The legacy of Ladan and Laleh.

On July 7, 2003, as people everywhere waited, watched and hoped for a modern miracle, 29-year-old Iranian craniopagous twins Laleh and Ladan Bijani died. The world mourned the passing of these courageous women.

The cause of death was blood loss during the final stage of a surgical attempt to separate the twin's conjoined skulls, dissect-apart their adherent brains and transform one common venous return from both brains into two individually complete blood flow avenues.

Many applauded the team of 28 physicians and 100 surgical assistants and technicians that operated for 50 hours to try to accomplish the nearly impossible. But some raised questions about whether or not the procedure, the first-ever attempt to separate adult craniopagus twins, should have been attempted.

Conjoined twins result from incomplete separation of monozygotic, monochorionic twins early in embryonic development, resulting in body parts of the otherwise normal twins remaining fused throughout development. Conjoined twins occur an estimated once in every 100,000 live births. Sites of fusion include hips (iliopagus twins), chest (thoracopagus twins), abdomen (omphalopagus twins) and head (cephalopagus twins).

Conjoined twins are sometimes called Siamese twins. Chang and Eng Bunker were thoracopagus twins born in Siam in 1811. After a long career as circus side show freaks, Chang and Eng (still conjoined) each married, settled in North Carolina and fathered a total of 19 children. (1)

Separating twins

For numerous reasons, conjoined twins have long been of medical, historical, and legendary interest. Biddenden, England is known not only for its winery but also for the legend of the 11th century Biddenden maids, sisters said to be iliopagus twins.

In their will they left property to the poor of the village. To this day in Biddenden, the Biddenden Dole takes place each Easter Monday. Cheese, bread and tea are distributed to the poor to celebrate this story, which may or may not be historic fact. (2)

Separation of conjoined twins is desirable for obvious reasons such as privacy, choice of place of residence, daily activities, educational path and career and the ability to interact socially in a normal manner rather than being considered a freakish sideshow attraction.

However, the surgical separation of conjoined twins is extremely delicate and dangerous. The main reason is the sharing of critical structures such as arteries, nerves, veins, kidneys or the liver.

Unique operative techniques must be devised and surgical skills must be meticulous in order to avoid death of one or both twins during handling of the critical structure, then to provide each twin a separate mechanism for supplying the need formerly met by the shared body.

Laleh and Ladan Bijani long sought relief from a situation they began to find intolerable. Intelligent women and attorneys, they were well aware of the technical details of separation surgery. They knew that one or both might die in a surgical attempt to separate them.

But they were also quite religious and felt that "If God wants us to live the rest of our lives as two separate independent individuals, we will." Ladan said they would spend the hours before the operation reading the Quran and performing ritual Muslim ablutions. "We feel closer to God that way," she said. (3)

The twins wrote an e-mail to Dr. Keith Goh at Raffles Hospital in Singapore asking that he evaluate them for possible surgery. They had heard of Goh's successful separation of 18-month old Nepalese cephalopagus twins in 2001.

After months of getting acquainted with Ladan and Laleh, assured that the twins wanted surgery at all costs, Goh decided to proceed with the operation and assembled a team of expert surgeons, anesthesiologists, radiologists, nurses and technicians. Notable among them was Benjamin Carson, MD, of Johns Hopkins University.

In 1987, Carson led the surgical team that separated craniopagus twins, both of whom survived, and in 1997 he guided a team of South African physicians who successfully separated craniopagus twins.

The operation

The first stages of the operation were successfully accomplished in spite of technical difficulties that are hard to imagine.

For example, successful millimeter-by-millimeter dissection of the twins' two matted brains is an incredible feat. To get a feel for the patience and skill required, put a foil-wrapped piece of chewing gum in your pocket, carry it around for two or three weeks, then get the foil wrapper off the gum in one piece without tearing the foil.

After 50 hours of painstaking surgery, during which episodes of unstable blood pressure and high intracranial pressure were successfully managed, the surgeons made the final cut. At that point massive uncontrollable fountain-like bleeding was encountered and both twins died of acute blood loss.

