Congenital Diaphragmatic Hernia Center | Acute & Long-term Care

When a baby is born with congenital diaphragmatic hernia (CDH), they are stabilized and then transferred from their birth hospital to Boston Children’s Medical-Surgical Intensive Care Unit (MSICU).

All babies with CDH require a breathing tube and support of a breathing machine called a ventilator immediately after birth. Through research and experience managing patients with CDH over decades, we have observed the importance of use of “gentle ventilation” to protect the small lungs as they develop. Extracorporeal membrane oxygenation (ECMO), a machine that does the job of the heart and lungs, is used when the ventilator and medicines do not provide enough support to the babies while their lungs are developing. About half of the babies we treat with CDH need ECMO.

Surgery is typically performed within the first week of life after the period of transition at birth. During this time we allow pulmonary hypertension (the high blood pressure in the lungs) to improve from the initial time of delivery and adjust the ventilator to optimize conditions for your baby to have surgery. Surgery is either performed with an incision in the abdomen (open approach) or with smaller incisions in the chest with a camera (thoracoscopic). At the time of surgery, the abdominal contents will be brought out of the chest back in to the abdomen and the diaphragm is closed. Sometimes there is enough muscle present to close the muscle to itself (primary repair), but more often a patch is required to close the hole. Once the repair is performed, then the lung has space to grow and develop.

Following surgery to repair CDH, your baby may need to stay in the hospital for several months to recover, depending on their individual situation. You will be part of your baby’s team and will be part of the discussion about their progress and plan of care each day. After the lungs have grown enough to come off of the ventilator and your baby has progressed, they will be transferred out of the ICU to a regular post-operative floor while they recover enough to go home. During this time they are working on feeding and weaning oxygen support that they are on through a cannula (small tube) in the nose. We encourage you to stay with your baby as much as possible so you will be comfortable before going home — and our team will teach you how to care for your baby at home when the time comes.

Boston Children’s Congenital Diaphragmatic Hernia Center offers a long-term clinic for children born with CDH. Established in 1991, our clinic is longest-running follow-up clinic of its kind in the world and monitors and treats the long-term health needs of CDH patients, from early childhood through young adulthood.

Our typical outpatient clinic runs weekly. During your visit, you and your child will see multiple clinicians from specialties including general surgery, pulmonology, nutrition, and developmental medicine. We also coordinate appointments with other necessary specialists, including cardiologists and neurologists, to limit your family’s time spent at appointments.

Your visits will occur frequently in the first years of your baby’s life. Most children with CDH improve as they grow, so you will have fewer visits over time. Your child will continue to be seen in our clinic into adulthood to ensure best possible outcomes.