Our Summary

This research paper discusses a rare case of a newborn baby who was born with birth defects - Tracheoesophageal fistula (TEF) and esophageal atresia (EA) and absence of one lung (pulmonary agenesis). These conditions occur in about 1 in 2500 to 3500 newborns. The baby was born prematurely at 33 weeks and experienced breathing difficulties after birth, requiring a breathing tube. Doctors were unable to pass a feeding tube through the baby’s nose to the stomach. Further tests revealed the baby had TEF, EA and was missing a lung.

The doctors initially tried to fix the TEF through a typical surgical approach but encountered difficulties due to the baby’s unique anatomy and fears of further compromising the baby’s breathing. They instead opted to use a different surgical approach, called a median sternotomy, which involves making an incision down the middle of the chest. This approach proved successful.

The authors note that this is only the second time this surgical approach has been used successfully to repair TEF and EA. They suggest that this method could be considered for similar complex cases in the future.

FAQs

  1. What is a tracheoesophageal fistula (TEF) and esophageal atresia (EA)?
  2. What is the median sternotomy approach used in the treatment of TEF and EA?
  3. How often does a child present with both TEF/EA and pulmonary agenesis?

Doctor’s Tip

A doctor might tell a patient undergoing sternotomy for a TEF/EA repair that this approach allows for better visualization and access to the affected area, leading to a successful repair. They may also advise the patient to follow post-operative care instructions carefully to ensure proper healing and recovery.

Suitable For

Patients who are typically recommended sternotomy for repair of congenital anomalies such as tracheoesophageal fistula (TEF) and esophageal atresia (EA) include those with complex pulmonary malformations that limit visualization and access via standard thoracotomy approaches. In cases where there is concern for significant ventilatory compromise during repair, a median sternotomy approach may be considered for definitive treatment. This approach may be particularly beneficial for neonatal patients with pulmonary agenesis or other anatomical variances that make traditional thoracotomy challenging.

Timeline

Before sternotomy:

  • Patient is born prematurely at 33 weeks gestational age
  • Patient experiences respiratory distress and is intubated
  • Nasogastric tube is unable to be passed, leading to suspicion of esophageal abnormality
  • Imaging confirms diagnosis of tracheoesophageal fistula (TEF) and esophageal atresia (EA) in conjunction with pulmonary agenesis
  • Planned staged repair with ligation of TEF via standard right thoracotomy approach

After sternotomy:

  • Significant ventilatory compromise is encountered during planned repair
  • Concern for ventilatory compromise and anatomical variance limiting visualization leads to decision to utilize median sternotomy approach
  • Median sternotomy approach is successful in exposing and repairing TEF/EA
  • This approach may be considered for cases with complex pulmonary malformation limiting standard thoracotomy
  • This is only the second reported case of successful TEF/EA repair using a median sternotomy approach.

What to Ask Your Doctor

  1. What is a sternotomy and why is it being recommended for my condition?
  2. What are the potential risks and complications associated with a sternotomy procedure?
  3. What is the success rate of sternotomy for repairing TEF and EA in conjunction with pulmonary agenesis?
  4. How long is the recovery time after a sternotomy procedure?
  5. Will I need any additional follow-up care or treatment after the sternotomy procedure?
  6. Are there any alternative treatment options to sternotomy for my condition?
  7. How experienced are you in performing sternotomy procedures for TEF and EA cases?
  8. Are there any specific precautions or lifestyle changes I should take after undergoing a sternotomy procedure?
  9. What are the long-term outcomes and prognosis for patients who undergo sternotomy for TEF and EA with pulmonary agenesis?
  10. Are there any support groups or resources available for patients undergoing sternotomy for TEF and EA?

Reference

Authors: Walker SC, Ma BL, Sawaya DE Jr. Journal: Am Surg. 2024 Aug;90(8):2089-2091. doi: 10.1177/00031348241241749. Epub 2024 Mar 28. PMID: 38549241