Multimedia: Congenital Diaphragmatic Hernia
References
Further Reading
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References

  1. Gross RE. Congenital hernia of the diaphragm. Am J Dis Child. 1946;71:579-592.

  2. Areechon W, Reid L. Hypoplasia of the lung associated with congenital diaphragmatic hernia. Br Med J. 1963;i:230-3.

  3. Klaassens M, de Klein A, Tibboel D. The etiology of congenital diaphragmatic hernia: Still largely unknown?. Eur J Med Genet. May 21 2009;[Medline].

  4. Jandus P, Savioz D, Purek L, Frey JG, Schnyder JM, Tschopp JM. [Bochdalek hernia: a rare cause of dyspnea and abdominal pain in adults]. Rev Med Suisse. May 13 2009;5(203):1061-4. [Medline].

  5. Fisher JC, Haley MJ, Ruiz-Elizalde A, Stolar CJ, Arkovitz MS. Multivariate model for predicting recurrence in congenital diaphragmatic hernia. J Pediatr Surg. Jun 2009;44(6):1173-9; discussion 1179-80. [Medline].

  6. Bryner BS, West BT, Hirschl RB, et al. Congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation: does timing of repair matter?. J Pediatr Surg. Jun 2009;44(6):1165-71; discussion 1171-2. [Medline].

  7. Deprest JA, Gratacos E, Nicolaides K, et al. Changing perspectives on the perinatal management of isolated congenital diaphragmatic hernia in Europe. Clin Perinatol. Jun 2009;36(2):329-47, ix. [Medline].

  8. Walsh-Sukys MC, Tyson JE, Wright LL, et al. Persistent pulmonary hypertension of the newborn in the era before nitric oxide: practice variation and outcomes. Pediatrics. Jan 2000;105(1 Pt 1):14-20. [Medline].

  9. Harrison MR, Keller RL, Hawgood SB, et al. A randomized trial of fetal endoscopic tracheal occlusion for severe fetalcongenital diaphragmatic hernia. N Engl J Med. Nov 13 2003;349(20):1916-24. [Medline].

  10. Jelin E, Lee H. Tracheal occlusion for fetal congenital diaphragmatic hernia: the US experience. Clin Perinatol. Jun 2009;36(2):349-61, ix. [Medline].

  11. Peetsold M, Huisman J, Hofman VE, Heij HA, Raat H, Gemke RJ. Psychological outcome and quality of life in children born with congenital diaphragmatic hernia. Arch Dis Child. Jun 15 2009;[Medline].

  12. Mitanchez D. [Neonatal prognosis of congenital diaphragmatic hernia.]. Arch Pediatr. Jun 2009;16(6):888-90. [Medline].

  13. [Guideline] Maisch B, Seferovic PM, Ristic AD, et al. Guidelines on the diagnosis and management of pericardial diseases. European Society of Cardiology. 2004.

  14. Albanese CT, Lopoo J, Goldstein RB, et al. Fetal liver position and perinatal outcome for congenital diaphragmatic hernia. Prenat Diagn. Nov 1998;18(11):1138-42. [Medline].

  15. Bohn DJ, Pearl R, Irish MS, Glick PL. Postnatal management of congenital diaphragmatic hernia. Clin Perinatol. Dec 1996;23(4):843-72. [Medline].

  16. Clark RH, Hardin WD Jr, Hirschl RB, et al. Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg. Jul 1998;33(7):1004-9. [Medline].

  17. Colvin J, Bower C, Dickinson JE, Sokol J. Outcomes of congenital diaphragmatic hernia: a population-based study in WesternAustralia. Pediatrics. Sep 2005;116(3):e356-63. [Medline]. [Full Text].

  18. Cortes RA, Keller RL, Townsend T, et al. Survival of severe congenital diaphragmatic hernia has morbid consequences. J Pediatr Surg. Jan 2005;40(1):36-45; discussion 45-6. [Medline].

  19. Finer NN, Tierney A, Etches PC, et al. Congenital diaphragmatic hernia: developing a protocolized approach. J Pediatr Surg. Sep 1998;33(9):1331-7. [Medline].

  20. Garred P, Madsen HO, Balslev U, et al. Susceptibility to HIV infection and progression of AIDS in relation to variant alleles of mannose-binding lectin. Lancet. Jan 25 1997;349(9047):236-40. [Medline].

  21. Kapur P, Holm BA, Irish MS, et al. Tracheal ligation and mechanical ventilation do not improve the antioxidant enzyme status in the lamb model of congenital diaphragmatic hernia. J Pediatr Surg. Feb 1999;34(2):270-2. [Medline].

  22. Kays DW, Langham MR Jr, Ledbetter DJ, Talbert JL. Detrimental effects of standard medical therapy in congenital diaphragmatic hernia. Ann Surg. Sep 1999;230(3):340-8; discussion 348-51. [Medline]. [Full Text].

  23. Kinsella JP, Parker TA, Ivy DD, Abman SH. Noninvasive delivery of inhaled nitric oxide therapy for late pulmonary hypertensionin newborn infants with congenital diaphragmatic hernia. J Pediatr. Apr 2003;142(4):397-401. [Medline].

  24. Lally KP. Extracorporeal membrane oxygenation in patients with congenital diaphragmatic hernia. Semin Pediatr Surg. Nov 1996;5(4):249-55. [Medline].

  25. Lally KP, Breaux CW Jr. A second course of extracorporeal membrane oxygenation in the neonate-- is there a benefit?. Surgery. Feb 1995;117(2):175-8. [Medline].

