For the first time, fetal medicine experts have performed prenatal heart surgery to remove a life-threatening tumor (intrapericardial teratoma) connected to the heart.
The patient, who underwent the operation at 24 weeks of gestation while in his mother’s womb, is now a healthy three-year-old preschooler.
This great work shows that scientists can accurately diagnose and provide a prognosis for this rare condition in utero, as well as perform fetal surgery.
Scientists from the Children’s Hospital of Philadelphia published their findings online in the American Journal of Obstetrics and Gynecology.
The research team, from CHOP and the University of Pennsylvania, reported a case series of 8 fetuses with suspected life-threatening tumor seen at CHOP between 2009 and 2015.
The disease is an extremely rare, rapidly growing tumor that can be detected during the fetal period. Arising in the pericardium, the sac surrounding the heart, it is lethal if untreated.
The current prenatal strategy is to drain pericardial fluid to buy time for the fetus to grow to a viable gestational age, followed by surgery to remove the teratoma shortly after delivery.
However, rapid prenatal growth of the tumor, along with fluid around the heart, which inflicts dangerous pressure on the heart, lead to disruption of normal fetal circulation and fetal death.
Therefore, an ideal treatment is to perform surgery prenatally, before cardiac dysfunction occurs. The CHOP team is the first to perform this prenatal surgery resulting in a healthy live born neonate.
Managing this type of tumor presenting before birth is a challenge, but the scientists show that survival with good outcome may be possible in some patients.
It was the ability to bring so many different disciplines to the table that made them confident enough to take the leap to offer in-utero surgery on a fetal heart as a viable option.
In addition, the current era offers technological advantages, such as improved prenatal imaging, improved cardiovascular surveillance through fetal echocardiography, and fetal surgery techniques.
A crucial factor is implementing treatment before fetal heart failure occurs.
It very important that doctors and nurses could promptly referring these cases to a multidisciplinary fetal therapy center during the optimal window of opportunity for both the fetus and mother.
Citation: Rychik J. et al. (2016). Fetal Intrapericardial Teratoma: Natural History and Management Including Successful in Utero Surgery. American Journal of Obstetrics and Gynecology, published online, DOI: http://dx.doi.org/10.1016/j.ajog.2016.08.010.
Figure legend: This Knowridge.com image is credited to Children’s Hospital of Philadelphia.