Localized persistent pulmonary interstitial emphysema

Abstract

A 3-week-old premature female infant was referred to our institution with progressively worsening respiratory distress. She was born at 29 weeks’ gestational age with radiologic evidence of mild respiratory distress syndrome. She required only minimal supplemental oxygen until her third week of life, when she developed worsening respiratory distress requiring mechanical ventilation. A new chest radiograph (Fig. 1) demonstrated hyperinflation of the left hemithorax with coarse reticular markings throughout the left lung. Computed tomography (CT; Fig. 2) revealed innumerable cystic spaces of varying sizes occupying the entirety of the left upper lobe, with no appreciable normal lung parenchyma interposed. She failed to wean from mechanical ventilation and underwent an urgent left thoracotomy. The entire upper lobe was grossly involved with cystic lung pathology (Fig. 3). This was treated by an uncomplicated left upper lobectomy, and the patient was weaned from mechanical ventilation over the next 3 days. The histopathology (Fig. 4) demonstrated cyst-like cavities lined by an irregular layer of cells within the lung parenchyma. These cells were mostly mononuclear admixed with foreign body–type multinucleated giant cells. The cysts were interspersed with essentially normal-appearing pulmonary tissue. Occasional air spaces were questionably dilated, particularly at the periphery of the lung beneath the pleural surface. However, widespread emphysematous changes were not apparent; neither was there any significant intraalveolar or interstitial inflammatory cell infiltrate. These findings were diagnosed as localized persistent pulmonary interstitial em