Based on a 20-week prenatal ultrasound, the expectant parents were referred to CHOP’s Center for Fetal Diagnosis and Treatment (CFDT) for further evaluation for congenital diaphragmatic hernia (CDH). Findings based on ultrasound and magnetic resonance imaging placed this baby in the severe spectrum of disease based on the findings of herniation of the fetal liver into the chest and a lung capacity of 34% expected.
P.V. was born at full term in CHOP’s Garbose Family Special Delivery Unit (SDU). P.V. was intubated immediately and transferred to the Neonatal Surgery (Neosurgery) team in the Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU). She was sedated and placed on mechanical ventilation using a small lung volume/rapid rate strategy allowing for permissive hypercapnia. She was started on inhaled nitric oxide for severe pulmonary hypertension. However, due to impaired ventilation and oxygenation she was placed on extracorporeal membrane oxygenation (ECMO) at bedside in the N/IICU within hours of birth. Venoarterial ECMO is used to bypass the significant pulmonary hypertension seen in CDH.
P.V. was decannulated on DOL 12. P.V. underwent repair of her diaphragmatic defect with a silastic patch at bedside on DOL 22. She weaned from mechanical ventilation by DOL 31. Feeds were slowly advanced with a postpyloric feeding tube. P.V. was unable to transition to gastric feeds due to persistent vomiting and gastroesophageal reflux. After failure of medical management, she underwent a fundoplication and gastrostomy tube placement. At the time of discharge (age 3½ months) she was taking fortified breastmilk by bolus during the day and overnight continuous feeds. She was also on sildenafil for pulmonary hypertension.
Since going home, P.V. has thrived. At 18 months her G-tube was removed. She makes regular visits to CHOP’s Pulmonary Hypoplasia Program (PHP). She is followed closely by Pulmonary and cardiologists specializing in pulmonary hypertension.
Care of babies with CDH, giant omphalocele, and congenital lung lesions at risk for pulmonary hypoplasia starts in the CFDT.
Special Delivery Unit
Delivery occurs in the SDU, the world’s first birth facility in a pediatric hospital specifically designed for healthy mothers carrying babies with known birth defects. These babies have immediate access to specialists, surgical procedures, and ECMO without undergoing postnatal transportation. Further, these babies are not separated from their mothers after birth. The SDU opened its doors in 2008 and now sees approximately 500 deliveries per year of babies with congenital anomalies.
Diagnosis-specific optimal care resuscitation guidelines beyond Neonatal Resuscitation Program (NRP) ensure safe and consistent perinatal transitions. From the moment of birth, babies are cared for by a special team of neonatologists, surgical nurse practitioners, and the pediatric surgeon who knows them well.
Newborn/Infant Intensive Care Unit
The Neosurgery team has grown from its inception in 2004 to a current census involving on average one-third of the total unit census, for an average of more than 33 patients per day under the care of the Neosurgery team. This model of a close working collaboration between Surgery and Neonatology has been a success.
Further, the unique construct of the multidisciplinary team, involving Neonatology, Surgery, Anesthesiology, Nursing, Nutrition, Respiratory Therapy, ECMO, Neonatal Pharmacy, Social Work, Case Management, and Child Life supports all the needs of the baby. Not only is there a focus on family-centered care, but each bed space is also capable of transforming into an operating room for the most critically ill neonates. Specialized personnel and equipment for surgery and ECMO all come to the baby.
This avoids the stress of travel to and from the operating room and reduces the need for transitions in care and handoffs. With a large concentrated volume of neonatal surgical patients, the team has also been able to focus on the perioperative experience and has refined care of the surgical neonate by working closely with our pediatric anesthesia colleagues.
Babies with CDH have pulmonary hypoplasia and pulmonary hypertension and are typically admitted to the N/IICU for weeks to months. Use of conventional mechanical ventilation and high-frequency oscillatory ventilation allowing permissive hypercapnia, aggressive treatment of pulmonary hypertension, and early use of ECMO as indicated has increased our survival to > 85%.
Continuing care after discharge
Ongoing developmental and medical assessments from a multidisciplinary team occur from N/IICU discharge through adolescence in the PHP clinic. Multidisciplinary evaluations take place in one day with cohesive plans and recommendations coming from the team as a whole to avoid fragmented care.
PHP specialists work together, partnering with primary care physicians, to provide the most comprehensive care for our patients. Many patients require supplemental tube feedings, therapies, and services that need coordination to optimize outcomes.
The PHP program continues to grow, providing care for the children who benefit from this specialized follow-up care.
Pulmonary Hypoplasia Program Enrollment
Total patients enrolled in PHP Research Study as of March 13, 2019: 817
Pulmonary Hypoplasia Program - Total Patients by Diagnosis
Total diagnoses: 856
Congenital Diaphragmatic Hernia (527 cases)
As of March 13, 2019
*Children with multiple diagnoses are counted in multiple categories.
For more information, visit the Pulmonary Hypoplasia Program website or call 215-590-2733.