Our Summary

This research paper is about a rare case where a 6-week old baby boy had a condition where parts of his organs were out of place from birth. This condition, known as bilateral congenital diaphragmatic hernia (CDH), is very rare and often results in high rates of newborn death. In this case, the baby’s liver had moved into an abnormal position on the right side and was unusually fused to the lung, a condition known as hepatopulmonary fusion (HPF). The baby also had abnormal blood flow from the lung to the liver and a hole in the wall that separates the two lower chambers of the heart.

The doctors decided to perform a surgery to fix the misplaced organs. They used a camera-assisted method to operate on both sides of the child’s body, but they had to make an additional cut on the right side. They carefully separated the liver from the lung using a tool that burns through tissue. They then used a mesh to close the hole that caused the organs to move in the first place. However, they left part of the area intact to avoid damaging the abnormal blood flow from the lung to the liver.

Two weeks after this procedure, the doctors performed another surgery to fix the hole in the baby’s heart. The baby had bleeding and needed help with breathing for an extended period after the surgery. However, there were no signs of the organs moving out of place again 9 months after the procedure.

FAQs

  1. What is congenital diaphragmatic hernia (CDH) and how common is it?
  2. How was the surgical repair of CDH performed in this case?
  3. What complications occurred during the postoperative course?

Doctor’s Tip

A helpful tip a doctor might tell a patient about congenital diaphragmatic hernia repair is to follow all postoperative care instructions closely, including keeping the surgical site clean and dry, taking any prescribed medications as directed, and attending follow-up appointments with the surgeon to monitor healing and prevent any complications. It is important to communicate any concerning symptoms or changes in the patient’s condition to the medical team promptly. Additionally, maintaining a healthy lifestyle and avoiding activities that could put strain on the repaired hernia can help promote successful healing and recovery.

Suitable For

Patients with congenital diaphragmatic hernia (CDH) are typically recommended for surgical repair, especially if they are experiencing symptoms such as respiratory distress, feeding difficulties, or failure to thrive. In the case of bilateral CDH, like the patient described in the abstract, surgical repair may be necessary to prevent complications such as herniation of abdominal organs into the chest cavity, respiratory compromise, or other associated anomalies such as hepatopulmonary fusion or cardiac defects. The decision to proceed with surgery will depend on the specific characteristics and severity of the hernia, as well as the overall health and stability of the patient.

Timeline

Before CDH repair:

  • Patient may present with respiratory distress, cyanosis, and difficulty breathing shortly after birth
  • Diagnosis of CDH is confirmed with imaging studies such as ultrasound or MRI
  • Patient may require respiratory support and monitoring in the neonatal intensive care unit
  • Surgical consultation is obtained for repair of the diaphragmatic hernia

After CDH repair:

  • Surgical repair of the diaphragmatic hernia is performed, either thoracoscopically or with a subcostal incision
  • In this case, the patient also had associated anomalies such as HPF, anomalous pulmonary venous drainage, and ventricular septal defect, which were repaired in subsequent surgeries
  • Postoperative complications may occur, such as bleeding and prolonged ventilation
  • Patient is monitored for hernia recurrence and overall respiratory and cardiac function
  • Long-term follow-up is required to assess for any complications or developmental delays related to the CDH repair.

What to Ask Your Doctor

  1. What is the prognosis for my child with bilateral congenital diaphragmatic hernia and hepatopulmonary fusion?

  2. How will the surgical repair of the hernia and HPF be performed? Will it be done thoracoscopically or through a subcostal incision?

  3. Are there any potential complications associated with the surgical repair of bilateral CDH and HPF?

  4. Will my child require any additional surgeries for associated anomalies, such as the anomalous drainage of the right pulmonary vein and ventricular septal defect?

  5. What is the expected recovery time and postoperative care for my child after the surgical repair?

  6. Will my child need any long-term follow-up or specialized care after the repair of bilateral CDH and HPF?

  7. Are there any lifestyle or dietary changes that my child will need to make following the surgical repair?

  8. What are the signs and symptoms of hernia recurrence that I should watch for in my child?

  9. Are there any genetic factors or risk factors that may have contributed to the development of bilateral CDH and HPF in my child?

  10. Are there any support groups or resources available for families of children with congenital diaphragmatic hernia and hepatopulmonary fusion?

Reference

Authors: Bawazir OA. Journal: Saudi Med J. 2019 Sep;40(9):949-953. doi: 10.15537/smj.2019.9.24378. PMID: 31522224