A case of pentalogy of Cantrell pathology in only one fetus of a twin pregnancy is reported; no congenital malformation was found in the second fetus. This fact would endorse epigenetic theories on its origin. Some thoughts on prenatal diagnosis, etiology and management of these cases are discussed.

A case of pentalogy of Cantrell pathology in only one fetus of a twin pregnancy is reported; no congenital malformation was found in the second fetus. This fact would endorse epigenetic theories on its origin. Some thoughts on prenatal diagnosis, etiology and management of these cases are discussed.

A case of pentalogy of Cantrell pathology in only one fetus of a twin pregnancy is reported; no congenital malformation was found in the second fetus. This fact would endorse epigenetic theories on its origin. Some thoughts on prenatal diagnosis, etiology and management of these cases are discussed.

Hydrothorax is a primary pleural effusion that occurs during prenatal life (called“primary chylothorax” after birth). In certain cases, this effusion is severe andproduces pulmonary and cardiac compression, and perinatal mortality remains high.Newborns with hydrothorax often require drainage, total parenteral nutrition andspecific medication for their recovery. However, prenatal interventions, mainly withthoraco-amniotic shunts, can improve these results. We report the case of a fetuswith severe hydrothorax who underwent thoracentesis and review the literature onits role in current prenatal management