Factors Associated with Neurodevelopmental Impairment in Bronchopulmonary Dysplasia.

OBJECTIVE: To identify factors associated with neurodevelopmental impairment (NDI) in patients with bronchopulmonary dysplasia (BPD). STUDY DESIGN: We identified 151 patients with moderate to severe BPD from 2010 to 2014 with complete Bayley Scales of Infant Development (BSID) scores at 24 months corrected age. We defined NDI as any diagnosis of cerebral palsy or ≥1 BSID composite scores of <80. RESULTS: The mean corrected age at BSID was 23 ± 1 months; 18% had a cognitive score of <80, 37% had a communication score of <80, and 26% had a motor score of <80. Cerebral palsy was diagnosed in 22 patients (15%); 84 (56%) patients did not have NDI. Patients with NDI had lower birth weight, but there was no difference in gestational age at birth, severe intraventricular hemorrhage (IVH), necrotizing enterocolitis, or patent ductus arteriosus ligation compared with patients with no NDI. Ventilator days were greater in patients with NDI than in patients without NDI. More patients with NDI received furosemide and systemic corticosteroids and the hospital length of stay was longer than in patients with no NDI. Logistic regression modeling demonstrated that for every additional 100 g of birth weight the odds of NDI decreased by 35% and for every additional hospital day the odds of NDI increased by 1.3%. CONCLUSIONS: In our cohort of patients with moderate to severe BPD, the majority had no NDI, and low birth weight and length of hospital stay were associated with increased risk of developing NDI. This finding suggests that there are potentially modifiable factors associated with better neurodevelopmental outcomes in patients with BPD that deserve further study.

Clinical phenotypes and management concepts for severe, established bronchopulmonary dysplasia.

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 of 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.

Pulmonary Vein Stenosis in Neonates with Severe Bronchopulmonary Dysplasia.

Objectives Pulmonary vein stenosis (PVS) is a rare, often lethal anomaly associated with poor outcomes. Given the association between bronchopulmonary dysplasia (BPD) and cardiovascular complications, we tested the hypotheses that (1) a subgroup of neonates with severe BPD develop PVS (BPD-PVS) and have worse outcomes than do neonates with severe BPD alone (BPD); (2) among a cohort of neonates with severe BPD-associated pulmonary hypertension (BPD-PH), PVS is an additional risk factor for adverse outcomes and mortality. Study Design We performed a retrospective review of neonates with severe BPD, based on the Eunice Kennedy Shriver National Institute of Child Health and Development (NICHD) criteria, at our institution between June 1, 2009, and June 30, 2013. PVS was determined based on serial review of echocardiograms performed during their hospitalization. Neonates with congenital heart disease or chromosomal anomalies were excluded. Results Of 213 patients with severe BPD, 10 (4.7%) were found to have PVS (BPD-PVS). Neonates with BPD-PVS had lower birth weight (634 ± 178 vs. 767 ± 165 g; p < 0.01) and were more likely to be intrauterine growth restricted (80 vs. 11%; p < 0.01) than neonates with BPD alone. Time on mechanical ventilation and length of hospitalization were longer in the BPD-PVS group than BPD group. Survival was lower in the BPD-PVS group than BPD group (5/10 [50%] vs. 196/203 [97%]; log-rank test p < 0.01). Among a subgroup of neonates with BPD-PH, survival was lower among infants with PVS than those without PVS (5/9 [56%] vs. 26/30 [86%]; log-rank test p = 0.01). Conclusions Compared with neonates with severe BPD alone, those with acquired PVS are at increased risk for worse outcomes, including higher mortality. Evidence-based recommendations regarding screening protocols and surveillance are needed in this high-risk subgroup of BPD neonates.