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With so few individuals actually surviving until birth, the only treatment option is surgery to try to remove the parasitic twin. Surgery, however, is very dangerous and has been successful only once. The problem with surgical intervention is that the arterial supplies of the head are so intertwined that it is very hard to control the bleeding, and it has been suggested that cutting off the parasitic twin's arterial supply might improve the odds of the developed twin's survival.
Craniopagus twins are conjoined twins that are fused at the cranium. This condition occurs in about 10–20 babies in every million births in the United States. Among this small group, cephalic conjoining, or craniopagus twinning, represents the rarest of congenital abnormalities, accounting for 2–6% of all conjoined twins. Additionally, conjoined twins are genetically identical and always share the same sex. The union in craniopagus twins may occur on any portion of the Calvary, but does not include either the face or the foramen magnum. The thorax and abdomen are separate and each twin has its own umbilicus and umbilical cord. The union may involve the entire diameter of the head or only a small portion. This suggests that although there are many different kinds of vulnerabilities already known in the scientific community, there are an infinite number of variations that can occur. Most of these variations are based on the rotation of one twin's skull to the other and the different phenotype sub-groups of craniopagus twins are based on all these rotational conformations. Each of these factors (rotation, spot of union) affects the development of the brain, the vascular system within the brain and overall wellness of life both of the twins have outside the womb. Relatively few craniopagus twins survive the perinatal period – approximately 40% of conjoined twins are stillborn and an additional 33% die within the immediate perinatal period, usually from organ abnormalities and failure. However 25% of craniopagus twins survive and can be considered for a surgical separation and several attempts occur yearly worldwide. In the last-half century, many advances in medicine including brain imaging, neuro-anesthesia and neurosurgical techniques have proven that a successful outcome is possible following separation of total craniopagus twins.
Only ten cases of craniopagus parasiticus have been reported in the medical research literature. Of those cases, only three have survived birth. The first case on record is that of Everard Home's Two-Headed Boy of Bengal, whose skull is preserved at the Hunterian Museum at the Royal Society of Surgeons.
Following methods could serve as prevention: carrying the infant and tummy time.
Preliminary research indicates that some babies with plagiocephaly may comprise a high-risk group for developmental difficulties. Other research suggests that while developmental delay is more commonplace among babies with plagiocephaly, it cannot be inferred that plagiocephaly is the cause of the delay.
Surgery to separate conjoined twins may range from very easy to very difficult depending on the point of attachment and the internal parts that are shared. Most cases of separation are extremely risky and life-threatening. In many cases, the surgery results in the death of one or both of the twins, particularly if they are joined at the head or share a vital organ. This makes the ethics of surgical separation, where the twins can survive if not separated, contentious. Alice Dreger of Northwestern University found the quality of life of twins who remain conjoined to be higher than is commonly supposed. Lori and George Schappell and Abby and Brittany Hensel are notable examples.
In 1955, neurosurgeon Harold Voris (1902-1980) and his team at Mercy Hospital in Chicago performed the first successful operation to separate Siamese twins conjoined (Craniopagus twins) at the head, which resulted in long-term survival for both. The larger girl was reported in 1963 as developing normally, but the smaller was permanently impaired.
In 1957, Bertram Katz and his surgical team made international medical history performing the world's first successful separation of conjoined twins sharing a vital organ. Omphalopagus twins John Nelson and James Edward Freeman (Johnny and Jimmy) were born to Mr. and Mrs. William Freeman of Youngstown, Ohio, on April 27, 1956. The boys shared a liver but had separate hearts and were successfully separated at North Side Hospital in Youngstown, Ohio by Bertram Katz. The operation was funded by the Ohio Crippled Children's Service Society.
Recent successful separations of conjoined twins include that of the separation of Ganga & Jamuna Shreshta in 2001, who were born in Kathmandu, Nepal, in 2000. The 197-hour surgery on the pair of craniopagus twins was a landmark one which took place in Singapore; the team was led by neurosurgeons Chumpon Chan and Keith Goh. The surgery left Ganga with brain damage and Jamuna unable to walk. Seven years later, Ganga Shrestha died at the Model Hospital in Kathmandu in July 2009, at the age of 8, three days after being admitted for treatment of a severe chest infection.
