Perfusão arterial reversa gemelar: revisão da literatura / Twin reverse arterial perfusion: literature review

A perfusão arterial reversa gemelar é uma anormalidade rara que pode ocorrer em gestações gemelares monocoriônicas. Consiste em uma alteração na circulação fetoplacentária, com desvio de sangue de um dos gemelares para o outro, por meio de anastomoses arterioarteriais e venovenosas na superfície placentária e anastomoses arteriovenosas em áreas de circulação placentária compartilhada. O feto bombeador pode desenvolver insuficiência cardíaca devido ao aumento do débito cardíaco, e o feto receptor, perfundido por sangue pobre em oxigênio por meio do fluxo reverso, é severamente malformado, incompatível com a vida extrauterina. Este artigo apresenta o caso de uma gestação gemelar monocoriônica diamniótica, com manejo clínico conservador. O objetivo é relatar um caso de complicação rara de gestações monozigóticas e revisar condutas para diagnóstico e manejo adequado.(AU)

Twin reverse arterial perfusion is a rare abnormality that can occur in monochorionic twin pregnancies. It consists of an alteration in the fetal-placental circulation, with blood diversion from one of the twins to the other, through arterio-arterial and veno- venous anastomosis on the placental surface and arterio-venous anastomosis in areas of shared placental circulation. The pumping fetus may develop heart failure due to increased cardiac output, and the recipient fetus, perfused by oxygen-poor blood through reverse flow, is severely malformed, incompatible with extrauterine life. This article presents the case of a monochorionic diamniotic twin pregnancy, with conservative clinical management. The objective is to report a case of rare complication of monozygotic pregnancies and review procedures for diagnosis and adequate management.(AU)

Síndrome de transfusão feto-fetal: revisão de literatura / Twin-twin transfusion syndrome: a literature review

A síndrome de transfusão feto-fetal é uma complicação das gestações gemelares monocoriônicas. Além de ocorrer comumente no segundo trimestre, apresenta elevada morbimortalidade fetal e neonatal, e taxas de incidência que variam de 10 a 15% dentre todas as gravidezes monocoriônicas. O objetivo deste estudo é realizar uma revisão de literatura a partir de levantamento bibliográfico acerca dos principais aspectos epidemiológicos, clínicos e terapêuticos da STFF. A base de dados PubMed foi consultada, uma vez que os termos de pesquisa utilizados foram "síndrome de transfusão feto-fetal", "diagnóstico" e "tratamento". Obtiveram-se sessenta e oito artigos de revisão de literatura e/ou revisão sistemática, sendo que apenas vinte e nove foram selecionados após aplicação dos critérios de elegibilidade. Em relação à fisiopatologia, a síndrome é explicada pela transferência sanguínea direta entre os fetos gemelares através de anastomoses arteriovenosas placentárias, conceitualmente determinando a existência de um feto receptor e outro doador. Embora as gestantes comumente se apresentam assintomáticas, as repercussões clínicas fetais costumam ser graves. O diagnóstico é exclusivamente ultrassonográfico e deve ser feito o mais precocemente possível, ressaltando-se a importância da detecção da corionicidade da gestação gemelar, além de acompanhamento ultrassonográfico seriado para rastreio do desenvolvimento da síndrome. Apesar de ainda não haver protocolo de tratamento bem estabelecido, a ablação dos vasos placentários a laser é tida como o padrão-ouro dentre as opções terapêuticas disponíveis. Apresenta elevada taxa de sobrevida de pelo menos um dos fetos e baixos índices de sequelas neurológicas neonatais, podendo ser realizada somente até a 26ª semana de gestação.

Twin-twin transfusion syndrome is a complication of monochorionic twin pregnancies. In addition to commonly occurring in the second trimester, it has high fetal and neonatal morbidity and mortality and incidence rates ranging from 10 to 15% among all monochorionic pregnancies. This study aims to perform a literature review based on a bibliographic survey about the main epidemiological, clinical and therapeutic aspects of TTTS. The PubMed database was consulted, as the search terms used were "twin-twin transfusion syndrome", "diagnosis", and "treatment". Sixty-eight literature review and systematic review articles were obtained, and only twenty-nine were selected after applying the eligibility criteria. About the pathophysiology, the syndrome is explained by direct blood transfer between the twin fetuses through placental arteriovenous anastomoses, determining the existence of a recipient fetus and another donor. Although pregnant women are usually asymptomatic, the clinical fetal repercussions are often severe. Diagnosis is exclusively ultrasonographic and must be made as early as possible, emphasizing the importance of detecting chorionicity in twin pregnancy, in addition to serial ultrasonographic follow-up to track the development of the syndrome. Although there is still no well-established treatment protocol, endoscopic laser ablation of vascular anastomoses is considered the gold standard among the available therapeutic options. It has a high survival rate for at least one of the fetuses and low rates of neonatal neurological sequelae and can only be performed until the 26th week of pregnancy.

Secuencia de perfusión arterial reversa en embarazo gemelar; coagulación de arteria nutricia. Reporte del primer caso en el país / Twin reversed arterial perfusion sequence; coagulation of the umbilical cord. A report on the first case in Uruguay / Seqüência reversa de perfusão arterial na gravidez gemelar; coagulação da artéria nutritiva. Relato do primeiro caso no país

Resumen: La secuencia de perfusión arterial reversa (TRAP) es una complicación muy poco frecuente y grave de los embarazos gemelares monocoriónicos. Generalmente ocurre cuando el corazón de un gemelo de apariencia normal sirve como bomba para uno o más gemelos dismórficos cuya cabeza, órganos torácicos y extremidades superiores no se desarrollan completamente o no se desarrollan en absoluto y, por lo tanto, carecen de actividad cardíaca. La arquitectura placentaria vascular anómala provoca un cambio en el flujo arterial hacia el gemelo acardíaco. Los mecanismos fisiopatológicos exactos que conducen a este fenómeno devastador no se conocen bien. Compartiremos el caso clínico de una paciente de 19 años, cursando un embarazo gemelar monocorial monoamniótico, en que realizamos diagnóstico de TRAPS, y realizamos la coagulación laser de la arteria nutricia del feto acárdico.

