ABSTRACT
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.
Subject(s)
Humans , Female , Pregnancy , Adult , Laser Coagulation , Fetal Heart/abnormalities , Fetofetal Transfusion , Placenta/pathology , Umbilical Arteries/surgery , Pregnancy, TwinABSTRACT
TRAP describe la perfusión crónica de un gemelo acardíaco por un gemelo de bomba a través de canales vasculares entrelazados permeables. La secuencia TRAP ocurre en 1 de cada 35.000 nacimientos o en 1 de cada 100 pares de gemelos monocigóticos. Se diagnostica mediante los hallazgos ecográficos de un feto de desarrollo normal y una masa amorfa con frecuencia con partes fetales perceptibles. El Doppler color revela el flujo sanguíneo reverso hacia el gemelo acardíaco dentro de la arteria umbilical lo que lleva a las complicaciones típicas del cuadro. El manejo expectante es razonable en ausencia de características pronósticas deficientes. El propósito de este artículo es revisar los aspectos básicos y el estado actual de esta condición, haciendo énfasis en el diagnóstico y el manejo expectante.
TRAP describes the chronic perfusion of an acardiac twin by a pump twin through permeable interlocking vascular channels. TRAP occurs in 1 in 35,000 births or 1 in 100 pairs of monozygotic twins. It is diagnosed by ultrasound findings of a normally developing fetus and an amorphous mass often with noticeable fetal parts. Color Doppler reveals the reverse blood flow to the acardiac twin within the umbilical artery, leading to typical complications of the condition. Expectant management is reasonable in the absence of poor prognostic characteristics. The purpose of this article is to review the basic aspects and current status of this condition, emphasizing the diagnosis and expectant management.
Subject(s)
Female , Adult , Fetus , Perfusion , Twins , BloodABSTRACT
Multifetal gestation is often a high-risk pregnancy and especially the monochorionic twin pregnancy significantly contributes to fetal morbidity and mortality. Acardiac twinning, earlier known as chorioangiopagus parasiticus, is the most extreme manifestation of this condition. An acardiac twin is a rare complication of multifetal pregnancy, in the literature reported at an incidence of 1% of monochorionic twin pregnancies, i.e. 1 of 35,000 pregnancies. Often results from abnormal placental vascular anastomoses. This leads to twin reversal arterial perfusion with complex pathophysiology. Here authors present a case of acardiac twin pregnancy presented at 26 weeks with the ultrasonography report suggested?? Placental teratoma of size 11×11×13 cm with polyhydramnios as there was no reason to suspect something else as the picture described in the USG report with the polyhydramnios was fitting with the diagnosis of placental teratoma but as the scan was done at taluka place and the images provided were not clear authors decided to confirm the diagnosis from fetal medicine specialist as MTP was not the option for the patient as she was 28 weeks who confirmed that as a case of acardiac twin pregnancy and the case was managed accordingly.
ABSTRACT
To report a case of twin reversal arterial perfusion sequence and its management by means of laser coagulation of the vascular malformation in the placenta. Twin reversed arterial perfusion sequence is a rare form of twin to twin transfusion syndrome occurring primarily in Monochorionic monoamniotic twins. The prevalence is about 1 in 35,000 pregnancies. The significance of this condition is that there is a normal foetus and an acardiac foetus. The blood is shunted from the normal twin to the acardiac twin through vascular malformations in the placenta. The normal twin faces a high risk of both morbidity and mortality due to cardiac failure. A case of twin reversal arterial perfusion sequence diagnosed at 22 weeks following a target scan underwent laser photocoagulation and gave birth vaginally at 30 weeks without any complications. Early detection of this condition can lead to timely intervention and thereby improve the outcome. In Twin reversal arterial perfusion sequence, the normal or the pump twin has a high chance of mortality due to cardiac failure. As the size of the acardiac twin increases, there is a higher chance of mortality of the pump twin. There is a need for regular follow up with ultrasonography and foetal echocardiography along with early therapeutic interventions to ensure the survival of the normal twin. In our case, despite the large size of the acardiac twin, we had a successful pregnancy outcome for the normal twin due to timely intervention.
