Craniopagus Twins

The miracle began at 730 am on a Thursday morning. The two tiny girls were tucked into bed-specially made for them and donated by Kinetic Concepts, Inc.- and put into a deep sleep by a team of anesthesiologists. The babies’ small skulls were bolted into place so there would be no movement whatsoever, not even a flinch, as the surgical team began its work. Renatta Osterdock, MD, Chief of Pediatric Neurosurgery at Loma Linda University Children’s Hospital and team leader on the case, had been preparing for this moment since long before the babies were born. When she and the team were finished, the twins would be separate individuals for the very first time.

What was to become known as the “Journey of Hope” began with a routine ultrasound three months into Blanca Cabrera’s pregnancy. Yes, she was carrying twins. But they were joined at the head, a condition known as craniopagus twins. Only 35 such cases have been reported since 1928.

Dr. Osterdock vividly remembers the call from the obstetrician: Can you come talk to the mom and tell her what’s going to happen?” Dr. Osterdock paused. In six years of residency, a year of fellowship, and then years of clinical practice, she had never even seen conjoined twins. But she knew from professional meetings that advances in CT and MRI were making separations more successful. She agreed to take the case and immediately began to research procedures, six months before the babies were due to arrive in the world.

“I started looking at what other people had done. What kind of technology did they use? What kind of studies needed to be done on the babies?” she recalls. A fetal MRI was reassuring. Thought the twins’ heads were joined, they did not appear to share brain tissue or blood vessels. That could change, though, as they grew. “Over time they would become more and more connected.” She explains. Separating them as early as possible would be an important key to success.

On July 16, 2004, Cristina and Crystal were born by C-section at Loma Linda, two months premature but with no complications. They were two beautiful lively little girls whose skulls were fused. They could see the world, but not each other.

Chrystal and Christina at a few months of age

Chrystal and Christina at a few months of age

Six months later the twins returned to Loma Linda to prepare for separate lives. Three-dimensional models created from CT scans and MRI’s provided free of charge by Medical Modeling in Colorado, allowed the surgical team to visualize exactly what they would encounter in the separation. The scans also confirmed the need for separation to take place as soon as possible. “By the third set of images, we saw the facial artery of one twin was beginning to drain into the facial vein of her sister,” says Dr. Osterdock.

Facial artery draining into opposite facial veins

Facial artery draining into opposite facial veins

MRI scans of the twins

MRI scans of the twins

Jeannie Martinez, a long-time case manager in the pediatric intensive care at the hospital, was planning to retire in a few weeks when she got a call from Janel Isaeff, executive director for patient care,that put her plan on hold. “There’s going to be a special project,” Isaeff said, “And I’d like you to head it, “She talked with Martinez about the twins and arranged for her to meet with Dr. Osterdock and with the hospital’s Media Relations staff. “OK Jeannie, you’re it,” said Isaeff a few days later. “Go ahead and form your team.” It would take 40 or more nurses, plus occupational, physical, and speech therapists, child life specialists, and a chaplain to see the girls through to their bran new life. Martinez put retirement on hold.

The twins were scheduled to check into the hospital on January 2, 2005, to begin two months of preparation for surgery. A key part of preparation would be tissue expansion. “There was another set of twins joined at the head that were separated very successfully in Italy,” Dr. Osterdock says. “Neurologically they did great. But they had skin grafts where their skulls were separated, so they ended up without hair, which is a pretty significant deformity. We wanted to use the twins’ own skin to cover the separation spot so they could have normal hair.”

There was just one major difficulty. Unlike adults, infants have soft skull. “People are often very afraid to put tissue expanders in babies,” Dr. Osterdock notes. “You can imagine that if you blow up a balloon under a baby’s skin, it’s not just going to push the skin out-it’ll push the skull in. So we capitalized on the place where the skulls were fused to stretch the skin, because the part where they were attached had relatively thick and firm bone.”

The need for tissue expansion was just one challenge to the nursing staff. Every other day a syringe would be inserted into the twins’ scalps and saline added to the expanders. Each injection was another opportunity for infection to set in.

Another challenge was finding the right kind of bed so there would not be too much pressure on any one part of their bodies. The answer was a specialty bed developed by KCI with a flotation mattress. Martinez arranged in-service training so the nurses could learn how to use it.

As the girls grew ready for surgery, the medical team continued to resolve technical dilemmas. One of the most vexing was how to secure the girls’ heads during the separation. “The way we typically fixate a head during surgery so that it doesn’t move is by putting pins with 60 pounds of pressure into the skull,” says Dr. Osterdock. “But if you put an infant’s skull under 60 pounds of pressure you just crush it. We had to come up with another way. “

The solution came to the surgical team one evening during a planning session over Thai food. What if they implanted small washers into the babies’ skulls several weeks before surgery? Bolts could then be threaded into the washers at the beginning of the separation surgery, and the bolts fastened to clamps. Dr. Osterdock drew the concept and sent it to an engineer at Mayfield Cooporation. Could he fabricate such a system? “Yeah!” he replied. Each of the pieces he made was imprinted with one of the twins’ names on it, because it was specifically designed just for her. “We were able to fixate an infant’s skull without any pressure,” says Dr. Osterdock. “It’s never been done before, that we know of.”

