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Many factors determine the optimal way to deliver a baby. A vertex presentation is the ideal situation for a vaginal birth, however, occiput posterior positions tend to proceed more slowly, often requiring an intervention in the form of forceps, vacuum extraction, or Cesarean section. In a large study, a majority of brow presentations were delivered by Cesarean section, however, because of 'postmaturity', factors other than labour dynamics may have played a role. Most face presentations can be delivered vaginally as long as the chin is anterior; there is no increase in fetal or maternal mortality. Mento-posterior positions cannot be delivered vaginally in most cases (unless rotated) and are candidates for Cesarean section in contemporary management.
Usually performing the Leopold maneuvers will demonstrate the presentation and possibly the position of the fetus. Ultrasound examination delivers the precise diagnosis and may indicate possible causes of a malpresentation. On vaginal examination, the leading part of the fetus becomes identifiable after the amniotic sac has been broken and the head is descending in the pelvis.
"'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.
Prenatal Diagnosis:
- Aymé, "et al." (1989) reported prenatal diagnosis of Fryns syndrome by sonography between 24 and 27 weeks.
- Manouvrier-Hanu et al. (1996) described the prenatal diagnosis of Fryns syndrome by ultrasonographic detection of diaphragmatic hernia and cystic hygroma. The diagnosis was confirmed after termination of the pregnancy. The fetus also had 2 erupted incisors; natal teeth had not been mentioned in other cases of Fryns syndrome.
Differential Diagnosis:
- McPherson et al. (1993) noted the phenotypic overlap between Fryns syndrome and the Pallister–Killian syndrome (601803), which is a dysmorphic syndrome with tissue-specific mosaicism of tetrasomy 12p.
- Veldman et al. (2002) discussed the differentiation between Fryns syndrome and Pallister–Killian syndrome, noting that differentiation is important to genetic counseling because Fryns syndrome is an autosomal recessive disorder and Pallister–Killian syndrome is usually a sporadic chromosomal aberration. However, discrimination may be difficult due to the phenotypic similarity. In fact, in some infants with 'coarse face,' acral hypoplasia, and internal anomalies, the initial diagnosis of Fryns syndrome had to be changed because mosaicism of isochromosome 12p was detected in fibroblast cultures or kidney tissue. Although congenital diaphragmatic hernia is a common finding in both syndromes, bilateral congenital diaphragmatic hernia had been reported only in patients with Fryns syndrome until the report of the patient with Pallister–Killian syndrome by Veldman et al. (2002).
- Slavotinek (2004) reviewed the phenotypes of 52 reported cases of Fryns syndrome and reevaluated the diagnostic guidelines. She concluded that congenital diaphragmatic hernia and distal limb hypoplasia are strongly suggestive of Fryns syndrome, with other diagnostically relevant findings including pulmonary hypoplasia, craniofacial dysmorphism, polyhydramnios, and orofacial clefting. Slavotinek (2004) stated that other distinctive anomalies not mentioned in previous guidelines include ventricular dilatation or hydrocephalus, agenesis of the corpus callosum, abnormalities of the aorta, dilatation of the ureters, proximal thumbs, and broad clavicles.
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
In France, Aymé, "et al." (1989) estimated the prevalence of Fryns syndrome to be 0.7 per 10,000 births based on the diagnosis of 6 cases in a series of 112,276 consecutive births (live births and perinatal deaths).
Because postmortem fetal extrusion is so rare, and occurs under highly idiosyncratic conditions regarding the individual and the ambient environment, this phenomenon has not been studied for possible applications to forensic investigation. Even if the study of postmortem fetal extrusion could lead to improved investigative methods, experimental research would be highly problematic. At present, forensic scientists have at their disposal an array of established techniques and procedures for a death investigation at the stages of decomposition when postmortem fetal extrusion typically occurs.
In archaeology, the study of mortuary context, that is, the interpretation of the postmortem treatment of the dead, whether an individual or as pertains to patterns within a group, has led to the development of hypotheses on social status and/or hierarchy regarding many cultures, ancient and extant. In addition, the determination of whether or not delivery actually occurred prior to death has a bearing on analyses of the mother's population, as the concentration of trace elements differ markedly between the skeletons of prepartum adult women (before giving birth) and women who are lactating; the identification of coffin birth would lead to more accurate analyses of the number of lactating women in a population or the rate of maternal mortality. It is therefore necessary for investigators to be able to recognize postmortem fetal extrusion when encountered in an excavated burial.
Radiographic analysis by performing a computed axial tomographic scan is the gold standard for diagnosing craniosynostosis.
