Extracorporeal membrane oxygenation in congenital diaphragmatic hernia
Presentation
Term newborn baby boy via vaginal delivery with respiratory distress requiring immediate intubation.
Patient Data
Multiple gas-filled bowel loops and the gas-filled stomach are seen herniated into the left hemithorax causing mass effect and mediastinal shift to the right. The left hemi-diaphragm is not visualized. There is diffuse compression of the right lung. The left lung is barely visualized on this exam. These findings are consistent with a congenital left diaphragmatic hernia (CDH).
The OG tube (orogastric tube) is seen terminating in the expected region of the GE junction. The ETT terminates at T3 level and is well above the carina.
The venoarterial (V-A) circuit ECMO catheters are visualized in the right neck. The venous cannula courses through the right internal jugular vein. Adjacent to the venous cannula is the arterial cannula, which courses through the right common carotid artery.
There is extensive diffuse edema of the soft-tissues. A (low) UAC (Umbilical arterial catheter) is seen terminating at L3 level in an appropriate position.
Multiple gas-filled bowel loops and the gas-filled stomach are seen herniated into the left hemithorax causing mass effect and mediastinal shift to the right. The left hemi-diaphragm is not visualized. There is diffuse compression of the right lung. The left lung is barely visualized on this exam. These findings are consistent with a congenital left diaphragmatic hernia (CDH).
The OG tube (orogastric tube) (yellow arrow) is seen terminating in the expected region of the GE junction. The ETT (green arrow) terminates at T3 level and is well above the carina.
The venoarterial (V-A) circuit ECMO catheters are visualized in the right neck. The venous cannula (blue arrow) courses through the right internal jugular vein. Adjacent to the venous cannula is the arterial cannula (red arrow) which courses through the right common carotid artery.
There is extensive diffuse edema of the soft-tissues. A (low) UAC (Umbilical arterial catheter) is seen terminating at L3 level in an appropriate position.
Case Discussion
A congenital diaphragmatic hernia is a birth defect of the diaphragm that allows abdominal organs to herniate and protrude into the chest cavity. The incidence is 1 in 2500 to 5000 live births. This anomaly is often discovered via ultrasound at 10-12 weeks gestation. A major complication from this abnormal growth is the underdevelopment of the lung and pulmonary vasculature causing pulmonary hypoplasia or pulmonary hypertension. Treatment requires surgical repair to fix the defect.
If pulmonary hypertension is severe and oxygen exchange is compromised, extracorporeal membrane oxygenation (ECMO) can be used for pulmonary hypertension to resolve or till the definite surgery can be performed.
ECMO is a treatment that offers respiratory and cardiac support, largely used in neonates with life-threatening lung and heart conditions. It is a modified form of cardiopulmonary bypass. In simplest terms, blood is removed from the body (extracorporeal), flows through an artificial lung (membrane), CO2 is removed and blood is oxygenated. This oxygen-rich blood is then returned to the body.
ECMO can be applied in several instances included but not limited to severe infection, sepsis, pneumonia, cardiac arrest from congenital heart disease, ARDS, severe pulmonary hypertension as seen in CDH, and any end state cardiac and respiratory failure.
Two types of ECMO are commonly used: veno-arterial (VA) and venovenous (VV). VA ECMO supports both heart and lung functions by diverting blood back to the arterial system. This allows time for the heart and lungs to rest and recover. Blood is removed via a venous cannula before it enters the heart. Oxygenated blood is returned to the aorta. VV ECMO supports lung function only and diverts blood to the venous system. Oxygen poor, carbon dioxide enriched blood is removed by a venous cannula. This blood flows through an oxygenator which oxygenates the blood while also removing carbon dioxide. This blood is then returned to the same place at which it enters the heart. Blood is pumped through the body with the patient's own heart.
Complications of ECMO include hemorrhage, blood clots, fluid overload, and infection. The child is given medication for pain and sedation. Heparin is used to prevent blood clots. Fluid overload is managed via diuretics or hemofiltration.
ECMO can be utilized for days to weeks and should only be used for reversible lung and heart conditions or in those who are transitioning to transplant or long term device. Average time on ECMO is 3 to 14 days. For some, it is a life-saving treatment.
This case was submitted with supervision and input from:
Soni C Chawla, M.D.
Associate Professor
Department of Radiological Sciences
David Geffen School of Medicine at UCLA
Olive View - UCLA Medical Center
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