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Facts About the Twins! !

Births of conjoined twins, whose skin and internal organs are fused together, are rare. Conjoined twins occur once every 200,000 live births, and their survival is anything but assured.! ! Approximately 40 to 60 percent of conjoined twins arrive stillborn, and about 35 percent survive only one day. The overall survival rate of conjoined twins is somewhere between 5 percent and 25 percent.! ! For some reason, female siblings seem to have a better shot at survival than their male counterparts. Although more male twins conjoin in the womb than female twins, females are three times as likely as males to be born alive. Approximately 70 percent of all conjoined twins are girls.! ! How They Are Formed! ! Conjoined twins are genetically identical, and are, therefore, always the same sex. They develop from the same fertilized egg, and they share the same amniotic cavity and placenta.! ! Twinning occurs one of two ways: either a woman releases two eggs instead of the usual one or she produces only one egg that divides after fertilization. If she releases two eggs, which are fertilized by separate sperm, she has fraternal twins. When a single, fertilized egg divides and separates, she has identical or paternal twins.! ! In the case of conjoined twins, a woman only produces a single egg, which does not fully separate after fertilization. The developing embryo starts to split into identical twins during the first few weeks after conception, but stops before the process is complete. The partially separated egg develops into a conjoined fetus.! ! A History of Conjoined Twins! ! One of the earliest documented cases of conjoined twins were Mary and Eliza Chulkhurst. They were born in Biddenden, County of Kent, England in the year 1100, and were joined at the hip.! ! The wealthy sisters, who were known as the Biddenden Maids, lived for 34 years. When they died, they left a small fortune to the Church of England. In honor of their generosity, it was customary for English citizens to bake little biscuits and cakes in the sisters' images and give them to the poor. ! ! Another set of famous conjoined twins was Eng and Chang Bunker, who were born in Thailand (then called Siam) in 1811. The term Siamese twins was coined as a reference to Eng and Chang, who achieved international fame shortly after leaving Siam as teenagers.! ! They were joined at the lower chest by a narrow band of flesh, which connected their livers. They were exhibited in circus shows around the world before settling in the United States, where they married two sisters and had nearly two dozen children. They were successful businessman and ranchers in Wilkes County, North Carolina, where they lived until 1874. They were 63 years old when they died.! ! The term Siamese twins is no longer considered appropriate. Conjoined twins aren't limited to any racial or ethnic group and indeed have been born all over the world.! ! Various Types of Conjoined Twins! ! There are nearly a dozen di"erent types of conjoined twins. One of the most common classifications is thoracopagus twins. These twins are connected at the upper portion of the torso.! ! Thoracopagus twins share a heart, which, depending on how closely they are joined, makes it nearly impossible to separate them and save them both. Thoracopagus twins make up about 40 percent of all conjoined cases.! ! Another common type of conjunction is called omphalopagus, where twins are connected from the breastbone to the waist. About 33 percent of all conjoined cases are categorized as omphalopagus. These twins may share a liver, gastrointestinal or genitourinary functions, but rarely share a heart.! ! One of the rarest types of conjoined twins is craniophagus twins, which are joined at the cranium or head. In fact, only 2 percent of all conjoined twins are joined in this way.! ! Separating Twins: No Easy Matter! ! The surgical separation of conjoined twins is a delicate and risky procedure, requiring extreme precision and care. Therefore, the decision to separate twins is a serious one.! ! Mortality rates for twins who undergo separation vary, depending on their type of connection, and the organs they share. For example, twins joined at the sacrum at the base of the spine have a 68 percent chance of successful separation, whereas, in cases of twins with conjoined hearts at the ventricular (pumping chamber) level, there are no known survivors.! ! Although success rates have improved over the years, surgical separation is still rare. Since 1950, at least one twin has survived separation about 75 percent of the time.! ! It is only after twins are born that doctors can use magnetic resonance imaging, ultrasound and angiography to find out what organs the twins share. In order to determine the feasibility of separation, doctors must carefully assess how the twins' shared organs function.! ! After separation, most twins need intensive rehabilitation because of the malformation and position of their spines. The muscles in their backs are constantly being flexed and they often have a di#cult time bending their backs forward and backwards and sitting up straight.! ! For patient inquiries or to make an appointment call 1-800-492-5538.! ! This page was last updated: July 23, 2013! ! Average rating (1013)! ! Give us feedback: (up to 255 characters)! ! ! !

