RESUMO
The case involves a 23-year-old Dominican woman's admission to Hospital Docente Nuestra Señora De la Altagracia for an elective cesarean section at 38 weeks gestation with conjoined twins. Despite effective treatment for syphilis in the third trimester, her medical history complicated the situation. The twins, thoraco-omphalopagus conjoined, share vital organs and exhibit congenital anomalies, posing unique diagnostic and management challenges. This case contributes to the scarce literature on conjoined twins, especially in the Dominican Republic. It highlights the complexities of diagnosis, prognosis, and management strategies for such rare cases. This emphasizes the importance of ongoing research and medical intervention in addressing these challenges.
RESUMO
Diprosopus is a congenital anomaly in which partial or complete duplication of craniofacial structures occurs. Because it is rare, the mortality rate is high, and information concerning this anomaly is scarce. This study describes a case of human diprosopus in a 9-year-old male individual, who has severe complications associated with the central nervous, cardiovascular, respiratory, and digestive systems. Since birth, he has been monitored in a specialized hospital environment, where he has undergone several surgeries and multidisciplinary treatments. Regarding the craniofacial aspects, he had agenesis of the corpus callosum, floor of the nasal cavity, and floor of the anterior cranial fossa, in addition to the presence of bone dysplasia, ocular hypertelorism and cleft palate with nasal and oral teratoma. Regarding dental characteristics, the patient has duplication of the maxilla, mandible, tongue, and some teeth. After complementary imaging exams, several supernumerary teeth were found, with some being impacted and in complex regions, with an indication for extraction due to the risks of impaction, irruptive deviation, root resorption, and associated cystic or tumoral lesions. Because of the numerous complications, knowledge, and preparation of the entire team is necessary for the correct management of the case.
Assuntos
Anormalidades Craniofaciais , Humanos , Masculino , Criança , Agenesia do Corpo Caloso , Fissura Palatina , Dente Supranumerário/diagnóstico por imagem , Dente Supranumerário/cirurgia , Teratoma/congênito , Teratoma/cirurgia , Teratoma/diagnóstico por imagem , Hipertelorismo , Anormalidades Múltiplas , Cavidade Nasal/anormalidades , Cavidade Nasal/diagnóstico por imagemRESUMO
BACKGROUND: The condition of monozygotic, monochorionic triplet fetuses with a pair of conjoined twins is extremely rare (close to one in a million births), presents challenges in its management, and with poor prognosis. CASE REPORT: We report a case of monochorionic diamniotic triplet pregnancy, ultrasound at 14 weeks shows a pair of conjoined thoracopagus fetuses, sharing heart, liver, and umbilical cord, in addition to omphalocele. The third fetus, without malformations, presents signs of early heart failure compatible with twin-to-twin transfusion syndrome. It was decided to carry out expectant management where at 18 weeks, intrauterine death of the three fetuses occurs. An abortion is performed by hysterotomy. CONCLUSIONS: The treatment in these cases is discussed, three management options have been proposed: expectant management, selective reduction of the conjoined fetuses, or termination of the pregnancy. A review of the literature found only 12 cases with this combination of pathologies, in which only 3 normal fetuses (25%) survived and none of the conjoined twins survived. To our knowledge, this case is the first of a monochorionic triplet pregnancy with conjoined fetuses complicated with early twin-to-twin transfusion.
Assuntos
Transfusão Feto-Fetal , Gravidez de Trigêmeos , Gêmeos Unidos , Feminino , Gravidez , Humanos , Transfusão Feto-Fetal/complicações , Morte Fetal/etiologia , Feto/anormalidadesRESUMO
Conjoined twins (CTs), popularly referred to as Siamese twins, are a rare anomaly due to monochorionic and monoamniotic twin pregnancies. Dicephalus dibrachius dipus, a type of parapagus conjoined twin which is characterized by possessing two arms, two legs, a single trunk and two heads, epidemiologically, is an even rarer occurrence of CTs. In this article, a rare, well-preserved anatomical specimen of a dicephalus dibrachius dipus conjoined twin is presented. This study was conducted in a specimen which is part of the collection of the Embryology Museum of the institution by donation and approved by the Research Ethics Committee (REC). The female conjoined twins were born at full-term by cesarean section in the 1970s and died hours after birth. A thorough anatomical description was made through observational analysis, computed tomography and 3D reconstructed images. Major abnormalities were observed in the cardiovascular, respiratory and digestive systems. The internal anatomy exhibited a heart with three atria, two ventricles, two aortic arches, two pulmonary arteries, one innominate venous trunk and a respiratory system with two tracheas and four lungs. No other report was similar to our three atria heart description. This article provides a thorough anatomical description of all systems, which is valuable information for further studies on CTs.
