A World of Solutions
In addition to cervical teratomas, the Fetal Care Center will be able to address a wide range of fetal problems with an equally wide range of tools and techniques. “There are, for example, ultrasound-guided procedures,” Dr. Crombleholme says. “Using a needle, we can do intrauterine transfusions. We can tap fluid spaces. We can do amnio infusions. We can put a laser fiber into a blood vessel feeding a lung tumor and photocoagulate the vessel to shrink that tumor. We can devascularize teratomas that have these enormous vessels that feed them. We can do ultrasound-guided procedures with a radio frequency device, which uses radio frequency to generate heat that can coagulate the feeding vessel.”
But that’s not all. There's a condition called twin reversed arterial perfusion sequence. In this situation, a normal multiple-fetus pregnancy has gone awry, and one of the twins is nothing more than a fetus-shaped mass with no head, no heart, and no nervous system. It is connected to the healthy fetus and draws blood from it to such a degree that the healthy baby can die from heart distress. Dr. Crombleholme addresses this condition by using ultrasound to guide a 19-guage needle to the cord that connects the healthy fetus and the malformed one. The needle has a special tip that uses radio frequencies to coagulate the cord. The unviable fetus dwindles, and the healthy fetus no longer loses nutrients and blood. “We have a 98 percent survival with that approach,” Dr. Crombleholme says.
A Womb with a View
“We can also do open fetal surgery,” Dr. Crombleholme says, “most commonly for repairing spina bifida, but also taking out masses like pericardial or mediastinal tumors. We can do amnioports for amnio infusion, and a whole host of EXIT procedures.”
EXIT stands for ex-utero intrapartum treatment, one of the most complex procedures the Fetal Care Center will perform and the one that addresses cervical teratomas—the tumor in a fetus’s neck that chokes off the airway. The surgeon makes an incision in the mother’s abdomen and uterus, then pulls the fetus partway out and performs the necessary surgery while the fetus is still receiving oxygen and nourishment from the placenta. When the procedure is done, the baby is fully delivered. In other open fetal surgeries, the fetus is returned to the womb, incisions sutured and the pregnancy continues.
The idea is audacious, and actually accomplishing it requires overcoming multiple hurdles, achieving precise timing and maintaining intensive monitoring and adjustment. Because the procedure involves two patients, the mother and the baby, it requires a wider-than-usual range of specialists, with as many as 40 people in the room. “You have myself,” Dr. Crombleholme says, “and a maternal-fetal-medicine specialist, another fetal surgeon, a cardiologist doing continuous echocardiographic monitoring plus a scrub team—at least one, sometimes two scrub teams—an obstetric anesthesiologist, a pediatric anesthesiologist, the neonatal resuscitation team, and, if you're going to continue operating after the baby's delivered, a whole other OR team and another setup.” That’s why a Fetal Care Center requires a larger-than-average operating room, to accommodate all those people and equipment necessary for the procedure.
Care that Starts Before the Beginning and Continues After the End
That pre-procedure education is the beginning of what Dr. Crombleholme calls vertically integrated care, which includes caring for the mother and child through diagnosis and treatment through delivery, through the Neonatal Intensive Care Unit, and then during long-term follow up, all with the same team of doctors and clinicians.
“If we can reduce the stress that these parents experience when their baby's in the nursery,” Dr. Crombleholme says, “then they can be a parent as opposed to a passive bystander with the nurses and the doctors hovered around the baby. They can be the mom and the dad. My experience has been that it makes an enormous difference in the neurodevelopmental outcome of these children, if their parents can be parents from the time the baby is born, rather than being so intimidated by the medical setting, the baby's going to have a better physical outcome.”
All of the conditions treated in the Fetal Care Center are rare to one degree or another. But there are some conditions that are far from rare and, as yet, have no treatment available. One of those is fetal growth restriction. As the name suggests, the fetus does not grow at the typical rate and is at risk of a whole host of medical problems. Many babies with this condition are stillborn. In fact, this condition is the second leading cause of mortality just before or after birth. But Dr. Crombleholme, in addition to his work in the operating room, is also a researcher, and he is working on a method of gene therapy that holds great promise to reverse this condition.
“It affects 7 percent of all pregnancies and there is no treatment for it,” Dr. Crombleholme says. “The fetus is not growing and they're doing very poorly. They get delivered severely premature with all the attendant risks of severe prematurity. In my basic research in mouse, rat and rabbit models, we’ve been able to do placental gene therapy to correct the growth restriction. We can expand the vascularity of the placenta and completely reverse the growth restriction in utero. And growth restriction in these animals has the same characteristics as human growth restriction. Not only is that animal a normal size when it’s born, but the non-treated growth-restricted fetuses, when they become adult animals, are obese, diabetic, and have a hypertensive cardiomyopathy. None of those things happen with the animals treated in utero. This is something that we’re very excited about, and we think that is just over the horizon, that we’re going to be able to make the transition from treating animal models to treating growth-restricted babies.”