Wow, there aren't just two-headed snakes, but two-headed sheep, pigs, cats, dogs, fish, and even people! With two separate heads (and two separate brains), how can they survive? Wouldn't the two heads fight with each other? And how would they control their body?! ! Well, they do have a lot of problems and most of them don't survive very well in the wild. They can't decide which direction to go and sometimes they even try to attack and swallow the other head!! ! Two-headedness is really a severe example of conjoined twins. These twins happen when identical twins don't separate completely. Instead of two twins, you end up with twins who are still attached.! ! The attachment can be as little joining as just a piece of skin or cartilage connecting the two twins. Or in more severe cases, they can share a whole body but separate heads!! ! Ending up with Two Heads! ! Identical twins happen when a single egg is fertilized but then splits into two separate embryos. Each embryo then goes on to grow into a separate person. Depending on when the split happens, twins can either grow in their own sac with their own placenta or they can share a sac and placenta.! ! Conjoined twins can happen in this second case. But it isn't common"$in humans it only happens in around 1 in 50,000 to 200,000 pregnancies.! ! There are two theories for how conjoined twins come about. One is that they happen when the fertilized egg splits incompletely. Another is that the embryo split is complete but for some reason the two new embryos re-fuse later on.! ! Twins can end up fused at various places. Where the twins end up attached depends on a lot of factors, like how they were oriented in the womb, the time and location of splitting, etc.! ! Sometimes, one of the twins can end up more dominant than the other. In this case, one of the twins can die and be absorbed by the other twin. If the absorption is complete, one of the twins vanishes. But, if the absorption is just partial, the remaining twin can have extra arms, legs, or even heads that are leftover from that other fetus!! ! Having conjoined twins doesn't usually run in families. This is because identical twinning doesn't run in families either. It is a random event.! ! But some cases of having extra limbs can be genetic. One of the best understood is polydactyly, which is when people have extra fingers and toes.! ! Genetic Causes of Duplication! ! There are lots of genetic ways to end up with extra fingers and toes. Here I'll focus on just one case.! ! Most cases of polydactyly can be accounted for by changes in just one of a few di"erent genes. This is surprising because making fingers is such a complicated task. Lots of di"erent genes all need to work together to make 10 fingers and 10 toes.! ! So how can just one change in a gene cause something like an extra finger? Because this one gene can a"ect lots of other genes, too.! ! Recall that genes are our basic instruction manual for building and running all the things in our body. These genes are "read" by cells and made into proteins. Each protein then goes on to do a specific job in the cell.! ! Some proteins like hemoglobin carry our oxygen. And others like insulin help us use the food we eat.! ! It's very important that the right proteins get made and in the right amount. If we don't have enough hemoglobin, we have trouble breathing. Or if we don't have enough insulin, we get the disease diabetes.! ! That's why cells have lots of ways to control when, where and how often a gene is read. One of the ways cells do this is by using proteins called transcription factors* (TFs).! ! TFs stick to a gene and control how often the gene is read. Lots of copies of each TF are made and they usually stick to and control a bunch of genes all at once.! ! Some TFs called activators make cells make extra proteins. And some TFs called repressors cause cells to make less protein.! ! Well, one reason polydactyly happens is because of changes in a single gene, Gli3. GLI3 is one of these repressor TFs.! ! One of its jobs is to keep two genes, dHand and Sonic Hedgehog (Shh) from being read too often. When GLI3 isn't doing its job right, too much dHAND and SHh proteins get made. And too many fingers and/or toes get made, too.! ! Some people with polydactyly have mutations in GLI3 that cause it either to not work at all or that cause it to work only very weakly. So basically, the embryo ends up with too much SHh and dHAND, causing the extra fingers and toes.! ! This kind of polydactyly can be inherited from your parents. It is an autosomal dominant trait.! ! Autosomal means that boys and girls are just as likely to get it (it isn't sex-linked). Dominant means that you only need to have one copy of the polydactyly version to end up with extra fingers and/or toes. In fact, if you have one copy, you will have extra digits!! ! So if just one parent has this mutation in Gli3, their kids have a 50% chance of getting it too. If both parents have it, their kids have a 75% chance of having extra fingers or toes!! ! Polycephaly (having multiple heads or faces), or polymelia (having extra limbs) can also have some genetic causes. But, these are usually associated with other serious genetic defects (it's not well understood for either of these cases), or more frequently, are the result of twinning or another problem during development.

