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Virtual Pediatric Hospital: Thoracopaedia: An Imaging Encyclopedia of Pediatric Thoracic Disease: Case 7 Thoracopaedia: An Imaging Encyclopedia of Pediatric Thoracic Disease

Case 7

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Chief Complaint:
Nine month old female with chronic lung disease.

Clinical History:
The patient was a 9 month old former 32 week premature female with a history of bronchopulmonary dysplasia and chronic lung disease that required home oxygenation therapy. The patient also has a history of pulmonary artery hypertension, patent foramen ovale and patent ductus arteriosus. Because of the patient's continued profound respiratory distress there was clinical concern of another underlying lung process and a chest CT exam was obtained to rule out congenital lymphangiectasia.

Clinical Physical Exam:
The patient was in chronic respiratory distress.

Clinical Labs:

Clinical Differential Diagnosis:
Chronic respiratory distress due to bronchopulmonary dysplasia or congenital lymphangiectasia.

Imaging Findings:
A chest film and chest CT exam performed on the same day better defined the nature of the patient's lung disease.

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Imaging Differential Diagnosis:
The findings were considered to be compatible with, but not diagnostic of, lymphangiectasia.

Operative Findings:
The patient underwent a left posterolateral thoracotomy and an open lung biopsy of the lingula.

Pathological Findings:
Focal septal and interstitial thickening with fibrosis, emphysema and cuboidal metaplasia of alveolar lining cells was seen, consistent with mild bronchopulmonary dysplasia. Moderate thickening of the peripheral pulmonary arterioles, consistent with moderate pulmonary artery hypertension, was also seen. Focal intra-alveolar and bronchial fibrin, RBC and iron laden macrophages with erythrophagocytosis were also noted. There was no evidence of alveolar proteinosis or lymphangiectasia.

Final Diagnosis:
Bronchopulmonary dysplasia

Follow-up and Prognosis:
The patient had an uncomplicated post-operative course.

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