Types II and III congenital pulmonary airway malformation with hydrops treated in utero with percutaneous sclerotherapy.

Abstract

To present outcomes of fetuses with congenital pulmonary airway malformation (CPAM) treated with sclerotherapy. Retrospective study of 8 patients with a prenatal diagnosis of CPAM type II or III with secondary hydrops treated with percutaneous sclerotherapy using 5% ethanolamine oleate (EO). All patients underwent ultrasonic measurement of the CPAM volume ratio. Results are expressed as median (range). Gestational age at initial sclerotherapy was 22.0weeks (19.6-31.4); 3 patients underwent 2 procedures. Intrauterine fetal demise (IUFD) occurred in 4 cases; 2 died on postoperative day #1 (one from inadvertent intravascular EO injection); 2 died >6weeks after the procedure. Preoperative CPAM volume ratio was 3.6 (1.6-7.8) in survivors and 2.7 (1.7-4.7) in those with IUFD. The volume of EO at the initial sclerotherapy procedure was 3mL (2-5) in survivors and 7mL (6-10) in IUFD cases. The gestational age at delivery of the 4 survivors was 38.4weeks (37.4-39.3); all underwent postnatal resection. The efficacy of percutaneous sclerotherapy for CPAM types II and III remains in question. Further studies are needed to determine the optimal dose of sclerotherapy agent and the safety and efficacy of this procedure.