Advances in prenatal and postnatal diagnosis, perioperative management, and
postoperative care have dramatically increased the number of scientific reports on
congenital thoracic malformations (CTM). Nearly all CTM are detected prior to birth,
generally by antenatal ultrasound. After delivery, most infants do well and remain
asymptomatic for a long time. However, complications may occur beyond infancy,
including in adolescence and adulthood. Prenatal diagnosis is sometimes missed and
detection may occur later, either by chance or because of unexplained recurrent or
persistent respiratory symptoms or signs, with difficult implications for family counseling
and substantial delay in surgical planning. Although landmark studies have been
published, postnatal management of asymptomatic children is still controversial and
needs a resolution. Our aim is to provide a focused overview on a number of unresolved
issues arising from the lack of an evidence-based consensus on the management of
patients with CTM. We summarized findings from current literature, with a particular
emphasis on the vigorous controversies on the type and timing of diagnostic procedures,
treatments and the still obscure relationship between CTM and malignancies, a matter
of great concern for both families and physicians. We also present an algorithm for the
assessment and follow-up of CTM detected either in the antenatal or postnatal period.
A standardized approach across Europe, based on a multidisciplinary team, is urgently
needed for achieving an evidence-based management protocol for CTM.