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Article: Shared Lives

From the types of joining to separation surgeries, the issues around conjoined twins are varied and complex.
 

Cynthia M Piccolo

With years of experience in healthcare recruitment and a love of the English language, Cindy provides a relevant editorial direction to the content side of MedHunters.com. Cindy's quick wit comes through clearly in her writing, and, though she doesn't like to be described as an intellectual, the number of letters after her name give her that distinction. Read more.

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Shared Lives

By Cynthia M Piccolo

Conjoined twins have long been a source of awe. The 16th-century French doctor Ambroise Paré developed numerous possible explanations for conjoined twins: God's wrath (or God's desire to show power); the devil's influence; something the pregnant woman looked at; too tight a womb; too tight clothes; and the way a woman sat while pregnant. Even today, there are contrasting explanations. Currently, most of the literature states that conjoined twins are formed when a fertilized egg fails to separate properly. However, in her 2003 book, Conjoined Twins: Developmental Malformations and Clinical Implications, Dr. Rowena Spencer says that, based on embryology, "the only logical explanation of the origin of conjoined twins would seem to be … the secondary union of two originally separate embryonic discs into one of only two areas – the dorsal neural tube or the ventral yolk sac." That is fusion, not improper fission.

Conjoined twins are rare – they occur approximately once every 50,000 to 100,000 births, and approximately 75% of conjoined twins are stillborn or die within 24 hours. The overall survival rate is estimated at between 5 and 25%. Although more male twins are conjoined in utero, females are three times more likely to be born alive, resulting in approximately 70% of conjoined twins being girls. And contrary to what was represented in the December 2003 movie Stuck on You, conjoined twins are always identical.

Conjoined twins are usually defined by where they are joined, using the suffix "pagus" (from Greek, pagos, which means "that which is fixed"). Modern definitions of conjoined twin types are:

Cephalopagus: About 11% of cases. These twins have ventral (frontal) union, involving both the head and the chest. Usually there are two faces on opposite sides of the head, but in very rare cases, there is one face or no face. These twins do not survive.
Craniopagus: About 5% of cases. The twins are joined at the dorsal (rear) or upper part of the skull. With advances in technology, more successful separations have occurred, but usually with some brain damage. An unsuccessful attempt at separation involved adult Iranian sisters Ladan and Laleh Bijani, who were joined at the side of the head and underwent surgery in July 2003. Meanwhile, a successful example is "the Egyptian Twins," toddlers Ahmed and Mohamed Ibrahim, who underwent surgery in Dallas, Texas, in October 2003.
Ischiopagus: About 11% of cases. The twins have ventral (frontal) union at the pelvis and usually share internal organs in the lower abdomen and of the genitourinary tract. The separation survival rate is about 63%, though excretory and sexual functions can be impaired after separation. An example of this type of twin is the Maltese babies, known as Jodie and Mary.
Omphalopagus: About 18% of cases. The twins have ventral (frontal) union at the abdomen and often share liver tissue. Twins of this type have about an 82% chance of successful separation. About 27% of this type of twins have congenital heart problems. The most famous of these were the first Siamese twins – Chang and Eng Bunker, born in Siam, who lived from 1811 to 1874, married two sisters, and had a total of 21 children.
Parapagus: About 28% of cases. These twins have lateral (side) union including the pelvis and some or all of the trunk and usually have three or four arms and two legs. Approximately 76% of this type of twins have some form of congenital heart problem. Separation sometimes succeeds. A thriving unseparated set of parapagus twins are Americans Abigail and Brittany Hensel, who were born in 1990. The girls share one trunk, two legs, and two arms. Each girl controls one leg and one arm.
Pyopagus: About 6% of cases. The twins have dorsal (rear) union at the pelvis. Separation for this type of twins has about a 68% success rate for survivability and a good outlook. The famed "Biddenden Maids," Mary and Elizabeth (or Eliza) Chulkhurst were born in England in 1100. They lived, joined, to age 34, when one twin died. Reportedly, when one sister died, doctors wanted to separate them, but the living twin refused saying, "As we came together, we will go together," and died shortly after. The local church inherited 20 acres of land at their death, and, in remembrance of the twins' generosity, food, imprinted with their image, was given to the poor each Easter. The tradition continues today.
Rachipagus: About 2% of cases. The twins are back-to-back, joined at the spine, generally from the mid portion. There have been no reported separations.
Thoracopagus: Approximately 19% of cases. These twins exhibit ventral (frontal) union at the chest, usually sharing a heart. They also have congenital heart problems. Separation has been attempted but none have survived.
Unique Cases: It is possible for conjoined twins to exhibit characteristics of more than one type (e.g., to be a cephalothoracopagus twin, joined at the head and chest). Also, there are cases of "parasites," where a child is born with part of a twin (always non-viable) attached to it. For example, in February 2004, seven-week-old Rebeca Martinez, of the Dominican Republic, underwent surgery to remove a parasitic head (belonging to an undeveloped craniopagus twin) from the top of her skull but sadly died hours afterward. There have been few cited reports of conjoined triplets, but the most famous case was of a male born in Italy, in 1831, with three heads.

