CDH is a complex disease that often requires lifelong follow-up care with many specialists. Children cared for by a multidisciplinary, integrated team have better outcomes and live healthier lives.

Seattle Children’s CDH program offers the best care and outcomes in the Northwest and is a destination center for families living in U.S. western states and abroad.  

12 Reasons to Choose the CDH Program at Seattle Children’s:

  1. One of the highest survival rates in the nation for children born with a CDH.
  2. One of the lowest rates worldwide for repeat hernia surgery.
  3. The only Level IV NICU in the Northwest.
  4. The largest extracorporeal life support (ECLS) program for babies, children and teens in the Northwest, providing all types of ECLS, including ECMO, VAD and total artificial heart.
  5. The only mobile ECMO program on the West Coast at a children’s hospital.
  6. Above-average survival rate for high-risk babies needing ECMO and a lower rate of babies needing to go on ECMO compared to CDH programs nationally, which translates to an overall higher survival rate.
  7. High-volume center, which studies show leads to better outcomes. Each year we see 15 to 20 new babies with CDH.
  8. pediatric surgery team that is one of the most experienced in the nation, with excellent outcomes, and is part of a multidisciplinary CDH team composed of specialists in maternal-fetal medicine, genetics, neonatology, cardiology, pulmonology, and feeding/nutrition.
  9. The same team of pediatric surgeons and neonatologists involved in a baby’s prenatal care will remain their same providers throughout their hospital stay. This allows us to get to know each family and baby very well and offer the very best care.
  10. Weekly care conferences; our specialists round on every CDH inpatient as a team to discuss their care and management (includes surgery, neonatology, cardiology, pulmonology, anesthesia, nutrition, care coordinators and OT/speech). Our surgery and neonatology teams check the babies multiple times per day to assess and manage their care.
  11. Comprehensive, long-term, multidisciplinary CDH follow-up clinic to both manage and prevent long-term complications from CDH.
  12. In 2020–2021, U.S. News & World Report ranked Seattle Children’s top in the Northwest and among the nation’s best programs in key CDH specialties: neonatology, cardiology and pulmonology.

Prenatal Diagnosis and Treatment

Care for babies with a CDH starts with our Prenatal Diagnosis and Treatment program, a large team of specialists that include maternal-fetal medicine doctors, fetal radiologists, genetic counselors, surgeons, neonatologists, cardiologists, nurses and care coordinators. Families will meet and work with all of these specialists before their baby is born.

Most prenatal visits take place at our Prenatal Clinic at the Springbrook Professional Center, a block from the main Seattle hospital campus.

The following Seattle Children’s locations offer fetal echocardiogram with cardiology consultation:

Our Bellevue Clinic and Surgery Center also offers fetal MRI and urology consultation.

When a fetus is diagnosed with CDH, we perform MRI and fetal ultrasound tests that are very helpful in determining the level of risk to the fetus. They also help our providers communicate in very precise terms with families about what outcomes to expect for their baby.

Seattle Children’s prenatal care is always evolving in response to the latest advances in CDH research. One measurement our prenatal team looks at closely is the “percent predicted lung volume” (PPLV) based on fetal MRI. The newest data show that a PPLV greater than 15% means that the baby is almost certain to survive. Their likelihood of needing ECMO is 10%, and these babies spend an average of just 37 days in the hospital after birth. When PPLV is below 15%, the survival rate drops to 40%, the risk for ECMO is high and these babies will likely be in the hospital for 101 days on average. It’s very helpful to the family as well as providers to have this level of specificity.

Delivering at UW Medical Center

When a baby has CDH and will be in our care, we recommend delivery at UW Medical Center. We require this because we partner with UW to follow a strict set of guidelines for the baby’s safety. These guidelines were developed in a multidisciplinary fashion with extensive literature review and input from multiple services. They standardize the minutiae of the management of these babies. Studies have shown time and again that this improves outcomes for these patients.

Sometimes families consider going to a non-children’s hospital because it’s important to them to deliver their baby closer to home; to use their own pregnancy provider; or for financial, convenience or other reasons. Because we want all children with a CDH to get the best care possible, we encourage community providers to advise their families to contact us to allow us to address their questions and concerns, no matter how small or non-concerning the CDH may appear. We offer a tour of our hospital to meet members of the CDH team and show families the areas where their baby would be cared for. We let them know about all the resources available Seattle Children’s offers for their family. We also made a video for families to show them how we safely transport fragile newborns from UW with the best safety protocols, equipment and care team in place.

Top Clinical Care for Newborns

The CDH field of medicine is continually evolving, and we are likewise always raising our level of care. One hot topic recently has been hernia repair and the timing of when to do it for babies on ECMO.  This has been a hard question to answer because each hospital only sees a small number of these sickest babies. Traditionally, hospitals have done the repair once the baby was off ECMO, if they were able to survive. Using the CDH registry data from dozens of hospitals, a study last year published compelling data showing early hernia repair for babies on ECMO significantly improved survival. In response, Seattle Children’s has made early repair our surgery strategy for these critically ill babies.