Up on the acute care pediatric unit, the staff was keeping the twins’ presence a secret, to ensure privacy. They did such a good job of it that one day when the girls’ nurse went to lunch, charge nurse Alane Allbee asked another regular staff nurse to watch the twins. “What twins?” came the mystified reply.

Those who did know about the unit’s special charges were falling in love with them. “Oh my goodness, they were precious,” Allbee gushes. “They were so good, and so much fun to work with.”

Dr. Osterdock and several therapists were known to stop by after work just to play with the girls. And, Allbee notes the caregivers were so meticulous that in nearly three months in the hospital, the twins did not have a single complication or infection.

Somewhere along the line the pediatric staff decided that the girls’ “Journey of Hope” needed to be documented so they and their parents could know how much love had enveloped them at Loma Linda. With the help of a digital camera and 20 or more enthusiastic scrapbookers, they created five books of pictures and stories, two for each girl and one for their parents.

Surgical Rehearsal

Surgical Rehearsal

Sunday, February 27, 2005-just four days remained before surgery. Dr. Osterdock promised that if the team involved in the surgery would come to a dress rehearsal, she’d cook lunch for them-all 40 of them. They came even though it was their day off and no one was paid for it. They practiced every step, from fitting the girls into the special beds that would rotate as the incision was made, to draping the tables, setting up the central lines, and carrying out each step of the operation. It took most of the day, but “it was awesome,” Dr. Osterdock remembers. “Everybody was just so excited. I was nervous, but I couldn’t believe how much people went out of their way to make this happen.”

Dr. Osterdock was able to use special models to determine the best method for separating the skulls.

Dr. Osterdock was able to use special models to determine the best method for separating the skulls.

Dr. Osterdock tells her residents that “being a surgeon is not a right. You don’t just get to go into everybody’s head because you have a title. You have to earn that privilege. It is an awesome and humbling responsibility.” On Thursday, March 3, 2005, she and the surgical team were entrusted with responsibility for the lives and futures of two little girls. At 6:30 am, they set to work.

The anesthesiologists went first, Drs. Linda Mason, Carol Lau, Elizabeth Ghazal, and Brenna Gustafson putting the girls into deep sleep. Plastic surgeons Andrea Raya and Brett Lehocky played the next role in the ongoing drama, making careful incisions to open the skin. Finally neurosurgeons Osterdock and Alexander Zouros began to cut through the bone.

The neurosurgeons expected their part to take six to eight hours. It took only two. “There was a period of about 10 minutes when we couldn’t see anything,” Dr. Osterdock recalls. “We started to separate the bones and then to get in between the two babies. We were feeling, feeling, feeling, and all of a sudden my instrument went all the way through. I said, ‘I think I just went through.’

“We both just looked at each other,” she continues, “and I said, ‘I think we need to separate the beds.’ Everybody looked at me like, ‘You’re kidding.’ I’m like, ‘No, I think we’re separated.’ Nobody would believe us!”

Despite surgeons’ best efforts to understand their patients’ condition before surgery, not everything can be known in advance. In one twin separation done in New York, the pre-surgical MRIs appeared to show no brain connection, but when the surgeons got into the operating room, they found that the brains were indeed connected. Fortunately, that was not the case with Cristina and Crystal. Only the dura, the lining of the brain under the skull, was connected, says Dr. Osterdock, who describes it as being “like filo dough. It has multiple layers. What we were able to do was separated the leaves of the dura so they both had intact dura. We never went into the brain at all.” The skin expanders worked; the plastic surgeons had plenty of skin to cover the separation sites. The girls could look forward to a normal future.

craniopagus12craniopagus11Two days later the twins, now in separate beds for the first time, were out of intensive care and back on the pediatric acute care unit. In just three weeks they were able to go home in their parents’ brand new van. And eleven months later they attended the Children’s Hospital gala with their parents-two perfectly beautiful, identical, and now completely separate little girls, continuing in their Journey of Hope.

Looking back, Dr. Osterdock admits that “sometimes you feel like you’re biting off more than you can chew.” Previous teams that separated conjoined twins were, she says, made up of the “the people we think of as world experts.” The team she led was young and primarily composed of women. “One of the biggest challenges is letting people know what we’re capable of…

Alane Allbee saw it in the care of the nurses who “put their heart and soul out there and gave 100 percent” in their care for the twins. Jeannie Martinez saw the miracle in “the smile on Mom and Dad’s faces when Dr. Osterdock came in to tell them that they had two little girls that were separated and they were just fine.”craniopagus13

For Cristina and Crystal, the miracle just may have been when they saw each other face to fac, for the very first time….craniopagus14

 

Taken from the Loma Linda University 2005 Annual Report, author unknown.