ABSTRACT
Twin reversed arterial perfusion sequence is a complication of monochorionic twin pregnancies in which one twin that exhibits lethal anomalies, including acardia, is perfused by the other 'pump' twin via anastomoses between placental arteries. This results in growth and characteristic anomalies of the acardiac twin, and possible heart failure of the pump twin. The exact pathophysiological mechanisms that lead to this devastating phenomenon are not well known. It occurs in about 1% of monochorionic pregnancies and in one out of 35 000 pregnancies. Mortality is usually due to anemia and cardiac complications which can lead to cardiac failure, appear early during pregnancy and cause the death of the pump twin. We report a case of twin reversed arterial perfusion in a 28-year-old patient with 12 weeks monochorionic-diamniotic pregnancy that was referred for absent cardiac activity in one twin. Ultrasound showed an acardiac twin with flow reversal seen in the umbilical artery and umbilical vein and a normal second twin. At 28 weeks an emergency cesarean section was done due preterm labor and transverse situation of the second twin. The first twin was malformed and the second twin died two days later due to complications of prematurity. Accurate prenatal diagnosis of twin reversed arterial perfusion sequence is essential to improve the prognosis of this rare entity.
La secuencia de perfusión arterial reversa en gemelos es una complicación del embarazo gemelar monocoriónico, en la que un gemelo muestra anomalías mortales, incluyendo acardia, y es perfundido por el otro gemelo 'bomba' a través de anastomosis arteriales placentarias, produciendo el crecimiento y las anomalías características del gemelo acárdico, con posible insuficiencia cardíaca en el gemelo bomba. Los mecanismos fisiopatológicos exactos que conducen a este fenómeno devastador no son bien conocidos. Ocurre en aproximadamente 1% de los embarazos monocoriónicos y en 1 de cada 35 000 embarazos. La mortalidad se debe generalmente a anemia y complicaciones cardíacas que pueden conducir a insuficiencia cardíaca, que aparecen temprano durante el embarazo y causan la muerte del gemelo bomba. Se presenta un caso de perfusión arterial reversa en gemelos en paciente de 28 años de edad con un embarazo monocoriónicodiamniótico de 12 semanas quien fue referida por ausencia de actividad cardíaca de uno de los gemelos. La ecografía mostró un gemelo acárdico con reversión de flujo visto en arteria-vena umbilical y un segundo gemelo normal. A las 28 semanas se realizó una cesárea de emergencia debido a parto pretérmino y situación transversa del segundo gemelo, obteniendo el primer gemelo marcadamente malformado y un segundo gemelo vivo que murió dos días después de las complicaciones de la prematuridad. El diagnóstico prenatal exacto secuencia de perfusión arterial reversa en gemelos es esencial para mejorar el pronóstico de esta rara entidad.
ABSTRACT
In general, twin pregnancy represents a high-risk pregnancy. The monozygous twin of all twin pregnancy is a real challenge for the obstetrician due to the severe complications that may arise during its development. An extremely uncommon, severe complication of monozygous twin pregnancy, which we recently experienced in Soonchunhyang University Cheonan Hospital, was a monochorionic diamniotic twin pregnancy with acardiac twin-twin reversed arterial perfusion sequence. An acardiac twin presented no heart, underdeveloped inferior part of the body, being transfused by the other fetus (pumping twin) by umbilical vessels. We report a 33-year-old pregnant woman, with a history of in vitro fertilization-embryo transfer in the second pregnancy, who presented with generalized edema, abdominal discomfort, and high blood pressure at 33 weeks' gestation. By ultrasonography, one of the babies is normally developed but in the other baby, head, upper extremities, and cardiac activity cannot be detected. The pumping twin was cardiomegaly, polyhydramnios, absent diastolic wave on the umbilical artery, and small for gestational age. We decided to terminate the pregnancy by primary cesarean section after administration of corticosteroids. The comprehension of this case is compulsory in order to provide maximum survival opportunity to the viable baby.