Abstract: Twin reversed arterial perfusion sequence (TRAPS) is rather an unusual and severe complication of monochorionic twin pregnancies. It usually occurs when the normal-appearance heart of a twin acts as a pump for one or more dysmorphic twins whose head, thoracic organs and upper limbs fail to totally develop or do not develop at all and thus, have no cardiac activity. The abnormal vascular architecture at the placenta changes the arterial flow towards the acardiac twin. The exact pathophysiological mechanisms that result in this devastating phenomenon are still unknown. The study presents the clinical case of a 19-year- old patient pregnant with monoamniotic, monochorionic twins and a diagnosis of TRAPS, treated by laser coagulation of the acardiac twin's umbilical cord.

Resumo: A seqüência reversa de perfusão arterial (TRAPS) é uma complicação muito rara e grave de gestações gemelares monocoriônicas. Geralmente ocorre quando o coração de um gêmeo de aparência normal serve como uma bomba para um ou mais gêmeos dismórficos cuja cabeça, órgãos torácicos e membros superiores não se desenvolvem totalmente ou não se desenvolvem e, portanto, não têm atividade cardíaca. A arquitetura vascular placentária anormal causa uma mudança no fluxo arterial para o gêmeo acardíaco. Os mecanismos fisiopatológicos exatos que levam a esse fenômeno devastador não são bem compreendidos. Descrevemos o caso clínico de uma paciente de 19 anos, portadora de gestação gemelar monocoriônica monoamniótica, na qual fizemos o diagnóstico de TRAPS e realizamos coagulação a laser da artéria nutritiva do feto acardíaco.

Seguridad de la anestesia neuroaxial en mujeres con embarazo gemelar y síndrome de transfusión feto-fetal, sometidas a fotocoagulación láser. Estudio de cohorte retrospectiva / Safety of neuraxial anesthesia in patients twin pregnancy and twin-to-twin transfusion syndrome taken to laser photocoagulation. Retrospective cohort study

Objetivo: describir las características clínicas y la frecuencia de complicaciones maternas, fetales y neonatales, según técnica de anestesia neuroaxial (AN) en mujeres con síndrome de transfusión feto-fetal (STFF) tratadas con fotocoagulación láser (FL). Materiales y métodos: estudio de cohorte retrospectiva descriptivo. Se incluyeron gestantes con STFF tratadas con FL y AN en la Fundación Valle del Lili, Cali (Colombia) entre 2013-2017. Se excluyeron pacientes con STFF estadio-V de Quintero. Se usó estadística descriptiva. El protocolo fue aprobado por el Comité de Ética de la institución. Resultados: 32 participantes cumplieron con los criterios de inclusión y de exclusión. La población estuvo constituida por mujeres jóvenes, multíparas. En el 87,5% de los casos se realizó intervención de urgencia. El 43,7% presentaba el estadio-III de Quintero y en el 56,2 % de las gestantes se utilizó anestesia epidural. Las variables hemodinámicas maternas exhibieron un comportamiento similar, acorde al momento de la cirugía y la técnica neuoraxial implementada. El 65,6 % de las gestantes presentó hipotensión sostenida y el 9,3 % desarrolló edema pulmonar. El 65,6 % de las pacientes experimentó parto pretérmino y el 18,7 % ruptura prematura de membranas. Se registraron 14 muertes fetales y cinco neonatales. No se registraron casos de mortalidad materna. Conclusiones: en pacientes con STFF que requieren FL, el uso de la anestesia epidural, espinal o combinada probablemente se asocia con un comportamiento similar al de las variables hemodinámicas maternas, durante los momentos de la cirugía. Los profesionales que brindan atención a estas gestantes deben estar alerta ante la frecuente aparición de complicaciones maternas, fetales y neonatales. Se requieren estudios prospectivos que evalúen la seguridad y la efectividad de las diferentes técnicas de anestesia neuroaxial en pacientes con STFF.

Objective: To describe the clinical characteristics and the frequency of maternal, fetal and neonatal complications in accordance with the neuraxial anesthesia (NA) technique in women with twin-to- twin transfusion syndrome (TTTS) treated with laser photocoagulation. Materials and Methods: Descriptive retrospective cohort study of pregnant patients with TTTS treated with laser photocoagulation under NA at Fundación Valle del Lili, Cali (Colombia), between 2013-2017. Patients with Quintero stage VTTTS were excluded. The protocol was approved by the institutional ethics committee. Results: Of the participants, 32 met the inclusion and exclusion criteria. The study population consisted of young, multiparous women. Urgent interventions were performed in 87.5% of cases; 43.7% were Quintero stage III and epidural anesthesia was used in 56.2% of the women. Maternal hemodynamic variables were similar, in accordance with the timing of surgery and the neuraxial technique used. Sustained hypotension occurred in 65.6% of the pregnant women and 9.3% developed pulmonary edema. Pre-term delivery occurred in 65.6% of the patients and 18.7% had premature rupture of membranes. There were 14 fetal demises and five neonatal deaths. There were no cases of maternal mortality. Conclusions: In patients with TTTS requiring laser photocoagulation, the use of epidural, spinal or combined anesthesia is likely associated with similar maternal hemodynamic variables at the time of surgery. Practitioners providing care to these pregnant women must be aware of the frequent occurrence of maternal, fetal and neonatal complications. Prospective studies to assess the safety and effectiveness of the different neuraxial anesthesia techniques in patients with TTTS are required.

