Craniopagus twins, a rare deformity in which the newborns are fused at the skull, occur with a frequency of one in approximately 2 million live births. Even more rare is the vertical craniopagus type 2, i.e., twins joined at the vertex within a single cranial cavity, with two separate but intimately opposed brains.

The likelihood of having two sets of conjoined twins with this type of cranial fusion born within one month of each other is almost unheard of. Not surprising, however, is the fact that two preeminent members of ASPS would be tasked with their surgical separation.

Ultimately, these surgeons found themselves on convergent paths, facing multifaceted financial, medical, ethical and social challenges with determination, courage and hope.

Guatemalan twins
Henry Kawamoto, MD, director of plastic surgery at UCLA, first met his twins through Jorge Lazareff, MD, director of UCLA’s pediatric neurosurgery program and an active member of Healing the Children Foundation (HTC), Spokane, Wash.

Dr. Lazareff’s work with HTC, an organization that arranges for medical screening, care, transportation and host-family support for children worldwide, took him to Guatemala’s Social Security Hospital. There he encountered conjoined twins Maria Teresa and Maria de Jesus Quieg-Alvarez, hospitalized since their birth in July 2001. The girls’ parents brought the dark-haired and lively “Two Marias” to the hospital from Belén, a poor coastal village. Not long after, Dr. Lazareff phoned Dr. Kawamoto, who agreed to evaluate the twins.

Dr. Lazareff and Dr. Kawamoto set about getting the philanthropic support they needed to transport the girls to America for a battery of tests. Once the Fundación Pediatricá Guatemala, an international partner with HTC, made arrangements to transport the girls to America, Drs. Kawamoto and Lazareff approached Mattel Children’s Hospital at UCLA to donate use of their facilities and nursing staff. Once UCLA was on board, recruiting the physicians and medical support team that would collaborate on the separation was easy.

“I had not separated any other craniopagus cases,” says Dr. Kawamoto, lead plastic and reconstructive surgeon on the team. “But we were in agreement that the surgery could be done. Once we had the financial support and top-ranked physicians willing to donate their services, we got down to the planning.”

Egyptian twins
Meanwhile, Kenneth Salyer, MD, founding chair and director of the International Craniofacial Institute and the Cleft Lip and Palate Treatment Center at Medical City Dallas Hospital, learned of yet another conjoined twins case.

Mohammed and Ahmed Ibrahim, conjoined twin boys, were born in June 2001 in a small, poor, rural village 500 miles south of Cairo. The twins were being cared for at Abu el-Reesh Hospital in Cairo, where doctors were unsure of their fate.

One of the few plastic and reconstructive surgeons in the world who had previously successfully separated craniopagus twins, Dr. Salyer had seen success in Lithuania – and was immediately interested in the Egyptian twins.

But, like Dr. Kawamoto, Dr. Salyer understood the costs. “We decided to get the World Craniofacial Organization involved,” said Dr. Salyer. The Dallas-based non-profit group is dedicated to helping children obtain life-changing craniofacial surgery.

“Plus, at our hospital, we’ve treated complex cases from 72 countries and every state in the U.S.,” Dr. Salyer points out. “We have a full-time multi-specialty team just for children and we have a motto at the hospital: ‘No child will ever be turned away.’”

Similar yet distinct challenges
As the two plastic surgeons moved forward with their respective cases, each shared similar yet distinct challenges: neural and vascular connections that had to be mapped, tracked and ultimately severed.

A host of preoperative tests on both sets of twins included CT scans, MRIs and customized, three-dimensional anatomical models and imaging systems donated by two companies invited to participate in the cases.

For Dr. Kawamoto and his team of specialists, planning was the key to a successful outcome. Preparations included gathering as much information as possible from world literature and specialists involved with other successful craniopagus separations in Toronto and in Africa. “What I learned is that each case is a little different,” says Dr. Kawamoto, “yet each case requires anticipatory planning – from the special bed required to cushion the twins’ tissue expanders to the backup plans you have for each step preoperatively, during surgery and postoperatively.”

Putting the plan to work
After studying the Guatemalan twins’ tests, cranial measurements – exact to the millimeter .- were used to create tissue expanders that would allow for the design of adequate flaps.

That challenge was exacerbated by a small tear in one of the twins’ saline-injected cranial balloons, notes Dr. Kawamoto. “That was a really big deal, but we had a backup plan for each step of the procedure, including the tissue expansion.”

What the surgical team didn’t anticipate was the children’s rapid growth rate.

