Pathological classification is academically important but is unnecessarily complex for clinical and research use. Our simple radiological-based system allows unambiguous comparison between the results of different studies and also guides the choice of necessary investigations specific to each group.
WMS is a rare but clearly identifiable syndrome with significant morbidity, predominantly affecting infants below 1,500 g birth weight. The earliest pathology appears to be alveolar air leak. Inflammatory activation induced by cystic interstitial air may cause the subsequent progressive respiratory disease. Management is supportive but should include investigation for pulmonary hypertension.
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