Plain radiography of the skull may be sufficient for diagnosing a single suture craniosynostosis and should therefore be performed, but the diagnostic value is outweighed by that of the CT-scan. Not only can the sutures be identified more accurately, thus objectively demonstrating a fused suture, but also evaluation of the brain for structural abnormalities and excluding other causes of asymmetric growth are possible at the same time. In addition to this, CT-scanning can visualize the extent of skull deformity, thereby enabling the surgeon to start planning surgical reconstruction.
Coffin birth, also known as postmortem fetal extrusion, is the expulsion of a nonviable fetus through the vaginal opening of the decomposing body of a deceased pregnant woman as a result of the increasing pressure of intra-abdominal gases. This kind of postmortem delivery occurs very rarely during the decomposition of a body. The practice of chemical preservation, whereby chemical preservatives and disinfectant solutions are pumped into a body to replace natural body fluids (and the bacteria that reside therein), have made the occurrence of "coffin birth" so rare that the topic is rarely mentioned in international medical discourse.
Typically during the decomposition of a human body, naturally occurring bacteria in the organs of the abdominal cavity (such as the stomach and intestines) generate gases as by-products of metabolism, which causes the body to swell. In some cases, the confined pressure of the gases can squeeze the uterus (the womb), even forcing it downward, and it may and be forced out of the body through the vaginal opening (a process called "prolapse"). If a fetus is contained within the uterus, it could therefore be expelled from the mother's body through the vaginal opening when the uterus turns inside-out, in a process that, to outward appearances, mimics childbirth. The main differences lie in the state of the mother and fetus and the mechanism of delivery: in the event of natural, live childbirth, the mother's contractions encourage the infant to emerge from the womb; in a case of coffin birth, built-up gas pressure within the putrefied body of a pregnant woman pushes the dead fetus from the body of the mother.
Cases have been recorded by medical authorities since the 16th century, though some archaeological cases provide evidence for its occurrence in many periods of human history. While cases of postmortem fetal expulsion have always been rare, the phenomenon has been recorded under disparate circumstances and is occasionally seen in a modern forensic context when the body of a pregnant woman lies undisturbed and undiscovered for some time following death. There are also cases whereby a fetus may become separated from the body of the pregnant woman about the time of death or during decomposition, though because those cases are not consistent with the processes described here, they are not considered true cases of postmortem fetal extrusion.
Prognoses for 3C syndrome vary widely based on the specific constellation of symptoms seen in an individual. Typically, the gravity of the prognosis correlates with the severity of the cardiac abnormalities. For children with less severe cardiac abnormalities, the developmental prognosis depends on the cerebellar abnormalities that are present. Severe cerebellar hypoplasia is associated with growth and speech delays, as well as hypotonia and general growth deficiencies.
Ilona and Judit Gófitz (Hungarian: "Gófitz Ilona" és "Judit"; in contemporary publications: "Helen" and "Judith of Szőny"), also known as the Hungarian Sisters, were conjoined twins from Szőny, Hungary who lived from October 19, 1701 to February 8, 1723. In the image they appear to be pygopagus.
Ganga and Jamuna Mondal (born Ayara and Jayara Ratun, 1969 or 1970), known professionally as The Spider Girls and The Spider Sisters, are conjoined twins from a Bengali family in Basirhat, West Bengal, India.
The outcome of this disease is dependent on the severity of the cardiac defects. Approximately 1 in 3 children with this diagnosis require shunting for the hydrocephaly that is often a consequence. Some children require extra assistance or therapy for delayed psychomotor and speech development, including hypotonia.
There are several ways to classify craniosynostosis.
- For example, one can consider the number of closed sutures. If only one of the four sutures is prematurely closed (single suture craniosynostosis), the craniosynostosis is referred to as 'simple' (or 'isolated'). Whereas when two or more sutures are no longer open, the craniosynostosis is 'complex'.
- A second classification scheme gives a clinical description of the resulting shape of the skull. This will be further discussed under phenotype.
- A third classification involves the presence or absence of an identified craniofacial syndrome. Craniosynostosis where no extracranial deformations are present, is called non-syndromic or 'isolated' craniosynostosis. When there are extracranial deformations present, for instance involving the limbs, heart, central nervous system or the respiratory tract, you may speak of a syndromic form of craniosynostosis. More than 180 identified syndromes show deformations due to craniosynostosis. The following syndromes are associated with fibroblast growth factor receptors:
In addition, the following syndromes have been identified:
The treatment of soft tissue parts of midface anomalies is often a reconstruction from a skin flap of the cheek. This skinflap can be used for other operations in the further, as it can be raised again and transposed again. In the treatment of midface anomalies there are generally more operations needed. Bone tissue reconstruction of the midface often occurs later than the soft tissue reconstruction. The most common method to reconstruct the midface is by using the fracture/ incision lines described by René Le Fort. When the cleft involves the maxilla, it is likely that the impaired growth will result in a smaller maxillary bone in all 3 dimensions (height, projection, width).