Assuntos
Gêmeos Unidos , Humanos , Gravidez , Feminino , Cesárea , Tomografia Computadorizada por Raios X , Imageamento TridimensionalRESUMO
BACKGROUND: Parapagus diprosopus are conjoined twins characterized by craniofacial duplication and only one body, representing one of the rarest types of these twins. Their occurrence has been recorded in different species of vertebrates, including humans, but few cases have been studied in domestic pigs. CASE: A pair of conjoined twin pigs was studied using x-rays, computed tomography, and necropsy. The abnormalities found were compared with those of the rare swine cases presented in the literature as well as with other species, and the different etiopathogenetic possibilities were addressed. The degree of duplication of the head bones decreased caudally, as did that of the structures of the central nervous system. In the two oral cavities, there was a complete cleft palate. All the cervical vertebrae and thoracic vertebrae up to T3 were partially duplicated. The heart and great vessels were normal, as were the other thoracic and abdominal organs. CONCLUSIONS: The conjoined twin pigs of this study are a case of parapagus diprosopus tetraophthalmus triotus, presenting the same pattern of abnormalities of human diprosopus and that of other species. The scarcity of detailed studies on craniofacial duplication in pigs and the lack of a definitive explanation on the etiology and pathogenesis of conjoined twins shows the need for further research and the publication of more cases.
Assuntos
Fissura Palatina , Gêmeos Unidos , Humanos , Suínos , Animais , Sus scrofa , Fissura Palatina/patologia , Tomografia Computadorizada por Raios XRESUMO
Background: Congenital anomalies are structural, functional, or metabolic defects caused by a combination of environmental, genetic, or even iatrogenic factors. Genetic defects, which can be inherited, are more common in purebred dogs. Teratogenic factors such as radiation, toxins, chemical agents, infectious diseases, mechanical influences, drugs given to the mother, and nutrition can affect the litter during gestational development. The incomplete division of a fertilized egg results in monozygotic, conjoined or Siamese twins, which are animals with complete or incomplete duplications. This paper reports on an adult bitch with monocephalus dipygus dibrachius and the surgical procedures. Case: A 2-year-old female Shih Tzu weighing 5 kg was admitted to a veterinary clinic, presenting with swelling and myiasis near the anus and several development disorders, characterized by 2 pelvises, 2 anuses, 2 vulvas, 2 forelimbs and 6 hindlimbs. Her physiological parameters were otherwise normal. Only the dog's myiasis was treated at this time due to the owner's financial straits. After 5 months, the owner brought the bitch back to the veterinary clinic because the animal presented with fecaloma in 1 of the anuses. Radiography revealed numerous alterations: seven lumbar vertebrae with marked vertebral axis deviation, reduced disc space, as well as ankylosis and fused ventral spondylosis at L6 and L7. Two pelvises fused medially by the wings of the ileum, with slight deviation and thinning of pelvic bones. Four hip joints and medial joints with pelvic avulsion and bone remnants of the pelvic limbs. Acetabular tearing slightly flattened femoral head and thickened femoral neck. Caudal vertebrae and vertebral axis located in left pelvis. Left lateral patella inserted in the trochlear groove and lateral dislocation of right patella. Right patellofemoral joint with smooth surface, preserved intra-articular density and cranial displacement of the tibia relative to the femoral condyles (cranial cruciate ligament rupture). An ultrasound analysis revealed 2 bladders. Two months later surgery was performed due to recurrent complications. During laparotomy 2 uteruses, 2 bladders and bifurcation of the intestine were observed. Ovariosalpingohysterectomy was performed in both uterus and enterectomy of the problematic intestinal portion. After 2 days of the surgery, blood transfusion was performed. After 2 days of the transfusion, there was extravasation of yellow fluid from the surgical cut and abdominal palpation was indicative of bladder rupture, so the patient was sent to emergency surgery. Unilateral nephrectomy and ureterectomy, and ruptured bladder cystectomy were performed. The dog remained hospitalized for 24 days after surgery, before it was released. Discussion: The classification of conjoined twins is based on the location of the junction and the number of limbs. Monocephalus dipygus dibrachius was diagnosed based on the fact that the dog had 1 skull, 2 thoracic limbs and 4 pelvic limbs, as well as the corresponding genitourinary and gastrointestinal tract alterations. Imaging scans are extremely important for a proper diagnosis to ensure appropriate surgery planning. The bitch was the result of inbreeding between a male dog and its offspring, which probably contributed to this malformation. There are very few reports of surviving adult conjoined animals, and even fewer descriptions of successful surgical treatments. To the best of knowledge of the authors, there are no previous reports of a surviving adult dog suffering from this malformation.