A case of particular interest was that of infants Rose and Grace ("Mary" and "Jodie") Attard, conjoined twins from Malta who were separated in Great Britain by court order over the religious objections of their parents, Michaelangelo and Rina Attard. The surgery took place in November, 2000, at St Mary's Hospital in Manchester. The operation was controversial because Rose, the weaker twin, would die as a result of the procedure as her heart and lungs were dependent upon Grace's. (The twins were attached at the lower abdomen and spine.) However, if the operation had not taken place, it was certain that both twins would die. Grace survived to enjoy a normal childhood.
In 2003 two 29-year-old women from Iran, Ladan and Laleh Bijani, who were joined at the head but had separate brains (craniopagus) were surgically separated in Singapore, despite surgeons' warnings that the operation could be fatal to one or both. Their complex case was accepted only because high tech graphical imagery and modelling would allow the medical team to plan the risky surgery. Unfortunately, an undetected major vein hidden from the scans was discovered during the operation. The separation was completed but both women died while still in surgery on July 8, 2003.
The exact nature of how conjoined twins develop inutero remains unclear. Embryologists have traditionally attributed identical twinning as "splitting or fission" of either the inner cell mass of pleuripotential cells or early embryonic disc at 13–14 days of gestation just before the primitive streak. Some theorists suggested that conjoined twins develop as a result of the failed fusion of a single fertilized ovum. However a new hypothesis suggests that cranial fusion occurs between two separate embryos prior to the end of the 4th week of gestation. This is able to happen because the cranial neuropore is still open which is responsible for the ultimate fusion and formation of the brain stem and central nervous system. Furthermore, this secondary fusion of embryonic discs could implicate that intact skin will not fuse to other intact skin, including the ectoderm of the embryo. This means that two embryonic discs could only unite in locations where the ectoderm is absent. Moreover, the fusion occurs from neural folds of two separate, dorsally oriented embryonic discs, and the union can occur only after the ectoderm is disrupted to allow the neural and surface ectodermal layers to separate from each other.
The union in craniopagus twins may happen at any portion of the calvarium. The juncture can involve either the entire diameter of the head or any portion of the head and can be positioned at a multitude of rotational angles. In fact, craniopagus twins are rarely found in a symmetrical union. Apart from this, the vertebral axes may have a straight line. Despite this, the angle of the vertebrae is the ultimate dictator in how the individuals heads actually face. The majority of twins face either the same way or the exact opposite direction.
Many reviews suggest a practical four-category system that breaks down the craniopagus twins on the basis of vertical or angular configuration or on the basis if there were shared dural venous sinuses. This scheme was applied to 64 cases and has adequately described sets of twins for over the last 86 years.
A parasitic twin (also known as an asymmetrical or unequal conjoined twin) is the result of the processes that also produce vanishing twins and conjoined twins, and may represent a continuum between the two. Parasitic twins occur when a twin embryo begins developing in utero, but the pair does not fully separate, and one embryo maintains dominant development at the expense of its twin. Unlike conjoined twins, one ceases development during gestation and is vestigial to a mostly fully formed, otherwise healthy individual twin. The undeveloped twin is defined as parasitic, rather than conjoined, because it is incompletely formed or wholly dependent on the body functions of the complete fetus.
The independent twin is called the autosite.
Embryo splitting in which zygote divide asexually,
to produce identical children, is blocked by mitosis inhibitor.
Only a few individuals who did not have fatal kidney and bladder complications are known to have survived beyond birth with this condition.
Colpocephaly is usually non-fatal. There has been relatively little research conducted to improve treatments for colpocephaly, and there is no known definitive treatment of colpocephaly yet. Specific treatment depends on associated symptoms and the degree of dysfunction. Anticonvulsant medications can be given to prevent seizure complications, and physical therapy is used to prevent contractures (shrinkage or shortening of muscles) in patients that have limited mobility. Patients can also undergo surgeries for stiff joints to improve motor function. The prognosis for individuals with colpocephaly depends on the severity of the associated conditions and the degree of abnormal brain development.
A rare case of colpocephaly is described in literature which is associated with macrocephaly instead of microcephaly. Increased intracranial pressure was also found in the condition. Similar symptoms (absence of corpus callosum and increased head circumference) were noted as in the case of colpocephaly that is associated with microcephaly. A bi-ventricular peritoneal shunt was performed, which greatly improved the symptoms of the condition. Ventriculo-peritoneal shunts are used to drain the fluid into the peritoneal cavity.
