Infants with congenital diaphragmatic hernia often require intensive treatment after birth, have prolonged hospitalizations, and have other congenital anomalies. After discharge from the hospital, they may have long-term sequelae such as respiratory insufficiency, gastroesophageal reflux, poor growth, neurodevelopmental delay, behavior problems, hearing loss, hernia recurrence, and orthopedic deformities. Structured follow-up for these patients facilitates early recognition and treatment of these complications. In this report, follow-up of infants with congenital diaphragmatic hernia is outlined.
INTRODUCTIONSurvival rates for patients with congenital diaphragmatic hernia (CDH) have increased during the past decade with the implementation of more "gentle" ventilation and physiology-specific strategies, high-frequency ventilation, extracorporeal membrane oxygenation (ECMO), and improved supportive care. 1-3 Improvement in survival rates has occurred for infants with CDH complicated by severe pulmonary hypoplasia, pulmonary hypertension, and chronic lung disease. 4 However, other significant morbidities, such as neurocognitive delay, gastroesophageal reflux, hearing loss, chest wall deformity, poor growth, hernia recurrence, and complications attributable to associated congenital anomalies, continue to affect the lives of many infants with CDH beyond the neonatal period. 1,5,6 Coordination of the complex medical and surgical needs of these infants is challenging. Comprehensive multispecialty clinics that aggregate specialty physicians and services are family-friendly and provide for collaborative evaluation and management planning. Same-site multidisciplinary service teams also improve coordination, communication, and support for the medical home pediatrician who is responsible for managing the general health care needs of the infant. Unfortunately, such multispecialty clinics are not available to all infants with CDH. The following information is intended to provide clinicians who care for infants with CDH with a template to organize a comprehensive plan for detection and management of associated morbidities.
PULMONARY MORBIDITYSurvivors with CDH may require treatment beyond the initial hospitalization for chronic lung disease, bronchospasm, pulmonary hypertension, aspiration, pneumonia, and pulmonary hypoplasia. Oxygen treatment beyond the initial hospitalization may be needed for many of these infants, especially those who are treated with ECMO and a prosthetic patch. 7-9 Many survivors not treated with ECMO also receive bronchodilators and inhaled steroids. 8 At least 4% of survivors require a long-term tracheostomy. 9,10 Nearly one fourth of infants with CDH who survive have obstructive airway disease at 5 years of age, 10,11 and some have pulmonary hypertension that persists for months or years. Pulmonary hypertension that persists for more than the first few weeks after birth is a risk factor for early death. 12 Persistent abnormalities in lung function also have been demonstrated on ventilation/per...