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Diprosopus (Greek , "two-faced", from , ', "two" and , ' [neuter], "face", "person"; with Latin ending), also known as craniofacial duplication (cranio- from Greek , "skull", the other parts Latin), is an extremely rare congenital disorder whereby parts (accessories) or all of the face are duplicated on the head.
Craniopagus parasiticus is an extremely rare type of parasitic twinning occurring in about 4 to 6 of 10,000,000 births. In craniopagus parasiticus, a parasitic twin head with an undeveloped body is attached to the head of a developed twin. Fewer than a dozen cases of this type of conjoined twin have been documented in the literature. Most infants with this condition are stillborn, or die shortly after birth.
In the past, the use of terminology when describing parasitic twins has been somewhat inconsistent. By definition, a parasitic twin is joined to another twin in a certain anatomical location or position on the developed twin's body. The underdeveloped twin is termed the parasite, and the developed twin is termed the autosite. The autosite can have some abnormalities, as well. For the most part, however, it has developed enough that it can live on its own.
Diprosopus often occurs in combination with other congenital disorders, particularly anencephaly, neural tube defect and cardiac malformations. When present, the brain may show abnormalities ranging from partial to complete duplication of brain structures, and/or underdevelopment of brain tissues.
Conjoined twins are typically classified by the point at which their bodies are joined. The most common types of conjoined twins are:
- Thoraco-omphalopagus (28% of cases): Two bodies fused from the upper chest to the lower chest. These twins usually share a heart, and may also share the liver or part of the digestive system.
- Thoracopagus (18.5%): Two bodies fused from the upper thorax to lower belly. The heart is always involved in these cases. As of 2015, separation of a genuinely shared heart has not offered survival to two twins; a designated twin may survive if allotted the heart, sacrificing the other twin.
- Omphalopagus (10%): Two bodies fused at the lower abdomen. Unlike thoracopagus, the heart is never involved in these cases; however, the twins often share a liver, digestive system, diaphragm and other organs.
- Parasitic twins (10%): Twins that are asymmetrically conjoined, resulting in one twin that is small, less formed, and dependent on the larger twin for survival.
- Craniopagus (6%): Fused skulls, but separate bodies. These twins can be conjoined at the back of the head, the front of the head, or the side of the head, but not on the face or the base of the skull.
Other less-common types of conjoined twins include:
- Syncephalus: One head with a single face but four ears, and two bodies.
- Cephalothoracopagus: Bodies fused in the head and thorax. In this type of twins, there are two faces facing in opposite directions, or sometimes a single face and an enlarged skull.
- Xiphopagus: Two bodies fused in the xiphoid cartilage, which is approximately from the navel to the lower breastbone. These twins almost never share any vital organs, with the exception of the liver. A famous example is Chang and Eng Bunker.
- Ischiopagus: Fused lower half of the two bodies, with spines conjoined end-to-end at a 180° angle. These twins have four arms; one, two, three or four legs; and typically one external set of genitalia and anus.
- Omphalo-Ischiopagus: Fused in a similar fashion as ischiopagus twins, but facing each other with a joined abdomen akin to omphalopagus. These twins have four arms, and two, three, or four legs.
- Parapagus: Fused side-by-side with a shared pelvis. Twins that are dithoracic parapagus are fused at the abdomen and pelvis, but not the thorax. Twins that are diprosopic parapagus have one trunk and two faces. Twins that are dicephalic parapagus have one trunk and two heads, and have two (dibrachius), three (tribrachius), or four (tetrabrachius) arms.
- Craniopagus parasiticus: Like craniopagus, but with a second bodiless head attached to the dominant head.
- Pygopagus (Iliopagus): Two bodies joined at the pelvis.
- Rachipagus: Twins joined along the dorsal aspect (back) of their bodies, with fusion of the vertebral arches and the soft tissue from the head to the buttocks
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.