Gran feto acardio: diagnóstico tardío de gestacion gemelar monocorial biamniótica complicada por secuencia TRAP: reporte de un caso y revisión de la literatura / Great acardiac fetus: late diagnosis of monochorionic diamniotic twin pregnancy complicated by sequence TRAP: case report and review of the literature

OBJETIVOS: reportar el caso de una paciente con gestación gemelar monocorial-biamniótica complicada por secuencia TRAP que dio lugar al nacimiento de un feto bomba de 1932 gramos sin malformaciones anatómicas y de un feto acardio anceps de 1800 gramos, y realizar una revisión sobre esta patología y la importancia de su diagnóstico y tratamiento precoces. MATERIALES Y MÉTODOS: se presenta el caso de un feto acardio en una gestante con embarazo sin control estricto en el Hospital San Pedro de Logroño en el año 2019, de interés por su diagnóstico tardío y elevado peso al nacimiento del feto acardio. Se realizó una búsqueda de la literatura en las bases de datos Medline vía PubMed, OVID, Embase y SciE-LO con las palabras clave DeCS y términos MeSH. Como criterios de inclusión se consideraron artículos tipo series y reportes de casos y artículos de revisión desde enero de 1950 hasta enero de 2020. RESULTADOS: la búsqueda incluyó 39 referencias bibliográficas sobre las que se repasaron las principales cuestiones teóricas a exponer. El peso del feto acardio de nuestro caso fue muy elevado sin provocar repercusión en el feto sano, en comparación con la bibliografía, lo que aporta singularidad al caso, siendo sólo equiparable la serie de casos de Brassard et al (1999), con pesos de los fetos acardio por encima de 1700 gramos y diferenciándose en 100 gramos del feto bomba. CONCLUSIONES: el feto acardio es una complicación infrecuente de embarazos gemelares monocoriales. Se requiere la presencia de anastomosis vasculares placentarias entre ambas circulaciones. El diagnóstico precoz es importante para disminuir la morbilidad y usar, en la medida de lo posible, técnicas terapéuticas no invasivas.

OBJECTIVES: to report the case of a patient with a monochorionic-biamniotic twin gestation complicated by TRAP sequence that gave rise to the birth of a pump fetus without anatomical malformations (1932 g) and an acardiac anceps fetus (1800 g), and to review this pathology and the importance of its early diagnosis and management. MATERIAL AND METHODS: the case of an acardiac fetus is presented in a pregnant woman without strict control at the Hospital San Pedro de Logroño in 2019, worthwhile because of its late diagnosis and high birth weight. A search of the literature was carried out in the Medline databases via PubMed, OVID, Embase and SciELO with the MeSH terms. As inclusion criteria, we considered series-type articles and case reports, cohorts and review articles from January 1950 to January 2020. RESULTS: 39 bibliographic references were included with the main theoretical questions to be reviewed. Our acardiac fetus weight was very high comparing with the bibiography and without causing repercussion in the healthy fetus, which contributes to the uniqueness of the case, only the series report by Brassard et al (1999) is comparable, with weights of the acardiac fetus above 1700 grams and differing by 100 grams from the pump fetus. CONCLUSIONS: the acardiac fetus is an infrequent complication of monochorionic twin pregnancies. The presence of placental vascular anastomoses between both circulations is required. Early diagnosis is important to decrease morbidity and to use, as far as possible, non-invasive therapeutic techniques.

Twin-twin transfusion syndrome - a University Hospital experience with intrauterine treatment / Síndrome da transfusão feto-fetal - experiência de um hospital universitário com tratamento intrauterino

ABSTRACT Introduction: twin-to-twin transfusion syndrome (TTTS), defined by combination of polyhydramnios-oligohydramnios, is the most prevalent (5%-35%) of the abnormalities due to placental vascular anastomoses and the most lethal (80%-100% mortality) if untreated. Fetoscopic laser ablation of abnormal vasculature using the Solomon technique is the gold standard approach. It consists of interrupting the intertwin blood flow. Objectives: to present our initial experience at the Fetal Surgery Service of the Hospital de Clinicas of the Federal University of Parana (HC-UFPR) and to compare our results with those reported in the literature. Methods: we conducted a retrospective analysis of pregnancies who had undergone laser ablation, assessing data on Quintero's staging, gestational age at diagnosis and at the time of the procedure, placental position, immediate post-procedure survival, and survival after the neonatal period. We then compared these data with the most recent data available in the literature. Results: we analyzed ten TTTS cases. The diagnosis was performed before the 26th week of pregnancy (median 20.8 weeks) and treatment occurred in a median of 9.5 days later. The distribution by the Quintero's staging was of three cases in stage II, five in stage III, and two in stage IV. In 50% of the gestations, at least one of the fetuses survived through the neonatal period. Conclusion: the treatment of TTTS in the HC-UFPR had a positive impact in the survival of the affected fetuses, although the results were worse than the ones reported in the literature, probably due to the delay in referencing the patients to our service, leading to a prolonged interval between diagnosis and treatment.

RESUMO Introdução: a síndrome de transfusão feto-fetal (STFF), definida pela combinação polidrâmnio-oligohidrâmnio, é a mais prevalente (5 a 35%) das anormalidades associadas às anastomoses vasculares placentárias e tem a maior letalidade (80 a 100%) se não tratada. A ablação a laser destes vasos por via fetoscópica com a técnica de Solomon é o tratamento de escolha atual para a interrupção das anastomoses vasculares. Objetivo: apresentar a experiência inicial do Serviço de Cirurgia Fetal do Hospital de Clínicas da Universidade Federal do Paraná (HC-UFPR) e comparar nossos resultados com os da literatura. Métodos: foram revisados os prontuários de todas as pacientes submetidas ao procedimento de ablação a laser, sendo analisados os dados referentes ao estadiamento de Quintero, à idade gestacional ao diagnóstico e ao procedimento, à posição placentária, à sobrevida imediata pós-procedimento e sobrevida após o período neonatal. Os dados foram então comparados com os mais recentes disponíveis na literatura. Resultados: dez casos de STFF foram analisados. Todos diagnosticados antes da 26a semana (mediana 20,79) e o intervalo diagnóstico-tratamento teve mediana de 9,5 dias. A distribuição pelo estadiamento de Quintero foi: três casos no estádio II, cinco casos no III e dois casos no IV. Em 50% das gestações pelo menos um dos fetos sobreviveu ao período neonatal. Conclusão: o tratamento da STFF no HC-UFPR impactou positivamente a sobrevida dos fetos acometidos. Entretanto, nossos resultados estão aquém dos reportados na literatura, possivelmente pelo demora no referenciamento das pacientes, com intervalo aumentado entre diagnóstico e tratamento.

