Our Summary

The treatment of newborns with congenital diaphragmatic hernia (CDH), where the diaphragm doesn’t develop properly, has improved over the last 30 years. The usual treatment now involves stabilizing the baby then performing surgery. However, some babies don’t respond well to medical stabilization attempts, and their survival rate is very low without extra treatment strategies. One such strategy is extracorporeal life support (ECLS), which is a type of life support that takes over the function of the heart and lungs. This has been shown to increase survival rates in certain cases.

The researchers looked back at six cases of newborns with CDH who had surgery while on ECLS, from September 2011 to November 2014. Out of 24 babies admitted with CDH, six (25%) needed ECLS. All of these six had surgery while on ECLS, and there were no complications related to blood loss or clotting. One baby died in the hospital, but the other five were able to come off ECLS and were discharged.

The researchers conclude that ECLS can help improve the survival rate of babies with severe CDH who might otherwise not survive. They also found that having surgery while on ECLS is safe. The use of ECLS along with early surgery, strategies to protect the lungs, and aggressive management of high blood pressure in the lungs were all associated with a good survival rate. They suggest that ECLS should be considered as an important part of the treatment options for babies with CDH.

FAQs

  1. What is the current standard of care for neonates with congenital diaphragmatic hernia (CDH)?
  2. How has the use of extracorporeal life support (ECLS) impacted the survival rates of neonates with CDH?
  3. Can CDH repair be safely performed during ECLS, and what are the associated risks or complications?

Doctor’s Tip

A helpful tip a doctor might tell a patient about congenital diaphragmatic hernia repair is to consider the option of extracorporeal life support (ECLS) for neonates who are not responding well to traditional medical management. ECLS has been shown to improve early survival in selected cases and can be used in conjunction with surgical repair of the hernia. Additionally, using lung protective ventilation strategies and aggressively managing pulmonary hypertension can also improve outcomes for patients with CDH. It is important to discuss all treatment options with your healthcare provider to determine the best course of action for your specific situation.

Suitable For

Patients with congenital diaphragmatic hernia (CDH) who fail to achieve adequate oxygenation with medical management, including high frequency oscillation and inhaled nitric oxide, are typically recommended for CDH repair. These patients have a high mortality rate without additional management strategies, such as extracorporeal life support (ECLS). ECLS is recommended for neonates with CDH who are extremely compromised and at high risk of mortality. Early repair during ECLS, along with lung protective ventilation strategies and aggressive management of pulmonary hypertension, have been associated with good early survival outcomes in these patients. ECLS should be considered as an integral part of the therapeutic approach for CDH in neonates.

Timeline

Before congenital diaphragmatic hernia repair:

  • Neonate is diagnosed with CDH through prenatal ultrasound or shortly after birth
  • Neonate may have difficulty breathing, cyanosis, and respiratory distress
  • Initial stabilization with respiratory support and possibly high frequency oscillation and inhaled nitric oxide
  • Neonate may fail to achieve adequate oxygenation with medical management, leading to consideration for ECLS
  • Neonate undergoes CDH repair during ECLS if deemed necessary

After congenital diaphragmatic hernia repair:

  • CDH repair is performed during ECLS without intra-operative bleeding complications
  • Use of ECLS, early repair during ECLS, lung protective ventilation strategies, and management of pulmonary hypertension contribute to good early survival
  • Neonate is weaned from ECLS and discharged home
  • Overall, ECLS is considered an integral part of therapeutic armamentarium for CDH in neonates with a high mortality rate without additional management strategies

What to Ask Your Doctor

Some questions a patient should ask their doctor about congenital diaphragmatic hernia repair may include:

  1. What is the current standard of care for managing congenital diaphragmatic hernia in neonates?
  2. How do you determine if my baby will require extracorporeal life support (ECLS) for CDH management?
  3. What are the potential risks and benefits of undergoing CDH repair during ECLS?
  4. How will the use of ECLS impact my baby’s recovery and long-term outcomes?
  5. What are the success rates for CDH repair during ECLS in neonates?
  6. What are the potential complications associated with CDH repair during ECLS?
  7. How will the medical team monitor and support my baby during the CDH repair procedure?
  8. What follow-up care and monitoring will be necessary after the CDH repair surgery?
  9. Are there any alternative treatment options or additional management strategies that may be considered for my baby’s CDH?
  10. What is the expected recovery timeline and prognosis for my baby after undergoing CDH repair during ECLS?

Reference

Authors: Prabhu S, Mattke AC, Anderson B, McBride C, Cooke L, Karl T, Alphonso N. Journal: ANZ J Surg. 2016 Sep;86(9):711-6. doi: 10.1111/ans.13466. Epub 2016 Mar 14. PMID: 26990599