Prenatal Catheter Placement for Fetal Cystic Adenomatoid Pulmonary Malformation: A Case Report

Abstract

Introduction: Fetal congenital cystic adenomatoid malformation is a pulmonary developmental anomaly arising from an overgrowth of the terminal respiratory bronchioles. This is such a rare malformation that it is not always thought of as a diagnostic possibility. Case: In the present case, after a large pulmonary cyst started deviating the mediastinum and following an increase in amniotic fluid volume in the fetus at 28 weeks of gestation, the placement of a catheter for continuous drainage was performed. Subsequent follow-up with ultrasounds and serial echocardiograms revealed normalization of the position of mediastinum, normal amniotic fluid levels, and the correct position of the catheter. The size of the cyst was decreasing daily, but 8 weeks after fetal invasive procedure, there was concern about the stabilization of its size, with the suspicion of obstruction of the catheter. Cesarean delivery was performed at 38 weeks’ gestation. The size of the cyst on the day of C-section was 3.0 × 2.9 × 2.1 cm. At delivery, the infant weighed 3,030 g, with no evidence of respiratory distress, revealing the correct location of catheter. At day 4 after delivery a superior right pulmonary lobe resection was performed. Conclusion: Large pulmonary cysts may produce deviation of mediastinum and can lead to cardiac tamponade, nonimmune hydrops and pulmonary hypoplasia. Precise prenatal imaging and different fetal therapeutic strategies may allow survival of affected fetuses.

Prenatal diagnosis and outcome of echogenic fetal lung lesions

“…The literature review identified another 18 fetuses with a large cyst causing major mediastinal shift that were treated by placement of a thoracoamniotic shunt (Table 5)1–3, 6, 74, 85–90. In five of these fetuses, thoracentesis was first performed, with subsequent rapid reaccumulation of fluid within the cyst.…”

Congenital cystic lung disease: contemporary antenatal and postnatal management

“…The infant underwent uneventful postnatal resection of the CPAM. To date, at least 28 CPAMs have been managed by thoracoamniotic shunting, with about a 70% survival [19,21,22,50,56,57,[59][60][61]. It is, however, important to note that despite successful prenatal cyst decompression, these infants often have significant pulmonary hypoplasia and mass effect at birth.…”

Perinatal outcomes of prenatal diagnosis of congenital pulmonary airway malformation: an experience

“…If there are any dominant cysts, even with a CVR <1.6, there is a risk of acute cyst growth and development of fetal hydrops. A thoracoamniotic shunt may be considered if signs of fetal decompensation at <32 weeks’ gestation are identified 15 . Microcystic lesions increase the risk of fetal hydrops, pulmonary hypoplasia, and polyhydramnios 16 .…”