1 Neonatal Unit and Radiology Department, Soroka Medical Center, Beer-Sheva, Israel
An atypical pulmonary aspiration syndrome occurred in two prematurely born siblings with congenital ichthyosis; pneumothorax and pneumomediastinum were seen in a third neonate with less severe ichthyosis. These pulmonary manifestations were probably complications of aspiration of amniotic fluid containing abnormally high concentrations of epithelial squames. The severity of the lung disease appeared to be correlated with the severity of the ichthyosis. Amniotic fluid enters the fetal respiratory tract either chronically, during physiological fetal respiratory movements or, acutely, by way of gasping initiated during fetal distress.