Subject(s)
Adult , Female , Humans , Pregnancy , Adrenal Cortex Hormones , Cardiomegaly , Cesarean Section , Comprehension , Edema , Fetus , Gestational Age , Head , Heart , Hypertension , In Vitro Techniques , Perfusion , Polyhydramnios , Pregnancy, High-Risk , Pregnancy, Twin , Pregnant Women , Twins , Ultrasonography , Umbilical Arteries , Upper ExtremityABSTRACT
Objetivo: Presentar un caso de gemelo acardio a término. Caso: Paciente de 28 años de edad, II gestas, I para, sin antecedentes patológicos personales ni noxas antes y durante el embarazo, ni antecedentes familiares; Inicia control prenatal donde se le realizó ecografía obstétrica evidenciando: Embarazo gemelar monocorial/biamniotico; primer feto: para 14 semanas por biometría fetal sin alteraciones anatómicas, pliegue nucal normal y hueso nasal presente, PowerDoppler normal; segundo feto: imagen ecomixta de bordes definidos compatible con feto acardio de 15 semanas por longitud de fémur, PowerDoppler sin evidencia de flujos; placenta en cara posterior media izquierda. Durante la gestación cursa con infección urinaria a las 26 semanas la cual fue tratada y a las 30 semanas con dengue sin complicaciones. Ecocardiograma fetal del feto 1: corazón funcional y anatomicamente normal. Se dio seguimiento expectante con flujometría Doppler continúa sin afección del feto 1 hasta las 39 semanas + 1 día, culminando el embarazo vía cesárea segmentaria obteniéndose recién nacido masculino aparentemente sano y feto acardio amorfo, al cual se le realizó estudio radiológico y autopsia, esta última reportó gemelo acardio amorfo (milicefalo). Conclusión: La mortalidad de los 2 fetos entre el primero y segundo trimestre es muy alta si no se diagnostica a tiempo y se la da un seguimiento adecuado.
Case presentation: Patient aged 28, II pregnancies, I para no medical history or personal insults before and during pregnancy to interrogation, nor a history of personal or familial congenital malformations. A prenatal obstetric ultrasound was performed showing: twins pregnancy monochorionic/diamniotic, first fetus: 14 weeks for fetal biometry without anatomic abnormalities, nuchal translucency and nasal bone present standard, power Doppler normal. Second fetus: image-edged ecomixta support acardiac fetus of 15 weeks for femur length, power Doppler no evidence of flows; placenta posterior left middle. During pregnancy the patient presents a urinary infection at 26 weeks which was treated and later, 30 weeks, the patient presents an uncomplicated dengue fever. First fetus echocardiogram: functional and anatomically normal heart. The first fetus was expectantly followed with continuous Doppler flowmetry without affecting the first fetus fetus up to 39 weeks + 1 day, culminating pregnancy via segmentary cesarean obtaining an apparently healthy newborn male and amorphous acardiac fetus, which underwent radiological and autopsy studies, reporting amorphous acardiac twin (milicefalo). Conclusion: The mortality of the two fetuses between the first and second quarter is very high if not diagnosed early and gives proper monitoring.