Fetal Survival Immediate after Fetoscopic Laser Ablation in Twin to Twin Transfusion Syndrome

BACKGROUND: The purpose of this study was to determine prognostic factors that can affect the fetal survival immediate after fetoscopic laser ablation. METHODS: The study population consisted of consecutive twin pregnant women who underwent fetoscopic laser ablation with the diagnosis of twin to twin transfusion syndrome (TTTS) from 2011 to 2018 in Seoul National University Hospital. Fetal survival immediate after procedure was defined as survival to 48 hours after procedure and neonatal survival was defined as survival to 28 days of life. Clinical characteristics and ultrasound findings were compared according to the fetal survival immediate after procedure. RESULTS: A total of 57 pregnant women with TTTS were included, and the overall fetal survival immediate after procedure was 71.1% (81/114) after fetoscopic laser ablation. Fetuses who survived immediate after procedure had higher gestational age at procedure and lower frequency of abnormal Doppler studies than those did not survive. However, the frequency of hydrops was not different between cases with fetal survival and those with fetal death. The earlier gestational age at procedure and the presence of abnormal Doppler studies were significant risk factors for fetal death even after adjustment. CONCLUSION: Based on this data, the fetal survival immediate after procedure (fetoscopic laser treatment) in TTTS can be affected by the gestational age at procedure and the presence of abnormal Doppler studies.

Manejo del síndrome de transfusión feto fetal en gestaciones gemelares monocoriales / Management of Fetal Fetus Transfusion Syndrome in Monochorionic Twin Gestations

El síndrome de transfusión feto fetal es una complicación grave de las gestaciones gemelares monocoriales. Su etiología se debe a la transfusión crónica de sangre del feto donante, al feto receptor, a través de las anastomosis vasculares entre los territorios placentarios de ambos. El cuadro clínico característico se evidencia en ambos fetos, receptor y donante, lo cual es consecuencia directa de las alteraciones en la volemia que padece cada uno. En el feto receptor se observa un polihidramnios relacionado con la poliuria derivada de un estado de hipervolemia constante, que acaba evolucionando hacia una insuficiencia cardiaca congestiva. En el feto donante el cuadro es inverso, caracterizado por oligoamnios, oliguria, crecimiento intrauterino retardado e hipovolemia, con tasas de mortalidad sin tratamiento de entre el 80-100% de los casos, que varían en función del grado de transfusión. El diagnóstico se basa en el estudio ecográfico exhaustivo de ambos fetos, procurando un diagnóstico precoz y un estadiaje correcto, ya que el pronóstico se verá muy influenciado por la actuación temprana. El tratamiento de elección es la coagulación láser de las anastomosis vasculares por fetoscopia, que se realiza idealmente entre la semana 18 y 26 de embarazo. El pronóstico es variable, en función de la disponibilidad de terapia fetal y de la edad gestacional en el momento del diagnóstico, siendo, en general, de muy mal pronóstico sin tratamiento efectivo(AU)

Twin-twin transfusion syndrome (TTTS) is one of the most serious complications of monochorionic multiple gestations. The etiology is due to a chronic blood transfusion from de donor twin to the recipient twin trough vascular anastomoses between both placenta territories. Main clinical presentation is present in both donor and recipient fetuses, as a direct consequence of a blood volume alteration in each one. The recipient twin shows polyhydramnios related to polyuria resulting from a state of constant fluid overload, finally evolving into congestive heart failure. In the donor twin, the clinical presentation is opposite and shows oligoamnios, oliguria, intrauterine retarded growth and hypovolemia. Untreated mortality rates are between 80-100 % of all cases, which may vary depending on the severity of the transfusion. Diagnoses is based on exhausting ultrasound examination of both fetuses, securing early diagnosis and correct staging, since prognosis will be greatly influenced by early actions. Fetoscopic laser ablation is generally the definitive treatment between 16 and 26 weeks of gestation. The prognosis is variable, depending on the availability of fetal therapy and gestational age at diagnosis. It is generally very poor prognosis without effective treatment(AU)

Resultados perinatales del embarazo gemelar en un Hospital de Alta Complejidad / Perinatal results of twin pregnancy in a high complexity hospital

The objective of this study was to describe the epidemiology and the perinatal outcomes of twin pregnancy in the Maternal-Infantile Departament of the National Hospital "Alejandro Posadas". During the period studied there were 2825 births. The prevalence of twin pregnancies was 3.2% (N= 91). From the total of twin pregnancies, 88 were doubles and 3 were triples. It is postulated that the monochorial monovular twins constitutes a different clinical entity, because of a higher proportion of complicatioms, and that the adequate planification of the actions and working connected the perinatal team will improve the care process

Fetoscopic laser photocoagulation for twin-to-twin transfusion syndrome treatment: initial experience in tertiary reference center in Brazil

OBJECTIVE: To evaluate the initial maternal and perinatal outcomes of fetoscopic laser photocoagulation for the treatment of twin-to-twin transfusion syndrome (TTTS) in a referral center in Brazil. METHODS: This prospective observational study analyzed 24 fetoscopic laser photocoagulation procedures at 18–26 weeks of gestation. TTTS severity was determined using the Quintero classification. Blood vessels that crossed the interamniotic membrane were nonselectively photocoagulated. The χ2 test and Mann-Whitney U test were used for the statistical analysis. RESULTS: The mean (±standard deviation) age of pregnant women, gestational age at surgery, surgical time, gestational age at birth, and newborn weight were 32.2±4.1 years, 20.7±2.9 weeks, 51.8±16.7 minutes, 30.5±4.1 weeks, and 1,531.0±773.1 g, respectively. Using the Quintero classification, there was a higher percentage of cases in stage III (54.2%), followed by stages IV (20.8%), II (16.7%), and I (8.3%). Ten (41.7%) donor fetuses died and 14 (58.3%) donor fetuses survived until the end of gestation. Placental insertion location (anterior vs. posterior) did not affect the incidence of iatrogenic septostomy, surface bleeding, and premature rupture of membranes until the end of gestation. The death rate of donor and recipient fetuses before 24th gestational week increased with severity of TTTS. CONCLUSION: The maternal and perinatal outcomes resulting from the implementation of a new minimally invasive surgical technique are in line with those obtained in major centers worldwide, considering the learning curves and infrastructures.

