The decision to separate conjoined twins would be easy if it guaranteed a better life for both babies. But the possibility of one or both twins dying or becoming severely disabled because of the surgery or the separation's effects weighs heavily on parents and doctors, according to a new report.
Two-year-old twins joined at the head were the focus of the report on the bioethics of separation surgery. The girls, who were unnamed, shared kidneys and veins that drain blood from their brains, making separation surgery a risky undertaking unlikely to benefit both of them equally. But leaving them joined could also threaten their health, not to mention their independence.
"In this case, every ethical principle is sort of turned on its head," said Dr. Devra Becker, a plastic surgeon at UH Case Medical Center in Cleveland and senior author of the report published Monday in Plastic and Reconstructive Surgery, the journal of the American Society of Plastic Surgeons. Those principles, including informed consent, the duty of doctors to heal and avoid harm, and the tenet that health care resources should be distributed fairly, form the framework of Becker's report.
The girls traveled with their parents to Rainbow Babies and Children's Hospital in Cleveland from Italy for separation surgery. They are craniopagus twins -- the rarest form of conjunction affecting one in 2.5 million births. Based on published cases, the odds of both twins surviving separation surgery are 33 percent -- the same odds for both twins dying.
"Few will debate the benefit of separation if the surgical risk is [zero]. Similarly, few will advocate for separation if the procedure guarantees the deaths of the twins," Becker and colleagues wrote in the report. "The ethics of separation becomes more complex when the morbidity of separation lies between [zero] and 100 percent or if one twin will benefit more from the separation than the other."
Following the risky, not to mention expensive procedure, the larger twin would need a kidney transplant or life-long dialysis to live. The smaller twin would be at risk for brain damage. But left together, the girls were at risk for kidney failure and cardiovascular disease.
The procedure could also give both twins the chance for a normal life.
"I think parents do want what's best for their kids," said Becker. But the assumption that separation would make life better may be incorrect.
"We tend to view certain conditions as deficits requiring repair," said Becker. "But people with those conditions may view it differently."
Chang and Eng Bunker of Siam (now Thailand) had two wives and 21 children between them, despite their being joined at the sternum. Abigail and Britney Hensel of Minnesota, whose bodies were joined but heads were separate, attended college at St. Paul's Bethel University. And craniopagus twins Lori and George Schappell of Reading, Pa., were award-winning musicians.
"It didn't slow them down," said Becker. "We think of it as a sort of limitation, but it may or may not be limiting to the people affected."
The risks of craniopagus twin separation surgery are great. In the 64 documented attempts since 1928, 32 twins died and 17 were neurologically impaired. In 2003, Iranian twins Laleh and Ladan Bijani died from surgical complications. They were successful lawyers before the procedure.
After thoroughly weighing the risks and benefits, the Italian twins' parents and the medical team decided to move forward with the procedure. The larger twin, who would be left without kidneys, would go on dialysis until she was strong enough for a transplant. And the risk of brain damage in the smaller twin would be minimized by doing the procedure in stages. The benefits of separation for both twins, both medical and otherwise, outweighed the risks.
But during the procedure, the surgeons noticed the layer of tissue covering the twins' brains was dangerously tight -- a twist that tipped the risk-benefit scale. The surgery was aborted, and both twins recovered.
"This is a compelling case; it's very dramatic," said Becker. "But I think it can be generalized, because everything we do potentially has effects on other people."
Becker said the nuances of the twins' case make it a good discussion point for ethics: The twins themselves could not consent; the medical and social risks and benefits for both babies were at times conflicting; the cost was exorbitant; and the outcome was uncertain.
"You can always cherry pick a success story of people who have stayed together or a success story of people who were separated, but that's not necessarily predictive of any one family," she said. "That's why having these discussions is so important."