Updates

Congenital Diaphragmatic Hernia Program

 Patient Resources

Delivery

When a baby has CDH, most women can have a vaginal delivery, unless other medical reasons require a cesarean delivery.

Babies with CDH are at higher risk of having problems with their lungs and having trouble breathing. They may need specialized treatment as soon as they are born. One kind of treatment is called ECMO (extracorporeal membrane oxygenation), a heart-lung bypass machine that performs the work of the heart and lungs so they have time to rest or grow. Even babies whose prenatal imaging looks good will need highly specialized care in an experienced neonatal intensive care unit (NICU).

We recommend that all babies with CDH be delivered in a center with a team who is experienced in taking care of the most critically ill newborns. This is important because a transfer to another hospital could be life-threatening transfer.

Delivery at a center like Texas Children’s Pavilion for Women provides for the best possible outcomes, because the mother and baby can receive expert care in one comprehensive location. We have a level IV NICU (the highest level), and babies delivered at Texas Children’s have immediate access to pediatric surgical care as soon as they are born. Our expert doctors and surgeons are well-equipped to manage a high-risk delivery and provide the specialized care these babies need. Texas Children’s also offers small, family-centered classes to help families prepare for their newborn’s hospitalization after they are born

For babies with CDH, their delivery and the care they receive after they are born need to be carefully planned and coordinated. At Texas Children’s, every member of our team of maternal-fetal medicine specialists, neonatologists and pediatric surgeons is highly experienced. They work together to provide the very best care for babies with CDH.

CDH Diagram Figure 1

Treatment after Birth

After your baby is born, a breathing tube will be placed, along with a tube through the mouth or nose into the stomach, to decompress the intestines within the chest so the lungs can expand. Until the hernia is corrected through surgery, your baby will need intravenous nutrition.

Every baby with CDH is different. Although we closely monitor and try to gauge how severe the CDH is while they are in utero, the amount of support and treatment that will be needed at birth and afterwards is uncertain.

At Texas Children’s, our neonatologists and pediatric surgeons specialize in the treatment of newborns across the full spectrum of CDH, from medications to treating pulmonary hypertension, to high-frequency oscillatory ventilators, to ECMO.

Surgical Repair

The timing of surgery to repair the hernia after birth, and the length of hospital stay, depends on the severity of your baby’s CDH and the amount of support needed. Typically, surgery is performed within the first week of life after your baby has stabilized from the birthing process, either on a ventilator or on ECMO.

During the surgery, an incision is made in the diaphragm just below the rib cage. The intestines and other abdominal organs are removed from the chest cavity and placed back in the baby’s abdomen. The hole in the diaphragm is then closed with stitches. In cases where the hole is very large, a prosthetic mesh patch is used to help close the defect.

After the repair, a chest tube — and sometimes a drain — are left so than any excess fluid can flow out of the baby’s chest to help the lungs expand.

Following surgery to close the hole, it will take time for your baby’s lungs to recover. It may be weeks or months before your infant is able to go home. Other treatments such as IV nutrition and antibiotics are often used to support your baby in the meantime.

CDH FETO Surgical Repair

CDH is often diagnosed before a baby is born during a routine ultrasound in the second trimester of pregnancy. The fetal ultrasound may show abdominal organs, such as the liver, in the chest or an increased amount of amniotic fluid, which may be caused by fetal lung abnormalities.

In some cases, CDH may not be detected until after the baby is born.

If CDH is diagnosed during pregnancy, you may be referred to a fetal center for comprehensive evaluation and specialized care.

We can arrange for you to visit Texas Children’s Fetal Center as quickly as possible to meet with our team of specialists who are experienced in treating CDH. Our team includes pediatric surgeons, maternal-fetal medicine physicians, neonatologists, genetic counselors and fetal imaging experts  who will care for you from the time of your diagnosis, through delivery and beyond.

Prenatal Evaluation

Testing may include:

We use measurements from your prenatal imaging to look at how severe your baby’s CDH is:

  • Fetal lung-to-head ratio
  • Amount of liver herniation into the chest: We will look at the position of the liver to see if any of it has pushed into the chest — and if so, how much. Larger defects tend to have more liver herniated into the chest.
  • Total fetal lung volume: We will measure how much lung your baby has from your fetal MRI.

At the end of the day, we will meet with you to go over your results. Based on the needs of your baby, we will help you make the most informed decisions for care and treatment.

Fetal Surgery for CDH

When a baby has severe CDH, our team may offer an experimental fetal surgery known as fetal endoscopic tracheal occlusion (FETO). FETO may be an option to potentially improve lung growth before birth. Texas Children’s CDH Program is one of the few centers in the nation to offer FETO, and we have one of the most skilled and experienced teams of physicians in this treatment.

