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

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.

Late secondary urological reconstruction of separated ischiopagus twins with exstrophic bladder and urinary incontinence / Reconstrução urológica tardia de gêmeos isquiópagos com extrofia de bexiga e incontinência urinária

ABSTRACT We report a case of secondary urinary reconstruction of previously separated conjoined twins with exstrophic bladder and urinary incontinence. Patients were male and aged 13-year-old. Twin one had a history of failed enterocystoplasty that extruded and was visible like an exstrophic neobladder. He underwent a procedure to close bladder neck and reconfigure abdominal wall. After the procedure the patient developed a fistula that was treated, but it persisted and, for this reason, a catheterizable pouch was constructed and native bladder was discarded. Twin two required the immediately construction of catheterizable pouch using the Macedo's technique. Currently, both patients are continent at 4 hour intervals. The mean follow-up was 8 months. Modern continent urinary diversion techniques offer new perspectives and hope for such complex population.

RESUMO Relata-se caso de reconstrução urinária em gêmeos siameses previamente separados com apresentação clínica de bexiga extrófica e incontinência urinária. Os dois pacientes eram do sexo masculino com idade de 13 anos. O primeiro gêmeo apresentava falha da enterocistoplastia com extrusão e visualização da neobexiga extrófica, tendo sido submetido ao fechamento do colo vesical e à reconfiguração da parede abdominal. Após o procedimento, o paciente desenvolveu fístula, que foi tratada, mas persistiu. Posteriormente, optamos por bolsa cateterizável, descartando a bexiga nativa. O segundo gêmeo foi submetido à construção imediata de bolsa cateterizável, por meio da técnica de Macedo. Atualmente, ambos os pacientes estão continentes em intervalos de 4 horas. O seguimento médio foi de 8 meses. As atuais técnicas de derivação urinária oferecem novas perspectivas e esperança para esta população complexa.

Anesthésie pour séparation d'un Craniopagus parasiticus

Le jumeau parasite est un type de siamois chez lequel un des jumeaux est représenté par une formation rudimentaire parasitant le jumeau principal. L'incidence des siamois est estimée de 1:50.000 à 1:100.000 naissances vivantes. Les siamois sont décrits en fonction des organes conjoints ou éventuellement partagés. La classification de Spencer est la plus classique. La séparation chirurgicale -si envisagée et réalisable- relève d'une prise en charge multidisciplinaire.La séparation d'un jumeau parasite est une intervention à risque per et post-opératoire élevé, nécessitant une exploration clinique, radiologique et physiologique notamment en ce qui concerne les organes conjoints ou fusionnés. Le risque anesthésique porte particulièrement sur l'intubation difficile, l'hémorragie, la transfusion massive, les troubles métaboliques. Pour partager notre expérience et discuter les principes de prise en charge, nous rapportons un cas d'anesthésie pour la séparation d'un cas de Craniopagus parasiticus réussi avec des suites post-opératoires sans complications

Manejo de gemelos unidos: experiencia con nueve casos en Medellín, Colombia / Management of conjoined twins: experience with nine cases in Medellín, Colombia

Introducción y metodología: los gemelos siameses son una anomalía muy rara, que ha generado gran interés por el reto que plantea la complejidad de su manejo. Se presenta la experiencia obtenida al respecto durante 40 años en la Sección de Cirugía Pediátrica de la Universidad de Antioquia, Medellín, Colombia, con base en la revisión de las historias clínicas y los archivos personales de los cirujanos de dicha Sección. Resultados: entre 1970 y 2010 se presentaron nueve casos de gemelos siameses, seis de ellos en el Hospital Universitario San Vicente de Paúl y tres en el Instituto de los Seguros Sociales. La distribución fue: tres pares de gemelos asimétricos (parásitos) y seis de gemelos simétricos, a saber: dos de toracoonfopagos, dos de onfaloxigopagos, uno de isquiopagos y uno de pigopagos. Discusión y conclusiones: la separación de los gemelos siameses es de muy rara ocurrencia en la práctica de la cirugía pediátrica. Por esto es importante que las experiencias sean compartidas y publicadas para mejorar los resultados a corto y largo plazo. La experiencia con estos complejos pacientes nos ha dejado enseñanzas como la importancia del diagnóstico prenatal apropiado, del enfoque preoperatorio certero, de la planeación rigurosa del acto quirúrgico y del seguimiento postoperatorio minucioso.

