(More Than) Twice As Nice

Yurelia and Fiorella Rocha-Arias

Maria Elizabeth Arias is glad to push two strollers and chase after her two daughters when  they run in two separate directions. These are just some of the “twos” Maria dreamed about before she brought her 2-year-old, conjoined twin daughters, Yurelia and Fiorella Rocha-Arias, to Packard Children’s to undergo high-risk separation surgery. Now, after sailing through surgery and recovery, the Costa Rican girls have returned home to share their miracle with family and friends. At the same time, the largest team of doctors and caregivers ever to collaborate on a single case at Packard Children’s—and the first of its kind for the hospital—reflects on how it went so well.
 
General pediatric surgeon Gary Hartman, MD, conducted the November 12 symphony—a 23-member, multidisciplinary team inside the operating room and countless others outside. The team’s harmony hinged on years of experience, careful choreography and detailed contingency plans.
 
Cardiothoracic surgeons, liver surgeons and plastic surgeons would work in turns to perform their part of the complex separation surgery. Anesthesiologists would be by the girls’ side throughout preparation, surgery and upon the twins’ awakening in recovery. But in this extraordinary case, because the girls’ hearts were connected, and because of Yurelia’s congenital heart defect, anesthesiologists with special training in pediatric cardiology care were required.
 
“Anesthesiology played a very special role,” said Hartman, who noted that in most cases the anesthesiologist is the last person patients and families see before a child is wheeled into surgery, and the first person the family sees when a child awakes. The anesthesiologist often becomes the “face” of the procedure. Gail Boltz, MD, pediatric cardiac anesthesiologist, was team leader.
 
“The anesthesiologist is the person to whom the parent hands their child,” says Boltz. “It’s a major psychological responsibility coupled with a very complicated physiological responsibility.” For patients who will be returning for several procedures, anesthesiologists are careful to set them up for success right at the beginning by creating an environment of calm and comfort, thus helping parents—who set the tone for their children—feel trusting and secure. “This positive psychological impact has an enormous payoff,” said Boltz.
 
Cardiac anesthesiologists like Boltz manage pain and maintain the life presence of patients while surgeons do their work. They monitor oxygenation of the blood, ventilation and blood pressure. They must also consider how the surgery itself will affect the patient, and how any factors like heart disease—and in this case, the very fact of being conjoined—may impact or be impacted by the surgical procedure. For the Rocha-Arias twins, these effects were hard to anticipate. How dependant was one girl on the other? How would medication provided to one twin affect the other?
 
For answers, Boltz and her team studied the few other cases of conjoined twins and contacted other doctors across the country with related experience, as well as the girls’ doctors in Costa Rica. In August, shortly after their arrival, the twins were anesthetized for an initial 6-hour imaging study of their structural make-up to determine if separation surgery could proceed. Doctors quickly made some fascinating discoveries. “We found that if we anesthetized one, we anesthetized both,” said Boltz. And that discovery was just the beginning.
 
"We also found that if we injected something into Fiorella’s left hand, it went straight up her arm through the coronary sinus (drainage of the heart) and straight into Yurelia, bypassing Fiorella’s system almost completely,” explained Boltz. They also learned that if Yurelia had her blood flow obstructed in any way—from outside pressure of her position or her sister’s leaning body, for example—her pulmonary (lung) blood flow would drop. These factors would be critical to monitor, especially during separation surgery, when organs would have to be lifted and held in a certain position for long periods of time. “These were important findings,” said Hartman.
 
In the weeks before surgery, the girls underwent surgical insertion of skin expanders to stretch their skin prior to separation, and also weekly injections of saline to enlarge the expanders. By the time of the separation surgery, Yurelia and Fiorella had been anesthetized at least five times.
 
Hartman, Boltz and their teams carefully planned the operating room logistics for the November 12 separation surgery. There would be two of everything, including medical staff, equipment and even two cardiopulmonary bypass machines. Carefully prepared plans determined which teams and equipment would be in the OR when, and how many power lines would be required to keep it all running. These plans also included how and when the twins, after separation, would move to separate operating rooms, allowing different surgery teams to complete surgery with each girl. The six-member cardiac anesthesia team had a strategy for almost every possible scenario, and understood all the complex factors that would be required to keep the twins stable during separation.
 
“The most exciting moment,” Boltz recalls of the separation surgery, “was in the final stage, when surgeons placed a clamp on the connection between the girls’ hearts.” The cardiac connection shared by the girls was the riskiest part of the procedure. It was simply unknown how much the girls needed each other to survive. “Immediately—as if someone had turned on a switch—their individual stats improved. Their blood pressures improved. It was clear the girls were going to be stronger apart.”
 
The twins mother Maria is now celebrating their miracle. “We are just so happy and so content,” she said, “because our girls have been born anew.”
 
Packard Children’s may have changed the Rocha-Arias family’s lives, but the twins also left their mark on the hospital. “This was just a remarkable experience for all of us,” said Boltz. “We may never see a case like this again. I’m really honored that we could give these girls our best.”
 
Gifts in support of Packard Children’s Hospital and the expert care it gives to children like Yurelia and Fiorella can be made by visiting http://www.lpfch.org/fundraising/. To find out more about Mending Kids International, visit http://www.mendingkids.org.