Acardius mylacephalus: un diagnóstico poco frecuente / Acardius mylacephalus: an infrequent diagnosis

Se presenta el caso clínico de una primigesta de 21 años de edad, a quien se le detectó un embarazo gemelar monoamniótico y monocigótico en el examen ecográfico en el Centro Provincial de Genética de Santiago de Cuba. El primer feto presentaba hidronefrosis bilateral predominantemente en el lado izquierdo, y el segundo estructura fetal rudimentaria y deforme, área cardíaca mal estructurada y latidos cardíacos solo arrítmicos y bradicárdicos, además de que no se definieron los órganos internos y todo se encontraba rodeado de linfangioma quístico grave con grandes quistes paravertebrales y un solo cordón umbilical con tres vasos. Lo descrito en la ecografía se corroboró con los resultados de la necropsia, lo cual se correspondió con la variedad Acardius mylacephalus

The case report of a 21 years primigravida woman is presented to whom a monoamniotic and monochorial twin pregnancy was detected in the echographic exam at the Genetic Provincial Center of Santiago de Cuba. The first fetus presented bilateral hydronephrosis predominantly in the left side, and the second rudimentary and deformed fetal structure, not well structured heart area and just arrhythmic and bradycardiac heart beats besides that the internal organs were not defined and everything was surrounded by severe cystic lymphangioma with big paravertebral cysts and a single umbilical cord with three vessels. Everything described in the echogram was corroborated with the results of the autopsy, which corresponded with Acardius mylacephalus variety

Síndrome de Transfusión Feto Fetal / Twin-to-Twin Transfusion Syndrome

RESUMEN El Síndrome de Transfusión Feto Fetal es una de las complicaciones más severas de los embarazos múltiples que amerita seguimiento estricto y tratamiento oportuno según las características de la patología en cada caso. Esta patología se presenta como una condición de fetos productos de embarazo múltiple monocorial, quienes tienen genotipos idénticos pero adoptan fenotipos cardiovasculares discordantes a consecuencia del desequilibrio en el flujo; esto debido a las múltiples anastomosis placentarias que suelen desarrollarse entre los fetos. Expertos en perinatología han abordado el tema y han postulado diversas alternativas diagnósticas y terapéuticas, por lo cual es fundamental conocer las consideraciones para abordar pacientes con dicha entidad patológica y brindar consulta oportuna a las madres afectadas. A continuación se presenta una revisión de la literatura acerca del Síndrome de Transfusión Feto Fetal enfocado hacia las características de presentación, reseñas epidemiológicas, criterios y métodos diagnósticos así como las alternativas terapéuticas desarrolladas para la disminución y manejo de las complicaciones. MÉD.UIS. 2016;29(3):61-71.

ABSTRACT Twin to Twin Transfusion Syndrome is one of the most serious complications on multiple pregnancy which deserves a strict monitoring and appropriated treatment according to the characteristics of the pathology in each case presented. This pathology is presented as a condition in foetus from multiple monchorionic pregnancies which have identical genotypes, but they adopt discordant cardiovascular phenotypes as a consequence of the disorder in the discharge due to multiple placental anastomoses usually developed between the foetuses. Perinatology experts have approached this topic and they have postulated different diagnostic and therapeutic alternatives, so it is fundamental to know the considerations to aboard patients with the above mentioned clinical condition and provide a timely consultation to the affected mothers. Below, it is presented a revision on different literature about Twin to Twin Transfusion Syndrome focused on the characteristics of its presentation, epidemiological reviews, criteria and methods of diagnosis as well as the therapeutic alternatives developed for the management of its complications and a summary of progress regarding post intervention prospective studies which demonstrate progress in terms of survival and neurological involvement in the twin survivors. MÉD.UIS. 2016;29(3):61-71.

Manejo del síndrome de transfusión feto fetal en gestaciones gemelares monocoriales / Management of twin-twin transfusion syndrome in monochorionic twin gestations

El síndrome de transfusión feto fetal es una complicación grave de las gestaciones gemelares monocoriales. Su etiología se debe a la transfusión crónica de sangre del feto donante, al feto receptor; a través de las anastomosis vasculares entre los territorios placentarios de ambos. El cuadro clínico característico se evidencia en ambos fetos: receptor y donante, lo cual es consecuencia directa de las alteraciones en la volemia que padece cada uno. En el feto receptor, se observa un polihidramnios relacionado con la poliuria derivada de un estado de hipervolemia constante, que evoluciona hacia una insuficiencia cardiaca congestiva. En el feto donante, el cuadro es inverso; caracterizado por oligoamnios, oliguria, crecimiento intrauterino retardado e hipovolemia, con tasas de mortalidad sin tratamiento de entre 80-100 por ciento de los casos, que varían en función del grado de transfusión. El diagnóstico se basa en el estudio ecográfico exhaustivo de ambos fetos, procurando un diagnóstico precoz y un estadiaje correcto, ya que el pronóstico se verá muy influenciado por la actuación temprana. El tratamiento de elección es la coagulación láser de las anastomosis vasculares por fetoscopia, idealmente entre la semana 18 y 26 de embarazo. El pronóstico es variable, en función de la disponibilidad de terapia fetal y de la edad gestacional en el momento del diagnóstico; al ser, en general, de muy mal pronóstico sin tratamiento efectivo(AU)

Twin-twin transfusion syndrome is one of the most serious complications of monochorionic multiple gestations. Its etiology is due to a chronic blood transfusion from de donor twin to the recipient twin through the vascular anastomoses between the placental territories of both fetuses. The characteristic clinical presentation appears in both donor and recipient fetuses, as a direct consequence of altered volemia in each one. Polyhydramnios related to polyuria resulting from a state of constant hypervolemia is observed in the recipient twin, finally evolving into congestive heart failure. The clinical presentation in the donor twin is reversed and characterized by oligoamnios, oliguria, retarded intrauterine growth and hypovolemia, with untreated mortality rates ranging 80-100 percent of all cases, which may vary depending on the severity of the transfusion. The diagnosis is based on exhaustive echographic examination of both fetuses to make an early diagnosis and correct staging, since the prognosis depends very much on early action. The treatment of choice is fetoscopic laser coagulation of vascular anastomoses between 18 and 26 weeks of gestation. The prognosis is variable, depending on the availability of fetal therapy and the gestational age at diagnosis, being prognosis generally very poor without an effective treatment(AU)