Assuntos
Animais , Feminino , Cães , Gêmeos Unidos/cirurgia , Gêmeos Unidos/patologia , Anormalidades Congênitas/veterináriaRESUMO
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.
Assuntos
Humanos , Feminino , Gravidez , Adolescente , Adulto , Gêmeos Unidos , Diagnóstico Pré-Natal , Cesárea , Ultrassonografia Pré-Natal , Gravidez de GêmeosRESUMO
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)
Assuntos
Animais , Gatos , Gêmeos Unidos , Gatos/anormalidades , Gatos/embriologia , Anormalidades Congênitas/veterinária , Gemelação EmbrionáriaRESUMO
OBJECTIVE: To review the perinatal and long-term outcomes of symmetric and ventrally conjoined twins evaluated prenatally by ultrasound and fetal magnetic resonance imaging (MRI). METHODS: From March 2010 to January 2019, cases of symmetric and ventrally conjoined twins, who were prenatally diagnosed and referred to the Clínica Diagnóstico por Imagem (CDPI), Rio de Janeiro, Brazil, for prenatal evaluation by ultrasound and MRI were selected. The postnatal information was collected from hospitals where the twins were born and/or treated and from parents' verbal and written information. RESULTS: Four cases of symmetrical and ventrally conjoined twins were selected. Of these, two were omphalopagi and two thoracopagi. One pair of thoracomphalopagus died early in utero and the other died 6 days after birth. The outcome of the two omphalopagus pairs were separation in emergency surgeries after birth, with neonatal demise of one of the twins due to congenital malformations. In cases of omphalopagi, fetal MRI presented important information of the twins' anatomy before emergency separation of both pairs. CONCLUSION: Despite the apparently similar conditions of twins with ventral fusion, ventrally attached twins have very different outcomes, most adverse for thoracomphalopagus and related to the singular anatomy of the pair, associated malformations and the extension of the adhesion, requiring individual evaluation of the cases. Fetal MRI is as an important tool for the postnatal surgery management of twin neonates, providing crucial information in cases where urgent separation is required.
Assuntos
Gêmeos Unidos , Brasil , Feminino , Humanos , Recém-Nascido , Imageamento por Ressonância Magnética , Gravidez , Ultrassonografia , Ultrassonografia Pré-NatalRESUMO
SUMMARY BACKGROUND: Asymmetric or heteropagus conjoined twins is a rare occurrence, with an incidence of one case in 1-2 million. Conjoined twins are classified according to their symmetry, place of fusion, and grade of duplication. METHODS: We report here an extremely rare presentation of parasitic twins not described before. We describe macro and micro anatomic alterations and discuss the aspects of this peculiar presentation and the importance of prenatal diagnosis. RESULTS: The case of a twenty-three-year-old patient, with monochorionic, monoamniotic asymmetrically-conjoined twin pregnancy, discovered at 29 weeks of gestational age. We believe that this report calls attention to this presentation and the importance of prenatal care and management. The twins were delivered vaginally without life. The twins' combined weight was 1.300 gr. They were bonded in the left cervical region. CONCLUSION: This report may help to find strategies for clinical decisions in future cases. Antepartum diagnosis is important to the management, preoperative planning, and outcomes. Prenatal imaging exams like echocardiography, CT, MRI, and ultrasonography are feasible and can provide relevant information about malformation severity and prognosis.
RESUMO BACKGROUND: Gêmeos parasitas assimétricos ou heterópagos são uma ocorrência rara, com incidência de um caso em 1-2 milhões. Os gêmeos siameses são classificados de acordo com sua simetria, local de fusão e grau de duplicação. MÉTODOS: Relatamos aqui uma apresentação extremamente rara de gêmeos parasitários não descritos anteriormente. São descritas alterações macro e microscópicas e discutidos aspectos relevantes dessa malformação e da importância do diagnóstico pré-natal. RESULTADOS: Um caso de uma paciente de 23 anos de idade, com gestação monocoriônica, monoamniótica de gêmeos siameses assimétricos diagnosticada com 29 semanas de idade gestacional. Acreditamos que este relato chama a atenção para esta apresentação e para a importância do cuidado e manejo pré-natal. Os fetos nasceram de parto vaginal já sem vida, pesando em conjunto 1.300 gramas, e eram unidos pela região cervical esquerda. CONCLUSÃO: Este relato pode ajudar a encontrar estratégias para a decisão clínica em casos futuros. O diagnóstico pré-natal é fundamental para o manejo e planejamento pré-operatório. Exames de imagem como ecocardiografia, tomografia computadorizada, ressonância magnética e ultrassonografia são factíveis e podem fornecer informações-chave sobre a gravidade e prognóstico da malformação.