ABSTRACT
Introducción: el gemelo acárdico es una malformación congénita de muy baja prevalencia, 1 en 35.000 nacimientos, y se encuentra aproximadamente en 1 de cada 100 gemelos monocigóticos. La asociación de un gemelo acárdico con un segundo gemelo con malformaciones mayores ocurre aproximadamente en el 10% de los casos. El objetivo de este artículo es el reporte de un caso de muy baja prevalencia y la revisión de la literatura publicada sobre la etiología y fisiopatología asociada a la acardia, así como de las anomalías congénitas asociadas a esta y a la infección por virus de la varicela. Materiales y métodos: se presenta el caso de un embarazo gemelar monocoriónico biamniótico con un gemelo acardius mylacephalus y un segundo gemelo anencefálico, con exposición de la madre a varicela en el primer trimestre del embarazo. Se realizó cariotipo con bandas G, 46 XY sin anormalidades estructurales. Se hace una revisión de la literatura publicada en los últimos 10 años en las bases de datos PubMed, Ovid e HINARI. Conclusión: la importancia de este trabajo radica en que en la literatura revisada sólo se encontró un único reporte de la asociación de gemelo arcádico, coexistente con gemelo anencefálico. Además, sería el primer reporte de la relación entre esta combinación de malformaciones mayores en el embarazo gemelar y la exposición a varicela en el primer trimestre del embarazo.
Introduction: an acardiac twin is a congenital malformation having low prevalence (1 in 35,000) births and is found in about 1 in every 100 monozygotic twins. The association of an acardiac twin with a second twin having greater malformations occurs in around 10% of cases. This article was aimed at reporting a case having very low prevalence and a review of the literature published about the etiology and physiopathology associated with acardiac anomaly, as well as the congenital anomalies associated with it and infection by the chicken pox virus. Materials and methods: the case of monochorionicdiamniotic twin pregnancy involving an acardius mylacephalus twin and a second anencephalic twin is presented, the mother having been exposed to chicken pox during the first trimester of pregnancy. G-band kariotyping showed 46 XY having no structural abnormalities. A search was made of the literature published during the last 10 years in the PubMed, Ovid and HINARI databases. Conclusion: the importance of this report lies in the fact that only one report of the association of acardiac twin coexisting with anencephalic twin was found in the pertinent literature. This is also the first report of the relationship between this combination of greater malformations in twin pregnancy and exposure to chicken pox during the first trimester of pregnancy.
Subject(s)
Humans , Female , Pregnancy , Anencephaly , Chickenpox , PregnancyABSTRACT
An acardiac twin is one of the very rare anomalies that occurs in monochorionic twins and the incidence of this is about one out of 35,000 births. We present the serial prenatal ultrasound findings, along with the postnatal histologic correlation, of an acardiac twin that manifested as a single lower extremity.
Subject(s)
Humans , Fetus , Incidence , Lower Extremity , Parturition , Ultrasonography, PrenatalABSTRACT
Acardiac twin is a rare anomaly that occurs 1% in monochorionic twins and 1 in 35,000 pregnancies overall. Acardiac twin, also known as twin-reversed arterial perfusion (TRAP) sequence, involves a "pump" or donor twin perfusing a recipient or "acardiac" twin through vascular (usually arterial-arterial and venous-venous) anastomoses. Perinatal mortality rate for the pump twin has been reported to be 50~75%, mainly as a result of polyhydramnios, preterm labor, and congestive heart failure. Therefore, occlusion of the circulation to the acardiac twin has been recommended to improve perinatal outcome of the pump twin. Radiofrequency ablation of the acardiac twin effectively protects the pump twin from high-output cardiac failure and death. We report our experience in the treatment of patients with TRAP sequence using radio frequency ablation to stop perfusion to the acardiac twin.
Subject(s)
Female , Humans , Pregnancy , Fetus , Heart Failure , Obstetric Labor, Premature , Perfusion , Perinatal Mortality , Polyhydramnios , Pregnancy, Twin , Tissue Donors , Umbilical CordABSTRACT
The acardiac twin, or twin reversal of arterial perfusion (TRAP) sequence is encountered in approximately 1% of monozygotic twins with an incidence of one in 35,000 births. The problem results from vascular anastomoses between the arterial and venous circulation of normal "pump" twin and that of recipient "perfused" acardiac twin. The recipient twin may display severe and lethal anomalies, including acardia and acephalus. The pump twin is structurally normal. Mortality of about 50-75% in cases without treatment is due to heart failure, prematurity or cord entanglement. We report a case of acardiac twin diagnosed by ultrasound prenatally.