"'Conjoined twins" are identical twins joined in utero. An extremely rare phenomenon, the occurrence is estimated to range from 1 in 49,000 births to 1 in 189,000 births, with a somewhat higher incidence in Southeast Asia and Africa. Approximately half are stillborn, and an additional one-third die within 24 hours. Most live births are female, with a ratio of 3:1.
Two contradicting theories exist to explain the origins of conjoined twins. The more generally accepted theory is "fission", in which the fertilized egg splits partially. The other theory, no longer believed to be the basis of conjoined twinning, is fusion, in which a fertilized egg completely separates, but stem cells (which search for similar cells) find like-stem cells on the other twin and fuse the twins together. Conjoined twins share a single common chorion, placenta, and amniotic sac, although these characteristics are not exclusive to conjoined twins as there are some monozygotic but non-conjoined twins who also share these structures in utero.
The most famous pair of conjoined twins was Chang and Eng Bunker (Thai: อิน-จัน, In-Chan) (1811–1874), Thai brothers born in Siam, now Thailand. They traveled with P.T. Barnum's circus for many years and were labeled as the Siamese twins. Chang and Eng were joined at the torso by a band of flesh, cartilage, and their fused livers. In modern times, they could have been easily separated. Due to the brothers' fame and the rarity of the condition, the term "Siamese twins" came to be used as a synonym for conjoined twins.
Polycephaly is the condition of having more than one head. The term is derived from the Egyptian stems "poly" (Greek: "πολύ") meaning "many" and "kephalē" (Greek: "κεφάλη") meaning "head". A polycephalic organism may be thought of as one being with a supernumerary body part, or as two or more beings with a shared body.
Two-headed animals (called bicephalic or dicephalic) and three-headed (tricephalic) animals are the only type of multi-headed creatures seen in the real world, and form by the same process as conjoined twins from monozygotic twin embryos.
In humans, there are two forms of twinning that can lead to two heads being supported by a single torso. In dicephalus parapagus dipus, the two heads are side by side. In craniopagus parasiticus, the two heads are joined directly to each other, but only one head has a functional torso. Survival to adulthood is rare, but does occur in some forms of dicephalus parapagus dipus.
There are many occurrences of multi-headed animals in mythology. In heraldry and vexillology, the double-headed eagle is a common symbol, though no such animal is known to have ever existed.
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.
Two-headed people and animals, though rare, have long been known to exist and documented.
There are two categories of craniopagus twins:
- Partial Although partial craniopagus is less common than total, it is still a division of craniopagus twins that is worth exploring. This type of twinning is defined as having limited surface area involvement, with either intact crania or cranial defects. In other words, it is a defect of the cranial "coverings." In partial craniopagus twins, the unions are usually frontal and less commonly occipital and vertical. Angular frontal junctions occur when the two twins are joined at any part of the forehead. Occipital twins are joined at the occipital lobe in the back of the head and vertical are joined on the top of the head and usually face opposite directions. The junctional diameter is often smaller in partial forms and occasionally an incomplete layer of bone may be present between the twins. Each child maintains independent calvarial convexities except at the common area of skull junction. The dura of both children may be intact or deficient and cortical gyri may interdigitate. Additionally shared dural venous sinuses is usually absent or if it is present it is negligible. These twins usually undergo successful separation and both twins may live to lead normal lives.
- Total Total craniopagus twins are defined as sharing extensive surface area with widely connected cranial cavities. Among total craniopagus twins, there are four main categories which are then further divided into several subcategories. Frontal, the first category, are when twins are facing each other with the axis of the bodies forming an acute angle. Temporoparietal craniopagi are joined immediately above the external auditory meatus. The third division is the occipital anomaly where the twins are connected in the occipital lobe causing the twins to face away from each other. The final variant is the parietal craniopagus which occurs when twins fuse at the vertex with the axis of the twins forming an obtuse angle. This category is perhaps the most important, or most interesting because the craniums of the two twins share the most veins, lobes and circuitry and is often described as one brain shared by two individuals.