Ethical aspects

According to the popular format of Beauchamp and Childress, the four major principles of health care ethics are autonomy, beneficence, nonmaleficence and justice. (4) Using that framework as a guide, here are a few questions about ethical aspects of the Bijani case and answers acceptable to most observers.

Autonomy

Were the twins' wishes respected? Absolutely.

The decision to proceed with the surgery was theirs. Ladan and Laleh, both intelligent women and law school graduates, had long sought an attempt at surgical separation because they found the status quo increasingly intolerable.

The sincere and often expressed wishes of Ladan and Laleh to attempt separation at all costs would not have been well served by a decision not to attempt an operation that, in the hands of this particular team, had a significant possibility of succeeding.

Was the twins' decision based on accurate information adequately shared? Yes.

Ladan and Laleh were aware that Goh's successes, like Carson's, were in infants and children and that the operation had never before been attempted on adults. So they knew that the procedure was in the realm of experimental surgery.

Death of one or both sisters was openly discussed as a definite possibility. Indeed, the surgical team's efforts to assure themselves that the Bijani twins knew they were placing themselves in extreme danger far exceeded traditional informed consent routines.

The only criticism to be heard is that the estimate of a 50-50 chance of success seems optimistic. Does this point really matter? I think not.

In 1997 a team of highly qualified German physicians refused to attempt the surgery because of the large shared common vein that Goh and Carson also feared from the very beginning. In spite of that 1997 opinion, the twins kept searching for a surgeon who would share the risks with them.

Clearly Ladan and Laleh were not going to take "No" for a final answer, regardless of the risks.

Beneficence

Was the operation done to benefit the twins? Few disagree that if the operation had been 100 percent successful, the beneficial effect on the Bijani twins' lives would have been great beyond measure.

Nonmaleficence

Was harm done? A dependable physician's primary watchword is "primum non nocere," first do no harm. How can an operation in which two patients die in the operating room be said not to have caused harm?

The answer is that, in this case, the surgeons had no clear choice between risking harm by operating and clearly avoiding harm by accepting the status quo.

Deterioration of the twins' medical condition, as well as their own perception of a worsening of the lifestyle imposed by their deformity, were not reversible or approachable by any means other than taking this surgical risk.

By the way, this point is generalized. That is, choosing between operating and not operating is often not a choice between risk and no risk. This fact is often ignored in conclusions about "unnecessary surgery."

Justice

A fine line must be walked between justice for individual patients and distributive justice.

Distributive justice in health care means avoiding the specter of denying needed care to some patients simply because care of one or two patients has consumed a disproportionate share of available money, staff time and other resources.

No one has questioned this aspect of the Bijanis' case. The hospital in which the surgery was done is staffed and equipped to continually meet the needs of Goh and his local and international colleagues. Money was not an issue because the hospital underwrote the costs of surgery and the surgeons donated their time and expertise.

Goh expects that the ethics debate and second-guessing of the surgeons' decision to proceed with this operation might go on forever. I don't think so. In fact, the ethics debate is over already.

It is clear to almost everyone that Goh, Carson, Laleh, and Ladan were highly conscious and respectful of the ethical impact of their chosen course. Their story is uncomfortable for us partly because it reminds us that we too often are not.

References

(1.) A social history of conjoined twins. http://zygote.swarthmore.edu/cleave4b.html

(2.) A local legend, www.biddendenvineyards.com

(3.) "Conjoined twins prepare lot surgery". Anchorage Daily News. July 5, 2003. adn.com

(4.) Beauchamp, TL and Childress, JF. Principles of Biomedical Ethics, Fifth Edition. Oxford University Press. New York and Oxford. 2001.

Richard E. Thompson, MD, is adjunct instructor of ethics at Drury University, Springfield, Mo. and president of Thompson, Mohr and Associates. Previously, he was an adjunct instructor of ethics at the Ethics Institute, St. Petersburg College, St. Petersburg, Fla. He can be reached by phone at [phone] or by e-mail at [e-mail]