3 R.I. doctors emerging as pioneers in
Providence Journal 08-03-2000
In the 17th week of her fourth pregnancy, Marjorie Campbell learned that something was amiss.
She already knew that she was having twins, and she knew twin pregnancies can be complicated. But the complication that doctors were talking about now was something quite chilling, something she had never heard of, never imagined.
Her twins had a deadly condition that occurs in one out of every 2,000 or 3,000 pregnancies. Trying to save them, Campbell, who lives in Mashpee, Mass., would travel to Providence and become one of the first patients of a pioneering program in fetal surgery.
Her twins were sharing a placenta and a blood supply, a situation that's not a problem in itself. The problem was that the blood vessels had formed an abnormal connection, with the artery of one twin connected to the vein of the other. Because arteries have stronger pressure than veins, one twin had become a blood donor to the other. The condition is called twin-to-twin transfusion syndrome.
About 4 of every 1,000 births involves identical twins sharing a placenta. Of those, about 10 percent have twin-to-twin transfusion syndrome.
As Campbell tells it, the doctors spared no details in describing what this means. The "donor twin" would keep pumping blood into his brother, expending so much energy making blood cells that he would have little left for growth. He would be too small and have too little amniotic fluid around him, eventually becoming entangled in the amniotic membranes.
The "recipient twin" would fare no better. He would end up with too much blood and water, bloated and surrounded by an excess of amniotic fluid, at risk of congestive heart failure.
If nothing was done, neither twin was likely to survive.
But what could be done?
The first line of treatment is amnioreduction - inserting a needle into the amniotic space of the larger, recipient twin, and draining off some fluid. This seems to help, possibly by relieving the pressure on the umbilical cord of the recipient twin, allowing for a better balance of fluids. With amnioreduction, there's a 60 percent chance that the larger twin will survive, although often with severe heart or brain damage.
And it's risky; each time the uterus is punctured by the needle, the risk for bleeding or infection increases.
Campbell had the amnioreduction performed seven times in five weeks, and it became clear that it wasn't going to work. The donor twin was growing too slowly, and showed signs of heart failure.
Campbell had already read about another option: surgeons could correct the problem at its source by entering the uterus and closing off the blood vessels linking the two fetuses.
THE FETUS is a new frontier in surgery. As diagnostic technology has improved, doctors are better able to detect abnormalities, and as surgical technology advances, they have the confidence to intervene.
The first attempts at fetal surgery, in the 1980s, involved cutting open the mother's abdomen, removing the fetus, operating on the tiny patient, and returning it to the womb. It was traumatic for mother and fetus, and the results were generally dismal. Many fetuses died, and many mothers delivered their babies prematurely.
Recent years have brought improvements in endoscopic surgery. Doctors can remove gall bladders and even repair hearts through tiny fast-healing incisions, thanks to minuscule cameras that bring Images of the body's interior to video screens. The same technology would allow entry into the once-inviolable womb.
The operation that offered the best hope to Campbell's twins had first been performed in 1995 by Dr. Julian DeLia. His results were promising but not impressive enough to catch on with American doctors. But doctors in London and Paris began doing the procedure with much smaller instruments than DeLia had used and started seeing better results: 70 to 80 percent survival rate for one twin, and 50 to 60 percent survival rate for both. Additionally, the surviving twin had fewer health problems at birth than those whose mothers underwent amnioreduction.
Campbell learned, from her own Internet research, that DeLia was on sabbatical. The U.S. medical center with the most experience at the surgery is in Florida. But her doctors at New England Medical Center in Boston, which is owned by the Providence-based Lifespan, told her that a few miles to the south, in Rhode Island, a team of doctors had performed the procedure.
"We decided to meet the surgeons here before we made a decision," Campbell says. "As soon as I met the three surgeons, I felt comfortable with them. I knew I'd stay here."
The three surgeons were Dr. Stephen R. Carr, an expert in fetal diagnosis from Women & Infants Hospital, and Dr. Francois I. Luks and Dr. Thomas Tracy, pediatric surgeons at Hasbro Children's Hospital.
