Abstract
Congenital pulmonary airway malformation (CPAM) is a rare congenital lung lesion that
usually remains asymptomatic during the fetal and neonatal period. However, it can
occasionally cause prenatal cardiocirculatory failure and fetal hydrops, requiring
a thoraco-amniotic shunt (TAS) placement. In other cases, it can also cause symptoms
at birth (such as respiratory distress) and may require urgent surgical intervention.
Thoracoscopic lobectomy for neonates is rarely reported. Here, we report a case of
right macrocystic CPAM causing fetal hydrops at 27 weeks of gestation. The fetus was
treated with a TAS placement that successfully resolved the hydrops. At 39 weeks of
gestation, a male neonate was born (weight 2,850 g). The TAS spontaneously displaced
during delivery, causing an open pneumothorax (PNX), initially treated with a drainage.
His condition gradually worsened, requiring ventilatory support. Computed tomography
(CT) scan showed different giant cysts in the context of the right lower lobe, left
mediastinal shift, and compression of the rest of the lung. An urgent surgical management
was required. A thoracoscopic right lower lobectomy was performed at 10 days of life
(weight 2,840 g). The postoperative course was uneventful; the child remained totally
asymptomatic and showed a good recovery. To the best of our knowledge, this is the
first reported case of open iatrogenic PNX following TAS positioning and the second
of neonatal thoracoscopic lobectomy in a newborn weighting less than 3 kg. The purpose
of this report is to indicate that minimally invasive surgery is feasible, safe, and
effective for the resection of CPAM, even in small newborns.
Keywords
congenital pulmonary airway malformation - thoraco-amniotic shunt - thoracoscopic
lobectomy - neonate - case report