- "Type 1": both children face in the same general directional axis so that the angle between twins is less than 40 degrees. These twins show relatively symmetric superior bi-parietal or vertex compressional flattening.
- "Type 2": both children face opposite directions so that the deformity shows an axial rotation between 140-180 degrees.
- "Type 3": in this variety axial rotation is intermediate between the first two types with a rotation of being between 40 and 140.
The acardiac twin is a parasitic twin that fails to properly develop a heart, and therefore generally does not develop the upper structures of the body. The parasitic twin, little more than a torso with or without legs, receives its blood supply from the host twin by means of an umbilical cord-like structure (which often only has 2 blood vessels, instead of 3), much like a fetus in fetu, except the acardiac twin is outside the host twin's body. Although the reason is not fully understood, it is apparent that deoxygenated blood from the pump twin is perfused to the acardiac twin. The acardiac twin grows along with the pump twin, but due to inadequate oxygenation it is unable to develop the structures necessary for life, and presents with dramatic deformities.
Although no two acardiac twins are alike, twins with this disorder are grouped into 4 classes: Acephalus, anceps, acormus, and amorphus.
- Acephalus – The most common type, lacking a head, though it may have arms. Thoracic organs are generally absent, and disorganized & unidentifiable tissues take their place.
- Anceps – The acardius has most body parts, including a head with face and incomplete brain. Organs, though present, are crudely formed.
- Acormus – This type has no apparent body and the umbilical cord is seemingly attached to the neck, but x-rays or dissection reveal thoracic structures in the apparent head. One had a leg apparently attached to the head. This may be due to embryopathy degenerating a once normal embryo.
- Amorphus – This extreme form not only lacks a head and limbs, but also any internal organs, and consists of tissues with blood vessels branching from the umbilical cord. Some may only be stem cell tumors.
The acardiac twin may also be described as a "hemiacardius", which has an incompletely formed heart, or a "holoacardius", in which the heart is not present at all.
The diagnosis is strongly suggested by ultrasound (sonogram), but definitive diagnosis requires histopathological examination. On ultrasound, the mole resembles a bunch of grapes ("cluster of grapes" or "honeycombed uterus" or "snow-storm"). There is increased trophoblast proliferation and enlarging of the chorionic villi. Angiogenesis in the trophoblasts is impaired as well.
Sometimes symptoms of hyperthyroidism are seen, due to the extremely high levels of hCG, which can mimic the normal Thyroid-stimulating hormone (TSH).
Molar pregnancies usually present with painless vaginal bleeding in the fourth to fifth month of pregnancy. The uterus may be larger than expected, or the ovaries may be enlarged. There may also be more vomiting than would be expected (hyperemesis). Sometimes there is an increase in blood pressure along with protein in the urine. Blood tests will show very high levels of human chorionic gonadotropin (hCG).
Teratomas maybe found in babies, children, and adults. Teratomas of embryonal origin are most often found in babies at birth, in young children, and, since the advent of ultrasound imaging, in fetuses.
The most commonly diagnosed fetal teratomas are sacrococcygeal teratoma (Altman types I, II, and III) and cervical (neck) teratoma. Because these teratomas project from the fetal body into the surrounding amniotic fluid, they can be seen during routine prenatal ultrasound exams. Teratomas within the fetal body are less easily seen with ultrasound; for these, MRI of the pregnant uterus is more informative.
Teratomas are not dangerous for the fetus unless there is either a mass effect or a large amount of blood flow through the tumor (known as "vascular steal"). The mass effect frequently consists of obstruction of normal passage of fluids from surrounding organs. The vascular steal can place a strain on the growing heart of the fetus, even resulting in heart failure, and thus must be monitored by fetal echocardiography.
Teratomas can cause an autoimmune illness called Anti N-methyl-D-aspartate (NMDA) Receptor Encephalitis. After surgery, there is a risk of regrowth in place, or in nearby organs.