Sirenomelia, alternatively known as Mermaid syndrome, is a rare congenital deformity in which the legs are fused together, giving them the appearance of a mermaid's tail as the nickname suggests.
This condition is found in approximately one out of every 100,000 live births (about as rare as conjoined twins) and is usually fatal within a day or two of birth because of complications associated with abnormal kidney and urinary bladder development and function. More than half the cases of sirenomelia result in stillbirth and this condition is 100 times more likely to occur in identical twins than in single births or fraternal twins. It results from a failure of normal vascular supply from the lower aorta in utero. Maternal diabetes has been associated with caudal regression syndrome and sirenomelia, although a few sources question this association.
VACTERL-H is an expanded form of the VACTERL association that concludes that this diagnosis is a less severe form of sirenomelia. The disorder was formerly thought to be an extreme case of caudal regression syndrome; however, it was reclassified to be considered a separate condition.
Stem cell therapy is considered a very promising treatment for patients with colpocephaly. Oligodendroglial cells can be used which will increase the production of myelin and alleviate symptoms of colpocephaly. Damage to the developing oligodendrocytes near the cerebral ventricles causes cerebral palsy as well as other demyelinating diseases such as multiple sclerosis and leukodystrophies. Demyelination reduces the speed of conduction in affected nerves resulting in disabilities in cognition, sensation, and motor. Therefore, by using oligodendrocyte stem cells the effects of cerebral palsy can be treated and other symptoms of colpocephaly can be alleviated.
In humans, as in other animals, partial twinning can result in formation of two heads supported by a single torso. Two ways this can happen are dicephalus parapagus, where there are two heads side by side, and craniopagus parasiticus, where the heads are joined directly.
Two-headed people and animals, though rare, have long been known to exist and documented.
For every 200,000 live births, conjoined twins are born. Conjoined twins are identical and of the same sex one hundred percent of the time and are more common in females than in males. For surgical separations the survival rate of at least one twin surviving is approximately 75%.
Anastasia and Tatiana Dogaru
born August 29, 2004) are craniopagus conjoined twins. They were scheduled to begin the first of several surgeries to separate them at Rainbow Babies and Children's Medical Center in Cleveland, Ohio. However, in August 2007 the surgery was called off as too dangerous.
The twins were born in Rome, Italy to Romanian parents, Alin Dogaru, a Byzantine Catholic priest, and Claudia Dogaru, a nurse. Their mother heard about the successful separation of Egyptian-born twins who were also joined at the head and hoped her children could also be successfully separated. The Dogaru family — who also have an older daughter, Maria, and younger son Theodor — were brought to north Texas by the World Craniofacial Foundation to have Anastasia and Tatiana evaluated for possible separation.
The girls are currently developing normally for their age and speak both Romanian and English. They get around with Anastasia leading the way and Tatiana following. The top of Tatiana's head is attached to the back of Anastasia's. Anastasia, whose kidneys don't function, relies on her sister's kidneys, and Tatiana on her sister's circulatory system. The girls also share blood flow to the back of the brain and some brain matter. Doctors estimated the twins had only a 50 percent chance of surviving the surgery. There were also risks of complications, such as brain damage, but the girls also risk early death if they remain conjoined. Their parents believed separation would give them their best chance at living a normal life.
In May 2007, doctors used a catheter to insert wire coils into the veins of the two girls, successfully redirecting their blood flow. It was the first time the procedure was attempted in conjoined twins. Doctors pushed back the first of the planned separation surgeries to June 2007 while studying the complex circulatory system of the twins, but, in August of that year, decided it was too risky.
Retrospective data of over 182,000 births, with the statistical power to determine even mild associations, suggest that a single or multiple nuchal cords at the time of delivery is not associated with adverse perinatal outcomes, is associated with higher birthweights and fewer caesarean sections in births. Although some studies have found that a tight nuchal cord is associated with short term morbidity, it is unclear whether such outcomes are actually a result of the presence of the nuchal cord itself, or as a result of clamping and cutting the cord
Management of a presenting nuchal cord should be tailored to prevent umbilical cord compression whenever possible. Techniques to preserve an intact nuchal cord depend on how tightly the cord is wrapped around the infant’s neck. If the cord is loose, it can easily be slipped over the infant’s head. The infant can be delivered normally and placed on maternal abdomen as desired. If the cord is too tight to go over the infant’s head, the provider may be able to slip it over the infant’s shoulders and deliver the body through the cord. The cord can then be unwrapped from around the baby after birth. Finally, if the cord is too tight to slip back over the shoulders, one may use the somersault maneuver to allow the body to be delivered. The birth attendant may also choose to clamp and cut the umbilical cord to allow for vaginal delivery if other methods of nuchal cord management are not feasible.