Resultados perinatales en el síndrome de transfusión feto fetal manejados con la técnica de Solomon: revisión de la literatura / Perinatal outcomes in fetal-fetal transfusion syndrome managed with the Solomon technique: literature review

INTRODUCCIÓN: Entre 15-20% de los embarazos gemelares monocoriales biamnióticos se complican con el síndrome de transfusión feto/fetal el cual se asocia con mortalidad superior a 90% y morbilidad significativa en el 50% del gemelo sobreviviente. La técnica láser que coagula la superficie de la placa coriónica entre los principales canales a lo largo del ecuador (técnica de Solomon), se ha sugerido para disminuir la recurrencia, prevenir complicaciones secundarias sin incrementar resultados adversos. Métodos: REVISIÓN de la literatura existente en las bases de datos MEDLINE, EBSCO, OVID, PROQUEST, COCHRANE, Lilacs, SciELO, desde el año 2000 al 2015. Se incluyeron los artículos de revisión e investigaciones originales que compararon la técnica estándar de fotocoagulación secuencial con láser y la técnica de Solomon, el resultado primario fue la reducción de la incidencia Secuencia Anemia Policitemia, recurrencia del síndrome de transfusión feto/fetal, mortalidad perinatal y morbilidad neonatal severa. RESULTADOS: Se encontraron 200 artículos, se seleccionaron seis: 1 ensayo clínico y su análisis secundario, 2 estudios de cohorte retrospectivos, 1 revisión sistemática y un estudio que compara los resultados del neurodesarrollo. Los estudios sugieren una mejoría en la sobrevida de algunos de los fetos con la técnica Solomon, menor recurrencia del síndrome de transfusión feto/fetal y Secuencia Anemia Policitemia, sin la presencia de eventos adversos. CONCLUSIÓN: La técnica de Solomon mejora la sobrevida de algunos gemelos, sin embargo no puede concluirse que haya mejoría en la mortalidad pues los estudios no tienen el suficiente poder para determinarlo.

INTRODUCTION: Between 15 to 20% of monochorionic diamniotic twin pregnancies are complicated by the twin-twin transfusion syndrome. It has a mortality greater than 90% and a significant morbidity, 50% in the surviving twin. The Solomon technique (laser photocoagulation of the main vascular channels of the chorio-nic plate surface along the entire vascular equator) has been suggested to reduce the recurrence, and pre-vent secondary complications without increasing adverse results. METHODS: Systematic review of electronic searches of the literature from 2000 to 2015 (MEDLINE, EBSCO, OVID, PROQUEST, COCHRANE, Lilacs, and SciELO). We included review articles and original investigations comparing the standard photocoagulation technique with laser ablation against the Solomon technique. The primary results were reduction of Anemia Polycythemia Sequence incidence, twin-twin transfusion syndrome recurrence, perinatal mortality and severe neonatal morbidity. RESULTS: Of 200 articles, we selected six: one clinical essay and its secondary analysis, two retrospective cohort studies, one systematic review and a study comparing neurodeve-lopmental outcomes. The studies suggested a survival improvement in some fetuses using the Solomon technique, less twin-twin transfusion syndrome recurrence and Anemia Polycythemia Sequence without the presence of adverse effects. CONCLUSION: Solomon technique improves the survival of some twins, although we cannot conclude there is mortality improvement, because the studies do not have enough power to determine that.

Twin-twin transfusion syndrome: neurodevelopment of infants treated with laser surgery / Síndrome de transfusão feto-fetal: neurodesenvolvimento de lactentes tratados com cirurgia a laser

ABSTRACT Objective To assess the neurodevelopmental functions of survivors of twin-twin transfusion syndrome (TTTS) treated by fetoscopic laser coagulation (FLC), during the first year of life, comparing them to a control group; and to verify the influence of specific variables on neurodevelopment. Method This was a prospective, longitudinal study. The sample comprised 33 monochorionic diamniotic twins who underwent FLC for treatment of TTTS and 22 full-term infants of single-fetus pregnancies. Bayley Scales of Infant and Toddler Development Screening Test were used for evaluation. Prenatal, perinatal and postnatal information were obtained. Results There was an increased frequency of infants in the TTTS group with inadequate performance compared to the control group. The identified variables (fetal donor, low economic income and cardiorespiratory disease) negatively impacted expressive communication and fine motor skills. Conclusion Although through follow-up is recommended in all TTTS survivors, particular attention is required for the high-risk group as defined in this study.

RESUMO Objetivo Avaliar o desenvolvimento neurológico de sobreviventes da sindrome de transfusão feto-fetal (STFF) submetidos à coagulação a laser por fetoscopia (CLF), durante o primeiro ano de vida, comparando estes ao grupo controle; e verificar a influência de variáveis específicas no desenvolvimento. Método Tratou-se de estudo prospectivo, longitudinal. A amostra foi composta por 33 gêmeos diamnióticos monocoriônicos submetidos à CLF para tratamento da STFF e 22 lactentes a termo de gestação única. Bayley Scales of Infant and Toddler Development Screening Test foram utilizadas para avaliação. Informações pré-natal, perinatal e pós-natal foram coletadas. Resultados Houve maior número de lactentes com desempenho inadequado no grupo STFF do que no controle. As variáveis identificadas (feto doador, baixa renda econômica e doença cárdio-respiratória) influenciaram negativamente a comunicação expressiva e as habilidades motoras finas. Conclusão Embora o acompanhamento seja recomendado para todos lactentes com STFF, especial atenção deve ser dada àqueles que apresentam fatores de risco.