Assuntos
Humanos , Feminino , Gravidez , Adulto Jovem , Gêmeos Unidos , Gravidez de Gêmeos , Diagnóstico Pré-Natal , Gêmeos , Ultrassonografia Pré-NatalRESUMO
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.
Assuntos
Humanos , Recém-Nascido , Diagnóstico Pré-Natal , Anormalidades Congênitas , Gêmeos Unidos , Ultrassonografia , Mortalidade Prematura , CardiopatiasRESUMO
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.
Assuntos
Humanos , Feminino , Gravidez , Adolescente , Gêmeos Unidos/cirurgia , Ultrassonografia Pré-Natal , Assistência Perioperatória/métodos , Anestesia/métodos , Respiração Artificial , Cesárea , Intubação Intratraqueal/métodosRESUMO
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 to 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 11hours of cesarean surgery, the pediatric surgical team chose to separate the twins. They were monitored with cardioscopy, oximetry, capnography, nasopharyngeal thermometer, urinary output, and noninvasive 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 (6mL.kg-1) and lumbar epidural (L1-L2) with 0.2% ropivacaine (2.5mg.kg-1) were used. The pediatric surgical team initiated the separation of the twins via sternotomy, ligation of hepatic vessels. After 2hours 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.
Assuntos
Anestesia/métodos , Assistência Perioperatória/métodos , Gêmeos Unidos/cirurgia , Ultrassonografia Pré-Natal/métodos , Adolescente , Cesárea , Feminino , Humanos , Intubação Intratraqueal/métodos , Gravidez , Respiração ArtificialRESUMO
BACKGROUND: Conjoined twins are among the most rare and challenging congenital malformations. The incidence varies from 1:30 000 to 1:200 000 live births. Pygopagus twins account for about 10 to 18% of all conjoined twins. CLINICAL CASE: We report the case of male conjoined pygopagus twins. They shared medullary canal, anal canal and sacrococcygeal region, without any degree of cross circulation. At the moment of the surgery, twin 1 had congenital heart disease without medical treatment. Twin 2 presented craniofacial malformations. The total organic separation was performed electively, by being driven by two teams of anesthesiologists with balanced general anesthesia, intravenous and sequential induction intubation. Non-invasive and invasive monitoring was used for both twins. During the transanesthetic period, twin 1 presented multiple hypercyanotic episodes associated to hypovolemia and hypotension. Twin 2 remained stable. During the final separation acute bleeding and hemodynamic instability showed up, requiring the use of inotropes, vasopressors and blood components. The twins were sent to intensive care where they remained under surveillance for three days. They were discharged a month and a half after the surgical event. CONCLUSIONS: Each type of siamese has specific characteristics depending on the type of union; comprehensive knowledge of the morphology and physiopathology of the twins is essential for optimal anesthetic management.
INTRODUCCIÓN: los gemelos unidos son una de las más raras y desafiantes malformaciones congénitas. Su incidencia varía de 1:30 000 a 1:200 000 nacidos vivos. Los gemelos pygopagus (pigópagos) representan del 10 al 18% de todos los gemelos unidos. CASO CLÍNICO: se presenta el caso de gemelos masculinos unidos pigópagos. Compartían canal medular, canal anorrectal y región sacrococcígea y no tenían ningún grado de circulación cruzada. Al momento del evento quirúrgico, el gemelo 1 era portador de cardiopatía congénita cianógena sin tratamiento médico. El gemelo 2 tenía malformaciones craneofaciales. La separación orgánica total se realizó de forma electiva; se llevó a cabo por dos equipos de anestesiólogos, con anestesia general balanceada, inducción intravenosa e intubación secuencial. Se empleó monitoreo no invasivo e invasivo para ambos. El gemelo 1 presentó durante el transanestésico múltiples episodios de hipercianosis asociados a hipovolemia e hipotensión. El gemelo 2 se mantuvo estable. Durante la separación definitiva se presentó sangrado agudo e inestabilidad hemodinámica, lo cual requirió el uso de inotrópicos, vasopresores y hemocomponentes. Los gemelos egresaron a terapia, donde permanecieron en vigilancia durante tres días. Fueron dados de alta un mes y medio después del evento quirúrgico. CONCLUSIÓN: cada tipo de siameses tiene características específicas dependiendo del tipo de unión; el conocimiento de la morfología y la fisiopatología es esencial para ofrecer un óptimo manejo anestésico.
