Allan Goldstein, 50, is the surgeon-in-chief at the Hospital for Children at Massachusetts General in Boston. After he conducted an operation to separate conjoined twins, his 14-year-old daughter asked: "Did you violate the Hippocratic oath?" He had to admit he didn't know the answer to the question. The highly complex procedure to separate 22-month-old, Siamese twins, was the subject of lengthy report this fall in the New England Journal of Medicine. Goldstein also shared his personal account of the surgical and ethical challenges involved with DER SPIEGEL.
It was the first time that I cried in the operation room. I had been confronted with death as we rarely experience it in pediatric surgery.
During the operation I had to execute a very difficult decision, perhaps the hardest in my career as a doctor. I was very grateful to receive ethical support while handling this case which was medically fascinating, technically challenging but also most painful for me.
It all started with an e-mail from Africa. The agent of a foundation contacted me asking whether we could help a pair of conjoined twins that he was taking care of. He also sent pictures. They showed two one-and-a-half-year-old girls who were attached just below the ribs. They had two heads, four arms, one belly, one pelvis and three legs.
They were born in a village in East Africa. I'd rather not name the country, because that might endanger the family. The people there consider abnormal babies like these twins to be a monstrosity, a sign of the devil. The parents were ostracized, stones were thrown at them and now, after the surgery, they are afraid for their lives -- I'm afraid, rightly so.
We looked closely at the pictures, wondering if we could separate these two children. It didn't appear hopeless to me. It was only later that I found out that 20 other clinics in the United States had rejected the case. It was probably better that I didn't know.
Of course, I had read about conjoined twins in the literature. But I had never before performed a separation myself. For a surgeon such an operation means an enormous and very fascinating challenge. Every case is unique, and every intervention has to be planned and prepared differently.
Apart from the photos, we had only a rudimentary CT scan and a written report. Later on, it turned out to be of particular importance that nobody had examined the two hearts more closely. I don't know what we would have done if we had known how weak one of them was. Maybe we would not have accepted the case. Without having more information, we agreed to help.
Then the family was flown to Boston. Right from the airport they sent me a text. They were so excited to be in America. Two or three days later, they came for their first visit to my office. The parents were desperate: They loved their children, but the situation was unbearable. They didn't know how to care for these twins. How should they feed or dress them? Customized strollers of this nature didn't even exist. And now imagine carrying around two joined children, with four arms and three legs.
We began with the examinations. The two girls were attached side by side. They couldn't walk, but one of them had begun to speak. She was a bit bigger, and also the livelier of the two.
'They Embraced Each Other'
They had very different personalities, even though they both were, like all conjoined twins, genetically identical: One was active and outgoing, the other more withdrawn. But they seemed to get along well: They played together and embraced each other.
We had to find out in as much detail as possible how they were attached. They clearly had two hearts, two lungs and two stomachs. But where did their guts join? How was the common pelvis organized? Which parts were supplied by the nerves and arteries of which body?
During separation it is important to follow the bloodstream. We learned that we would have to give the third, though malformed leg to the bigger, stronger one of the two girls, because it was her body that supplied it with blood.
CT scans and a 3-D model of the twins' skeletonFoto: The New England Journal of Medicine
In the echocardiogram and the cardiac CT scan, we discovered that the smaller, weaker of the two girls suffered from congenital heart disease. The oxygen content of her blood was rather low. Also, her complexion wasn't healthy. They were both black, of course, but one had a pink, robust color, while her sister looked more ashen.
Then she became ill, an infection of the respiratory tract. Usually this would not have been very severe, but her body could not tolerate it, because her lungs had very poor blood supply. The twins had to be moved to the intensive care unit. The oxygen content in her blood sank to a life-threateningly low level and she turned bluish. At this point, we realized that she was going to die. Her heart was just too weak to take care of her body alone. She was hanging on her sister's drip.
But what did that mean for the upcoming operation? For one girl, her seriously ill sister posed a life-threatening danger. If she died, the other would live only a few more hours. For the other, however, the healthy sister was a life support. The 3-D model we made of the skeleton and the blood vessels of the twins clearly shows the artery running from one body to the other right across the lower chest, supplying it with oxygen-rich blood. We knew that if we separated them, we would have to cut that lifeline.
We sought advice from the pediatric ethics committee of our hospital. In many extensive conversations, I learned how important it is how to frame such a situation: Our intent was not to end the life of one girl, but rather to save the other's. The difference is subtle, because the result would be the same: We would push two living children into the operating room and leave it with only one.
Not all of us followed the argument. Three doctors -- two surgeons and one anesthesiologist -- stepped back. They said they could not participate in such a thing. The idea that our intervention would likely lead to the death of the weaker girl seemed unfathomable to them. We respected this decision.
We also discussed the case at length with the parents. We had to be sure that they wanted the separation, even if it meant the death of one of their daughters. And we wondered how the girl herself would have decided: If she had to die herself, would she have wanted her sister to live? Or would she have preferred that they die together? Although I will never know the answer, it comforts me to believe that I acted in her interest.
For the operation we put together a team of around 50 surgeons, anesthesiologists, nurses and technicians. When we started the skin incision we knew that the chances of survival for the weaker twin sister were very low. But we were not without hope: Maybe we would be able to keep the girl stable for at least a few hours after separation, so that the parents could say goodbye to her.
The operation proceeded just as we had planned it. We had separated the twins' fused liver, we had divided the small intestine, and we had split the complexly organized pelvis. In the case of the skin it was difficult to determine exactly which parts belonged to which child. We injected fluorescent dye into the vessels of the stronger girl. Then we switched off the light and marked the glowing area. The parts that glowed stayed with the stronger girl.
Finally, more than 13 hours had passed, and the children were almost entirely separated, except for some tissue and skin at their backside. And the condition of both was still amazingly good. Peoples' hope was increasing: Wow, we thought, they might both survive!
However, we had left the transection of the crucial artery to the end, because we did not want to unnecessarily risk an early death of the weaker sister. But now, at this point, the cut had to be made. First, we squeezed the vessel as a test. The blood pressure in the smaller girl's body dropped immediately. The effect was dramatic.
I don't remember who made the crucial cut, but it was me who tied it down. Actually, she died very abruptly. The anesthesiologist tried to stabilize her condition with fluids and medication. But very soon he had to give up.
It took another 15 or 20 minutes for the plastic surgeons to cut the back muscles. Then we pushed the dead body into a neighboring operation room. There, we closed the body and wrapped her in a way that the parents could only see the face, which looked fine.
A team came to bring the girl up to her parents. I, for myself, sought refuge in a third operation room, and I cried there.
The surviving girl is doing great. She's happy. She has one major problem: One of her legs is a so-called "duplicated leg." The foot ends in a big toe, with three little toes on each side of it. This leg has also two tibias and no knee joint. She will never be able to use it.
The article you are reading originally appeared in German in issue 4/2018 (January 20th, 2018) of DER SPIEGEL.
After the operation, we recommended amputation. We told the parents that children at such early age learn to use a prosthesis almost as well as their own limb. But for the parents, there was no question: "That is the last thing we're going to do," they said. Once again, I realized that this case is challenging not only for surgical and ethical reasons but also because of the complicated cultural component.
We see her regularly for follow-up examinations. Once, when she visited us about three months after the operation, I gave her a stuffed animal. She smiled and said something to her father. He translated it for me: "She says: 'This is my sister.'"
Somewhere deep inside, she seems to remember that once there was a twin sister with her.