Antenatal Diagnosis of Parapagus Conjoined Twins: 3D Virtual and 3D Physical Models / Diagnóstico antenatal de gêmeos unidos parapagus: Modelos 3D virtuais e físicos

Abstract Conjoined twins (CTs) are a rare complication from monochorionic and monoamniotic twin pregnancies. We describe the use of 3D technologies, including 3D virtual and 3D physical models on prenatal evaluation of one parapagus CT. A 16-year-old G1P0 woman was referred for fetal magnetic resonance imaging (MRI) anatomical evaluation of a CT at 28 weeks of gestation. 3D images of the fetal surface were generated by the software during the examination for spatial comprehension of the relationship between the fetal parts. The pair of CTs died at the 32nd week of gestation, a few hours after cesarean section. 3D technologies are an important tool for parental counseling and preparation of the multidisciplinary care team for delivery and neonatal assistance and possible surgical planning for postnatal separation in CTs cases.

Resumo Gêmeos unidos (GUs) são uma complicação rara de gestações gemelares monocoriônicas e monoamnióticas. Descrevemos o uso de tecnologias 3D, incluindo modelos 3D virtuais e físicos, na avaliação pré-natal de GU parapagus. Mulher de 16 anos, G1P0, foi encaminhada para avaliação anatômica por ressonância magnética (RM) fetal de GU com 28 semanas de gestação. Imagens 3D da superfície fetal foram geradas pelo software durante o exame para compreensão espacial da relação entre as partes fetais. O par de GUs morreu na 32ª semana de gestação, poucas horas após a cesariana. As tecnologias 3D são uma importante ferramenta de aconselhamento dos pais e de preparação da equipe multiprofissional para o parto e a assistência neonatal, além de possível planejamento cirúrgico para separação pós-natal em casos de GUs.

Deradelphous Cephalothoracoomphalopagus in kittens: case report / Cefalotoraconfalopago derafelfo em gatos: relato de caso

The present report describes a case of conjoined twins of the cephalo-thoraco-omphalopagus deradelphous type in cats. A feline female was transferred to our veterinary hospital as an emergency for dystocic labor. The stillborn was subjected to radiographic evaluation, and a single skull and two complete distinct vertebral columns were found. Anatomopathological examination revealed that the twins presented the head, chest, and umbilicus as the main points of union and were classified as the cephalo-thoraco-omphalopagus type. In addition, the twins had unique and well-developed faces, which allowed them to be classified as deradelphous. This malformation is rare in domestic animals, and to the best of our knowledge, this type has not been reported in felines. Further studies are warranted on this embryonic alteration, primarily because its etiology remains unknown.(AU)

O presente relato descreve um caso de gêmeos siameses do tipo cefalotoraconfalopago deradelfo em gatos. Uma fêmea felina foi atendida na emergência do hospital veterinário em trabalho de parto distócico. Os natimortos foram encaminhados para avaliação radiográfica e constatou-se que apresentavam um único crânio e duas colunas vertebrais completas e distintas. O exame anatomopatológico evidenciou que os gêmeos possuíam cabeça, tórax e umbigo como principais pontos de união, sendo classificados como cefalotoraconfalopago. Além disso, os gêmeos apresentaram face única e bem desenvolvida, o que permitiu classificá-los como deradelfos. Esse tipo de malformação é raro em animais domésticos, e não foi encontrado nenhum trabalho em felino com a mesma classificação do presente relato. Há necessidade de mais estudos sobre essa alteração embrionária, pois a etiologia do processo ainda não foi esclarecida.(AU)

A rare case of dicephalic parapagus conjoined twins

Dicephalic parapagus are one of the rare forms of conjoined twins. Their prognosis is usually very poor. Early prenatal diagnosis of these serious congenital abnormalities remains a real challenge for health systems in the low-income countries. The late antenatal diagnosis of a case of this abnormality was presented. The diagnosis was made at the 33rd week on the only ultrasound performed during the pregnancy. The pregnancy was interrupted with the agreement of the couple and delivery was performed by caesarean section.

Thoracopagus: Autoptic and radiologic study of 2 cases

Conjoined twins are a very rare complication of monozygotic twinning, most common being thoracopagus. Here we report about two cases of thoracopagus male twins illustrating the autopsy details of one case and the prenatal magnetic resonance imaging (MRI) details of another case. While the first case was misdiagnosed as separate twins on antenatal USG, only to be later confirmed as thoracopagus twins after birth, the antenatal MRI done in the second case helped in accurate detection of thoracopagus twins. Bilateral peripheral cortical cysts with dysplasia was noted in one of the twins of the first case, which has not been reported earlier in conjoint thoracopagii. Early prenatal diagnosis of conjoined twins is essential for better counselling of parents regarding post natal surgical management or termination of pregnancy. Importance of prenatal MRI for accurate detection of these cases is thereby highlighted.