Levels of Complexity

Until the mid-20th century, it was rare for conjoined twins, who survived birth, to be separated. And twins who lived were usually put on display – the sideshow life being generally the only career option. It was, at least, quite lucrative, as seen by the case of the pygopagus Hilton sisters, Violet and Daisy, who were born in England in 1908. They were purchased by an acquaintance of their mother, trained in singing, dancing, and music and exhibited in Europe and North America. After their "mother" died, the money-generating sisters were "inherited" by their "mother's" daughter and son-in-law. In 1931, they escaped from the two and, in a court case, won their independence. They died in 1969.

But as separation becomes more of an option, issues arise. Is quality of life better together or apart? Consider the example of the craniopagus sisters Ladan and Laleh Bijani, who underwent their ultimately fatal separation surgery at Raffles Hospital in Singapore in July 2003. The sisters, born in Iran in 1974, said in a letter to well-wishers, written just before surgery, "We have been praying every day for our operation. We are excited about it, as we've waited 28 years for it!" Told of the risks of surgery – a strong chance of the death and/or disability of one or both of them – they nonetheless wanted to proceed. In some respects, they had a very good quality of life while joined, even graduating from law school. But for them, taking a risky chance at independence was more important than continuing the life they were living. And as Laleh said in a June 2003 press conference, "We want to see each other – face-to-face."

Both Bijani sisters wanted separation, but what about children who are not involved in the choice? Mary and Liam Holton brought their daughters, Katie and Eilish, who were joined at the chest, abdomen, and pelvis, to Great Ormond Street Hospital in London, when the children were three. The girls, born in Ireland in 1989, were healthy, but their parents became concerned about their eventual quality of life and wanted to explore the risks and merits of separation. Ultimately, they decided on surgery, which occurred in 1992. It was later discovered that Katie had a weak heart, and she suffered heart failure and died four days after separation. Eilish lived. Professor Lewis Spitz, speaking on the BBC, said, "Following separation Eilish was clearly devastated that her twin had disappeared and for one year after surgery she would not talk to me … I regret losing Katie, but I don't regret saving Eilish for a good quality of life." A two-part documentary on the girls' story – Katie and Eilish: Siamese Twins – follows the Holtons as they make their decision and shows six-year-old Eilish, three years later, starting school.

And what about separation of infants, especially when children share vital organs? The most famous example from recent history is that of the Maltese babies (mentioned earlier), known as Jodie and Mary, who were born on August 8, 2000. The babies were of the ischiopagus type, joined at the lower abdomen and sharing a spine, though both had a nearly full set of internal organs. However, Mary's lungs and heart were not sufficiently developed, and they shared an aorta, so Jodie's heart and lungs maintained both sisters. The doctors felt that Jodie's organs could not support both babies for more than a few weeks, so wanted to separate them. The parents did not want separation, which contained some risk for Jodie and certain death for Mary; instead, they wanted to care for the babies and let nature take its course. The doctors took the case to court, and they prevailed, performing the separation surgery at St. Mary's Hospital in Manchester, on November 7, 2000. Mary, as predicted, died. Jodie survived and is expected to lead an active life, though she will most likely need further surgeries.

A few years earlier, there was the case of Angela and Amy Lakeberg, who were born in Indiana on June 29, 1993. Sharing a liver and a heart, they could not survive together, so the parents and doctors made the difficult decision to sacrifice one for the other. The stronger of the two, Angela, was chosen to be the survivor. Angela, however, succumbed to pneumonia 10 months after surgery and never made it home.

Costs

While doctors learn from successful, and unsuccessful, procedures, not only are ethical questions raised, there are high emotional and financial costs. The cost of the Lakeberg babies' surgery, which took place at Children's Hospital in Philadelphia, was more than a million dollars.

Even in cases when positive outcomes are expected for both children and the quality of life without surgery is anticipated to be poor, the prohibitive cost cannot be borne by parents. Instead, it must be borne by outside sources: healthcare workers, hospitals, charities, donations, and/or governments. The craniopagus "Egyptian Twins" Ahmed and Mohamed Ibrahim are undergoing long-term recovery at the North Texas Hospital for Children at Medical City, which is donating all hospital-related costs. But while the World Craniofacial Foundation raised the US$125,000 for Children's Medical Center Dallas (where the surgery was performed, as a down payment, the surgery alone cost upwards of US$2 million. And the website devoted to the boys says, "they will also require intensive physical therapy and rehabilitation after surgery. The physical therapy is very important to their full recovery and there is no way they can receive this care in Egypt because of the fact that the family lives 500 miles from Cairo. Therefore, there will be large and ongoing expenses to the World Craniofacial Foundation and your support is needed in any amount."

And there is the case of craniopagus Guatemalan sisters Maria Teresa and Maria de Jesus Quiej Alvarez, born July 25, 2001. Their August 2002 separation surgery at UCLA – not including the cost of the doctors, who donated their care, but including nursing, hospitalization, and medical expenses – will cost more than $1.5 million. While Maria de Jesus is doing well, Maria Teresa contracted meningitis eight months after surgery, which has caused anatomical damage to her brain.

As modern technology – MRI, CT, and advanced surgical techniques – progresses, more and more conjoined twins will be separated. But the issues involving conjoined twins remain as complex as the condition. And that is, perhaps, why stories of conjoined twins hold court in the media. Their situation and fate cause us to ask ourselves tough questions. What is the price of a life – or two? How do we define quality of life? What would you want done to you? What would you choose for your children?


Conjoined Twins International is a support group for families and twins.

Published on August 22, 2004.

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