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      Conjoined Thoracopagus Twins: A Systematic Review of the Anomalies and Outcome of Surgical Separation

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          Abstract

          Introduction:

          Conjoined twin is an extremely rare condition and requires a thorough knowledge of anatomy, and a multidisciplinary approach is essential to successfully separate the twins. Thoracopagus twins lie face to face and are attached from chest to upper abdomen. They are the most common among all the varieties but have a poor survival rate.

          Materials and Methods:

          This study is a review of literature from 2019 to the oldest via PubMed and Google Scholar using keywords: Conjoined twins, Thoracopagus twins, Thoracoomphalopagus and Thoraco-omphalopagus twins. The articles were reviewed for the description of the anatomy of shared organs, management and outcome of these twins.

          Results:

          One hundred and fifty-eight sets of thoracopagus and thoraco-omphalopagus twins including our twins were included in this study. Out of 158 reported thoracopagus twin sets in literature, with M: F ratio of 1:2.3, 71 sets were found to be non-operable and all of them subsequently expired; 82 sets were operated upon, out of which 83 babies survived, suggesting an overall surgical success rate of about 50%.

          Conclusion:

          Thoracopagus twins have a dismal prognosis. The most important decisive parameter for successful separation is the extent of sharing of organs between twins. The role of a motivated multidisciplinary team is also indispensable and cannot be overemphasised.

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          Most cited references51

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          Conjoined twins: a worldwide collaborative epidemiological study of the International Clearinghouse for Birth Defects Surveillance and Research.

          Conjoined twins (CT) are a very rare developmental accident of uncertain etiology. Prevalence has been previously estimated to be 1 in 50,000 to 1 in 100,000 births. The process by which monozygotic twins do not fully separate but form CT is not well understood. The purpose of the present study was to analyze diverse epidemiological aspects of CT, including the different variables listed in the Introduction Section of this issue of the Journal. The study was made possible using the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) structure. This multicenter worldwide research includes the largest sample of CT ever studied. A total of 383 carefully reviewed sets of CT obtained from 26,138,837 births reported by 21 Clearinghouse Surveillance Programs (SP) were included in the analysis. Total prevalence was 1.47 per 100,000 births (95% CI: 1.32-1.62). Salient findings including an evident variation in prevalence among SPs: a marked variation in the type of pregnancy outcome, a similarity in the proportion of CT types among programs: a significant female predominance in CT: particularly of the thoracopagus type and a significant male predominance in parapagus and parasitic types: significant differences in prevalence by ethnicity and an apparent increasing prevalence trend in South American countries. No genetic, environmental or demographic significant associated factors were identified. Further work in epidemiology and molecular research is necessary to understand the etiology and pathogenesis involved in the development of this fascinating phenomenon of nature. Copyright © 2011 Wiley Periodicals, Inc.
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            Theoretical and analytical embryology of conjoined twins: part I: embryogenesis.

            R. Spencer (2000)
            A review of over 1,800 publications concerning the embryology and pathologic anatomy of conjoined twins provides convincing evidence that they all result from the secondary union of two originally separate monovular embryonic discs. This "fusion" theory seems to be confirmed by the adjustments to union and the pattern and incidence of specific anomalies at the proposed sites of conjunction in more than 1,200 cases, all of which can be arranged in two uninterrupted series of cases, the one united dorsally (in the neural tube) and the other, ventrally (over a shared a yolk sac). No theoretical "fission" of the vertebrate embryo at any stage of development, in any plane, in any direction can explain (1) the selection of the observed sites of fusion, (2) the details of the union, or (3) the limitation to the specific areas in which the twins are found to be joined. Part I of this disquisition deals with the pertinent normal and theoretical embryology, the adjustments to union, and the parasitic cases, as well as conjoined triplets and quadruplets, and a comparison of oviparous and viviparous embryos. Part II (in a subsequent issue of this journal) will compare and correlate the abnormalities of the various organ systems involved in 1,200 cases. Copyright 2000 Wiley-Liss, Inc.
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              Anatomic description of conjoined twins: a plea for standardized terminology.

              The function of language is communication, but accurate communication is impossible unless the language is mutually understood. In an extensive review of the literature of conjoined twins, considerable difficulty was encountered in interpreting the variations in nomenclature--the designation of the different types of union as well as the identification of the individual twins and their anatomic relationship to one another. For example, the term thoracopagus often includes twins with either one or two hearts; the former rarely (if ever) is capable of surviving, but the latter often do survive. Labeling them as thoracopagus and omphalopagus, respectively, will permit more meaningful comparison between the two types. A simple, logical system of terminology is proposed.
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                Author and article information

                Journal
                Afr J Paediatr Surg
                Afr J Paediatr Surg
                AJPS
                Afr J Paediatr Surg
                African Journal of Paediatric Surgery: AJPS
                Wolters Kluwer - Medknow (India )
                0189-6725
                0974-5998
                Jul-Sep 2023
                19 January 2023
                : 20
                : 3
                : 157-165
                Affiliations
                [1]Department of Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
                Author notes
                Address for correspondence: Dr. Rahul Saxena, Department of Pediatric Surgery, All India Institute of Medical Sciences, Jodhpur - 342 005, Rajasthan, India. E-mail: drrahulsaxena@ 123456gmail.com
                Article
                AJPS-20-157
                10.4103/ajps.ajps_77_22
                10450114
                37470549
                70213c4e-7e2b-4670-b1b1-300c0db89991
                Copyright: © 2023 African Journal of Paediatric Surgery

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 27 June 2022
                : 12 August 2022
                Categories
                Systematic Review

                conjoined twins,the survival of thoracopagus twins,thoraco-omphalopagus twins,thoracopagus twins

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