Texas Children’s Fetal Center is also one of the few programs in the country with permission from the U.S. Food and Drug Administration (FDA) for the moderate TOTAL (Tracheal Occlusion To Accelerate Lung Growth) trial, a pilot studying the use of FETO for moderate cases of CDH.

FETO is a minimally invasive procedure where a small camera, about the size of a straw, is inserted through a small incision through the mother’s abdomen and into the uterus. A small inflatable balloon is placed into the fetal trachea to temporarily block the airway.

Blocking the fetal trachea allows fluid to build up in the lungs with the goal of promoting growth and development during the remainder of the pregnancy. The balloon is removed before delivery, often around 34 weeks gestation.

After delivery, surgery can be performed to close the hole in the diaphragm.

Because the FETO procedure temporarily blocks the baby’s airway, unexpected delivery can be life-threatening. The mother must remain near the fetal center for close monitoring and follow-up throughout the pregnancy, with immediate access to specialized care in case of preterm delivery.

At Texas Children’s, our CDH FETO team is on standby 24/7 for when our patients need us.

Long-term follow-up care is a critical component of care for babies with CDH, as many children often have long-term health issues. These issues depend on the severity of the defect and any related problems.

At Texas Children’s, our core group of pediatric surgeons, pulmonary (lung) specialists and dedicated practice providers follow your baby through the years as they grow.

Our team works closely with your family to assess your baby’s changing needs and monitor the child for long-term health issues, including:

  • Lung problems – Potential issues may include the inability to wean off the ventilator, a continuing need for oxygen support, chronic lung disease, asthma and persistent pulmonary hypertension (high blood pressure in the lungs).
  • Feeding difficulties - Many babies with CDH need a feeding tube placed in their nose or stomach to get adequate nutrition. Even with a feeding tube, some babies have difficulty gaining weight, especially in the first year of life.
  • Heartburn - Gastroesophageal reflux is common in babies with CDH. Heartburn is often treated with medicine, but some babies may need a separate operation to treat the reflux.
  • Development - Potential developmental problems may include hearing loss and developmental delays. Babies who require ECMO are at greatest risk for more serious neurological problems.
  • Scoliosis - Some children develop scoliosis, an abnormal curvature of the spine.

Before your infant is discharged from our NICU, we will schedule your appointment at our multidisciplinary clinic, where all of your baby’s CDH care is coordinated in one location.

Texas Children’s CDH Outcomes Clinic and Complex CDH Clinic

Two clinics that provide comprehensive, multidisciplinary postnatal care for patients with CDH

Texas Children’s CDH Outcomes Clinic

Texas Children’s Hospital offers two postnatal follow-up clinics for patients with congenital diaphragmatic hernia, the CDH Outcomes Clinic and the Complex CDH clinic. Both clinics allow our patients to be seen by a multidisciplinary team, including pulmonary hypertension and pediatric surgery.

The CDH Outcomes Clinic launched in 2018 to provide routine follow-up care and monitor outcomes after surgery for stable CDH patients who do not need any supplemental oxygen or pulmonary medication. At each appointment, parents can expect their child to undergo follow up testing which may include growth measurement, chest x-ray, and pulmonary function testing. Results will be reviewed by pediatric surgery and pulmonary care teams for evaluation of surgical outcomes, pulmonary function, nutrition, developmental pediatrics and more. Other topics reviewed are the current status of developmental milestones and neurological development. Patients under the age of 1 are typically seen every 3 months. Patients 1-2 years old are seen every 6 months. Patients who are 3-18 years old are seen annually. However, this schedule can vary based on each patient and their needs. Parents can discuss any questions or concerns they have regarding continued prognosis for their child with congenital diaphragmatic hernia.

Complex CDH Clinic

The Complex CDH Clinic opened in April 2019 to extend multidisciplinary care to CDH patients who require additional oxygen supplementation, chronic ventilator support or pulmonary hypertension medications. These patients are more complex, with more postnatal complications and require closer follow-up care and support. Similar to CDH Outcomes Clinic, parents can expect testing to include measurement of growth, chest X-ray and pulmonary function testing. Bloodwork, echocardiography and a six-minute walk test may also be performed. In addition to pulmonologists who are specialized in pediatric pulmonary hypertension and management of tracheostomy/ventilator-dependent children, the clinic is staffed by members of Pediatric Surgery and respiratory therapists.

For more information about Texas Children’s Hospital’s congenital diaphragmatic hernia programs, please call the following numbers:

  • CDH Complex Clinic 832-822-3696
  • CDH Outcomes Clinic 832-822-3135

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