In spring 1999, the three had formed the Fetal Treatment Program, believing they possessed an unusual combination of expertise: the Hasbro doctors' skills in pediatric surgery and microsurgery, and Carr's knowledge of fetal development and prenatal diagnosis. For more than a year, the program had no patients, but the doctors, nurses and technicians were busy refining their technology and techniques, and practicing on sheep.
In particular, Luks said, the Fetal Treatment Program developed a flexible scope that could curve around bends. By chance, it would prove crucial in the first two cases that finally came their way.
The surgeons told Campbell that they had performed the procedure in a Providence woman on June 7, and considered it successful. Campbell demanded to know: Did both twins survive?
The answer was no. The smaller one died two weeks after the operation, unable to recover from the work of pumping so much blood into its twin. But the mother, whose water broke, is in her 30th week of pregnancy at Women & Infants, expecting a healthy baby some day soon.
Campbell and her husband, Glenn, decided to go ahead with the procedure. She entered an operating room at Hasbro Children's Hospital on June 30, and went under general anesthesia.
The surgeons' primary tool was a telescope the width of pencil lead (one-16th of an inch), with a miniature video camera at the tip and a hair-thin laser fiber running alongside it.
Campbell's placenta, like that of the Providence woman's, happened to be located in the front, right where the surgeons would usually make their incision. This condition, anterior placenta, occurs in 25 to 30 percent of pregnancies. Having a telescope that could curve a little proved a crucial advantage.
The surgeons punctured her abdomen and maneuvered the telescope around the placenta to locate the joined blood vessels. They were guided by a three-dimensional map of Campbell's entire uterus made the day before with an MRI device usually used in brain surgery, and a real-time ultrasound that pinpointed the telescope's location as it navigated the uterus.
Once the telescope reached a blood vessel that needed to be closed off, hot-pink laser beams were blasted into it. Then it moved to another, sealing it off with laser coagulation. The procedure took about two hours. A single stitch closed the incision.
"You're fine. Both babies are fine," Campbell remembers the doctors telling her when she regained consciousness.
The donor twin started growing almost immediately; Campbell could feel the difference within two days. An ultrasound found them to be almost the same size.
THE FIRST two surgeries of the Fetal Treatment Program were technically not fetal surgery: the procedure was done on the placenta, not the fetus. As he looks to the future, Luks is not sure what will come next. In other parts of the country, fetal surgery has been tried to correct spina bifida (an opening in the fetal spinal cord that causes multiple problems on birth), but Luks is not convinced that it's worthwhile.
A year ago, he was talking about a procedure to help fetuses who have holes in their diaphragms through which abdominal organs protrude, preventing proper lung development. The plan was to block the fetus's throat, causing the lungs to fill up with fluid and thus hold their own against the press of other organs. At birth, the obstruction would be removed and later, the diaphragm repaired.
"Technology-wise, we are ready to do it," Luks says. "We're just waiting for an appropriate patient."
But you can't justify invading the womb unless you're correcting a problem that can't be fixed after birth. And treatments for babies with inadequate lungs have been improving rapidly, as have many treatments for newborns.
"That raises the bar so much higher," Luks says. "Yes, we want to do it, but does it really make sense?"
At the same time, he adds, doctors are getting better at diagnosing problems earlier in pregnancy, and some may lend themselves to surgical intervention. The proper role of fetal surgery, Luks says, has become "a moving target."
Two weeks ago, Campbell was admitted to Women & Infants Hospital because her water broke, putting her at risk for premature labor. That also puts her at risk for infections. Yesterday, in her 28th week of pregnancy, the doctors decided the risk of infection was too great to wait any longer. They delivered the babies by Caesarean section.
The boys weighed almost exactly the same: one was 2 pounds 1 ounce, the other was 2 pounds 3 ounces. They are in stable condition in the intensive care unit, breathing with the help of respirators.
Unfortunately, one twin was born with an unexpected problem that Luks described as not life-threatening and not related to the surgery. He could not tell whether it was related to the twin-to- twin transfusion syndrome. The family did not want any details divulged.
The boys are named Christopher and Alexander.