“Two days before the surgery in early August, we cut out sample pieces on felt and laid them out, but it wouldn’t fit. In less than two months’ time, the girls’ heads had grown roughly 10 percent, so we had to start over with new a flap design,” Dr. Kawamoto explained.

The team, tired and frustrated, decided to come back the next morning – the day before surgery – and attempt a new design. Luckily, the new design fit.

In a crowded surgical theater where the coordination of medical personnel could easily be compared to a finely tuned orchestra, seven pediatric anesthesiologists, four neurosurgeons and four plastic surgeons, plus a host of other critical surgical support staff, worked in shifts for more than 15 hours.

During the girls’ surgery, experts in brain mapping monitored brain-wave activity and vascular specialists tracked each vessel as it was clamped off, ready to rebuild vessels that did not tie off properly. Both infants had an incomplete small sagittal sinus, but the surgeons felt that the collateral run-off could be recruited.

After the neurosurgeons completed their task, Dr. Kawamoto and his team began work on covering the girls’ new skulls. “As soon as you make that first incision at the beginning of the operation, you are committed to a path of no return,” Dr. Kawamoto warns, “because if you are short a little bit, the tissue won’t fit to cover the exposed brain.”

The operation itself went like clockwork, reports Dr. Kawamoto, who credits an “unbelievable” support team. “Postoperatively, the girls remain in serious condition but recovery is on track. We cleaned debris from both the girls’ skin flaps and applied a special skin substitute that encourages cell growth and promotes rapid healing.”

A complex quandary
Dr. Salyer’s twins, however, remain conjoined. As of press time, the test results, coupled with the risks inherent in Dr. Salyer’s twins case, have created a crucial dilemma: Should they operate?

“Our twins are more complex in terms of the venous drainage situation,” explains Dr. Salyer. “Neither of our boys has a normal sagittal sinus. In fact, each is dependent on the other’s circulation (see photo, top of page 24). We are trying to come up with a plan that provides them with venous drainage independently, without causing extensive brain damage or death. That’s my greatest concern and we don’t have that solved yet.”

Dr. Salyer admits that as he learns more about the twins’ potential outcome, deliberation to perform the surgery is forcing him to make one the most difficult decisions of his personal and professional life.

“We have five top pediatric neurosurgeons on our team from our Pediatric Neurosurgeons Group, and although they have come up with a consensus, even they are not all in agreement at this point,” emphasizes Dr. Salyer. “After much deliberation, they came up with an opinion, and to be honest, I don’t like the opinion, because it says that both children stand a good chance of not making it. So it’s a major struggle for me.”

Sharing information
Like Dr. Kawamoto, Dr. Salyer has gathered expert information and counsel from a variety of sources. One such source is Benjamin Carson, MD, director of pediatric neurosurgery and professor of neurosurgery, oncology and pediatrics at Johns Hopkins University Hospital, Baltimore. Dr. Carson, whose experience in separating craniopagus twins is renowned, has been helpful to Dr. Salyer and Dr. Kawamoto’s neurosurgery team, and in some ways, offers renewed hope.

“Until 1997 when I separated a similar case of twin girls from South Africa, none of the type 2 craniopagus surgeries prior to that had been successful,” says Dr. Carson. “All had either died or had severe neurological impairment. But the twins from South Africa are doing well and are in preschool now.”

Furthermore, Drs. Salyer and Kawamoto have stayed in contact, sharing information about their cases, test results and statistics. After Dr. Kawamoto’s separation was complete, he phoned Dr. Salyer to discuss the unexpected pitfalls that he experienced, especially in the measurements of the flaps.

All the education, state-of-the-art technology, expert advice and planning have done little to reassure Dr. Salyer, however.

“The chances of having both of these children make it are slim, and those are chances that neurosurgeons are used to,” admits Dr. Salyer. “But as a reconstructive surgeon, I’m not comfortable with going into an operation where I think I may lose two chilíren who right now are alive and viable. These boys are endearing, loving and compassionate - and they are two separate individuals, which is amazing to me. So I’ve fallen in love with them, like I do with many of the kids I deal with.”

Before making the decision to separate the twins, Dr. Salyer plans to communicate with the twins’ father directly. “I want to look him in the eye and make sure he understands the risks, because they are extremely high,” he says. “Should one or both live, the father needs to understand that there is the possibility of neurological damage.”

Dr. Salyer also is committed to making sure that Egypt will provide continued care and support for any disability the boys may have.

-Plastic Surgery News
October/November 2002