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      Successful Surgical Separation of Thoraco-Omphalopagus Symmetrical Conjoined Twins in Iran: Two Case Reports

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          Abstract

          Conjoined twins are derived from the division of a single fertilized ovum; a phenomenon accompanied with multiple congenital anomalies. Such twins are identical, of the same sex, and more likely to be female. Most twins die during the embryonic period, and only 18% survive longer than 24 hours. There are complex anomalies in thoraco-omphalopagus twins that makes them unlikely to live long enough to undergo separation. Treatment of this uncommon condition presents both surgical and anesthetic challenges. The management of rare anomalies is difficult even for skilled surgeons. Therefore, it is logical to use the knowledge gained from previous experiences.

          We herein present the first successful surgical separation of two pairs of thoraco-omphalopagus conjoined twins at the Pediatric Surgery Center of Namazi Hospital (Shiraz, Iran). In both cases, the neonates had separate hearts and common pericardium. Contrast-enhanced computed tomography of two sets of twins showed fusion of sternum, pericardium, diaphragm, and left lobe of liver. Critical steps of the surgical separation were identified and contingency plans were made for possible partial liver donation and cross-circulation between twins. The separation procedure and reconstruction of the abdominal wall were successfully performed. Both pairs of twins, now 6- and 8-year-old, are healthy and have normal growth and are under follow-up.

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          Surgical experience with thirteen conjoined twins.

          Conjoined twins occur in approximately one in 50,000 or so births, and most do not survive. The authors report herein their experience with 13 conjoined twins over the last 30 years, involving those of the following forms: thoracopagus (4 cases), omphalopagus (1 case), ischiopagus (4 cases), pygopagus (1 case), craniopagus (1 case), and incomplete or parasitic varieties (2 cases). The various diagnostic and imaging studies used are described in detail for each form of twinning. Separation is best delayed until such infants are relatively mature (i.e., 6-12 months of age). Operative survival was 50% in those operated on in the neonatal period, but 90% in those over 4 months of age. Ten separations were attempted in 13 sets of twins, with 16 operative survivors. Significantly, up to 10 years after surgery, there were six late deaths due to serious associated congenital anomalies, predominantly cardiac. Improved recent survival is probably the result of the availability of more accurate imaging studies and better anesthetic and operative techniques, with great emphasis on performing immediate reconstruction whenever possible. Use of skin expanders and prosthetic mesh has facilitated wound closure. In the future, ex vivo cardiac reconstruction and autotransplantation may permit separation of twins with complicated conjoined hearts.
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            Conjoined twins: theoretical embryologic basis.

            A theoretical basis for the embryology of conjoined twins was formulated from clinical experience with ten cases and extensive review of pertinent embryologic and clinical literature, including over 500 cases. Regarding the age old question of fusion or fission, it is concluded that there is no known embryologic process by which conjoined twins can be formed by fission but firm evidence to support fusion in all cases. Whether the fusion occurs between embryos on one embryonic disc or on two is of no consequence since they are all monovular. Intact ectoderm will not fuse to intact ectoderm, and all seven types of conjoined twins are explained by seven possible sites of union in the early embryo. One new term is proposed: parapagus, from the Greek para, meaning "side," combined with pagus, meaning "fixed"; this is the group formerly called dicephalus or diprosopos. These anterolaterally united parapagus twins must result from two nearly parallel notochords in close proximity; craniopagi and pygopagi from fusion at the cranial and caudal neuropores, respectively; cephalopagi and ischiopagi from union at the pharyngeal and cloacal membranes, respectively; thoracopagi from merging of the cardiac anlage; and omphalopagi from fusion of the umbilicus or of the edges of two embryonic discs in any area not including the above sites. Parasitic twins result from embryonic death of one twin, leaving various portions of the body vascularized by the surviving autosite. The rarity of cases (2) not easily explained by the above theories, and the nearly 6% of twins with two umbilical cords arising from the placenta would seem to support these conclusions. Should one wish to learn the methods of a conjurer, he might vainly watch the latter's customary repertoire, and, so long as everything went smoothly, might never obtain a clue to the mysterious performance, baffled by the precision of the manipulations and the complexity of the apparatus; if, however, a single error were made in any part or if a single deviation from the customary method should force the manipulator along an unaccustomed path, it would give the investigator an opportunity to obtain a part or the whole of the secret.(ABSTRACT TRUNCATED AT 400 WORDS)
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              Conjoined twins: From conception to separation, a review.

              Conjoined twins are suggested to result from aberrant embryogenesis. The two main theories proposed to explain the phenomena are fission and fusion. The incidence rate is 1 in 50,000 births; however, since about 60% of the cases are stillborn, the true incidence is approximated at 1 in 200,000. There is a higher predisposition towards female than male gender with a ratio of 3:1. Conjoined twins are classified based upon the site of attachment. The extent of organ sharing, especially the heart, determines the possibility and prognosis of a separation procedure. Meticulous preoperative evaluation, planning, and preparedness of the team are crucial for a successful separation. Separation of conjoined twins poses several technical, legal, and ethical issues. Clin. Anat. 30:385-396, 2017. © 2017 Wiley Periodicals, Inc.
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                Author and article information

                Journal
                Iran J Med Sci
                Iran J Med Sci
                Iranian Journal of Medical Sciences
                Shiraz University of Medical Sciences (Iran )
                0253-0716
                1735-3688
                March 2020
                : 45
                : 2
                : 143-147
                Affiliations
                [1 ] Department of Pediatric Surgery, Namazee Hospital, Shiraz University of Medical Science, Shiraz, Iran
                [2 ] Shiraz Transplant Medical Center, Namazee Hospital, Shiraz University of Medical Science, Shiraz, Iran
                [3 ] General Surgery Resident, Department of General Surgery, Shiraz University of Medical Science, Shiraz, Iran
                [4 ] Shiraz Anesthesiology and Clinical Care Research Center, Shiraz University of Medical Science, Shiraz, Iran
                [5 ] Maternal Fetal Medicine Research Center, Perinatology Ward, Shiraz University of Medical Science, Shiraz, Iran
                [6 ] Student Research Committee, Department of Otorhinolaryngology, Shiraz University of Medical Science, Shiraz, Iran
                Author notes
                Corresponding author: Zahra Beizavi, MD; General Surgery Resident, Department of General Surgery, Faghihi Hospital, Zand Ave, Postal Code: 71348-44119, Shiraz, Iran. Tel: +98 9173014584, Fax: +98 71 34846114.
                Article
                IJMS-45-2
                10.30476/ijms.2019.81060.
                7071550
                32210492
                f93804c1-6a87-43fb-a623-3e86aa62e99d
                Copyright: © Iranian Journal of Medical Sciences

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 06 April 2019
                : 28 April 2019
                : 19 January 2019
                Categories
                Case Report

                Medicine
                twins, conjoined,congenital abnormalities,reconstructive surgical procedures
                Medicine
                twins, conjoined, congenital abnormalities, reconstructive surgical procedures

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