Lung Pathology in Patients with Congenital Diaph

Authors: Amy E Heerema Joseph T Rabban Roman M Sydorak Micheal R Harrison Kirk D Jones

Publish Date: 2003/11/05

Volume: 6, Issue: 6, Pages: 536-546

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Abstract

Fetal intervention for congenital diaphragmatic hernia was developed to lessen the high morbidity and mortality of pulmonary hypoplasia Lung pathology and morphometry in patients treated with fetal intervention have not been described We report clinical and autopsy findings as well as basic lung morphometry in 16 cases of congenital diaphragmatic hernia with fetal intervention 12 cases tracheal occlusion 4 cases hernia repair and 19 cases of congenital diaphragmatic hernia without fetal intervention All patients who underwent fetal intervention were born premature Lung enlargement with increased lungtobody weight ratio was observed with fetal tracheal occlusion accompanied by lower than normal radial alveolar counts and increased alveolar size Patients treated with tracheal occlusion also had early alveolar development at 298 306 and 309 wk postconceptual age as well as mucous fluid pooling in airways and alveoli All cases showed severe alveolar septal widening more extensive in patients without fetal intervention When grouped by postconceptual age no statistically significant difference was found between patients with and without fetal intervention with respect to lungtobody weight ratio radial alveolar count mean alveolar length and relative arteriolar media thickness Lung enlargement has been observed with fetal tracheal occlusion sonographically our studies suggest that this is due in part to emphysema and mucous fluid pooling The lung remains abnormal with low radial alveolar counts and increased alveolar size Tracheal occlusion did not prevent development of lung pathology associated with pulmonary hypoplasia

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