**4.4. Prenatal management**

In the case of unilateral hypoplasia, no karyotyping is required, but the birth is recommended to occur in a tertiary center because of the risk of orotracheal intubation immediately after the delivery. Bilateral lung agenesis is incompatible with life. In the case of the primary bilateral pulmonary hypoplasia or associated with skeletal dysplasia, the importance of a correct diagnosis is not only for the current pregnancy that will evolve to the exitus of the fetus but also for a future pregnancy because skeletal dysplasia may not occur sporadically but exhibit recessive inheritance. In unilateral pulmonary hypoplasia associated with the scimitar syndrome, neonatal ventilation may be required. Fetal MRI may be useful in distinguishing between the pulmonary agenesis and CCAM [23, 24]. At the same time, the exclusion of associated fetal abnormalities can be done by MRI, in particular: ipsilateral upper extremities, mandible, face, or kidneys. There is no fetal intervention available in pulmonary agenesis.
