What is CDH?
Congenital diaphragmatic hernia, or CDH, is a birth defect in which the diaphragm has a hole that allows organs from the abdomen to push up into the chest. During the baby’s development, a portion or all of the diaphragm on one side fails to form, creating the hole. Abdominal contents in the chest prevent lung growth on the affected side. With CDH in babies, the airways and blood vessels of the lungs also develop abnormally. The exact cause of CDH in babies is unknown. CDH occurs in approximately 1 of every 3,000 pregnancies.
What can severe CDH cause?
Babies with CDH can have severe breathing problems, as well as pulmonary hypertension, a condition that makes it difficult for blood to circulate through the underdeveloped lungs. The severity of symptoms and the baby’s survival depends on the size of the birth defect in the diaphragm, the amount and types of organs pushing up into the chest and the amount of lung tissue that can develop. In addition to issues with lung growth, problems with other organs can also affect the survival of babies with severe CDH.
Diagnosis of CDH
CDH is typically identified by fetal ultrasound. When detected prenatally you will be referred to a perinatologist, a pediatric surgeon and a neonatologist to discuss what type of CDH your baby has and what to expect. Your baby will be followed by ultrasound. A fetal echocardiogram will be performed to evaluate the structure of the heart. A magnetic resonance imaging exam will also be arranged. Your baby’s delivery will be planned at a tertiary medical center with direct transfer to Rady Children’s Comprehensive Congenital Diaphragmatic Hernia Center.
Sometimes CDH is diagnosed after the baby is born. Your baby will be transferred directly to Rady Children’s as soon as the diagnosis is made.
What happens after delivery of potential CDH patients?
Most babies with CDH will need a breathing tube and may need help from a ventilator for many weeks. Your baby will be seen by a neonatologist and a pediatric surgeon. Consults to other specialties including genetics, cardiology, pulmonary hypertension, pulmonology, and gastroenterology will occur as needed. Once your baby has been stabilized, surgery from healthcare professionals can be done to repair the defect in the diaphragm and replace the intestines and other organs back in the abdomen. The timing of repair will be carefully decided based on your baby’s individual case.
An umbilical venous catheter or a peripherally inserted central catheter will be placed for health professionals to give IV (intravenous) medications, including blood pressure medications and IV nutrition. An umbilical arterial catheter or a peripheral arterial line will be placed to accurately measure blood pressure and for frequent lab draws.
Babies with CDH can have other problems outside of lung growth issues. Your baby will have imaging of their brain, heart and kidneys.
Your baby may also be extremely sick. One therapy that can be offered is ECMO (extracorporeal membrane oxygenation), a heart-lung bypass treatment that can allow time for pulmonary hypertension to improve. Other treatments include inhaled nitric oxide and Heliox, an inhaled helium-oxygen mixture that improves removal of carbon dioxide and eases the work of breathing.
What happens after CDH patients have surgery?
After the CDH has been repaired, respiratory support will gradually be weaned. When your baby no longer needs assistance from the ventilator, the breathing tube will be removed. Your baby may temporarily need CPAP (continuous positive airway pressure) or a nasal cannula to make his/her breathing easier. In some cases, babies need to go home with oxygen after seeing healthcare providers.
Babies with CDH cannot be fed until after the hernia is repaired by healthcare professionals. Your baby will receive full IV nutrition from TPN (total parenteral nutrition) while awaiting surgery. After surgery, your baby will be monitored feeding readiness. Once it appears that their intestines are ready, small feeds will be started, either by mouth or via a nasogastric tube (a small flexible tube passed from the nose down to the stomach) if considerable respiratory support is still required. Your baby will be followed by an occupational therapist, lactation consultant and registered dietician to support feeding and nutrition.
The length of your hospital stay will vary depending on the severity of your baby’s CDH.
Follow-up Care
All babies need a pediatrician for well-child care. For babies with CDH, a pediatrician is also needed to coordinate care from multiple specialists.
After you leave the hospital, your baby will be seen by by health professionals in our Pediatric Surgery program and the NICU Neurodevelopmental Clinic to focus on developmental care. Babies with CDH may continue to have medical issues after leaving the hospital. Your baby may also require referral to the Rady Children’s Pulmonary Hypertension Program to be seen by a cardiologist and pulmonologist.