Subject(s)
Humans , Heart Failure , Incidence , Mortality , Parturition , Perfusion , Twins, Monozygotic , UltrasonographyABSTRACT
The acardiac twin is very rare congenital malformation of monozygotic multiple gestations affecting 1% of monozygotic twins, or 1 in 35000 births. The acardiac twin has a parasitic existence and depends on the donor twin for its blood supply via placental anastomoses and retrograde perfusion of the acardiac umbilical cord. Acardiac twin places the normal twin at risk for high output cardiac failure and in utero fetal death and may lead to polyhydramnios and its related complications. The mortality rate is reported to be between 50% and 75% for the normal twin. We recently experienced a case of acadius anceps with a normal twin death in uterus, so present with a brief review of the literature.
Subject(s)
Humans , Fetal Death , Heart Failure , Mortality , Parturition , Perfusion , Polyhydramnios , Tissue Donors , Twins, Monozygotic , Umbilical Cord , UterusABSTRACT
Acardiac twin is a rare complication of multifetal pregnancy. The literatures report an incidence of 1% among monochorionic twin pregnancies, i. e. 1 of 35,000 pregnancies. The absence of identifiable fetal heart structures in one twin and reduction anomalies in many organ systems suggest the diagnosis. It has been hypothesized that in the presence of artery-to-artery and vein-to-vein anastomoses in a monozygotic placenta, blood is perfused by hemodynamically advantaged pump-twin to the recipient twin by retrograde flow. The principal perinatal problems associated with acardiac twinning are congestive heart failure of pump-twin, maternal polyhydramnios, preterm delivery and intrauterine death. The outcome is invariably fatal for the acardiac twin and 50-75% of the normal twin. Management options include observation, medical therapy, and selective termination of acardiac twin. The most appropriate intervention for the various clinical presentations of this disorder is undetermined, and conservative nonintervention is often appropriate. Long-term follow-up data on surviving pump twins are lacking. We experienced a case of acardiac twin gestation which showed satisfactory outcome with conservative management, so we present the case with a brief review of the literature.
Subject(s)
Humans , Pregnancy , Diagnosis , Fetal Heart , Follow-Up Studies , Heart Failure , Incidence , Placenta , Polyhydramnios , Pregnancy, TwinABSTRACT
Acardiac twin is a rare complication of multifetal gestation occurring in 1% of monozygotic twin pregnancies and 1 of 35,000 pregnancies. Acardia shows various other defects in addition to the absence of heart and the most common form is the acardius, acephalus, in which there is an absence of the fetal head and thoracic organs. The presence of an acardiac twin requires the normal (or "pump") twin to provide circulation for itself, as well as the acardiac (or "perfused") sibling. The mortality is 100% for the acardiac or perfused twin and for pump twin is about 50%, resulting from heart failure, prematurity, or cord entanglement. We report two cases of acardiac anomalies at 32 weeks gestation in 30 year-old primigravida woman and 26 weeks gestation in 30 year-old multigravida. A brief review of the literature is included.
Subject(s)
Adult , Female , Humans , Pregnancy , Head , Heart , Heart Failure , Mortality , Siblings , Twins, MonozygoticABSTRACT
Acardiac twinning affects 1 in 100 monozygotic twin pregnancies and 1 in 35,000 pregnancies overall. This condition is characterized by the absence or rudimentary development of fetal heart, and associated with various anomaly. The presence of an acardiac twin requires the normal (or "pump") twin to provide circulation for itself, as well as the acardiac sibling. The acardiac malformations are uniformly fatal in the affected twin, and mortality in the co-twin is as high as 55%. The principal perinatal problems associated with acardiac twinning are pump-twin congestive heart failure, maternal hydramnios, and preterm delivery. We recently experienced a case of acardius anceps associated with a normal male infant, so present with a brief review of the literature.