The twins are joined at the abdomen and pelvis, have four arms, and three legs, two of which are fused into a single, nine-toed limb, which remains concealed behind their body. They share a stomach, but have two hearts, two kidneys, one liver, and one reproductive tract.
The twins have been examined by doctors to determine whether or not they can be separated. However, they have no plans to do so because they feel it would be against God's will, carry too great a medical risk, and would rob them of their employment. They have stated ""We are happy as we are. The family will starve if we are separated.""
Ladan and Laleh Bijani (in Persian: ) (January 17, 1974 – July 8, 2003) were Iranian conjoined twin sisters. They were joined at the head and died immediately after their complicated surgical separation. Coincidentally, the twins were born 100 years to the day of the deaths of Chang and Eng Bunker, also conjoined twins, famously known as the "original" Siamese twins.
Ultrasound is the often chosen to examine the duct and determine the presence and size of any cysts or abnormalities. Fine-needle aspiration cytology can also be used to confirm the diagnosis.
The nose anomalies found in facial clefts vary. The main goal of the treatment is to reconstruct the nose to get a functional and esthetic acceptable result. A few possible treatment options are to reconstruct the nose with a forehead flap or reconstruct the nasal dorsum with a bone graft, for example a rib graft. The nasal reconstruction with a forehead flap is based on the repositioning of a skin flap from the forehead to the nose. A possible downside of this reconstruction is that once you performed it at a younger age, you can’t lengthen the flap at a later stage. A second operation is often needed if the operation is done on early age, because the nose has a restricted growth in the cleft area. Repair of the ala (wing of the nose) often requires the inset of cartilage graft, commonly taken from the ear.
The Number of the Beast (, "Arithmos tou Thēriou") is a term in the Book of Revelation, of the New Testament, that is associated with the Beast of Revelation in chapter 13. In most manuscripts of the New Testament and in English translations of the Bible, the number of the beast is 666. Papyrus 115 (which is the oldest preserved manuscript of the "Revelation" ), as well as other ancient sources like "Codex Ephraemi Rescriptus", give the Number of the Beast as 616 (), not 666; critical editions of the Greek text, such as the Novum Testamentum Graece, note 616 as a variant.
Stimmler syndrome is a rare autosomal recessive congenital disorder first described by Stimmler et al. in 1970. It is characterized by dwarfism, diabetes, a small head, and high levels of alanine in the urine.
Symptoms for the disease include microcephaly, a low birth weight, dwarfism, small teeth, and diabetes. The symptoms of Stimmler syndrome are closely related to a disease studied by Haworth et al. in 1967 as well as Leigh subacute necrotizing encephalopathy with lactic acidosis
Millie McCoy and Christine McCoy (July 11, 1851 – October 8, 1912) were American conjoined twins who went by the stage names "The Carolina Twins", "The Two-Headed Nightingale" and "The Eighth Wonder of the World". The Twins traveled throughout the world performing song and dance for entertainment.
Berdon syndrome, also called Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIH syndrome), is an autosomal recessive fatal genetic disorder affecting newborns. In a 2011 study of 227 children with the syndrome, "the oldest survivor [was] 24 years old." The Ann Arbor News reported a five year old survivor at the end of 2015.
It is more prevalent in females, 7 females to 3 males, and is characterized by constipation and urinary retention, microcolon, giant bladder (megacystis), intestinal hypoperistalis, hydronephrosis, and dilated small bowel. The pathological findings consist of an abundance of ganglion cells in both dilated and narrow areas of the intestine. It is a familial disturbance of unknown cause.
Walter Berdon "et al." in 1976 first described the condition in five female infants, two of whom were sisters. All had marked dilatation of the bladder and some had hydronephrosis and the external appearance of prune belly. The infants also had microcolon and dilated small intestines.
In order to prevent further cysts and infections from forming, the thyroglossal duct and all of its branches are removed from the throat and neck area. A procedure, known as the Sistrunk procedure, is considered to be the standard procedure and involves removal of portions of the hyoid bone and core tissue of the suprahyoid region. Cysts will often reoccur if the entire duct is not removed, so reoccurrence requires a wider range of tissue to be removed in a subsequent surgery.
Delaying the surgical procedure almost always leads to recurrent infections, which will continue to delay the needed treatment. The Sistrunk procedure has a reoccurrence rate of less than 5%, proving it is extremely effective at removing the majority of traces of the persistent thyroglossal duct.