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Updated: Dec 31, 2025
Development of Obliterative Bronchiolitis in a Murine Model of Orthotopic Lung Transplantation
Published on: July 10, 2012
Lung Transplantation for Bronchopulmonary Dysplasia in Adults: A Clinical and Pathologic Study of 3 Cases
Natalia Liu1, Oscar W Cummings1, Amir Lagstein2
1Departments of Pathology and Laboratory Medicine.
Insights
Long-term outcomes for bronchopulmonary dysplasia (BPD) survivors are poorly understood. Adult BPD lungs show airway fibrosis, elastosis, and pulmonary hypertension, explaining clinical findings.
Area of Science:
- Pulmonary Medicine
- Pathology
- Neonatology
Background:
- Bronchopulmonary dysplasia (BPD) affects premature infants requiring mechanical ventilation and oxygen.
- Adult BPD survivors frequently experience respiratory rehospitalizations, yet long-term outcomes are understudied.
- Limited data exists on adult BPD pulmonary function, radiographic findings, and pathology.
Observation:
- Three adult BPD lung transplant recipients were analyzed.
- Clinical data included presentation, imaging, pulmonary function tests, and cardiac catheterization.
- Explant lung pathology was examined using H&E, elastic stains, and CD31 immunohistochemistry.
Findings:
- Consistent clinical and radiologic features observed: prematurity history, long-term ventilation, hyperexpanded lungs with air trapping, mosaic attenuation, severe obstructive changes, and pulmonary hypertension.
- Pathologic findings revealed enlarged, simplified alveoli, fibrosis (peribronchial, subpleural, septal), small airway narrowing/obliteration (elastosis, muscular hypertrophy), venous wall thickening, and bronchitis/bronchiolitis.
- Cholesterol granulomas were present in two cases.
Implications:
- The shared pathologic features correlate with observed clinical and radiologic findings in adult BPD.
- Further research is needed to fully characterize adult BPD's clinical and pathologic spectrum.
- Improved understanding will aid in developing optimal management strategies for adult BPD patients.
Abstract:
Bronchopulmonary dysplasia (BPD) is usually seen in premature infants who require mechanical ventilation and oxygen therapy for acute respiratory distress. Although most patients wean from oxygen therapy by the ages of 2 to 3, rehospitalization for respiratory problems is common in these patients in adulthood. There have been few studies that document the long-term outcomes of BPD survivors and information about the pulmonary function and radiographic findings of adult BPD are limited. Data on pathologic features of adult BPD are scarce. Three adult patients who underwent recent lung transplantation for BPD from 2 institutions were identified. Clinical data including clinical presentation, chest radiographic images, pulmonary function tests, cardiac catheterization, and echocardiography were retrieved from the electronic medical records. Hematoxylin and eosin and selective elastic stained sections of the explant lungs were examined. CD31 immunohistochemical stain is performed on representative sections. All 3 cases had similar clinical and radiologic features including the history of prematurity and long-term mechanical ventilation after birth, hyperexpanded lungs with air trapping and mosaic attenuation on chest computed tomographic scan, severe obstructive changes on pulmonary function test, and pulmonary hypertension. Pathologic examination showed common features including enlarged and simplified alveoli, peribronchial, subpleural, and interlobular septal fibrosis, narrowing/obliteration of the small airways by elastosis and muscular hypertrophy, thickening of venous walls by fibromuscular hyperplasia, and bronchitis/bronchiolitis. Cholesterol granulomas were seen in 2 cases. The common pathologic findings in the lungs explain the clinical and radiologic findings. Future studies are warranted to further characterize the clinical and pathologic features of adult BPD to develop optimal management strategies for these patients.