Introduction and methodology: Conjoined twins are a rare anomaly that has generated a great deal of interest because of its complex management. We present the experience achieved during 40 years at the Section of Pediatric Surgery, University of Antioquia, in Medellin, Colombia. Results: In the period between 1970 and 2010, nine cases of conjoined twins were treated by our group, six of them at Hospital Universitario San Vicente de Paúl and three at the Social Security Institute. Their distribution was as follows: three pairs of asymmetric twins (parasites); two pairs of thoracoonphalopagus, two pairs of omphaloxigopagus, one pair of pygopagus and one pair of ischiopagus. Discussion and conclusions: Separation of conjoined twins is a rare situation in the practice of pediatric surgery. Therefore, it is important to share and publish the experience achieved in order to improve the short and long-term outcomes. Our experience has taught us the importance of proper prenatal diagnosis, safe preoperative approach, judicious planning of surgery and rigorous postoperative follow-up.

Conjoined twins: twenty years' experience at a reference center in Brazil

OBJECTIVE: This study reports on the experience of one hospital regarding the surgical aspects, anatomic investigation and outcomes of the management of 21 conjoined twin pairs over the past 20 years. METHODS: All cases of conjoined twins who were treated during this period were reviewed. A careful imaging evaluation was performed to detail the abdominal anatomy (particularly the liver), inferior vena cava, spleen and pancreas, either to identify the number of organs or to evaluate the degree of organ sharing. RESULTS: There were eight sets of ischiopagus twins, seven sets of thoracopagus twins, three sets of omphalopagus twins, two sets of thoraco-omphalo-ischiopagus twins and one set of craniopagus twins. Nine pairs of conjoined twins could not be separated due to the complexity of the organs (mainly the liver and heart) that were shared by both twins; these pairs included one set of ischiopagus twins, six sets of thoracopagus twins and one set of thoraco-omphalo-ischiopagus twins. Twelve sets were separated, including seven sets of ischiopagus twins, three sets of omphalopagus twins, one set of thoracopagus twins and one set of craniopagus conjoined twins. The abdominal wall was closed in the majority of patients with the use of mesh instead of the earlier method of using tissue expanders. The surgical survival rate was 66.7%, and one pair of twins who did not undergo separation is currently alive. CONCLUSION: A detailed anatomic study of the twins and surgical planning must precede separation. A well-prepared pediatric surgery team is sufficient to surgically manage conjoined twins.

Siameses / Siamese twins

El embarazo gemelar ha sido motivo de interés en todas las épocas, y es conocido que existen dos tipos: los dicigóticos o fraternos, que representan el 70 por ciento, y los monocigóticos o de un mismo huevo fecundado, que son alrededor del 30 por ciento...

Gêmeos unidos: diagnóstico pré-natal, parto e desfecho após o nascimento / Conjoined twins: prenatal diagnosis, delivery and postnatal outcome

OBJETIVO: analisar a evolução dos casos de gêmeos unidos do diagnóstico pré-natal ao desfecho após o nascimento. MÉTODOS: análise descritiva, retrospectiva, dos casos de gêmeos unidos diagnosticados por exame ultrassonográfico durante o pré-natal. Foram avaliadas as características maternas, os exames ultrassonográficos e de ecocardiografia fetal do período antenatal, os dados do parto e dos recém-nascidos, bem como os resultados da separação cirúrgica e anatomopatológico. Os gêmeos foram classificados segundo o tipo de união e dados referentes aos aspectos ultrassonográficos, parto, evolução pós-natal e de sobrevida foram analisados. RESULTADOS: quarenta casos de gêmeos unidos foram incluídos no estudo. Observou-se 72,5 por cento de toracópagos, 12,5 por cento de parápagos, 7,5 por cento de onfalo-isquiópagos, 5 por cento de onfalópagos e 2,5 por cento de cefalópagos. A autorização judicial para interrupção da gestação foi solicitada em 58,8 por cento dos casos. Todos os casos em que não se realizou a interrupção judicial da gestação, o parto foi cesárea, em idade gestacional média de 35 semanas. Todos nasceram vivos com mediana do peso de 3.860 g e 88 por cento evoluíram para óbito pós-natal. Dos nascidos vivos, 10 por cento foram submetidos à separação cirúrgica com sobrevida de 60 por cento. A sobrevida geral foi de 7,5 por cento e a pós-natal, de 12 por cento. A avaliação antenatal da letalidade e da possibilidade de separação cirúrgica pós-natal foi precisa. Não foram observadas complicações maternas relacionadas ao parto. CONCLUSÃO: a gemelidade imperfeita apresenta prognóstico sombrio, relacionado, principalmente, às fusões cardíacas complexas presentes na maioria dos toracópagos. Em centros de referência, a avaliação ultrassonográfica e ecocardiográfica antenatal delineia com acurácia o prognóstico de letalidade e de possibilidade de separação cirúrgica pós-natal.

PURPOSE: the aim of this study was to analyze conjoined twins in terms of antenatal, delivery and postnatal aspects. METHODS: a retrospective descriptive analysis of prenatally diagnosed conjoined twins. Prenatal ultrasound and echocardiography, delivery details, postnatal follow-up, surgical separation and post mortem data were reviewed. The twins were classified according to the type of fusion between fetal structures. The following data were analyzed: ultrasound and echocardiographic findings, antenatal lethality and possibility of surgical separation, delivery details and survival rates. RESULTS: forty cases of conjoined twins were included in the study. There were 72.5 percent cases of thoracophagus, 12.5 percent of paraphagus, 7.5 percent of omphalo-ischiophagus, 5.0 percent of omphalophagus, and 2.5 percent of cephalophagus. Judicial termination of pregnancy was requested in 58.8 percent of the cases. Cesarean section was performed in all cases in which pregnancy was not terminated. The mean gestational age at delivery was 35 weeks; all twins were live births with a mean birth weight of 3,860 g and 88 percent died postnatally. Ten percent of the live borns were submitted to surgical separation with a 60 percent survival rate. The total survival rate was 7.5 percent and postnatal survival was 12 percent. Antenatal evaluation of lethality and possibility of surgical separation were precise. There were no maternal complications related to delivery. CONCLUSION: conjoined twins present a dismal prognosis mainly related to the complex cardiac fusion present in the majority of cases with thoracic sharing. At referring centers, prenatal ultrasound and echocardiographic evaluation accurately delineate fetal prognosis and the possibility of postnatal surgical separation.

Gemeralidade conjugada tipo onfalópagos - Avaliação radiológica / Omphalopagus conjoined twins – Radiologic evaluation

Este trabalho descreve a gemelaridade conjugada tipo onfalópagos em que os métodos de imagem foram essenciais para a definição da fusão anatômica e o diagnóstico de possíveis anormalidades associadas, com o objetivo de conferir o correto planejamento cirúrgico e sobrevida adequada aos pacientes.

This paper reports on the omphalopagus conjoined twins in which imaging methods were essential for defining anatomical connection and diagnosis of possible associated abnormalities with the objective of providing accurate surgical planning and their survival.

Anestesia para separação cirúrgica de emergência de onfalópogos / Anesthesia for emergency separation of omphalopagus conjoined twins

JUSTIFICATIVA E OBJETIVOS: A taxa de mortalidade da cirurgia de separação de gêmeos unidos no período neonatal é de 50 por cento, podendo chegar a 75 por cento se ocorrer em situação de emergência. O planejamento da cirurgia de separação é meticuloso e envolve exames de imagem, avaliação da circulação cruzada e até a realização de outros procedimentos cirúrgicos de preparação, como a expansão de pele. RELATO DO CASO: Gêmeas onfalópogas com 11 dias de vida foram separadas em caráter de emergência devido ao óbito da irmã por sepse associada à cardiopatia. O fígado era um órgão comum e foi separado. A gêmea sobrevivente veio a óbito seis dias depois. CONCLUSÕES: A separação no período neonatal deve ser evitada devido à imaturidade dos sistemas orgânicos dos pacientes. Contudo, situações de emergência como esta podem impor a realização do procedimento.

BACKGROUND AND OBJECTIVES: The rate of mortality during the surgical separation of conjoined twins in the neonatal period is 50 percent and can reach up to 75 percent if it occurs in emergency situations. The planning of the surgical separation procedure is detailed and involves imaging assessment, evaluation of cross-circulation and even other surgical preparation procedures, such as skin expansion. CASE REPORT: Eleven-day-old female omphalopagus conjoined twins underwent emergency surgical separation due to the death of one twin caused by sepsis associated to cardiopathy. The liver was shared by the twins and was separated. The surviving twin died six days later. CONCLUSIONS: Surgical separation of conjoined twins in the neonatal period must be avoided due to the patients' organic system immaturity. However, emergency situations such as the one described herein can require the procedure to be carried out.

JUSTIFICATIVA Y OBJETIVOS: La tasa de mortalidad de la cirugía de separación de gemelos unidos en el período neonatal es de un 50 por ciento y puede alcanzar el 75 por ciento si ocurre en situación de emergencia. La planificación de la cirugía de separación es meticulosa y exige exámenes de imagen, evaluación de la circulación cruzada e incluso la realización de otros procedimientos quirúrgicos de preparación como la expansión de la piel. RELATO DEL CASO: Gemelas onfalópagas con 11 días de vida, que fueron separadas en carácter de emergencia debido al óbito de la hermana por sepsis asociada a la cardiopatía. El hígado era un órgano común y fue separado. La gemela que sobrevivió falleció seis días después. CONCLUSIONES: La separación en el período neonatal debe ser evitada debido a la inmadurez de los sistemas orgánicos de los pacientes. Sin embargo, situaciones de emergencia como esa, pueden exigir la realización del procedimiento.

Gêmeos conjugados: experiência de um hospital terciário do sudeste do Brasil / Conjoined twins: an experience of a tertiary hospital in Southeast Brazil

OBJETIVO: analisar a ocorrência de gemelaridade conjugada em um hospital universitário de referência terciária em perinatologia durante 25 anos (janeiro de 1982 e janeiro de 2007) e descrever a separação bem sucedida de um dos pares. MÉTODOS: para este fim, utilizamos retrospectivamente o banco de dados do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo, obtendo o número de pares de gêmeos conjugados, sua frequência, classificação, gênero, forma de resolução da gravidez, tentativa de separação cirúrgica, diagnóstico pré-natal e sobrevida. RESULTADOS: detectamos 14 pares de gêmeos conjugados (1/22.284 nascidos vivos e 1/90 pares de gêmeos nascidos vivos) nascidos neste período (seis masculinos, sete femininos e um com sexo indeterminado). O diagnóstico pré-natal foi realizado em todos os gêmeos, e os nascimentos ocorreram por cesariana. A separação só foi possível em um dos pares, que sobrevivem em ótimas condições de saúde após oito anos. Dos 13 restantes, dez morreram no mesmo dia do nascimento e três sobreviveram apenas alguns meses (menos de um ano). CONCLUSÃO: apesar de este estudo evidenciar um número anormalmente alto de gêmeos conjugados, trata-se de um fenômeno raro, de prognóstico perinatal reservado e dependente do compartilhamento de órgãos entre os gêmeos e malformações associadas, em especial relacionadas ao coração dos fetos. Devido ao mau prognóstico dos pares e do comprometimento reprodutivo materno pela necessidade de realização de cesariana com grandes incisões uterinas, propomos que, com base nestes números, seja solicitada a interrupção precoce destas gestações, como ocorre com outras doenças fetais incompatíveis com a sobrevida extrauterina. Desta forma, a confirmação diagnóstica e a resolução da gravidez de gêmeos conjugados deve ser realizada em centro de atendimento terciário tanto obstétrico quanto perinatal, e a autorização para a interrupção da gestação obtida por via judicial.

PURPOSE: to analyze the occurrence of conjoined twins at a tertiary perinatology reference university hospital over a period of 25 years (January 1982 to January 2007) and to describe the successful separation of one of the pairs. METHODS: we consulted retrospectively the database of the University Hospital of the Medical School of Ribeirão Preto, University of São Paulo, Brazil, in order to determine the number of pairs of conjoined twins, their frequency, classification, gender, type of pregnancy resolution, attempted surgical separation, prenatal diagnosis and survival. RESULTS: we detected 14 pairs of conjoined twins (1/22,284 live births and 1/90 pairs of twin live births) born during this period (six males, seven females and one of indeterminate sex). The prenatal diagnosis was performed in all twins and all births were accomplished by cesarean section. The separation was possible in only one pair, which survives in excellent health conditions after eight years. Of the remaining 13, ten died on the day of birth and three survived only a few months (less than one year). CONCLUSION: Although our study revealed an abnormally high number of conjoined twins, this is a rare phenomenon, with a poor perinatal prognosis depending on the organs shared by the twins and associated malformations, especially those related to the fetal heart. Due to the poor prognosis of these pairs and to the maternal reproductive impairment caused by the need to perform body cesareans, we suggest that, based on these numbers, early interruption of these pregnancies be legally granted, as in the case of other diseases incompatible with fetal survival outside the uterus. Thus, the confirmation of a diagnosis of conjoined twins and the resolution of pregnancy should be performed at a tertiary obstetric and perinatal care center, and an authorization for the interruption of pregnancy should be obtained by judicial means.

Casos quirúrgicos relevantes: separación de gemelos siameses thoraco-omphalopagus en Guatemala / Separation of thoraco-omphalopagus siamese twins in Guatemala

Este reporte describe la separación de gemelos siameses Thoraco-omphalopagus y su manejo. ambas siameses compartían el hígado y la bolsa pericárdica y se encontraban unidas desde el tórax, en el manubrio esternal hasta su inserción baja del ombligo en el abdomen. Se describen sus estudios pre-operatorios de TAC y RMI demostrando la presencia de una arteria nutricia que atravesaba el hígado de la gemela A a la B la cual alimentaba a esta última, quien además presentaba un corazón rudimentario con la presencia de solamente una aurícula y un ventrículo.

Successful separation of thoracopagus conjoined twins with a single extra-hepatic biliary system.

A pair of thoracopagus conjoined twins were separated at the age of 3 months at Khon Kaen Regional Hospital, Thailand on November 19, 2004. Pre-operative investigations showed separate hearts, joined duodenum, and fusion of the livers. Separation of the extra-hepatic biliary systems was suspected. Operative findings revealed fusion of the intestines from the second part of the duodenum to the terminal ileum with two normal colons. An intussusception was found at the terminal ileum. Fusion of the livers with only one extra-hepatic biliary system was noted In one of the twins, the gastrointestinal tract was anastomosed with Roux-en- Y enteric loop to one area of good bile drainage at the cut surface of liver Post operative course was hectic but both twins recovered satisfactorily. Both are doing well at present, two years after the separation. This was the first reported case of thoracopagus conjoined twins with complex biliary tract anomalies in Thailand. From the literature, pre-operative investigations in most cases of these conjoined twins failed to define the precise anatomy of the biliary system and may be misleading as in the presented case. The mortality rate remains high. Meticulous pre-operative planning, decision-making in the operative field and postoperative management as well as a multidisciplinary team are very important for a successful separation.

Separation of thoraco-omphalopagus Siamese twin.

A pair of female thoraco-omphalopagus twins, with two pairs of lungs, common diaphragm and separate pleural cavities was separated at the age of 5(1/2) months after a parasitic relationship had developed between them. Before separation both the babies developed recurrent respiratory tract infection and frequent diarrhea. They were treated with medical therapy and made fit for anesthesia. The combined weight of the twins was 4(1/2) kg at birth and the combined weight at the time of separation was 9(1/2) kg. One baby was smaller in growth compared to the other baby. The closure of anterior abdominal wall was difficult in both babies. However, we could close it without use of any biodegradable patches or tissue expander. Both the babies survived and are doing well after 3 months of separation.

Ischiopagus tripus conjoined twins: a case report

Conjoined twins are one of the rarest congenital anomalies. We present a case of Ischiopagus tripus conjoined twins. The twins were fused at abdomen and pelvis, having two well formed lower limbs and one abnormal lower limb. There was only a single fused pelvis and only one set of well formed male external genitalia and absent anal opening. Anoplasty was done for imperforate anus and later on surgical separation attempted

Conjoined twins: surgical separation in 11 cases.

Eleven pairs of symmetrically conjoined twins underwent surgical separation at the Queen Sirikit National Institute of Child Health. Six were omphalopagus, 4 were thoracopagus and 1 was pygopagus. Eight were female and 3 were male. Three pairs were separated on emergency or semi-emergency bases, and the remaining 8 pairs were separated electively at an older age. Of the 3 pairs who had early emergency separations, one pair, whose combined birth weight was only 2,500 g, underwent emergency separation at the age of 44 days after the death of one twin. The second twin also expired one hour after the separation. In the remaining 2 pairs, early separation was done because of the deterioration of one twin due to complex cardiac anomalies. In both cases, the infants with cardiac anomalies expired but the others survived the separation satisfactorily. In one pair of thoracopagus conjoined twins, one twin had cyanotic cardiac anomalies. They were electively separated at the age of 2 years and 9 months. The twin with cardiac anomalies expired 2 hours after surgery, but the other survived the separation satisfactorily. In the remaining 7 pairs who underwent elective separations, both twins of each pair survived the separation satisfactorily. However, one twin expired unexpectedly 10 days after the separation.