What Are Conjoined Twins?! Twins whose bodies are connected are called conjoined twins.! Conjoined twins begin as a single fertilized egg. Usually a single fertilized egg develops into a single baby. Sometimes a single egg divides in half during the first one to two weeks after it’s fertilized. This creates a set of identical twins.! The exact cause of conjoined twinning is not known. There are two theories. One is that the egg divides late and does not divide completely. The other is that the egg divides completely but then fuses (joins) back together.! The connection between the twins’ bodies may be fairly simple. They may share only a small amount of tissue, and both children may have all the organs and other structures they need. For example, the twins may be joined at the belly with a “bridge” that connects their livers.! Usually the connection is more complex, and sometimes it is very complex. The children may share:! Vital organs, like one heart! Many structures, like several parts of their digestive, genital and urinary systems! A large segment of their body, like all of their lower body! Part of the brain and skull! Types of conjoined twins! Doctors group conjoined twins based on where they are joined.! Joined at the chest, called thoracopagus. This is the most common type. About 40% of conjoined twins are in this group. These twins are face to face. In about 75% of cases, these twins share one heart. Twins joined at the chest may also share their liver, biliary tract (which carries bile from the liver to the small intestine) and upper digestive tract.! Joined from the breastbone to the waist, called omphalopagus or xiphopagus. These twins are face to face. They may share their liver, biliary tract and upper digestive tract. About 35% of conjoined twins are in this group.! Joined at the sacrum and buttock area, called pygopagus. These twins are back to back. They may share part of their lower digestive tract and parts of their skeleton, nervous system and genitals. About 20% of conjoined twins are in this group.! Joined in the pelvic area, possibly up to the breastbone, called ischiopagus. These twins may be oriented to each other in di"erent ways. In general, they partly face each other. They may share their liver and biliary tract, part of their upper and all of their lower digestive tract, their genital and urinary systems and part of their skeleton. About 6% of conjoined twins are in this group.! Joined at the head, called craniopagus. These twins may share their skull, brain and other parts of their nervous system. About 2% of conjoined twins are in this group.! Outlook for Conjoined Twins! Most sets of conjoined twins do not survive because their organs cannot support them. About 40% of conjoined twins are not alive when they are delivered (stillborn). About 35% die within a day after they are born.! Survival! Even so, more conjoined twins survive now than in the past. Advances in imaging, surgical techniques and anesthesia have helped improve chances for survival. We also have tools to detect their condition before birth (prenatally). This means their families and doctors can plan their early care before they are born.! Usually this includes scheduling delivery by cesarean section (C-section) a month before their due date. This is because a vaginal birth is too hard for mother and babies.! Among the conjoined twins who survive more than a day after birth, some continue to live for days, weeks, months or years while conjoined. Some live into adulthood still physically connected to each other.! Some have surgery to be separated, usually in the first year of life. The success of this surgery depends on many factors, mainly where the twins are connected and which structures they share. In some cases, both twins survive after surgery. In some cases, only one survives, or neither does.! Making decisions! Each set of conjoined twins is unique. Decisions about their care are complex and often hard for their families and their healthcare providers. The chance for survival is one important factor in these decisions. Another important factor is the likely quality of the twins’ lives if they stay conjoined or are separated.! Parents who find out they are pregnant with conjoined twins can have imaging studies to learn more about their twins’ condition. Usually these are ultrasound and MRI (magnetic resonance imaging). They can consult with doctors who care for conjoined twins about what to expect, including whether separation might be possible. Some parents continue their pregnancy, and some decide to end their pregnancy.! For parents continuing their pregnancy, doctors will suggest careful monitoring and planning. This helps their healthcare team prepare for the diagnostic tests and provide the treatment the twins may need once they are born.! After delivery, the twins will likely need many types of imaging studies and tests to learn as much as possible about how they are connected. This information will help the family and healthcare team decide how to care for the twins.! Symptoms of Conjoined Twins! Conjoined twins may be connected to each other in many di"erent ways. Their health and any symptoms depend on how each child developed, which structures they share and how well their organs work.! Most conjoined twins are born