Síndrome de transfusión feto fetal: una mirada global a la enfermedad / Fetofetal transfusion syndrome: a comprehensive look at the disease / Síndrome de transfusão feto no feto: um olhar mais abrangente sobre a doença

Introducción: El síndrome de transfusión feto- feto es una enfermedad compleja producida aproximadamente en 15% de los embarazos monocoriales; a pesar de su baja incidencia, su importancia radica en las graves complicaciones perinatales. Su fisiopatología está basada en el proceso por el cual los gemelos comparten las dos circulaciones a través de vasos sanguíneos anómalos, con posterior alteración hemodinámica del gemelo receptor y donante. El diagnóstico es primordialmente ecográfico y su tratamiento definitivo actual, consiste en estrategias in útero realizadas con el fin de ubicar y sellar los vasos, proporcionando una circulación individual, buscando contrarrestar su mal pronóstico, que puede llegar a una mortalidad de hasta un 100% en aquellos sin tratamiento. Objetivo: Realizar una revisión de la literatura sobre el síndrome de transfusión feto - feto abarcando una actualización en los diferentes componentes que incluyen: epidemiología, fisiopatología, avances en Diagnóstico clínico y tratamiento. Metodología: Se hizo una búsqueda en bases de datos como PubMed, Cochrane, Sciencedirect y Google académico; se encontraron 78 artículos, de los cuales finalmente cumplieron con los criterios de actualización, pertinencia y calidad 32 artículos; se revisó fisiopatología, diagnóstico y tratamiento. Se descartaron artículos que no cumplían con criterios de inclusión. Resultados: Se seleccionó la información más actualizada y completa de las bases de datos encontradas, respecto a clasificación, etiología, fisiopatología, diagnóstico, tratamiento y pronóstico. Conclusiones: El síndrome de Transfusión Feto-Feto consiste en un desbalance hemodinámico de anastomosis placentarias, niveles aumentados de vasopresina y finalmente cambios en el eje renina angiotensina aldosterona, que requiere un diagnóstico temprano con tratamiento oportuno para mejorar el pronóstico y prevenir complicaciones fetales.

Introduction: Fetofetal transfusion syndrome is a complex disease produced in about 15% of monochorionic pregnancies, despite its low incidence, its importance lies in its serious perinatal complications. Its pathophysiology is based on the process in which twins share the two circulations through abnormal blood vessels, with a subsequent hemodynamic alteration of the receptor twin and donor twin. Its diagnosis is primarily with an ultrasound scan and its current definitive treatment consists in-utero strategies performed in order to locate and seal the vessel, providing a single movement, seeking to counteract its poor prognosis, which can lead to a mortality rate of up to 100% in those patients without treatment. Objective: To review the literature about fetofetal transfusion syndrome, covering an update about various components including epidemiology, pathophysiology, clinical advances in its clinical diagnosis and treatment. Methodology: A search was conducted in databases such as PubMed, Cochrane, Sciencedirect and Google scholar and 78 articles were found, but only 32 of them finally achieved the criteria for updating, relevance and quality; also the pathophysiology, diagnosis and treatment were revised in each one. The articles that did not fulfill the inclusion criteria were discarded. Outcomes: The most updated and comprehensive information found in databases was selected according to its classification, etiology, pathophysiology, diagnosis, treatment and prognosis. Conclusions: Fetofetal transfusion Syndrome is a hemodynamic imbalance of placental anastomosis, increased levels of vasopressin and finally, changes in the renin angiotensin aldosterone axis, which requires an early diagnosis and treatment to improve the prognosis and prevent fetal complications.

Introdução: A doença de transfusão feto-fetal é complexa e aproximadamente 15% das gestações monocoriônicas, padece deste síndrome, embora a incidência seja baixa, sua importância esta nas complicações perinataies. A sua patofisiologia baseia-se no processo pelo qual os gêmeos partilham duas circulações através de vasos sanguíneos anormais, com alteração na hemodinâmica subsequente do gêmeo receptor como do doador. Seu diagnóstico é ultrassonográfico e atualmente o seu tratamento consiste em realizar as estratégias uterinas para localizar e selar os vasos, a fim de cambiar o seu mau prognóstico, caso contrário pode ocasionar a norte até de 100% dos pacientes. Objetivo: Revisar a literatura, sobre el síndrome de transfusão feto – feto, incluindo uma atualização nos diferentes componentes como epidemiologia, fisiopatologia, avanços clínicos no diagnóstico e tratamento. Metodologia: se investigou a base de dados como PubMed, Cochrane, Sciencedirect e Google acadêmico, entre os 78 artigos pesquisados, 32 apresentaram os criterios de atualização, pertinência e qualidade científica, também foi revisto fisiopatologia, diagnóstico e tratamento. Se descartaram, os artigos que não continham os critérios requeridos. Resultados: se escolheu a informação mais atualizada e completa da base de datos pesquisada, com relação à classificação, etiologia, fisiopatologia, diagnóstico, tratamiento e pronóstico.

The Impact of Introduction of Fetoscopic Laser Surgery on Twin-to-Twin Transfusion Syndrome

PURPOSE: Fetoscopic laser surgery (FLS) is considered an optimal therapeutic strategy for twin-to-twin transfusion syndrome (TTTS). We aimed to determine the clinical outcomes of TTTS patients since the introduction of FLS. METHODS: A retrospective study of TTTS patients born between January 2005 and December 2015 was conducted. Mortality and morbidity were compared in the FLS and non-FLS groups. The FLS group was divided into selective FLS and Solomon technique group, and subgroup analysis was performed. RESULTS: Of 70 pregnancies diagnosed with TTTS during the study period, FLS was performed for 35 (50%). Survival of at least one infant at discharge was achieved in 100% (35/35) of pregnancies in the FLS group and 91.4% (32/35) in the non-FLS group (P=0.028). Subgroup analysis revealed that the Solomon technique group had a higher dual survival rate than the selective FLS group (81.3% vs. 57.9%, P=0.036). Neonatal mortality was significantly lower in the FLS group than in the non-FLS group (1.7% vs. 16.4%, P=0.005). Severe intraventricular hemorrhage more than grade 3 was less frequent in the FLS group than in the non-FLS group (0% vs. 18.2%, P= 0.001). The FLS group showed smaller inter-twin differences in birth weight (24.4% vs. 33.7%, P= 0.032) and lower incidence of twin anemia-polycythemia sequence (0% vs. 43.8%, P<0.001). CONCLUSION: Since the introduction of FLS, the survival rate of TTTS patients has improved. The Solomon technique resulted in better dual survival rates than selective FLS.