Assuntos
Anestesia/métodos , Gêmeos Unidos/cirurgia , Humanos , Lactente , MasculinoRESUMO
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.
RESUMO
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.
Assuntos
Humanos , Masculino , Adolescente , Gêmeos Unidos/cirurgia , Incontinência Urinária/cirurgia , Extrofia Vesical/cirurgia , Epispadia/cirurgia , Reoperação/métodos , Falha de Tratamento , Procedimentos de Cirurgia Plástica/métodos , Ilustração MédicaRESUMO
Se denominan siameses a aquellos gemelos que comparten estructuras anatómicas en grado variable. La incidencia varía entre 1:50 000 a 1:100 000 embarazos, 1:4 de los casos fallecen in utero y 40-60 por ciento mueren poco después del parto.Los hallazgos en autopsias médico-científicas de gemelos unidos cefalópagos, que son aquellos que presentan fusión cefálica con dos hemicaras, son el cuarto tipo de siameses en ocurrencia y su incidencia está estimada en 1 por cada 3'000.000 de embarazos o 1/58 siameses. El objetivo es presentar los hallazgos de una autopsia médico-científica de gemelos unidos cefalópagos. Se trata de gemelos cefalópagos, con terminación de embarazo por incompatibilidad con la vida extrauterina. Presentan cordón umbilical único de cinco vasos, tenían cráneos fusionados a nivel frontofacial con dos caras en lados opuestos de la cabeza (Janiceps), con fusión ventro-ventral de los ejes somáticos hasta debajo del ombligo sobre la pelvis, con sistemas respiratorios, cardiaco y genitourinario independientes, el sistema digestivo superior esta fusionado hasta el intestino medio, con duplicación a partir de la tercera porción del duodeno. Los gemelos siameses cefalópagos no son los más infrecuentes, pero es difícil encontrar series de casos y revisiones sistemáticas de sus características y patogénesis en Latinoamérica. Son escasas las estadísticas sobre siameses y no se ha descrito previamente ningún caso como éste. Se hace necesaria la realización de estudios de autopsias para mejorar la descripción y caracterización de estos casos y poder determinar si existen otras condiciones asociadas además de las teorías de unión embriológica(AU)
It is denominated siamese to those twins that share anatomical structures to a variable degree. The incidence varies between 1/50.000 and 1/100.000 pregnancies, 1:4 of the cases die in the uterus and 40-60 percent die shortly after birth. Findings in a medical-scientific autopsies of conjoined cephalopagus twins, those that presenting cephalic fusion with two hemi faces, are the fourth type of Siamese in occurrence and their incidence is estimated in 1 every 3´000.000 pregnancies or 1/58 siamese. It is about cephalopagus twins, with termination of pregnancy due to incompatibility with extrauterine life. They presented a five vessels common umbilical cord, fused craniums at frontofacial level with two faces in opposed sides of the head (Janiceps), ventro-ventral fusion of the somatic axis until below the umbilicus and above the pelvis, independent respiratory, cardiac and genitourinary systems. The superior digestive system was fused until the medium intestine, with duplication parting from the third portion of the duodenum. Conjoined cephalopagus twins are not the most frequent but it is hard to find a series of cases and systemic revisions of their characteristics and pathogenesis in Latin America. Statistics on Siamese twins are scarce and it has not been previously described any case like this one. Autopsy studies becomes necessary to improve the description and characterization of these cases and to determine the existence of any other associated conditions different to those of the embryologic union theory(AU)
Assuntos
Humanos , Feminino , Gravidez , Adulto , Gêmeos Unidos/patologia , Cabeça/anormalidadesRESUMO
Se denominan siameses a aquellos gemelos que comparten estructuras anatómicas en grado variable. La incidencia varía entre 1:50 000 a 1:100 000 embarazos, 1:4 de los casos fallecen in utero y 40-60 por ciento mueren poco después del parto.Los hallazgos en autopsias médico-científicas de gemelos unidos cefalópagos, que son aquellos que presentan fusión cefálica con dos hemicaras, son el cuarto tipo de siameses en ocurrencia y su incidencia está estimada en 1 por cada 3'000.000 de embarazos o 1/58 siameses. El objetivo es presentar los hallazgos de una autopsia médico-científica de gemelos unidos cefalópagos. Se trata de gemelos cefalópagos, con terminación de embarazo por incompatibilidad con la vida extrauterina. Presentan cordón umbilical único de cinco