Pulmonary stabilisation followed by delayed surgery results in favourable outcome in congenital cystic lung lesions with pulmonary hypertension.

Objective: Congenital cystic lung lesions associated with fetal hydrops and polyhydramnios are rare, and reported to have greater than 50% mortality, can this be reversed? To propose a period of pulmonary stabilisation and delayed surgery for neonates with congenital cystic lung lesions and pulmonary hypertension.

Results: Four neonates with antenatal diagnosed congenital cystic lung lesions with associated fetal hydrops and maternal polyhydramnios, presented with pulmonary hypertension due to lung hypoplasia. Contrast spiral computerised tomography scan was diagnostic. Three had congenital cystic adenomatoid malformation and one extra-lobar pulmonary sequestration with anomalous blood supply from the abdominal aorta. All four were pre-operatively ventilated for 9.8+/-0.9 days on conventional mechanical ventilators. Definitive surgery was performed at 10.8+/-0.8 days following stabilisation of lung function (preductal PO(2) of greater than 60torr with a SaO(2) of 90-100%) and resolution of pulmonary hypertension with absence of persistent fetal circulation on echocardiography. Optimal timing of delayed surgery was in the range of 216-360h. All four are thriving with absent respiratory complications.

Conclusions: Delayed surgery following pulmonary stabilisation results in favourable outcome.