CCCAM, sequestration, hydrothorax: fetal intervention

Congenital cystic adenomatoid malformation (CCAM) is diagnosed prenatally when an ultrasound shows a cystic or solid lung tumor. Type I and II CCAM appear as cystic, fluid-filled masses while Type III appears as a solid mass.
Several researchers have demonstrated a survival rate of approximately 50% for hydropic fetuses with microcystic CCAM after surgery. Hydropic fetuses treated with steroids, however, have survival rates near 85%. If hydrops persists or emerges past 32 weeks, EXIT and neonatal resection remain options.
Macrocystic lesions that cause hydrops can be treated with catheter-based drainage techniques of the dominant cyst. Simple aspiration of the cyst is usually a temporizing measure but can slow down disease progression and help determine if a thoracoamniotic shunting will be effective. In lesions without a significant solid component, placement of a thoracoamniotic shunt can effectively decrease the CVR and reverse hydrops.
Broncopulmonary sequestration (BPS) is a rare congenital malformation of the lower respiratory tract, consisting of a non-functioning mass of lung tissue lacking normal communication with the tracheobronchial tree. Its appearance on fetal ultrasound mimics a CCAM of the microcystic type. The diagnosis, however, can be made by identifying a separate systemic artery from aorta feeding the sequestration.
For a large BPS, the presence of the systemic artery, however, opens a less invasive treatment option: minimally invasive coagulation of the blood supply. This may result in shrinking of the lesion and recovery of the fetus.
The incidence of fetal hydrothorax is estimated to be 1 in 15,000 pregnancies. Isolated hydrothorax is most often caused by congenital chylothorax, a primary lymphatic abnormality. Accumulation of fluid in the pleural space may lead to pulmonary hypoplasia, compression of the heart and obstruction of venous return with subsequent development of hydrops and compression of the esophagus leading to polyhydramnios. Untreated, the reported perinatal mortality is 22–53%. By far the most described procedure for treatment of fetal hydrothorax is placement of a thoraco-amniotic shunt. The vast majority of fetal shunt procedures have been done using a silicone double pigtail shunt as described by Rodeck, which is inserted under ultrasound guidance. In bilateral hydrothorax, shunts are usually placed on both sides. The most common complications of thoraco-amniotic shunting are either failure or the need for reintervention (ranging from 6 to 33% in various series), PROM (15% in the largest recent series), and direct fetal loss (5–10%).
Overall survival rate was 63%, ranging from 54% for single thoracocentesis to 80% in the 5 cases treated with pleurodesis. Survival rate was ranging from 61 to 67% for shunt-placement with or without prior thoracocentesis.
In conclusion, the fetus with a lung mass but without hydrops has an excellent chance for survival with maternal transport, planned delivery, neonatal evaluation and fetal surgery.

Anahtar Kelimeler