vasos, tenían cráneos fusionados a nivel frontofacial con dos caras en lados opuestos de la cabeza (Janiceps), con fusión ventro-ventral de los ejes somáticos hasta debajo del ombligo sobre la pelvis, con sistemas respiratorios, cardiaco y genitourinario independientes, el sistema digestivo superior esta fusionado hasta el intestino medio, con duplicación a partir de la tercera porción del duodeno. Los gemelos siameses cefalópagos no son los más infrecuentes, pero es difícil encontrar series de casos y revisiones sistemáticas de sus características y patogénesis en Latinoamérica. Son escasas las estadísticas sobre siameses y no se ha descrito previamente ningún caso como éste. Se hace necesaria la realización de estudios de autopsias para mejorar la descripción y caracterización de estos casos y poder determinar si existen otras condiciones asociadas además de las teorías de unión embriológica(AU)
It is denominated siamese to those twins that share anatomical structures to a variable degree. The incidence varies between 1/50.000 and 1/100.000 pregnancies, 1:4 of the cases die in the uterus and 40-60 percent die shortly after birth. Findings in a medical-scientific autopsies of conjoined cephalopagus twins, those that presenting cephalic fusion with two hemi faces, are the fourth type of Siamese in occurrence and their incidence is estimated in 1 every 3´000.000 pregnancies or 1/58 siamese. It is about cephalopagus twins, with termination of pregnancy due to incompatibility with extrauterine life. They presented a five vessels common umbilical cord, fused craniums at frontofacial level with two faces in opposed sides of the head (Janiceps), ventro-ventral fusion of the somatic axis until below the umbilicus and above the pelvis, independent respiratory, cardiac and genitourinary systems. The superior digestive system was fused until the medium intestine, with duplication parting from the third portion of the duodenum. Conjoined cephalopagus twins are not the most frequent but it is hard to find a series of cases and systemic revisions of their characteristics and pathogenesis in Latin America. Statistics on Siamese twins are scarce and it has not been previously described any case like this one. Autopsy studies becomes necessary to improve the description and characterization of these cases and to determine the existence of any other associated conditions different to those of the embryologic union theory(AU)
Assuntos
Humanos , Feminino , Gravidez , Adulto , Gêmeos Unidos/patologia , Cabeça/anormalidadesRESUMO
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.
Assuntos
Humanos , Doenças em Gêmeos , Autopsia , Teratoma , Gêmeos Unidos , Colômbia , Diagnóstico Diferencial , NatimortoRESUMO
BACKGROUND: Diprosopus is a subtype of symmetric conjoined twins with one head, facial duplication and a single trunk. Diprosopus is a very rare congenital anomaly. METHODS: This is a systematic review of published cases and the presentation of two new cases born in Argentina. We estimated the prevalence of conjoined twins and diprosopus using data from the National Network of Congenital Anomalies of Argentina (RENAC). RESULTS: The prevalence of conjoined twins in RENAC was 19 per 1,000,000 births (95% confidence interval, 12-29). Diprosopus prevalence was 2 per 1,000,000 births (95% confidence interval, 0.2-6.8). In the systematic review, we identified 31 diprosopus cases. The facial structures more frequently duplicated were nose and eyes. Most frequent associated anomalies were: anencephaly, duplication of cerebral hemispheres, craniorachischisis, oral clefts, spinal abnormalities, congenital heart defects, diaphragmatic hernia, thoracic and/or abdominal visceral laterality anomalies. One of the RENAC cases and three cases from the literature had another discordant nonmalformed twin. CONCLUSION: The conjoined twins prevalence was similar to other studies. The prevalence of diprosopus was higher. The etiology is still unknown. The presence of visceral laterality anomalies may indicate the link between diprosopus and the alteration or duplication of the primitive node in the perigastrulation period (12-15 days postfertilization). Pregnancies of more than two embryos may be a risk factor for diprosopus. Given the low prevalence of this defect, it would be useful to perform studies involving several surveillance systems and international consortiums. Birth Defects Research (Part A), 2016. © 2016 Wiley Periodicals, Inc. Birth Defects Research (Part A) 106:993-1007, 2016. © 2016 Wiley Periodicals, Inc.