Congenital Diaphragmatic Hernia Repair: Lillian Jane’s CDH Story, Prenatal Diagnosis to Home

Описание: Children's Hospital of Philadelphia cares for more babies born with congenital diaphragmatic hernia than any hospital in the world. http://www.chop.edu/cdh

In This Video:
2:09 What is congenital diaphragmatic hernia?
2:43 Evaluation, diagnosis and delivery planning for CDH baby
4:50 CDH care requires an experienced, multidisciplinary team
5:30 Delivery and immediate stabilization after birth
7:11 Experience with ECMO
7:40 Getting strong enough for surgery
9:27 How do you fix CDH? Surgical repair of the diaphragm
10:35 Recovery in the NICU and discharge home
11:30 Committed to helping families

This video follows Laura, Nick and their daughter, Lillian Jane, on their journey from prenatal diagnosis of congenital diaphragmatic hernia (CDH), care at the Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment — including pregnancy management, CDH labor and delivery, and postnatal surgery — and discharge home.

CDH is a birth defect that occurs when the diaphragm, the muscle that separates the chest from the abdomen, fails to close when the baby is in the womb. This opening allows the stomach, intestines and/or liver to move into the chest, impacting lung development. Small lungs (pulmonary hypoplasia) that have less developed blood vessels can cause high blood pressure in the lungs (pulmonary hypertension).

Since 1995, the Wood Center for Fetal Diagnosis and Treatment at Children’s Hospital of Philadelphia (CHOP) has been at the forefront of understanding, diagnosing and treating CDH. The center’s team continues to advance innovations that improve outcomes for mothers and babies diagnosed with this condition.

This video features members of CHOP’s expert CDH clinical team, including Holly L. Hedrick, MD, Julie S. Moldenhauer, MD, Juliana Sanchez Gebb, MD, and Natalie E. Rintoul, MD. This team answers questions like what is CDH, what causes CDH in babies, pregnancy management, diaphragmatic hernia treatment options, the specialized team that cares for babies immediately after birth, and the importance of long-term multidisciplinary follow-up.

CDH is a congenital anomaly that occurs in 1 of every 3,000 live births. To ensure an accurate CDH diagnosis and the best outcome, it is important to visit a fetal therapy center experienced in evaluating and caring for pregnancies affected by CDH.

The CFDT has cared for more babies with CDH than any other team in the U.S. The center's large multidisciplinary team, including maternal-fetal medicine specialists, neonatologists, advanced practice nurses, fetal therapy nurse coordinators, genetic counselors, fetal imaging specialists, labor and delivery nurses, anesthesiologists, and fetal cardiologists, has a level of ongoing collaboration that is unmatched.

The center’s fetal imaging specialists use advanced prenatal imaging techniques to make a precise diagnosis. In most cases, the pregnancy is followed closely by the center’s obstetrical team until planned delivery. For select cases of severe CDH, the center offers fetoscopic endoluminal tracheal occlusion (FETO), a fetal surgery procedure that may improve outcomes by allowing the lungs to grow more before birth.

Babies are born in the Garbose Family Special Delivery Unit, the world’s first birth facility in a freestanding pediatric hospital designed for healthy mothers carrying babies with known birth defects. After delivery, the CDH baby is immediately stabilized by a specialized Newborn/Infant Stabilization Team and Neonatal Surgical Team — the only team of its kind in the world.

The baby is taken to CHOP’s Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU), ranked among the best in the nation. Once the baby is strong enough, congenital diaphragmatic hernia repair of the hole in the diaphragm occurs at the N/IICU bedside, as it’s safer for the baby not to be transported to another location.

Babies born with CDH may require extracorporeal membrane oxygenation (ECMO), a heart/lung bypass machine. CHOP’s ECMO Center has been designated a Center of Excellence by the Extracorporeal Life Support Organization since 2008. The center has had more than 1,500 ECMO cases since opening in 1990, nearly 250 being babies with CDH.

CHOP’s unique Pulmonary Hypoplasia Program (PHP) provides comprehensive, interdisciplinary follow-up care for nearly 600 children with CDH well into school age. The team is made up of specialists from general surgery, developmental pediatrics, pulmonology, cardiology, psychology, nutrition, audiology, social services and others as needed.

The center offers support services to help families overcome challenges in receiving care, cope with their baby's diagnosis and treatment, and access resources closer to home. A dedicated psychosocial services team, including a clinical psychologist and social workers, provides emotional support, individualized and couple's counseling, and supportive therapy.