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      Minimally Conjoined Omphalopagus Twins with a Body Stalk Anomaly

      case-report

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          Abstract

          Introduction This report will discuss a case of minimally conjoined omphalopagus twins (MCOTs) with a body stalk anomaly (BSA).

          Case Report We experienced monochorionic diamniotic (MD) twins born at 31 weeks. One infant was suspicious of BSA before birth, and another infant was normal. But normal infant had anal atresia with small intestine which was inserted behind the umbilicus. Twins had very short common umbilicus and infant with BSA had intestinal conjunction, two appendixes at the site of the colon, and a blind-ending colon. We diagnosed MCOTs.

          Discussion On the basis of the Spencer hypothesis, the etiology of MCOTs was that MD twins shared a yolk sac. However, this could not explain the presence of a BSA. It is necessary to consider the possible reasons for a singleton BSA. In addition, intestinal fusion occurred unequally in this case, although two appendixes were found in the same place, which might have occurred because of the balanced fusion.

          Most cited references14

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          The embryology of conjoined twins.

          H Kaufman (2004)
          Attention is drawn to the spontaneous incidence of twinning, both dizygotic and monozygotic in different mammalian species. Conjoined twinning, however, only arises when the twinning event occurs at about the primitive streak stage of development, at about 13-14 days after fertilisation in the human, and is exclusively associated with the monoamniotic monochorionic type of placentation. It is believed that the highest incidence of conjoined twinning is encountered in the human. While monozygotic twinning may be induced experimentally following exposure to a variety of agents, the mechanism of induction of spontaneous twinning in the human remains unknown. All agents that are capable of acting as a twinning stimulus are teratogenic, and probably act by interfering with the spindle apparatus. The incidence of the various types of conjoined twinning is discussed. Information from the largest study to date indicates that the spontaneous incidence is about 10.25 per million births. The most common varieties encountered were thoraco-omphalopagus (28%), thoracopagus (18.5%), omphalopagus (10%), parasitic twins (10%) and craniopagus (6%). Of these, about 40% were stillborn, and 60% liveborn, although only about 25% of those that survived to birth lived long enough to be candidates for surgery. Conjoined twinning occurs by the incomplete splitting of the embryonic axis and, with the exception of parasitic conjoined twins, all are symmetrical and "the same parts are always united to the same parts". Fusion of monozygotic twins is no longer believed to be the basis of conjoined twinning. Accounts are provided of the anatomical features of each of the commonly encountered varieties.
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            Limb body wall complex: I. Pathogenesis.

            Twenty-five fetuses with limb body wall complex (LBW complex) were evaluated. The diagnosis was based on two out of three of the following: exencephaly/encephalocele with facial clefts; thoraco- and/or abdominoschisis; and limb defect. Ninety-five percent (24/25) of the fetuses had associated internal structural defects. In 72% (18/25) the internal defects have been recognized as being secondary to vascular disruption. Concordance was not found between the side and location of the body wall defect versus the limb, internal, and cranial defects. In 85% there was evidence for persistence of the extraembryonic coelom by examination of the placenta. In this same group (85%) there was persistence of the ectodermal-amnion margin, with the amnion being continuous with the skin of the body wall defect. In 40% (10/25) there were tags and amniotic adhesions at other sites. There was no difference in the types or incidence of internal defects between those with and those without amniotic bands. The abnormalities in this collection and experimental animal models support vascular disruption during 4-6 weeks' gestation as an etiology for LBW complex. There is disruption and loss of existing tissues, persistence of embryonic structures, and secondary malformations. Persistence of the extraembryonic coelom may lead to the typical amniotic tags, ring constrictions, and adhesions seen in some specimens.
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              Body stalk anomaly at 10-14 weeks of gestation.

              In a multicenter project of screening for chromosomal defects by fetal nuchal translucency thickness and maternal age at 10-14 weeks, 14 of 106,727 fetuses examined had body stalk anomaly. The ultrasonographic features were a major abdominal wall defect, severe kyphoscoliosis and a short umbilical cord. In all cases, the upper part of the fetal body was in the amniotic cavity, whereas the lower part was in the celomic cavity. The nuchal translucency thickness was above the 95th centile in ten (71.4%) of the cases, but the fetal karyotype was normal in all 12 fetuses evaluated. The findings suggest that early amnion rupture before obliteration of the celomic cavity is a possible cause of the syndrome.
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                Author and article information

                Journal
                AJP Rep
                AJP Rep
                10.1055/s-00000169
                AJP Reports
                Thieme Medical Publishers (333 Seventh Avenue, New York, NY 10001, USA. )
                2157-6998
                2157-7005
                08 May 2015
                October 2015
                : 5
                : 2
                : e124-e128
                Affiliations
                [1 ]Department of Pediatrics, Kochi Health Sciences Center, Kochi, Japan
                [2 ]Department of Diagnostic Pathology, Kochi Health Sciences Center, Kochi, Japan
                [3 ]Department of Pediatric Surgery, Kochi Health Sciences Center, Kochi, Japan
                [4 ]Department of Obstetrics and Gynecology, Kochi Health Sciences Center, Kochi, Japan
                Author notes
                Address for correspondence Hidehiko Maruyama, MD, PhD Department of Pediatrics, Kochi Health Sciences Center 2125-1 Ike, Kochi 781-8555Japan maruyamahidehiko@ 123456gmail.com
                Article
                150011
                10.1055/s-0035-1549300
                4603844
                553c0e08-c4f5-49ff-aa4f-54a1e4227b06
                © Thieme Medical Publishers
                History
                : 03 February 2015
                : 05 March 2015
                Categories
                Article

                body stalk anomaly,intestinal conjunction,minimally conjoined omphalopagus twins

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