  26. Lally KP, Lally PA, Langham MR, et al. Surfactant does not improve survival rate in preterm infants with congenitaldiaphragmatic hernia. J Pediatr Surg. Jun 2004;39(6):829-33. [Medline].

  27. Langham MR Jr, Kays DW, Ledbetter DJ, et al. Congenital diaphragmatic hernia. Epidemiology and outcome. Clin Perinatol. Dec 1996;23(4):671-88. [Medline].

  28. NINOS. Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia. The Neonatal Inhaled Nitric Oxide Study Group (NINOS). Pediatrics. Jun 1997;99(6):838-45. [Medline]. [Full Text].

  29. Nobuhara KK, Lund DP, Mitchell J, et al. Long-term outlook for survivors of congenital diaphragmatic hernia. Clin Perinatol. Dec 1996;23(4):873-87. [Medline].

  30. Nobuhara KK, Wilson JM. Pathophysiology of congenital diaphragmatic hernia. Semin Pediatr Surg. Nov 1996;5(4):234-42. [Medline].

  31. O'Toole SJ, Irish MS, Holm BA, Glick PL. Pulmonary vascular abnormalities in congenital diaphragmatic hernia. Clin Perinatol. Dec 1996;23(4):781-94. [Medline].

  32. Reickert CA, Hirschl RB, Atkinson JB, et al. Congenital diaphragmatic hernia survival and use of extracorporeal life support at selected level III nurseries with multimodality support. Surgery. Mar 1998;123(3):305-10. [Medline].

  33. Slavotinek AM. The genetics of congenital diaphragmatic hernia. Semin Perinatol. Apr 2005;29(2):77-85. [Medline].

  34. Stege G, Fenton A, Jaffray B. Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia. Pediatrics. Sep 2003;112(3 Pt 1):532-5. [Medline]. [Full Text].

  35. Steinhorn RH, Kriesmer PJ, Green TP, et al. Congenital diaphragmatic hernia in Minnesota. Impact of antenatal diagnosis on survival. Arch Pediatr Adolesc Med. Jun 1994;148(6):626-31. [Medline].

  36. Stolar CJ. What do survivors of congenital diaphragmatic hernia look like when they grow up?. Semin Pediatr Surg. Nov 1996;5(4):275-9. [Medline].

  37. Van Meurs K. Is surfactant therapy beneficial in the treatment of the term newborn infantwith congenital diaphragmatic hernia?. J Pediatr. Sep 2004;145(3):312-6. [Medline].

  38. Weinstein S, Stolar CJ. Newborn surgical emergencies. Congenital diaphragmatic hernia and extracorporeal membrane oxygenation. Pediatr Clin North Am. Dec 1993;40(6):1315-33. [Medline].

  39. Wilcox DT, Irish MS, Holm BA, Glick PL. Pulmonary parenchymal abnormalities in congenital diaphragmatic hernia. Clin Perinatol. Dec 1996;23(4):771-9. [Medline].

  40. Wilson JM, Lund DP, Lillehei CW, Vacanti JP. Congenital diaphragmatic hernia--a tale of two cities: the Boston experience. J Pediatr Surg. - Lillehei CW;32(3):401-5. [Medline].

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Keywords

congenital diaphragmatic hernia, CDH, Bochdalek hernia, posterolateral Bochdalek hernia, the anterior Morgagni hernia, hiatus hernia, pulmonary hypoplasia, pulmonary hypertension, cardiac maldevelopment, ventricular hypoplasia, Cornelia de Lange syndrome, Fryns syndrome, cleft lip, cleft palate, pneumothorax, polyhydramnios, respiratory distress, cyanosis, trisomy 13, trisomy 18, trisomy 21, Turner syndrome, Pallister-Killian syndrome, aortic stenosis, treatment, diagnosis

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Contributor Information and Disclosures

Author

Robin H Steinhorn, MD, Raymond and Hazel Speck Berry Professor of Pediatrics, Division Head of Neonatology, Associate Chair of Pediatrics, Northwestern University School of Medicine
Robin H Steinhorn, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Heart Association, American Pediatric Society, American Thoracic Society, and Society for Pediatric Research
Disclosure: Ikaria (INO Therapeutics) Consulting fee Consulting

Medical Editor

David N Sheftel, MD, Director, Division of Neonatology, Clinical Associate Professor, Department of Pediatrics, Lutheran General Children's Hospital of Park Ridge, Chicago Medical School
David N Sheftel, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine and American Academy of Pediatrics
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Arun K Pramanik, MD, MBBS, Professor of Pediatrics, Director of Neonatal Fellowship, Louisiana State University Health Sciences Center
Arun K Pramanik, MD, MBBS is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, National Perinatal Association, and Southern Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Carol L Wagner, MD, Professor of Pediatrics, Medical University of South Carolina
Carol L Wagner, MD is a member of the following medical societies: American Academy of Pediatrics, American Chemical Society, American Medical Women's Association, American Public Health Association, American Society for Bone and Mineral Research, American Society for Clinical Nutrition, Massachusetts Medical Society, National Perinatal Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Ted Rosenkrantz, MD, Professor, Departments of Pediatrics and Obstetrics/Gynecology, Division of Neonatal-Perinatal Medicine, University of Connecticut School of Medicine
Ted Rosenkrantz, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Pediatric Society, Connecticut State Medical Society, Eastern Society for Pediatric Research, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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