FLASHPATHH A Z E M A L I

PULMONARY INTERSTITIA

L EMPHYSEMA

H A Z E M A L I

CLINICAL• Rare condition commonly occur in preterm infants who

receives high pressure mechanical ventilation as a treatment for respiratory distress

• Characterized by air leakage into interstitial tissues– Due to alveolar rupture from barotrauma

• Causing persistant respiratory distress manifestations

• Air leakage can also occurs in– Pleura “pneumothorax”– Mediastinum “pneumomediastinum”– Peritoneum “pneumoperitoneum”

CLINICAL• Other mechanical ventilation – related problems:

– Bronchopulmonary dysplasia– Retinopathy of prematurity

• Incidence of all these diseases decreased with the use of synthetic surfactant and modern ventilation techniques

• Interstitial pulmonary emphysema have two clinical types:– Acute– Chronic “persistent”

GROSS• Pleura:

– Lines of bubbles are seen through the pleura• (outlining the lung lobules)

– Pleural blebs may be seen– Pneumothorax

• air bubbles when open the pleura under water

• Lung:– Multiple small cysts within parenchyma– If pneumothorax occurred

• lung collapse and mediastinal shift

MICROSCOPY• Pattern:

– Multiple cystic spaces– Have NO lining– Expand (widen) surrounding connective tissue

• Distribution:– Sub-pleural– Interlobular septa– Peri-bronchovascular

• Adjacent parenchyma may show:– Hyaline membrane disease– Bronchopulmonary dysplasia

MICROSCOPYAcute Chronic

“persistent” Giant cell reaction Absent PresentFibrosis Absent Present

DIFFERENTIAL DIAGNOSIS“ O t h e r c o n g e n i t a l / c y s t i c l u n g d i s e a s e s ”• Congenital:

– Pulmonary sequestration– Congenital pulmonary cysts– Congenital pulmonary airway malformation– Congenital lobar emphysema

• Acquired:– Emphysema– Healed abscess– Honeycombing

• Mixed:– Cystic fibrosis

DIFFERENTIAL DIAGNOSISPulmonary lymphangiectasis• Expansion of sub-pleural, Interlobular septa, and peri-bronchovascular

connective tissues by multiple cystic spaces that– Contain lymph– Have endothelial lining “ D2-40 positive “

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