Babies born with CDH are at high risk for respiratory distress. Immediately following delivery, doctors will carefully evaluate the baby. A neonatologist (a specialized doctor for high-risk babies) will begin providing breathing support and other necessary care. Depending on the severity of breathing difficulties, the baby may require a breathing tube. The newborn will be transferred to the neonatal intensive care unit for further evaluation and management.
Most infants will be stable after birth and will be closely monitored. Although breathing difficulties may require mechanical ventilation, the newborn may not require additional support and can be stabilized until surgery. A small group of infants with CDH may continue to develop problems with breathing and problems maintaining heart function and blood pressure. Your baby's doctors will provide many different methods to improve the condition of your baby. In the most severe cases, your newborn may need to be supported by using ECMO, a heart-lung bypass machine.
Surgical repair of the CDH is not an emergency. In most cases, the pediatric surgeon will wait until the infant is stable and able to handle the stresses of surgery. The pediatric surgeon will evaluate the baby for surgery, and once he or she has been stabilized for a period of time, surgery will be performed to repair the CDH. In some cases, the baby will stabilize quickly enough to undergo surgery in a few days after delivery. In more severe cases, it may be longer before it is safe to perform the surgical repair. The surgeon will determine the safest time for the surgical repair. Typical hospital stays for a newborn with CDH range from a few weeks to several months.
Children born with congenital diaphragmatic hernia range from those who appear normal at birth to those who are so sick they immediately need extracorporeal membrane oxygenation (ECMO) to support heart and lung function. Although other factors are involved in this complicated disease, much of the severity of CDH is related to the size of the hole in the diaphragm. This can only be determined during the operative repair of your child’s diaphragm. In general, the larger the hole, the more care your child will need.
Doctors classify congenital diaphragmatic hernia according to the size of the defect – A, B, C or D. A very small hole is classified as A; a classification of D means your child was born with a very large hole in the diaphragm (see diagram below).
Surgery to close the hole in the diaphragm is typically performed within two days to two weeks after birth. Open surgery involves a single, longer incision underneath the ribs. Depending on the size of the defect, a synthetic patch may be used to close the hole. In some cases, pediatric surgeons may perform a minimally invasive repair through three or four incisions, each less than 1 centimeter, all near the patient’s armpit. The appropriate approach will be determined based on each individual patient’s needs. Overall, the repair operation usually takes about two hours.
The affiliated team will choose the best approach for the individual child. With either approach, the overall goals are the same: reposition the organs that are in the chest back into the abdomen where they belong and rebuild a new diaphragm. Sometimes the diaphragm can be sewn together using sutures only (also called a “primary” repair), and sometimes the diaphragm repair requires a special patch (usually GoreTex®) to close the diaphragm (also called a “patch” repair).
About one-third of children born with CDH require ECMO.
Medical advances have made it possible for more than 70% of infants born with CDH to survive. Babies who survive can have ongoing challenges with breathing, feeding and growth. Survival rates and quality of life depend on the size of the hole, which doctors determine during surgery, and also on how well the baby’s lungs and blood vessels have developed. The outlook tends to be better for infants who have enough working lung tissue and no other problems.
The outcome also depends on whether there are any other abnormalities at birth. Other congenital anomalies are present in about one-third of babies with CDH. Heart lesions account for nearly two-thirds of these anomalies and have a major effect on the risk of death and disability. These and any other anomalies present at birth add to the complexity of CDH and the need for specialized care.
Specialists at the High-risk Multidisciplinary Clinic for congenital diaphragmatic hernia, affiliated with Children’s Memorial Hermann Hospital, track a range of outcomes across various subsets of its patient population, creating a rich source of long-term data that enables the team to identify the best interventions for each case. Data collected can be tied to decisions about future research, enabling the physician researchers.
Those who have received fetal intervention, those who have received ECMO, and/or those with a patch repair are at highest risk and require specialized long-term follow-up by a multidisciplinary team of medical, surgical and developmental specialists to identify and treat problems before they result in disability. The more common complications include underdevelopment of the lung tissue, which can cause difficulty breathing; poor weight gain and growth; gastrointestinal challenges; pulmonary issues; recurrent diaphragmatic hernias; neurodevelopmental delays; hearing loss; and cardiac problems. Attention deficit hyperactivity disorder (ADHD) is emerging as a very common diagnosis among children with CDH.
CDH is an embryonic (before birth) defect with many facets. Along with underdevelopment of the lung tissue, the lung vasculature is compromised. Most children who survive severe CDH have long-term pulmonary problems. While the specialists at The Fetal Center can assess how much pulmonary hypoplasia infants are likely to have at birth, there are critical aspects of patient survival related to the lung vasculature that are difficult to measure and predict. The risk of death is related to the size of the diaphragmatic defect, associated anomalies, and the lung development.
Babies with CDH go to the Level IV Neonatal Intensive Care Unit (NICU) where they are stabilized, undergoing a process that usually takes between 48 hours and two weeks. Based on international data, the total length of your child’s hospital staycan be estimated based on the size and severity of the diaphragmatic hernia: A, B, C or D. These include:
Please note these are only averages and can vary widely, depending on the individual patient.
Within that time period, children with more severe CDH may need to be supported on ECMO. After being taken off ECMO, they may be on a mechanical ventilator for a period of time, depending on the severity the diaphragmatic hernia. Ultimately, most babies are able to breathe without a breathing machine. After their hospital stay, babies go home and transition to the High-Risk Multidisciplinary Clinic for CDH for comprehensive care of their needs.
Usually, doctors see babies in the clinic two to four weeks after their NICU stay.
The Fetal Center at Children’s Memorial Hermann Hospital is a national referral center and an international leader in fetal diagnosis, fetal intervention and comprehensive fetal care for infants with congenital anomalies or genetic abnormalities. The affiliated physicians at The Fetal Center offer patients the full array of prenatal testing and fetal interventions through a coordinated program for both mother and baby. From the initial prenatal consultation through pediatric follow-up visits, specialists at The Fetal Center work closely with mothers, families and their referring physicians to provide evidence-based care and treatment, as well as provide support, counseling and education.
Home to an integrated CDH team that includes pediatric surgeons, neonatologists, pediatric anesthesiologists, pediatric sub-specialists, and maternal-fetal medicine specialists, The Fetal Center provides optimal and seamless care from prenatal diagnosis to delivery and through postnatal care. Physicians at The Fetal Center take a very aggressive approach to the management of the condition, and are one of a handful of teams in the country that operate on 93% of all patients, repairing the hole in the diaphragm surgically to give each child a chance of survival.
Thanks to more accurate prenatal diagnosis and the work of the International CDH Study Group and Registry, which resides at Children’s Memorial Hermann Hospital and McGovern Medical School at UTHealth, the global survival rate of infants born with CDH is steadily rising. Based on risk-adjusted data, The Fetal Center’s outcomes are in the top 10% or higher.
Physician researchers at The Fetal Center are faculty at McGovern Medical School at UTHealth, and are actively engaged in an intensive research program focused on the mechanisms, treatment, and cure of CDH and other fetal disorders. A national leader in research, The Fetal Center is one of only three U.S. centers to hold membership in all three key maternal-fetal research networks: the National Institute of Child Health and Human Development (NICHD) Neonatal Research Network, NICHD’s Maternal-Fetal Units (MFMU) Network and the North American Fetal Therapy Network (NAFTNet).
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