The legacy of Ladan and Laleh - Ethical Aspects - surgery to separate conjoined twins

Physician Executive, Sept-Oct, 2003 by Richard E. Thompson

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.