Safa and Marva were born with spliced skulls, but thanks to technology and a group of specialists they got a chance for a new life.
In mid-July 2019, a three-stage operation to separate Siamese twins Safa and Marwa ended in a London hospital. They were born with accrete skulls, a condition that is extremely rare. Their department was occupied by leading experts in this field, but even with the availability of modern technology and a large team, the total operation took more than 50 hours. The story of Safa and Marva told the BBC edition.
Initially, the family was not going to separate the girls, and the money was allocated for the operation by a businessman unfamiliar to them.
In January 2017, a resident of Pakistan, Zainab Bibi, gave birth to twins at a local hospital. She knew that they would probably be Siamese, but that did not bother her – before that she had already given birth to and raised seven children. She was not allowed to see the babies right away, as she still came to herself after giving birth. The first that the girls were born with accrete skulls (this state is known as Craniopagus twins), learned their grandfather Mohammad Sadat Hussain.
“I was happy to see them, but did not know what to do with them because of the fused heads,” – said the man BBC. According to Bibi, she fell in love with her daughters at first sight. “They were beautiful: beautiful hair and white skin. I did not even think that they had grown together. God gave them, ”said Pakistani. The girls were named Safa and Marvoy – in honor of the two hills in Mecca, which played an important role in the Quran.
A month after giving birth, the family decided to go to the hospital to have an operation to separate the twins. Similar procedures took place at a local military hospital, but doctors warned that one of the girls would probably die during the operation. Bibi refused to risk.
Three months later, the family received a new chance: they contacted the children’s neurosurgeon Owase Jeelani from the leading children’s hospital in London, Great Ormond Street. He grew up in the Pakistani region of Kashmir and contacted his family by offering help. But the operation should be carried out before the girls were one year old to maximize the chances of success.
The family did not have time. In August 2018, when the twins went 19 months, the operation has not even been planned. The problem was that the London hospital did not want to allocate money for such an operation, and with its own forces, Geelani raised funds only for paying the visa and renting a hospital ward for the family.
“They flew in early August, then the money we needed was quite a bit. The children were under stress. At this stage, I perceived [what was happening] as a personal responsibility, ”the doctor recalled. Help came from an unexpected side: at lunch in a cafe, a neurosurgeon told a lawyer friend about difficulties in collecting money for an operation. She called someone at the same time.
Someone” was a Pakistani businessman Murtaza Lakhani, who listened to the story and promised to pay for the surgery and treatment. “Children were born in Pakistan, just like me. But the main reason was not this, but the fact that the operation would save two children. For me it was a simple decision, this is how the future is created, ”the entrepreneur told.
Siamese twins develop in the same egg, so they always resemble each other. There are two theories why sometimes they grow together: either the separation of two embryos occurs later than usual, and the twins are partially separated from each other, or after splitting, parts of the embryos remain connected, and these parts merge as they grow.
Often in these cases, the twins are “bound” on the chest, abdomen or pelvis. But Safa and Marwa were joined by skulls. The girls have two different brains, but they are deformed: the right hemisphere of each sticks out at 90 degrees, speaking into the brain cavity of the other twin. The main problem for doctors in the case of surgical separation is how to deal with a complex network of veins and arteries. Each twin supplies the other with blood, and breaking the bond carries the risk of brain exhaustion and stroke.
It clarifies the BBC BBC, official statistics on cases where Siamese twins have grown their heads, no. Some studies indicate that one case accounts for 2.5 million births. Most die in the first 24 hours. There is no accepted methodology for separating such twins; each case is considered separately.
Since 1952, when the first recorded operation of this kind occurred, they were performed only about 60 times. Great Ormond Street is considered the world leader in the number of such procedures. The local team of doctors believes that the best outcome is possible if the separation occurs during several operations, and not one, so that the body recovers.
