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      Anesthesia and perioperative challenges for surgical separation of thoraco-omphalopagus twins: case report Translated title: Anestesia e desafios perioperátorios para cirurgia de separação de gêmeos toraco-onfalópagos: relato de caso

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          Abstract

          Background and objectives

          Conjoined twins are monozygotic twins physically joined at some part of the body. This is a rare phenomenon, estimated between 1:50,000 and 1:200,000 births. The objective of this report is to present the anesthetic management and the perioperative challenges for a separation surgery.

          Case report

          Thoraco-omphalopagus twins were diagnosed by ultrasound and were followed by the fetal medicine team of the service. After 11 h of cesarean surgery, the pediatric surgical team chose to separate the twins. They were monitored with cardioscopy, oximetry, capnography, nasopharyngeal thermometer, urinary output, and non-invasive blood pressure. We chose inhaled induction with oxygen and 4% Sevoflurane. T1 patient was intubated with a 3.5 uncuffed endotracheal tube, and, after three unsuccessful intubation attempts of patient T2, a number 1 laryngeal mask was used. After securing the twins’ airway, the induction was supplemented with fentanyl, propofol, and rocuronium. Mechanical ventilation in controlled pressure mode (6 mL.kg −1) and lumbar epidural (L1–L2) with 0.2% ropivacaine (2.5 mg.kg −1) were used. The pediatric surgical team initiated the separation of the twins via sternotomy, ligation of hepatic vessels. After 2 hours of procedure, the separation was completed, continuing the surgical treatment of T1 and the support of T2 until his death.

          Conclusions

          Conjoined twin separation surgery is a challenge, which requires planning and coordination of a multidisciplinary team during all stages.

          Resumo

          Justificativa e objetivos

          Gêmeos conjugados são gêmeos monozigóticos conectados por alguma parte do corpo. Esse é um fenômeno raro, estimado entre 1:50.000 a 1:200.000 nascimentos. O objetivo deste relato é apresentar o manejo anestésico e os desafios perioperatórios para cirurgia de separação.

          Relato de caso

          Gêmeos toraco-onfalópagos foram diagnosticados por ultrassonografia e acompanhados pela equipe de medicina fetal do serviço. Após 11 horas da cesárea, a equipe cirúrgica pediátrica optou pela separação dos gêmeos. Foram monitorados com cardioscopia, oximetria, capnografia, termômetro nasofaríngeo, débito urinário e pressão arterial não invasiva. Optou-se por indução inalatória com oxigênio e sevoflurano a 4%. O G1 foi intubado com tubo orotraqueal 3,5 sem cuff e após três tentativas de intubação do G2 sem sucesso usou-se máscara laríngea número 1. Após obtenção da via aérea nos gêmeos, complementou-se indução com fentanil, propofol e rocurônio. Ventilação mecânica no modo pressão controlada 6 ml.kg −1 e peridural lombar L1-L2 com ropivacaína 0,2% (2,5 mg.kg −1). A equipe cirúrgica pediátrica iniciou a separação dos gêmeos através de esternotomia, ligadura de vasos hepáticos. Após duas horas de procedimento, a separação foi concluída, prosseguiram-se o tratamento cirúrgico de G1 e os cuidados de G2 até o óbito.

          Conclusões

          A cirurgia de separação de gêmeos conjugados é um desafio, requer planejamento e coordenação de uma equipe multidisciplinar durante todos os estágios.

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          Most cited references4

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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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              Anestesia para separação de gêmeos isquiópagos no período neonatal: relato de caso

              JUSTIFICATIVA E OBJETIVOS: A separação de gêmeos unidos causa grande interesse devido à complexidade da anestesia e cirurgia, à raridade da patologia e às poucas chances de sobrevida. O objetivo desta descrição é o de contribuir para a literatura existente, relatando os desafios encontrados por nossa equipe no atendimento à cirurgia-anestesia de separação de gêmeos isquiópagos. RELATO DO CASO: Pacientes gêmeos, nascidos a termo, de parto cesariano, pesando juntos 5.100 g, classificados como isquiópagus tetrapus. Duas equipes anestésico-cirúrgicas estavam presentes, sendo o procedimento anestésico esquematizado com aparelho de anestesia, cardioscópio, capnógrafo, oxímetro de pulso, termômetro elétrico, estetoscópio esofágico, todos em dobro. Realizou-se indução anestésica com halotano e fentanil, com os gêmeos em posição lateral e com rotação da cabeça em 45º para facilitar a intubação traqueal. Os recém-nascidos foram mantidos em ventilação controlada manualmente, utilizando o sistema de Rees-Baraka. A anestesia foi mantida com halotano, oxigênio e fentanil. Durante o per-operatório, foram encontrados órgãos abdominais duplos, com exceção do cólon, que era único. As bexigas e os ísquios estavam ligados. Ao final da cirurgia as duas crianças apresentavam-se com sinais vitais estáveis. Os gêmeos permaneceram na Unidade de Terapia Intensiva (UTI) Neonatal por quatro semanas e receberam alta em bom estado geral. CONCLUSÕES: Ressalta-se a importância do entrosamento da equipe, do estudo retrospectivo multidisciplinar, da monitorização adequada e acurada observação clínica; todos esses fatores contribuíram para a boa evolução e alta dos gêmeos.
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                Author and article information

                Contributors
                Journal
                Braz J Anesthesiol
                Braz J Anesthesiol
                Brazilian Journal of Anesthesiology
                Elsevier
                0104-0014
                2352-2291
                13 November 2018
                Mar-Apr 2019
                13 November 2018
                : 69
                : 2
                : 214-217
                Affiliations
                [a ]Universidade Federal de Pernambuco (UFPE), Recife, PE, Brazil
                [b ]Instituto de Medicina Integral Professor Fernando Figueira (IMIP), Recife, PE, Brazil
                [c ]Universidade Estadual Paulista (Unesp), São Paulo, SP, Brazil
                [d ]Universidade de Pernambuco (UPE), Recife, PE, Brazil
                Author notes
                [* ]Corresponding author. linhohb@ 123456hotmail.com
                Article
                S0104-0014(18)30143-X
                10.1016/j.bjane.2018.10.004
                9391815
                30097185
                e4a5fa52-7207-467b-94d5-c02f0abd1b2c
                © 2018 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 16 November 2017
                : 15 June 2018
                Categories
                Clinical Information

                conjoined twins,pediatric anesthesia,multidisciplinary care,gêmeos conjugados,anestesia pediátrica,cuidados multidisciplinares

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