A vanishing twin, also known as fetal resorption, is a fetus in a multi-gestation pregnancy which dies in utero and is then partially or completely reabsorbed. In some instances, the dead twin will be compressed into a flattened, parchment-like state known as "fetus papyraceus".
Vanishing twins occur in up to one out of every eight multifetus pregnancies and may not even be known in most cases. "High resorption rates, which cannot be explained on the basis of the expected abortion rate...suggest intense fetal competition for space, nutrition, or other factors during early gestation, with frequent loss or resorption of the other twin(s)."
In pregnancies achieved by IVF, "it frequently happens that more than one amniotic sac can be seen in early pregnancy, whereas a few weeks later there is only one to be seen and the other has 'vanished'."
Supernumerary body parts are most commonly a congenital disorder involving the growth of an additional part of the body and a deviation from the body plan. Body parts may be easily visible or hidden away, such as internal organs.
Many additional body parts form by the same process as conjoined twins: the zygote begins to split but fails to completely separate. This condition may also be a symptom of repeated occurrences of continuous inbreeding in a genetic line.
Krista and Tatiana Hogan (born October 25, 2006) are Canadians who are conjoined craniopagus twins. They are joined at the head (the top, back, and sides). They were born in Vancouver, British Columbia and are the only unseparated ones of that type currently alive in Canada. They live with their mother, Felicia Simms, in Vernon, British Columbia and often travel to Vancouver for care at BC Children's Hospital and Sunny Hill Health Centre for Children.
Based on statistics, the twins were given a 20% chance of survival at birth . At birth at B.C. Women's Hospital & Health Centre, they were described as "wriggly, vigorous and very vocal." They weighed twelve and a half pounds, not six and a half pounds as reported by some media outlets, when they were born by caesarean section.
If left untreated, the pump twin will die in 50–75% of cases.
After diagnosis, ultrasound and amniocentesis are used to rule out genetic abnormalities in the pump twin. A procedure may then be performed which will stop the abnormal blood flow. The acardiac twin may be selectively removed. The umbilical cord of the acardiac twin may be surgically cut, separating it from the pump twin, a procedure called fetoscopic cord occlusion. Or a radio-frequency ablation needle may be used to coagulate the blood in the acardiac twin's umbilical cord. This last procedure is the least invasive. These procedures greatly increase the survival chances of the pump twin, to about 80%.
The pump twin will be monitored for signs of heart failure with echocardiograms. If the pump twin's condition deteriorates, the obstetrician may recommend early delivery. Otherwise, the pregnancy continues normally. Vaginal birth is possible unless the fetus is in distress, although it is recommended that the delivery take place at a hospital with NICU capabilities.
Maria and Teresa Tapia (born April 8, 2010) were conjoined twins born in the Dominican Republic. The twins were joined by their lower chest and abdomen and were therefore classified as omphalopagus sharing a liver, pancreas, and a small portion of their small intestine. On November 7, 2011, the twins underwent a successful separation surgery at the Children's Hospital of Richmond in Virginia.
Twin reversed arterial perfusion sequence—also called TRAP sequence, TRAPS, or acardiac twinning—is a rare complication of monochorionic twin pregnancies. It is a severe variant of twin-to-twin transfusion syndrome (TTTS). The twins' blood systems are connected instead of independent. One twin, called the "acardiac twin" or "TRAP fetus", is severely malformed. The heart is missing or deformed, hence the name acardiac, as are the upper structures of the body . The legs may be partially present or missing, and internal structures of the torso are often poorly formed. The other twin is usually normal in appearance. The normal twin, called the "pump twin", drives blood through both fetuses. It is called "reversed arterial perfusion" because in the acardiac twin the blood flows in a reversed direction.
TRAP sequence occurs in 1% of monochorionic twin pregnancies and in 1 in 35,000 pregnancies overall.