Multifaceted Long-Term Follow-Up

Some babies with a CDH have no long-term problems, but many need additional care until their teen years or longer. Given the myriad of risk factors they face, our outpatient CDH population is an incredibly heterogenous group with a wide clinical spectrum of CDH-associated morbidities that include:

  • High blood pressure in their lungs. Thirty percent of CDH patients at Seattle Children’s are discharged on oxygen for pulmonary hypertension or other respiratory support and will require many follow-up visits, homecare support and a prolonged oxygen wean.
  • Chronic lung disease. Studies report a 16% to 54% incidence of chronic lung disease in babies with a CDH requiring bronchodilators, diuretics, oxygen supplementation or ventilatory support.
  • Gastroesophageal reflux (GER). Common in children born with a CDH, reflux can cause discomfort, make feeding harder and worsen some breathing symptoms. It may require medication or, rarely, surgery. About 40% to 81% of patients experience GER in the first year; 30% of our patients have G-tube placement.
  • Nutritional concerns and feeding problems. Forty to fifty percent of infants with a CDH weigh below the 25th percentile at 2 years of age. Our occupational therapists help babies develop feeding skills. At Seattle Children’s, 80% of CDH patients are discharged on partial or full NG feeds and will require close outpatient monitoring from specialists in nutrition and occupational therapy.
  • Heart defects, such as being born with a hole in their heart.
  • Higher incidence of recurrent pneumonia and frequent, serious colds with slower recovery because their lungs are smaller and stiffer than normal during the first few years of life. We recommend special vaccines to prevent the most serious infections. Children who are traveling by airplane or to higher altitudes need to take appropriate precautions.
  • Asthma. Our team watches for early signs of asthma to be sure it is treated correctly.
  • Curved spine (scoliosis). Forty percent of CDH survivors will have musculoskeletal deformities, including a 10% to 27% incidence of scoliosis.
  • Problems with how the chest wall forms (pectus carinatum or pectus excavatum).
  • Neurodevelopmental concerns. Sixty percent of babies have motor delay at 1 year, 73% by age 3; we see speech delays in 18% at 1 year and 60% by age 3, and sensorineural hearing loss up to 60% and abnormal muscle tonicity.
  • Long-term surgical complications in children with a CDH most frequently include reherniation and bowel obstruction requiring reoperation.

Seattle Children’s provides a single place to get check-ups and coordinated follow-up treatment from all the specialists a child needs in our multidisciplinary CDH clinic.

Support for the Whole Family

Our doctors, nurses, child life specialists and social workers help families through the challenges of a CDH and connect them to community resources and support groups. Whether the family lives nearby or far away, we can help with financial counseling, schooling for other children in the family, housing, transportation, interpreter services and spiritual care.

Advances in Care

Clinical care: Our CDH team is committed to providing state-of-the-art care and services. Currently, they are:

  1. Developing a clinical standard pathway for respiratory support for inpatients
  2. Optimizing nutrition and feeding care for patients
  3. Improving the way we diagnose and manage neurodevelopmental deficits in patients

Clinical research: Seattle Children’s is part of an international CDH study group that is a consortium of 80 centers from 16 countries with a registry that has data on over 10,000 children with CDH.

Dr. Rebecca Stark, Seattle Children’s CDH program director, is a site PI for these current studies:

  • Lung perfusion studies as predictors of long-term lung function in children with CDH
  • Outcomes in CDH babies with severe pulmonary hypertension treated with prostacyclin
  • Right-sided CDH associated with trisomy 21 and anorectal malformations
  • Video fluoroscopic swallow studies and dysphagia in neonates with CDH

The CDH registry not only homes in on inpatient management, but also looks at risk factors following discharge that affect patient outcomes like race, income, language spoken at home, etc. Many children with a CDH require extensive therapy at home, but that care is harder for some families to access than others. During the COVID-19 pandemic, many home-based therapies have stopped for CDH kids, and we are seeing a clear discrepancy between families who speak English and those who don’t. The registry will help to identify and address these inequities.

Seattle Children’s CDH Team

Leadership:

  • Rebecca Stark, MD, is the CDH program director and a specialist in pediatric surgery.
  • Robert DiGeronimo, MD, is the CDH program medical co-director and director of our NICU.
  • BreAnna Kinghorn, MD, MS, runs the long-term, follow-up clinic for CDH patients along with Dr. Stark.

Cardiology:

Neonatology:

Nursing:

  • Allie Schneider, RN

Nutrition:

  • Jenny Stevens, RDN, CD, CNSC

Prenatal care team:

Pulmonology:

Surgery:

 

More Information

Please call the Prenatal Clinic at 206-987-5629 (866-987-2000 toll-free) if you have any questions.