What is a congenital diaphragmatic hernia?A congenital diaphragmatic hernia is a condition in which a hole in the diaphragm allows abdominal organs to move into the chest and restrict lung development. The diaphragm is a thin layer of muscle and tissue that separates the chest and abdominal cavity. It is the muscle that the body uses to breathe. The contents of your child’s abdomen, including the stomach, intestines, liver and spleen may go through the hole and into his or her chest. A congenital diaphragmatic hernia (CDH) affects about 1 in every 2,500 babies. It is usually diagnosed during a routine ultrasound at 18-20 weeks of gestation. If it is not diagnosed during pregnancy, it is usually diagnosed shortly after birth when the baby is having trouble breathing. Two types of CDH are a Bochdalek hernia and Morgagni hernia.
Each child may experience symptoms differently, but possible symptoms of a diaphragmatic hernia may include: difficulty breathing, fast breathing, fast heart rate, cyanosis (blue color of the skin), abnormal chest development, with one side being larger than the other or an abdomen that appears caved in (scaphoid). A baby born with a Morgagni hernia may or may not show any symptoms.
How is it treated?The treatment for congenital diaphragmatic hernia is surgery. Your baby may have the surgery soon after birth, or the surgeons may wait days or weeks to do the operation depending on baby’s condition. The outlook for babies born with CDH is increasingly positive with new surgical techniques and ways to support babies as they heal. It is possible that your baby can have long term problems and need regular follow up care after going home from the hospital. If a CDH is suspected based upon prenatal ultrasound, an appointment should be made with a pediatric surgeon to review test results, confirm the diagnosis, explain the treatment and potential outcomes, and answer any questions you may have.
What will happen in the hospital?Most babies with congenital diaphragmatic hernia are delivered vaginally. The neonatal team is present at the delivery since the baby will require immediate stabilization and, often, specialized ventilation (breathing) assistance. After birth, a chest x-ray is done to look at the abnormalities of the lungs, diaphragm, and intestine and to reconfirm the diagnosis of CDH. A blood test known as an arterial blood gas is often performed to evaluate the baby's breathing ability.
A child with a diaphragmatic hernia requires care in a neonatal intensive care unit (NICU). Babies with congenital diaphragmatic hernia are often unable to breathe effectively on their own because their lungs are underdeveloped. Most babies will need to be placed on a breathing machine called a mechanical ventilator to help their breathing. Some infants may need to be placed on a temporary heart/lung bypass machine called ECMO if they have severe problems. ECMO does the job that the heart and lungs would be doing: putting oxygen in the bloodstream and pumping blood to the body. ECMO may be used temporarily while a baby's condition stabilizes and improves.
When the baby's condition has improved, the congenital diaphragmatic hernia will be repaired with an operation. The infant’s lungs must be able to deliver adequate oxygen to the body and maintain good blood flow through the vessels prior to the operation. Surgical repair of CDH depends on the baby’s progress in the days following birth, and occurs as soon as the baby is stable.
Surgery is done while your child is under general anesthesia. The surgeon makes an incision in the abdomen under the rib cage on the side of the CDH to be able to reach the organs of the abdomen. Then the surgeon gently pulls these organs down into place through the opening in the diaphragm and into the abdominal cavity. The surgeon repairs the hole in the diaphragm. If the hole is small, it may be repaired with stiches, if it is too large; a patch is used to cover the hole in the diaphragm. The space created in the chest allows the lungs to continue to grow. Your child’s lung size should slowly increase over time. At the time of surgery some babies may need a chest tube. If needed, the tube allows any fluid, blood or air that collects in the chest to drain out.
Many babies will need to remain in the NICU after surgery. Although the abdominal organs are now in the correct place, the lungs still remain underdeveloped. The baby will usually need to have breathing support for a period of time after the operation. Once the baby no longer needs help from a breathing machine (ventilator), he/she may still need oxygen and medications to help with breathing.
When will my child be discharged?The length of stay in the NICU can vary widely. Some infants need mechanical ventilation only briefly, have the surgical repair and can go home in a relatively short period of time. Others may need ventilation or ECMO for prolonged periods and require longer hospital stays. If your child goes home quickly, a follow up visit with be scheduled two weeks after the operation to ensure the incision is healing well. If your baby stays in the hospital longer, the pediatric surgery team will stay closely involved until they are discharged.
What will be my child's recovery?Post operatively, patients will remain in the NICU to recover. Many babies will remain in the hospital for several weeks after the surgery. If the baby’s lungs are underdeveloped or if breathing problems persist after the surgery, a ventilator may be necessary during the recovery. The length of time your baby spends in the NICU is largely dependent upon how severe the hernia was, if there was any organ damage and how well developed the organs are. Once the baby is taken off the breathing machine, he or she will still likely need oxygen to adequately ventilate the lungs. Feedings will start once your baby’s bowels start working. Feeding are usually done through a feeding tube from the mouth into the stomach until your child can take the food he or she needs by mouth. On occasion, feeding difficulties can be significant and prolong the hospitalization.
What should I be looking out for after the operation?All patients with congenital diaphragmatic hernia require ongoing follow-up. Post operatively, it is important to monitor for signs or symptoms or infection such as redness, swelling around the surgical site or fever. The steri-strips should remain in place for at least ten days after the surgery.
Babies born with CDH can have long-term problems and often need regular follow-up after going home from the hospital. Depending on the severity of the initial defect and the type of repair, follow up may be managed by the child’s primary pediatrician or may require more specialized care.
Many babies have pulmonary hypoplasia, a condition characterized by small, under developed lungs that affect breathing. This requires long term follow up. Many babies will have gastroesophageal reflux. Acid and fluids from the stomach move up into the esophagus (the tube that leads from the throat to the stomach), and can cause heartburn, vomiting, feeding problems, or lung problems. Since gastroesophageal reflux is so common, almost all infants and children with CDH are given acid controlling medications.
Some babies will have difficulty growing. This is known as failure to thrive. The children with the most serious lung problems are most likely to have growing problems. They often require more calories than a normal baby in order to grow and get healthier. The outcome of this surgery depends on how well your baby’s lungs have developed. Usually the outlook is good for infants who have well developed lung tissue. With advances in medicine, the outlook for these infants continues to improve.