early (prematurely). This means their lungs probably have not finished developing. So breathing problems are common in many conjoined twins.! Conjoined Twins Diagnosis! In most cases, doctors see that twins are conjoined during a routine ultrasound late in the first trimester of pregnancy or in the second trimester. Once your doctor can see by ultrasound where the twins are joined, the doctor will have some idea of which structures the twins are likely to share. Other imaging studies can also take pictures of the twins before birth. They include echocardiography and prenatal MRI (magnetic resonance imaging). These may give your doctor more details.! After birth, conjoined twins need many other imaging studies and tests to learn about their health and the connections between them. Your doctor will want to find out more about their anatomy, or structure, and their function, or how well their bodies work. It will be important to check all of their systems.! The exact studies and tests your babies need will depend on where they are joined and how their health seems. Heart (echocardiography and electrocardiography) and lung (pulmonary) tests are a few of the common tests for conjoined twins.! Conjoined Twins Treatment Options! The treatment that conjoined twins may need depends on the health of each twin and where and how they are joined.! Creating a treatment plan! The connection between the twins’ bodies may range from fairly simple to very complex. Both children may have all the organs and other structures they need, or they may share vital organs, like their heart, or other structures. Their bodies may be able to support both their lives, or it may be hard for one or both to survive because of health problems. So treatment for each set of twins is unique.! Before birth and in the months soon after birth, your twins’ healthcare team will gather as much information as they can about your children’s anatomy, or structure, and their function, or how well their bodies work. The team will use this information to create a detailed treatment plan. The main goal is to give both children the best chance for a good quality life whenever possible.! Deciding about separation surgery! Your twins’ treatment plan may or may not include surgery to separate them. Separation surgery may give the best or only chance of survival for one or both twins. However, in some cases, staying joined gives the twins their best or only chance to live.! Your healthcare team will also talk with you about what your twins’ lives may be like whether they are joined or separated. They will help you consider which option is best for your twins.! Some conjoined twins continue to live for days, weeks, months or years — even into adulthood — still physically connected to each other. It may be hard for many of us to imagine what this might be like or how this could be a good life. For most of us, having a distinct, separate body is basic to our experience as people; it’s our “normal.” For conjoined twins, being connected to their sibling is “normal.” Some conjoined twins have happy, healthy, full lives by staying connected. For some twins, separation surgery is too di#cult. So some stay connected because it’s the only real option.! Some conjoined twins do have surgery to be separated. Usually this takes place in the first 6 to 12 months of life. This may be the best option for both children’s physical health, while also giving them the chance to have independent bodies and lives.! Sometimes separation surgery is the best option because the twins’ shared organs cannot support both of them. Or it may be the best option because one twin is unlikely to survive and separation is the only way to give the other twin a chance to live.! In some cases, conjoined twins need to be separated before they are 6 months old. Sometimes they need to be separated right after they are born because of a health emergency or an urgent problem that cannot wait.! When separation is planned! Surgery to separate conjoined twins requires detailed planning whenever possible. To prepare for surgery, your twins will have a large team of healthcare providers who are experts in many di"erent types of care. They will work together to plan the steps and timing of the surgery and the care your children will need before and after. One doctor will oversee the team and coordinate all parts of the plan.! In most cases, doctors will wait to do the separation surgery until your twins are at least 4 months old. This gives their bodies a chance to grow so it’s easier for doctors to do the surgery and easier for the twins to withstand the surgery. This also allows time for the many imaging studies and tests needed to learn about your twins’ bodies.! Each twin will probably have his or her own complete team for the surgery. Once your twins are separated, each may need more surgery to correct some of the anatomic (structural) problems they may have.! Follow-up care! Your twins will need long-term follow-up visits to check their growth and development whether they stay connected or are separated. Most will need surgeries or other types of treatment for health issues that arise over time. Families of conjoined twins can expect to keep a close relationship with the children’s healthcare team throughout ! their childhood.