Transfusión intrauterina, hidrops fetal no inmune: reporte de un caso / Intrauterine transfusion, non-immune fetal hydrops: case report / Transfusão intrauterina, hidropisia fetal não imune: relato de caso

Introducción: el hidrops fetal es grave, de mal pronóstico y alta morbimortalidad, a pesar de mejoras diagnósticas y terapéuticas desarrolladas en los últimos tiempos. El pronóstico estará determinado por la etiología y posibilidades terapéuticas asociadas a mejores resultados, a la edad gestacional, al diagnóstico y al nacimiento, si bien cabe destacar que no existen suficientes estudios de seguimiento a largo plazo. El diagnóstico ecográfico es confirmatorio, siendo la principal complejidad identificar la etiología y plantear la estrategia terapéutica adecuada. Descripción del caso: presentamos una paciente con diagnóstico de hidrops fetal de tipo no inmune y su abordaje terapéutico. La causa del hidrops correspondió a anemia fetal severa, requiriendo la realización de tres procedimientos con exanguinotransfusión parcial intrauterina mediante cordocentesis. A las 33 semanas, se decidió la finalización del embarazo, con buena evolución neonatal. Conclusión: el hidrops fetal aumenta la morbimortalidad fetal y neonatal, siendo un enorme desafío para el equipo tratante, que requiere de un equipo asistencial interdisciplinario. El conocimiento de esta patología permite realizar un abordaje completo, orientar a la etiología, realizando un diagnóstico oportuno y la selección adecuada del tratamiento. Como en este caso, al identificar la anemia severa como causa del hidrops, es mandatorio definir el manejo para los fetos candidatos a terapia intrauterina.

Introduction: fetal hydrops is a serious condition with a poor prognosis and high morbidity and mortality, despite improvements in diagnostics and therapeutics in recent years. Prognosis is determined by the etiology and therapeutic options associated with better outcomes, gestational age, diagnosis, and birth, although it should be noted that there are not enough long-term follow-up studies. Ultrasound diagnosis is confirmatory, with the main challenge being to identify the etiology and propose the appropriate therapeutic strategy. Description of the case: we present a patient diagnosed with non-immune fetal hydrops and its therapeutic approach. The cause of hydrops was severe fetal anemia, requiring 3 procedures with intrauterine partial exsanguination transfusion through Cordocentesis. At 33 weeks, the decision was made to terminate the pregnancy, with good neonatal outcomes. Conclusions: fetal hydrops increases fetal and neonatal morbidity and mortality, posing a significant challenge for the treating team and requiring an interdisciplinary healthcare team. Understanding this condition allows for a comprehensive approach, guiding the etiology, providing timely diagnosis, and selecting appropriate treatment. As in this case, identifying severe anemia as the cause of hydrops mandates defining the management for fetuses eligible for intrauterine therapy.

Introdução: a hidropisia fetal é grave, com mau prognóstico e elevada morbimortalidade, apesar das melhorias diagnósticas e terapêuticas desenvolvidas nos últimos tempos. O prognóstico será determinado pela etiologia e possibilidades terapêuticas associadas a melhores resultados, idade gestacional, diagnóstico e nascimento, embora se deva salientar que não existem estudos suficientes de seguimento a longo prazo. O diagnóstico ultrassonográfico é confirmatório, sendo a principal complexidade identificar a etiologia e propor a estratégia terapêutica adequada. Descrição do caso: apresentamos uma paciente com diagnóstico de hidropisia fetal não imune e sua abordagem terapêutica. A causa da hidropisia correspondeu a anemia fetal grave, sendo necessária a realização de 3 procedimentos com exsanguineotransfusão intrauterina parcial por meio de cordocentese. Às 33 semanas foi decidida a interrupção da gravidez, com boa evolução neonatal. Conclusão: a hidropisia fetal aumenta a morbimortalidade fetal e neonatal, sendo um enorme desafio para a equipe responsável pelo tratamento, necessitando de uma equipe de atendimento interdisciplinar. O conhecimento desta patologia permite uma abordagem completa, orientação sobre a etiologia, diagnóstico atempado e seleção do tratamento adequado. Assim como neste caso, quando se identifica anemia grave como causa da hidropisia, é obrigatória a definição do manejo para os fetos candidatos à terapia intrauterina.

Intrauterine transfusion of a hydropic fetus with anemia due to a giant chorioangioma: A case report.

Giant chorioangiomas are a potentially life-threatening condition that may require intrauterine therapy. We describe a case of a large chorioangioma (>4cm) diagnosed at 30 weeks of gestation causing severe fetal anemia and hydrops. An intrauterine blood transfusion was performed at 31 weeks with reversal of the anemia and hydrops. The neonate was born at 37 weeks showing respiratory distress syndrome that required neonatal intensive care unit admission but was discharged at 30 days of life. Further evaluation at two months of age showed no signs of abnormal neurodevelopment. When timely indicated, intrauterine transfusion of a hydropic fetus with anemia due to a giant chorioangioma is a potentially life-saving therapy that shows good neurodevelopment of the surviving fetus.

Transfusión intrauterina en hidrops fetal por Parvovirus B19: a propósito de un caso clínico / Intrauterine transfusion for hydrops fetalis by parvovirus B19 infection: a case report

Hidrops fetal no inmunológico diagnosticado a las 22 semanas de gestación, secundario a infección por Parvovirus B19, tratado exitosamente con cinco transfusiones intrauterinas. Parto vaginal con recién nacido de término sin estigmas de enfermedad. Enfatizamos la importancia de sospechar el diagnóstico, el manejo basado en Vmax de ACM y la capacidad actual de tratamiento exitoso a través de transfusiones intrauterinas.

Non immunologic hydrops fetalis diagnosed at 22 weeks of gestation, secondary to infection by Parvovirus B19, successfully treated with five intrauterine transfusions. Vaginal delivery at 37 weeks without stigmata of disease. We emphasize the importance of suspecting the diagnosis, management based on Vmax of ACM and the current capacity of successful treatment by intrauterine transfusion.

[Intrauterine transfusión in alloimmunization Rh in México 1987-2008]. / Transfusión intrauterina en aloinmunización rhD en México 1987-2008.

BACKGROUND: Diagnosis, care and prevention of hemolytic disease in fetuses and newborns is the most prominent historical example of a successful medical procedure aimed to abate perinatal morbidity and mortality caused by a disease which for centuries was described only unknown origin. OBJECTIVE: To review the perinatal outcome with intrauterine transfusion (IUT) in severe alloimmunization RhD over 21 years in a referral center of Mexico. The overall survival rate of fetuses and the relations with gestational age, and presence or absence of hydrops was analyzed. The authors present data about alloimmunization and a historical synopsis about IUT in México. MATERIAL AND METHOD: A retrospective study was conducted from January 1, 1987, to January 31, 2008. It was collected only RhD immunizations. Primary outcome variables included gestational age and presence or absence of hydrops, type and number of IUT in each case, and we studied fetal and neonatal morbidity. RESULTS: A total of 531 IUTs were performed in 150 fetuses. Severe hydrops was found at start of intrauterine treatment in 67 cases (45%). The survival rate was closely related to absence or presence of hydrops (88 and 60%), respectively. There were 123 liveborn fetuses and the procedure-related fetal loss rate was low (1.9%). CONCLUSIONS: This study confirmed good outcome with IUT for fetal anemia and the loss rate was low and similar to another publications. The hydrops was the principal factor in the survival rate because late detection and referral of fetuses is critical for fetal and neonatal outcome.

Reversion of the Ballantyne syndrome despite fetal hydrops persistence.

INTRODUCTION: The Ballantyne syndrome (or mirror syndrome) is a gestational proteinuric hypertension associated with fetal hydrops. This report describes a case in which Ballantyne syndrome reversion occurred despite fetal hydrops persistence. CASE REPORT: A 24-year-old woman showed fetoplacental hydrops at 28 2/7 gestational weeks. Severe Rh(D) alloimmunization and fetal hemolytic anemia (fetal hematocrit 15.4%) were confirmed by cordocentesis, and an intrauterine transfusion was performed. She also revealed hypertension (160/100 mm Hg), edema and proteinuria (845 mg/day). After four intrauterine transfusions, blood pressure was normalized; urinary proteinuria was not significant, and the edema vanished completely. Fetal hydrops persisted until delivery at 32 gestational weeks, but a partial reduction of placental hydrops was noted. DISCUSSION: Total or partial reduction of the placental edema may be responsible for the reversal of the Ballantyne syndrome despite the fetal hydrops persistence.

Fetal hydrops and other variables associated with the fetal hematocrit decrease after the first intrauterine transfusion for red cell alloimmunization.

