Cases reported "Twins, Conjoined"

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1/8. Diprosopus (partially duplicated head) associated with anencephaly: a case report.

    Craniofacial duplication (diprosopus) is a rare form of conjoined twin. A 16 year old mother with a twin pregnancy delivered one normally formed baby boy and one diprosopus male. The malformed baby was 33 weeks of gestation with a single trunk, normal limbs and various degrees of facial duplication. Of the following structures there were two of each: noses, eyes, ears (and one dimple), mouths, tongues and, with bilateral central cleft lips and cleft palates. This was associated with holoprosencephaly and craniorachischisis. Internal organs showed no duplication. There were multiple congenital anomalies including diaphragmatic hernia, small lungs, two lobes of the right lung, ventricular septal defect, small adrenal gland and small left kidney with short ureter. The body also had a short neck, small chest cavities and kyphosis. X-ray revealed duplication of the vertebral column. The case presented here represents a type II of diprosopia of Rating (1933) and is the least common type reported. We also reviewed 22 recently reported cases of diprosopus. In addition to facial duplication, anencephaly, neural tube defect and cardiac malformations represent the more common congenital abnormalities associated with diprosopus. The pathogenesis of diprosopus is not well understood. Factors that play a role in diprosopus are probably similar to those factors (genetic, environmental and abnormal placental circulation) which affect monozoygotic twins as observed in this case report. Early ultrasonography diagnosis of diprosopus permits one to consider a vaginal therapeutic abortion.
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2/8. Monocephalus diprosopus, a rare form of conjoined twins, and associated congenital anomalies.

    Craniofacial duplication (diprosopus) is a rare form of conjoined twins. A case of monocephalus diprosopus with anencephaly, cervicothoracolumbar rachischisis, and duplication of the respiratory tract and upper gastrointestinal tract is reported. The cardiovascular system remained single but the heart showed transposition of the great vessels. We present this case due to its rarity, and compare our pathologic findings with those already reported.
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3/8. Dicephalus dibrachius with anencephaly.

    We present a case of inferior conjunction in a rare type of conjoined twins, dicephalus in a male fetus. The male fetus was born to a 24-year-old, gravida 2, and para 0, who had medical abortion at 15 weeks of gestation due to anencephaly with meningoencephalocele revealed by ultrasound examination. The fetus was born with 2 anencephalic heads with a bifurcation of the vertebral column and presence of 2 spinal cords. The other viscera and limbs were normal in number and location as for a male singleton. This case illustrates the relationship between conjoined twining, and neural tube defect more particularly anencephaly with a male zygote, which is an unusual presentation for this type of zygote gender.
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4/8. Anencephalic conjoined twins.

    A case of anencephaly in female thoracophagus conjoined twins is presented and discussed.
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5/8. Aberrant twinning (diprosopus) associated with anencephaly.

    A case of Monocephalus diprosopus, associated with craniorachischisis and duplication of most of the foregut derivates is presented. The major part of the cardiovascular system remained single but the heart exhibited severe defects, including a complete persistent atrioventricular canal, transposition of the great arteries and atresia of the pulmonary valve. This report further supports the hypothesis that certain-types of incomplete twinning and neural tube defects may be caused by a single teratogenic mechanism.
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6/8. early diagnosis of severe congenital malformations by ultrasonography.

    The significance of ultrasound examinations in early stages of pregnancy is illustrated by the detection of four severe congenital malformations within the first trimester and one malformation syndrome within the first part of the second trimester of pregnancy. We report on the diagnosis of a fetal POTTER syndrome (13 weeks), an anencephalic fetus (13 weeks), MECKEL-GRUBER syndrome (16 weeks), fetal exomphalos (12 weeks) and finally "Siamese twins" (11 weeks). Characteristic ultrasonographic findings are presented and described in detail. The incidence of these severe fetal abnormalities vary between 1:1000 (anencephaly), 1:6000 (exomphalos), 3:10,000 (POTTER-syndrome), 1:50,000 (MECKEL-GRUBER syndrome) and 1:250,000 (conjoined twins) live births. The sonographical diagnosis of all these malformation syndromes could be established by thorough and repeated inspection of the fetal head and fetal body with longitudinal and transversal scans. It is concluded, that the high and still increasing reliability congenital structural anomalies renders routine systemic ultrasound screening an attractive possibility already at this "early" stage of pregnancy.
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7/8. anatomy and embryology in cephalothoracopagus twins.

    Anatomical features are described in a case of cephalothoracopagus female twins with laterally fused heads. There was a single foregut, shared equally by the two individuals. The tracheae, lungs, hearts, livers, and gallbladders were also shared by the two individuals. They were located in the anterior and posterior regions of the joined bodies. These peculiarities occur in other cases of cephalothoracopagus twinning reported in the literature, and may have been caused by spatial problems in the developing twin embryos. In particular, each heart is considered to be a composite organ, formed by tissue from both individuals. The occipital region of the skull was double, but the remainder of the cranium was single. The oral cavity was divided in two by a midline mandibular body thought to represent the fused medial portions of two mandibles. Each compartment so formed contained a tongue. This midline body is considered responsible for a cleft lip and palate. Finally, we propose that anencephaly in this and other cases of laterally fused heads is associated with mechanical difficulty in closing the rostral neuropore.
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8/8. Asymmetry and discordance for congenital anomalies in conjoined twins: a report of six cases.

    Six pairs of conjoined twins have been studied. The first case was a pair of 13-week-old omphalopagus fetuses. One was a holoacradius amorphus and the other had rachischisis and anencephaly. The second case was a pair of omphalopagus twins. One of the twins was macerated and corresponded to a developmental age of 13-14 weeks, while the other was developed to 28-30 weeks of gestation and exhibited urogenital and gastrointestinal defects not found in the smaller twin. In the third case, that of a thoracoomphalopagus, one had cleft lip and palate, pulmonic stenosis, and atresia of the ileocecal valve, while the other did not show these anomalies. In the fourth cae, also omphalopagus twins, one had a lumbosacral meningomyelocele and severe gastrointestinal and urogenital anomalies not found in the second twin. The fifth case was a pair of thoracoomphalopagus twins, sharing a common heart with asymmetrical anomalies. The sixth case was a diprosopus anencephalic conjoined twin. The first pairs of conjoined twins were discordant for several abnormalities in nonshared organs, in addition to having abnormalities of the conjoined organs. It seems that discordance in conjoined twins is not a rare finding. The factors that play a role in discordance of anomalies in conjoined twins are probably similar to the factors in monozygotic twins--i.e., environmental, genetic, and abnormal placental and/or fetal circulation.
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