BACKGROUND:

The bronchopulmonary sequestration (BPS) is usually diagnosed prenatally as an intrathoracic hyperechoic, homogeneous, solid mass usually located in the lower pulmonary lobe. The pathognomonic sign is the demonstration of its systemic feeding artery into the lung mass by using color Doppler ultrasound. Natural history data reports that a significant proportion of BPS cases usually regress in the intrauterine period. However, a proportion of cases showed a rapid and progressive growth with development of massive pleural effusion and hydrops. Such cases present the poorest prognosis and high perinatal mortality and therefore, fetal intervention should be considered to improve prognosis. Different fetal surgery procedures have been attempted for such purposes. However, the optimal fetal therapeutica strategy remains controversial.

OBJECTIVE:

In the present manuscript, we describe and discuss the perinatal outcome of the different published fetal interventions in fetuses complicated with BPS.

METHODOLOGY:

We search in the English and Spanish literature (Medline and PubMed) for cases complicated with BPS and treated with any fetal surgical intervention including the key words “broncopulmonary sequestration” and “fetal surgery” from 1990 to 2019.

RESULTS:

A total of 150 references were reviewed including only 26 studies describing pregnancies with prenatal diagnosis of bronchopulmonary sequestration that were treated with any fetal surgical intervention. Several fetal surgery procedures have been attempted for the management of complicated fetuses with severe BPS. These include open fetal surgery, placement of thoracoamniotic shunts, and occlusion of the feeding blood vessel by ultrasound-guided intrafetal laser coagulation, radiofrequency ablation, coil embolization, sclerotherapy with intravascular alcohol injection and fetal bronchoscopy.

CONCLUSIONS:

Laser coagulation of the feeding artery appears to be the best intervention, avoiding fetal death, neonatal pulmonary morbidity and the need for postnatal surgery in virtually all cases postulating itself as the only potential curative management.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )