Twin reversed arterial perfusion sequence: the heartless twin

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.

Síndrome de perfusión arterial reversa de feto a feto / Reverse Arterial Perfusion Syndrome from Fetus to Fetus

RESUMEN Introducción: Uno de los temas que presenta más interés en los obstetras es el relacionado con la gestación gemelar. Su diagnóstico nos permite clasificarla como una gestación de riesgo, en la cual el estudio ecosonográfico es muy importante al proporcionar datos relacionados con el tipo de placentación. Presentación de caso: Se realiza ecografía prenatal del I trimestre para confirmación de diagnóstico presuntivo de onfalocele, donde se constata embarazo gemelar, monoamniótico, monocorial. Se realiza el diagnóstico de síndrome de perfusión arterial reversa feto a feto. El estado anormal del "corazón" del feto podría identificarse como una holoacardia; pero el grado de malformación que presenta no se ajusta a ninguno de los criterios de esta clasificación. Teniendo en cuenta el hallazgo precoz y la elevada mortalidad que se asocia en estos casos se sugirió la opción de interrumpir el embarazo. Conclusiones: El asesoramiento genético referido a las posibles terapias a emplear estaría limitado en este caso, debido a la alta morbimortalidad perinatal, la falta de experiencia en relación con el tratamiento medicamentoso, y la escasa disponibilidad de técnicas para la intervención fetal(AU)

ABSTRACT Introduction: One of the topics of most interest to obstetricians is related to twin pregnancy. Its diagnosis allows to classify it as a risk gestation, in which the echo-sonographic study is very important because it provides data related to the type of placentation. Case report: First trimester prenatal ultrasound is performed to confirm the presumptive diagnosis of omphalocele, revealing twin, monoamniotic, monochorial pregnancy. The diagnosis of fetus-to-fetus reverse arterial perfusion syndrome is made. The abnormal state of the fetus´s heart could be identified as a holoacardia; but the degree of malformation it presents does not fit any of the criteria of this classification. Taking into account the early finding and the high mortality associated in these cases, the option of interrupting the pregnancy. Conclusions: Genetic counseling regarding possible therapies to be used would be limited in this case, due to the high perinatal morbidity and mortality, the lack of experience in relation to drug treatment, and the limited availability of techniques for fetal intervention(AU)

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

Diagnostic value of 3D-ultrasound for cord entanglement in monoamniotic twins / 实用医学杂志

Objective To discuss the diagnostic value of 3D-ultrasound for cord entanglement in monoam-niotic twins. Methods From November 2013 to March 2016,a total of 65 monoamniotic twinsunderwent routine obstetric ultrasonography to screen fetal abnormalities in our hospital. The cord was meticulously observedusing a systematic continuous sequence approach. Fetuses who showed cord entanglement by 2-D ultrasound or 3-D flow Doppler were all recruited. The gestational weeks,fetal malformation,ultrasonic image feature of cord entangle-ment,and clinicaloutcome were recorded and analyzed retrospectively. Results 4 monoamniotic twins showed cord entanglement. Three-dimensional color Doppler clearly delineated thefeatures of entanglement. At following-up of 8 fetuses,3 fetuses were found intrauterine fetal death and 3 fetuses with structural abnormalities except for 2 well infants.Conclusion Cord entanglement may threaten the safety of fetusesin monoamniotic pregnancies.In aid of 2-D ultrasound or 3-D flow Doppler in monoamniotic twins can help to find it in time ,monitor it carefully and provide evidence for obstetric treatment .

One case of monoamniotic twin pregnancy with cord entanglement and fetus survival at 32+4 weeks after successful medical amnioreduction and intensive ultrasound surveillance / 대한산부인과학회지

Monoamniotic twin pregnancies are relatively rare, but perinatal mortality is high about 30-70%. The major cause of fetal death is cord accident, congenital anomalies, twin-twin transfusion syndrome, and intrauterine growth restriction. Especially cord entanglement occurs; perinatal mortality is about 50%. So the active management of monoamniotic twins to reduce the complication of cord entanglement is important. This is a case of monoamniotic twin pregnancy with cord entanglement and both fetuses survival by elective cesarean section at 32+4 weeks after medical amnioreduction and intensive fetal surveillance.

One case of monoamniotic twin pregnancy without cord entanglement and both fetus survival / 대한산부인과학회지

Monoamniotic twin pregnancies are relatively rare, occuring in 1 percentage. But perinatal mortality is 50-60 percentage. The high mortality rate has been attributed to preterm delivery, cord entanglement, twin-to-twin transfusion syndrome, intrauterine growth retardation and congenital anomalies. However, the desirable management plan of the monoamniotic twin pregnancies is still not established and there are controversies regarding the proper antepartum care of monoamniotic twins and the optimal timing and mode of delivery. We have experienced one case of monoamniotic twin without cord entanglement and both fetuses survival by cesarean section at 36+3 weeks, is reported with a brief review of the literatures.