Siameses toracópagos: Reporte de un caso / Thoracic Siamese: Case Report

Los gemelos siameses son una entidad rara, cuya incidencia varía de 1 en 50.000 a 1 en 100.000 nacidos vivos; solo el 18 % sobrevive y aproximadamente el 35 % de los nacidos vivos muere en las primeras 24 horas. Se reportó un caso de siameses toracópagos, con diagnóstico prenatal mediante ultrasonografía a las 32.1 semanas de gestación, cuyas cabezas y extremidades son individuales; poseen genitales femeninos; a nivel del tórax se observó la existencia de un corazón que envía un vaso arterial a lo que se asemeja a una masa cardiaca inerte y rudimentaria al feto contralateral; comparten los órganos abdominales. El embarazo se interrumpió a las 32.1 semanas de gestación y los pacientes sobrevivieron dos horas, luego de lo cual fallecieron por la cardiopatía compleja.

Siamese twins are rare, occurring 1 in 50 000 to 1 in 100 000 live births; only 18% survive and approximately 35% of live births die in the first 24 hours. We report a case of thoracopagu twins. The prenatal diagnosis was made by ultrasound at 32 weeks of gestation. They had separated heads and extremities; they were females; at the joint thorax there was a single heart that sent an arterial vessel to what resembles a rudimentary cardiac mass in the contralateral fetus. They shared abdominal organs. Pregnancy was interrupted at 32.1 weeks of gestation; patients survived 2 hours.

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.

Gemelos unidos (siameses): descripción de hallazgos anatomopatológicos / Conjoined twins (siamese): description of anatomopathological findings

Resumen OBJETIVO: Describir los hallazgos anatomopatológicos y placentarios en una serie de autopsias de 5 siameses. CASOS CLÍNICOS: Serie de casos de autopsias efectuadas entre 2013 y 2018 de pacientes entre 14 y 30 semanas de embarazo, con diagnóstico de gemelos unidos. RESULTADOS: Se encontraron 5 casos de gemelos unidos, hijos de madres multíparas, 3 tuvieron muerte intrauterina y 2 fallecimiento a los pocos minutos de vida. Tres casos correspondieron a uniones ventrales (2 toracoonfalópagos y 1 cefalópago) y dos a uniones laterales de tipo parápagos, dicéfalos. Hubo corazón único en los gemelos toracoonfalópagos y sistemas cardiacos separados en los tres restantes, donde hubo fusión aórtica distal. En 4 casos el hígado se encontró fusionado, con dos vías biliares independientes. Los cordones umbilicales de los casos de unión ventral tenían 5 vasos sanguíneos. Los de unión lateral tenían cordón umbilical trivascular. Los discos placentarios mostraron grados variables de hipoxia preplacentaria y cambios de malperfusión vascular materna. CONCLUSIONES: Las fusiones ventrales tienen grados variables de estructuras compartidas; entre mayor sea la fusión de los órganos internos, menor es el número de vasos umbilicales. De las teorías etiológicas propuestas, la esférica es la que mejor soporta los grados variables de fusión.

Abstract OBJECTIVE: Description of the anatomopathological and placental findings in a series of autopsies of 5 conjoined twins. MATERIALS AND METHODS: Series of cases of autopsies performed between 2013-2018 of patients between 14-30 weeks of gestation, with diagnosis of conjoined twins. RESULTS: Five cases of conjoined twins, product of multiparous mothers, 3 of these presented intrauterine death and 2 deaths within a few minutes of life. Three cases corresponded to ventral junctions (2 thoracoomphalopagus and 1 cephalopagus) and two to lateral junctions of parapagus type. A single heart was observed in the thoracoomphalopagus twins and separated cardiac systems in the remaining three where there was distal aortic fusion. In 4 cases the liver was found fused with two independent bile ducts. The umbilical cords of the cases of ventral union presented 5 blood vessels; those with lateral junction presented a trivascular umbilical cord. The placentas showed varying degrees of preuterine hypoxic pattern and changes of Maternal Vascular malperfusion of the Placental Bed. CONCLUSIONS: Ventral fusions have varying degrees of shared structures; the greater the fusion of the internal organs, the smaller the number of umbilical vessels will be. Of the proposed etiological theories, it is the so-called spherical theory, which best supports the variable degrees of fusion and how it can be saltatory on the vertical axis.

Enfoque de las gestaciones múltiples de diagnóstico difícil durante la autopsia perinatal / Approach to Perinatal Autopsies in Multiple Gestations with Difficult Diagnoses / Enfoque das gestações múltiplas de diagnóstico difícil durante a autopsia perinatal

Resumen Introducción: Durante el examen de pediatría, genética, ecográfico o de anatomía patológica de fetos de gestaciones gemelares, suelen aparecer interrogantes en el diagnóstico diferencial, especialmente cuando los casos se asocian con maceración, malformaciones o duplicación de estructuras. Objetivo: Proponer un algoritmo para el enfoque de la autopsia perinatal en gestaciones múltiples de difícil diagnóstico. Materiales y Métodos: Estudio observacional descriptivo del universo de gestaciones gemelares vistas en el Departamento de Patología del Hospital Universitario San Ignacio (HUSI) entre 2007 y 2013. Definimos como "gestación múltiple de difícil diagnóstico" aquella en la que el examen del (de los) feto(s) planteó uno de los diagnósticos diferenciales: feto macerado/evanescente, gemelo siamés, fetus in fetu, feto acárdico o teratoma congénito. Se realizó el estudio anatomopatológico y junto con la literatura revisada, los gemelos se analizaron y clasificaron según el algoritmo propuesto. Resultados: Hubo 59 casos de gestaciones gemelares en las que al menos uno de los fetos requirió autopsia perinatal. Las principales causas de muerte fueron las infecciones ascendentes seguidas del síndrome de transfusión feto-fetal. Como gestaciones múltiples de difícil diagnóstico observamos tres fetos acárdicos y ocho fetos macerados. Se aplicó el algoritmo propuesto en este artículo. Conclusiones: El diagnóstico en las gestaciones gemelares puede ser en ocasiones difícil. Identificamos los aspectos más importantes a tener en cuenta de la historia clínica y de la autopsia perinatal para llegar al diagnóstico definitivo. Desarrollamos un algoritmo para ser aplicado en estas circunstancias durante las autopsias perinatales.

Abstract Introduction: During pediatric, genetic, sonographic, or pathological anatomy examination on complicated twin gestational fetuses, questions arise concerning differential diagnosis, especially when cases are associated with malformations, maceration of several months duration, or complex structural duplications. Objective: To propose an algorithm for approaching perinatal autopsy in complicated multiple gestations. Materials and Methods: Descriptive observational study on twin gestations seen in the Hospital Universitario San Ignacio (HUSI) in 2007-2013. We defined "complicated twin gestation" as one in which an examination of the fetus or fetuses raised one of the following differential diagnoses: macerated fetus, conjoined fetuses, fetus in fetu, acardiac fetus, or congenital teratoma. An anatomopathological study was performed and twins were classified according to the proposed algorithm. Results: Analysis of 59 cases in which at least one fetus required a perinatal autopsy. The leading causes of death were ascending infections and fetal-twin transfusion syndrome. Among complicated multiple gestations we observed eight fetuses including three acardiac macerated fetuses. We applied the algorithm proposed in this article. Conclusion: Diagnosis in twin pregnancies can sometimes be difficult. We identify the most important aspects of medical records and perinatal autopsy to be considered in reaching a definitive diagnosis; we develop an algorithm to be applied in these circumstances.

Resumo Introdução: Durante o exame de pediatria, genética, ecográfico ou de anatomia patológica de fetos de gestações gemelares, costumam aparecer interrogantes no diagnóstico diferencial, especialmente quando os casos se associam com maceração, com malformações ou com duplicação de estruturas. Objetivo: Propor um algoritmo para o enfoque da autopsia perinatal em gestações múltiplas de difícil diagnóstico. Materiais e Métodos: Estudo observacional descritivo do universo de gestações gemelares vistas no Departamento de Patologia do Hospital Universitário San Ignacio (HUSI) entre 2007 e 2013. Definimos como "gestação múltipla de difícil diagnóstico" aquela na que o exame do(s) feto(s) apresentou um dos diagnósticos diferenciais: feto macerado/evanescente, gêmeo siamês, fetus in fetu, feto acárdico ou teratoma congênito. Realizou-se o estudo anatomopatológico e junto com a literatura revisada, os gêmeos se analisaram e classificaram segundo o algoritmo proposto. Resultados: Houve 59 casos de gestações gemelares nas que pelo menos um dos fetos requereu autopsia perinatal. As principais causas de morte foram as infeções ascendentes seguidas da síndrome de transfusão feto-fetal. Como gestações múltiplas de difícil diagnóstico observamos três fetos acárdicos e oito fetos macerados. Aplicou-se o algoritmo proposto neste artigo. Conclusões: O diagnóstico nas gestações gemelares pode ser em ocasiões difícil. Identificamos os aspectos mais importantes a ter em conta da história clínica e da autopsia perinatal para chegar ao diagnóstico definitivo; desenvolvemos um algoritmo para ser aplicado nestas circunstâncias durante as autopsias perinatais.

Anesthetic management for separation of thoracopagus twins with complex congenital heart disease: a case report / 대한마취과학회지

Although thoracopagus twins joined at the upper chest are the most common type of conjoined twins, the separation surgery in these cases has a higher mortality rate. Here, we describe an anesthetic management approach for the separation of thoracopagus conjoined twins sharing parts of a congenitally defective heart and liver. We emphasize the importance of vigilant intraoperative hemodynamic monitoring for early detection of unexpected events. Specifically, real-time continuous monitoring of cerebral oximetry using near-infrared spectroscopy allowed us to promptly detect cardiac arrest and hemodynamic deterioration.

Conjoined twins: report of two cases and review of literature.

Two pairs of conjoined twins were encountered at the SCB medical college cuttack, Odisha, India during the past 3 years. In the first set the prenatal diagnosis was not established and the case was referred to us from other hospital with diagnosis of twin pregnancy and prolonged second stage of labour. Emergency caesarean section done and a pair of thoraco-omphalopagus twins delivered. Both babies died soon after birth. In the second case the conjoined twins were diagnosed prenatally at 20 weeks of gestationon in routine anomaly scan as thoraco-omphalopagus twin with fetuses were positioned face-to-face and fused from sternum to umbilicus. Fetuses were found to have separate hearts, a fused liver and separate upper and lower gastrointestinal tracts. Each twin had two normal appearing kidneys and a urinary bladder. The couples were counselled about the various management options and referred to higher centre. But she was admitted at 34 weeks of gestation with preterm labour and emergency caesarean section done and a pair of male thoraco-omphalopagus twins delivered. Both babies were alive and referred to higher centre for further management. The prenatal identification of conjoined twins is of cardinal importance for the planning of delivery and possible separation.

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.

Eighteen Year Follow-Up Results of Accessory Lower Limb Disarticulation and Pelvic Bone Reconstruction for Monocephalus Tripus Tribrachius / 대한정형외과학회잡지

Monocephalus tripus tribrachius, a type of conjoined twins with one head and three upper and lower extremities, is a rare congenital disorder. To date, no long-term follow-up results of surgical procedures for this condition have been reported in Korean literature. We experienced a case of monocephalus tripus tribrachius, which had been surgically managed with an accessory lower limb disarticulation and pelvic bone reconstruction to manage this accessory limb and accompanying comorbidities in hip joint and pelvis. Subsequently, ipsilateral Syme amputation was done for intractable deformity of foot, and later, ipsilateral femoral varus derotational osteotomy was done for inadequate coverage of femoral head observed in follow-up. We report 18-year follow-up results of the procedures with a review of literatures.

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 bicéfalos / Conjoined twins

Los gemelos siameses son individuos que se desarrollan unidos entre sí por alguna región de su anatomía, están conectados vascularmente y pueden compartir uno o varios órganos. Se presenta un caso de siameses bicéfalos, con el objetivo de describir los hallazgos anatomopatológicos, exponer aspectos relacionados con las posibles causas que los originaron, así como el diagnóstico prenatal de estos defectos congénitos.

Conjoined twins are individuals who develop together by some region of his anatomy, vascularly connected and they can share one or more organs. We report a case of two-headed conjoined twins, with the aim of describing the pathologic findings, exposing aspects of possible causes of these accidents and prenatal diagnosis of these defects.

Siameses / Siamese

Introducción: el nacimiento de siameses es siempre un evento raro e inevitablemente ha deslumbrado a especialistas y personas ajenas a la Medicina. las primeras siamesas Mery y Eliza fueron reportadas en el año 1100. Objetivo: exponer el primer caso reportado en Cuba en el 1er. trimestre del embarazo. Paciente: se reporta un caso de siameses onfalópagos diagnosticado en el 1er. trimestre del embarazo en el Centro Municipal de Genética de San Miguel del Padrón. Se detectó incremento de la translucencia nucal en ambos gemelos y defecto de cierre del tubo neural en uno de ellos, en el municipio. En el nivel terciario se confirman estos marcadores y se detectan alteraciones del flujo del ductus venoso en ambos fetos. El estudio citogenético informó fetos cromosómicamente normales. Se interrumpe el embarazo a las 17 sem. Resultados: el estudio anatomopatológico confirmó la presencia de siameses onfalópagos, de sexo femenino. Ambos fetos compartían hígado y porción de intestino delgado. Se observó riñón izquierdo hipoplásico en el primer feto. El segundo gemelo presentó defecto del tubo neural, mielomeningocele sacrococcígeo y agenesia de sínfisis del pubis. Conclusiones: se comprueba la eficacia del programa cubano para la detección temprana de malformaciones congénitas

Introduction: conjoined twins are always a rare event, and inevitably it has puzzled specialists and people outside of medicine. The first Siamese Mery and Eliza were reported in 1100. Objective: to describe the first case reported in Cuba in the 1st. trimester of pregnancy. Patient: It is reported a case of omphalopagus conjoined twins diagnosed in the 1st. trimester of pregnancy in the Municipal Center of Genetics, San Miguel del Padrón. in the township consultation, we detected increased nuchal translucency in both twins, as well as defects in neural tube closure in one of them. At the tertiary level of care, these markers are confirmed, and changes in the venous ductus flow on both fetuses were detected. The cytogenetic study reported chromosomally normal fetuses. This pregnancy was interrupted at 17 weeks. Results: the pathologic study confirmed the presence of female omphalopagus conjoined twins. Both fetuses shared liver and a portion of small intestine. Hypoplastic left kidney was observed in the first fetus. The second twin presented neural tube defect, myelomeningocele and sacrococcygeal agenesis of the symphysis pubis. Conclusions: the effectiveness of the Cuban program for early detection of congenital malformations is confirmed

Registro de un Monstruo Diprósopo de Tiburón Azul (Prionace glauca) en Chile / Record of a Diprosopus Monster of Blue Shark (Prionace glauca) from Chile

Un espécimen de siameses de tiburón azul, Prionace glauca, fue recolectado durante actividades de pesca en el norte de Chile. Según una clasificación basada en el grado de duplicación y el sitio de fusión de los gemelos, el espécimen corresponde a un monstruo del tipo diprósopo tetroftalmo. El individuo tiene una duplicación craneofacial parcial con presencia de cuatro globos oculares e igual número de narinas. Las cavidades orales están interconectadas convergiendo en una única faringe. El tronco es simple; la columna presenta lordosis torácica y una torsión helicoidal en su porción caudal. Este es el primer reporte de siameses en condrictios chilenos y primer registro mundial de diprosopa en tiburones.

A conjoined twins specimen of blue shark Prionace glauca was collected during fishing activities in Northern Chile. According to a classification based on duplication degree and fusion site of the twins, it represents a monster of diprosopus tetrophthalmus type. The specimen has a partial craniofacial duplication with presence of four eyeballs and an equal number of nostrils. Oral cavities are interconnected, converging in a single pharynx. The trunk is simple; the vertebral column presents thoracic lordosis and helical torsion in its caudal portion. This is the first Chilean record of conjoined twins to chondrichthyan and first world record of diprosopia in sharks.

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.

Thoracopagus conjoined twin - a case report.

A patient at her 38+ wks of pregnancy as a ultrasonographically diagnosed case of conjoined twin admitted in our department with ruptured membrane. Two live female babies joined at the chest were delivered by caesarean section. The parents refused a separation operation and the mother and the babies were discharged from hospital at 6th post natal day. A review of the literature suggests that early diagnosis by a combination of ultrasound and MRI is essential for management as it provides prognosis for viability and process of surgical separation and also the opportunity for early counseling of parents and termination if indicated.

Dicephalus dipus tribrachius: A case report of unusual conjoined twins.

A conjoined twin is a rarity. It occurs 1 in 50,000 to 1 in 2,00,000 fetuses. Forty percent of the conjoined twins are stillborn and an additional one-third die within 24 h of birth. They result from late twinning events about 14th day after fertilization. We report a case of stillborn conjoined twins sent for autopsy. The bodies of the fetuses were fused from the thorax to the pelvis. There were two heads, three upper limbs and two lower limbs, with fusion of the thoracic, abdominal and pelvic regions. On systemic examination, some organs were fused and some were separate. A multilocular cyst with milky fluid was seen in the pelvic region. This case is reported in view of its rarity.