Sequência anemia-policitemia em gêmeos: definição, classificação e tratamento / Twin anemia-polycythemia sequence: definition, classification and treatment

A presença de anastomoses vasculares placentárias nas gestações gemelares monocoriônicas favorece o surgimento de complicações graves como a Síndrome de Transfusão Feto-Fetal (STFF) e a Sequência Anemia-Policitemia em Gêmeos (TAPS). STFF e TAPS são ambas formas crônicas de transfusão feto-fetal. TAPS, descrita em 2007, é caracterizada por anemia crônica no gêmeo doador e policitemia no gêmeo receptor, na ausência de discordância no volume de líquido amniótico, característica da STFF clássica. O diagnóstico pode ser feito pré- e pós-natal. O critério diagnóstico pré-natal é baseado na discordância intergemelar da medida do Doppler do pico de velocidade sistólica da artéria cerebral média (PSV-ACM). O diagnóstico pós-natal é baseado na grande diferença da hemoglobina intergemelar, ao nascimento, e reticulocitose no gêmeo doador.(AU)

The presence of placental vascular anastomoses in monochorionic twins favors the development of several complications like Twin-Twin Transfusion Syndrome (TTTS) and Twin Anemia-Polycythemia Sequence (TAPS). TTTS and TAPS are both chronic forms of fetofetal transfusion. TAPS, described on 2007, is characterized by chronic anemia in the donor twin and polycythemia in the recipient twin, in the absence of amniotic fluid volume discordances, characteristic of classical TTTS. The diagnostic can be made pree posnatally. The prenatal diagnostic criteria is based on the inter-twin discordance in Doppler measurement of peak systolic velocity in the middle cerebral artery (MCA-PSV). The postnatal diagnosis is based on large intertwin hemoglobin difference at birth and retoculocytosis in the donor twin.(AU)

Desenlaces materno-fetales de los embarazos atendidos en el Hospital Universitario de Santander, Bucaramanga (Colombia), 2007-2011. Estudio de cohorte / Maternal and foetal outcomes in twin pregnancies seen at Hospital Universitario de Santander, Bucaramanga (Colombia), 2007-2011. A cohort study

Los embarazos gemelares implican mayor riesgo de complicaciones materno-fetales que los embarazos únicos, particularmente en los monocoriales. El objetivo del trabajo fue describir las características clínicas y los desenlaces materno-fetales, por tipo de placenta (monocorial o bicorial), de los embarazos gemelares atendidos en el Hospital Universitario de Santander (HUS), institución de tercer nivel de complejidad localizada en Bucaramanga (Colombia). Materiales y métodos: estudio descriptivo de cohorte, se incluyeron las pacientes que terminaron un embarazo gemelar entre 2007 y 2011 en el HUS, hospital general de referencia de la región centro-oriental del país. Muestreo consecutivo. Se evaluó la edad gestacional en la primera consulta al hospital, los hallazgos clínicos en la evaluación inicial, la terminación del parto y los resultados perinatales. Se hace análisis descriptivo por tipo de corionicidad. Resultados: se incluyeron un total de 248 gestantes con embarazo gemelar en el periodo de estudio. La mediana de la edad gestacional en la primera atención en el hospital en fue de 34 semanas. Al ingreso, 127 (51,2 %) pacientes se diagnosticaron con embarazo monocorial, pero solo en dos terceras partes coincidió el diagnóstico prenatal de corionicidad con el del posparto. Se diagnosticó RCIU con más frecuencia en embarazos monocoriales que en bicoriales (22,3 vs. 7,5 %), y el doppler se encontró alterado con mayor frecuencia en fetos de embarazo monocorial (7,8 vs. 1,1 %). Los neonatos > 24 semanas de edad gestacional de embarazo monocorial pesaron, en promedio, 109 g (IC 95 %: 34-184) menos que los bicoriales. Conclusiones: los resultados de este estudio sugieren un problema de salud pública en este grupo de pacientes, con inicio tardío de control prenatal, de remisión tardía a centros especializados y capacidad insuficiente para definir corionicidad. Se requiere plantear estrategias de atención que incluyan considerar los embarazos gemelares como alto riesgo y garantizar la atención oportuna y adecuada, orientada por una guía de cuidado diferencial para este grupo de gestantes...

Twin pregnancies, especially monochorionic placentations, are associated with a higher rate of maternal and foetal complications when compared to singleton pregnancies. The objective of this work was to describe the clinical characteristics and the maternal and foetal outcomes according to the type of placentation (monochorial or dichorial) of twin pregnancies delivered at Hospital Universitario de Santander (HUS), a level III institution located in Bucaramanga, Colombia. Materials and methods: Descriptive cohort study. The cohort consisted of patients delivered of a twin pregnancy between 2007 and 2011 at the HUS, a general referral hospital for the central-eastern region of the country. The assessment included gestational age at the time of initial presentation to the hospital, clinical findings during the initial assessment, delivery completion, and perinatal results. Descriptive analysis by chorionicity type. Results: A total of 248 women with a twin pregnancy during the study period were included. The mean gestational age on the first visit to the hospital was 34 weeks. On admission, 127 patients (51.2%) were diagnosed with a monochorionic pregnancy, but only in two thirds of the cases was the prenatal chorionicity diagnosis consistent with the post-partum finding. IUGR was diagnosed more frequently in monochorionic than in dichorionic pregnancies (22.3% v. 7.5%) and abnormal Doppler findings were more frequent in monochorionic foetuses (7.8% v. 1.1%). Neonates > 24 of gestational age in monochorionic pregnancies weigh in average 109 gr (IC 95%: 34-184) less than dichorionic twins. Conclusions: The results of this study suggest a public health problem in this group of patients who come late for their prenatal visits and are late referrals to specialized centres, when there is already a limited ability to determine chorionicity. There is a need to develop care strategies in which twin pregnancies are included as a high-risk condition, and to ensure timely and adequate care provision in accordance with differential care guidelines for this group of pregnant women...