Clinical phenotypes and management concepts for severe, established bronchopulmonary dysplasia

Abstract With advances in care, the bronchopulmonary dysplasia phenotypes have evolved, so that infants who would have died in previous eras are now surviving with significant pulmonary and neurologic morbidities. The spectrum of bronchopulmonary dysplasia phenotypes is broad, however, ranging from very mild to very severe disease, and management strategies used in previous eras of care may not be appropriate for the most severe phenotypes. The pathophysiology depends largely on the gestational age at birth, but disease progression and long-term outcome depends on the net sum of antenatal, perinatal and postnatal exposures. There is no single management strategy for the wide spectrum of clinical presentations of BPD; care must be individualized. Regardless of the phenotype, the support apparatus should match the disease physiology. Here we describe an interdisciplinary approach to management in terms of achieving clinical stability and progress along a continuum, from diagnosis at 36 weeks corrected gestational age to convalescence. The clinical trajectory depends on the balance of factors related to support of respiration, healing of the lungs, and return of organ growth and development. The overall treatment strategy should optimize positive influences that lead to a pro-growth state, while minimizing exposures that interfere with lung growth and development. This is best achieved by use of a multi-disciplinary team, with feedback loops that inform clinical decision-making regarding respiratory stability, tolerance for cares and activities, the clinical response to changes in the care plan, and progress in growth and development.