In total, the hospital team consists of 100 specialists, including two bioengineers, a 3D modeller and a virtual reality designer. In the case of Safa and Marwa, Geelani took the lead in separating the girls’ brain and blood vessels, and plastic surgeon and professor David Dunaway began to restore their heads and form the upper part of the skull.
To prepare for the operation, doctors used a 3D printer and virtual reality glasses.
The first operation, attended by 20 doctors, began on October 15, 2018. Before the start of the campaign, Djeleani once again spoke about the action plan, but the participants understood how much depends on them. In case of failure, one or both of the twins could die or suffer serious brain damage.
To begin with, doctors removed three large layers of skin and bones to reach the brain. Then Djeleani went to the operating microscope, through which the microstructure of the blood vessels of the twins was viewed. By 2:30 pm, after more than five hours of operation, the specialist reached the artery of Safa, supplying Marva’s brain, and clamped it.
This is a tense moment, because every such action threatens to damage the brain. The specialists waited five minutes, made sure that the brain was fine and did not feel a lack of blood, and continued. The procedure lasted a few more hours, and in parallel the second team, headed by Dunaway, created a rigid frame of three parts of the skull: the pieces of bones were sealed with a metal grid of screws. All this in the future became the basis for the creation of full-fledged twin skulls.
As soon as the specialists cut the arteries feeding the twins’ brains, and were convinced that the body had coped with the deprivation, the first stage of the operation was over. It took her 15 hours. Two days later, the patients went home from intensive care.
As Dunway said, the case of Safa and Marwa is the most difficult of those that the team encountered. “The first two times were much easier, we were lucky. But in this case, we underestimated the complexity of connecting parts of the brain; moreover, they are older, and the older, the more difficult, ”he said. According to London experts, the ideal time for the separation of Siamese twins passes in the first 6-12 months. At this time, the brain, skin and bones recover much faster.
By the time of the operation, the Pakistani twins had already passed this threshold, but since about 2011, doctors have developed new visualization and modeling technologies that simplify the process. The development team in this area is led by Juling Ong: according to him, local devices allow you to create realistic 3D models to study the anatomy of twins in detail and plan the operation.
“These are unique cases, they are not taught this in medical schools,” explains the specialist. When the 3D model is built, it is printed on the 3D printer there. Due to this, by the beginning of the operation, the doctors received detailed samples of the skulls of patients with strips of skin.
Then Jeelani tried on virtual reality glasses, and studied the twisted vascular system. “Undoubtedly, this is the future. We are blessed here from the point of view of software engineers and specialists; we, the doctors, do not have the skills that they bring, ”says Geelani.
After the second operation, one of the girls almost died, and in the last procedure the twins constructed new skulls.
The second operation took place a month after the first. It took the doctors to separate the common veins from the girls’ brains. Each such damage threatened with a stroke, so that specialists had to act with extreme caution.
The problems began at the very beginning – the separation of part of the girls’ skulls caused bleeding, and since the last operation, blood clots have formed in the veins of the neck of Safa. They limited the flow of blood from her brain, so blood passed over to Marwa. As a result, the blood pressure of one twin became very high, while that of the other was dangerously low.
For the team, it became clear that Marva was a weaker twin. Her condition rapidly deteriorated, so she was left with one connecting vein in order to increase her chances of survival. The problem is that for Safa this meant a risk of stroke, but the doctors decided that there was no other option. After 20 hours of operation, the operation is completed.
“I sighed with relief. We thought we would lose Marv at some point, but if they wake up, as we hope, everything will be fine, ”Ong said. Later, Geelani called from home to the hospital to find out about the condition of the twins. At the other end of the wire, it was reported that Safe was bad: she was not breathing, and her skin had acquired a strange shade.
“I thought Safa was dead,” recalls the doctor. From fatigue and shock, he sat down on the floor and began to cry. “My poor wife never saw me in such a state.” She took the phone from her husband and called Danae. Soon, both surgeons returned to the intensive care unit, did a CT scan of the dying Safa, and realized that they had made a mistake during the operation.
The left connecting vein provoked a new thrombus, which led to a stroke in Safa. The next two days the girl was in a critical condition, but then her position stabilized. She again began to breathe on her own, but as a result of a stroke her left arm and leg weakened.
“For me, the most crucial moment will come when she starts to walk and fully use her left hand, because I know that I am guilty of this weakness, it’s hard for me,” shared Geelani. The doctors had to find a way to cover the upper part of the girls’ heads after separation. To do this, they used the extenders of the tissues in which they pumped saline, so that each sister’s skin eventually stretched out on top.
By February 2019, the girls had experienced a total of 35 hours on the operating table. With the arteries and veins that bind the head, it was finished, and the skin was stretched enough to cover the crown of the twins after separation. There was a final operation, during which the doctors separated the remaining joints of the bone, brain and tissues.
After this, the twins were bound only by the dura mater – it covers the brain and spinal cord and fuses with the periosteum of the base of the skull. When the last binding element was removed, the doctors gently lifted the girls and spread them in different directions. After two years of life, they were able to exist independently of each other.
They put the girls on different tables, hooked them up to life-tracking devices, and began to construct new skulls for them, using their own twin tissue. “The skull has a three-layer structure: the inner and outer layers are very dense, but it seems that there are [honeycomb] honeycombs between them, it is easy to break them. This means that we need to cover almost the whole head with a bone, ”recalled Dunaway.
Separate pieces of the girls’ new skull were fastened with disposable sutures, thus a full-fledged skull was formed from small fragments, albeit consisting of debris. The gaps between them were filled with bone cells, which in the following months closed the crevices. In the final, the reconstructed skulls pulled the skin with dilators. After 17 hours of surgery, the doctors announced that the separation was successful.
Safa and Marva are on the mend, they are taught to tumble and keep their heads
While Safa and Marwa were recovering, Jeelani and Dunaway went to Ireland to visit Rital and Ritai Gabur, twins born with the same characteristics. In 2011, when the girls were 11 months old, the doctors successfully separated them, and since then the only visible reminder of the surgical intervention remains a slight asymmetry in the shape of their heads.
Ritala and Ritan were born in 2010 in Sudan. They were taken to a London hospital in critical condition, without surgery to separate them, they were threatened with death. As the doctors recall, their case turned out to be the most successful. Unlike Safa and Marwa, their brains were not deformed. A four-stage separation operation was carried out in three months. A few weeks left for recovery.
In September 2019, girls will be nine years old – Ritali was diagnosed with autism, she goes to a special school, and her sister demonstrates a standard level of development for her age and goes to a regular school. For the first time, Djeelani talked with the girls’ family when they were three months old, but due to lack of funding, the operation could not be carried out earlier.
The specialist is convinced that if the money had been found earlier, the result would have been better. “We assume that they will have problems with movement and intellectual functions. But the mom [of the girls] is glad that everything ended as it ended. She understands that they would die if we did not give them a chance, ”says the surgeon.
In mid-July 2019, it was time to discharge Safa and Marwa. During the five months of recovery, extra skin was transplanted to the back of their heads, and they also receive daily physiotherapy sessions, where they learn to ride, sit and hold their heads. They feel good and began to show the same playful mood. When the family left the hospital, they were escorted by Djeelani and Dunaway, as well as other team members.
The family will spend the next six months in London, where the girls will continue to attend physiotherapy sessions and go to hospital checks. In early 2020, they plan to return to Pakistan. Although their mother, Bibi, from birth did not embarrass the condition of girls, she recognized that their separation was the right decision. “I’m very glad. With God’s grace, I can hold one for an hour and then another. God answered our prayers, ”she concludes.
Surgeons Djeelani and Dunaway, who led the operation to separate Safa and Marwa, also created the Gemini Untwined Charitable Foundation to raise funds for research on Siamese twins and future operations