OBJECTIVE: To evaluate the influence of fetal hydrops and other variables on fetal hematocrit (Hct) decrease after the first intrauterine transfusion (IUT) in alloimmunized pregnancies. METHODS: From 1996 to 2006, the data of all alloimmunized pregnancies submitted to IUT were assessed. Exclusion criteria included: fetuses submitted to intraperitoneal transfusion; pregnancies complicated by other fetal abnormalities; pregnancies submitted to only one IUT, and cases in which posttransfusion or pretransfusion blood samples were not obtained. Linear regression models were implemented to assess the relationship between the rate of Hct fall after the first IUT and the following variables: fetal hydrops; antibody titer; gestational age at the first IUT; number of days between the first and second IUT; pretransfusion and posttransfusion fetal Hct values. RESULTS: Fifty fetuses fulfilled the study criteria. The fetal Hct decrease after the first IUT was 1.21 (range 0.18-2.3) %/day. The variables independently associated with the fetal Hct drop after the first IUT were the fetal hydrops (p = 0.000), the pretransfusion fetal Hct (p = 0.001) and the posttransfusion fetal Hct (p = 0.016). CONCLUSION: Fetal hydrops, pretransfusion fetal Hct and posttransfusion fetal Hct seem to influence the fetal Hct decrease between the first and second IUT. These findings may be helpful for estimating the rate of fetal Hct drop and programming the following IUT.

Hidrops fetal: diagnóstico etiológico y manejo / Fetal hydrops: etiologic diagnosis and management

El hidrops fetal es una seria condición, la que tiene una compleja fisiopatología y se asocia a una variada gama de etiologías y un incierto pronóstico perinatal (mortalidad entre 60-90%). Pese a los avances en el campo de la Medicina Fetal, sólo existe posibilidad terapéutica en no más del 30% de los casos. Es necesario seguir investigando en las bases fisiopatológicas de la enfermedad, así como también estimular el diagnóstico precoz, especialmente de las causas relacionadas con malformaciones cardíacas y de paso, favorecer el asesoramiento genético a las parejas en riesgo.

Hydrops fetalis is a serious condition due to a complex pathophysiology, which is associated with a wide range of etiologic mechanisms leading to a poor pregnancy outcome (mortality of 60-90% of the cases). Management and therapeutic options are limited and can only be offered to one third of the cases. Investigation of the pathophysiologic basis as well as an early diagnosis would probably help in improving the outcome and genetic counseling of parents at risk for this condition.

Septostomy with amniodrainage in the treatment of twin-to-twin transfusion syndrome: a 16-case report.

OBJECTIVE: To report the treatment results of 16 monochorionic and diamniotic gestations cases, which had complications due to the twin-to-twin transfusion syndrome (TTTS), the neonatal and other possible complications of the septostomy associated to the amniodrainage. METHOD: Based on ultrasonographic findings, 16 pregnant women were diagnosed with the twin-to-twin transfusion syndrome (TTTS). These cases were divided in two groups: one of them included the fetuses without hydrops and the second included the "recipient" fetuses with hydrops. The therapy measures included septostomy with or without amniodrainage. The following parameters were evaluated: gestational age at the time the septostomy was performed, volume of drained amniotic fluid, gestational age at delivery, birth weight, postnatal evolution, and procedure complications. RESULTS: The average gestational age for this procedure was of 23.6 weeks (from 14 weeks and 1 day to 33 weeks). The gestational age for the septostomy until the delivery was of 8.18 weeks (from 1.0 to 21.3 weeks). The survival rate in the group without hydrops was of 68.7%, while in the second group it was of 25%. Some of the complications were as follows: two cases of premature membrane rupture and one case of preterm labor. CONCLUSION: Septostomy with amniodrainage, when performed on the initial stages and on earlier gestational ages has good perinatal results.

La transfusión intrauterina por cordocentesis: Una alternativa terapéutica con expectativa de vida / Intrauterine transfusion by cordocentesis: A therapuetic alternative with life expectancy

La enfermedad hemolítica por anti D es una grave problemática de nuestro medio que es reconocido como causa de muerte perinatal. Conociendo que las pacientes con enfermedad hemolítica severa, las posibilidades de viabilidad fetal son nulas a pocas semanas de gestación donde otros tratamientos no fueron suficientes, nuestro trabajo demuestra que la transfusión intrauterina por cordocentesis (TIUPC) es una técnica que logra alta expectativa de vida ya que permite no solo reabsorber el hidrops fetal alcanzando embarazos a término con fetos viables, sino también evolución de los recién nacidos sin necesidad de internaciones prolongadas, con menor costo, en relación a los gastos ocasionados por el manejo multidisciplinario que requerían los neonatos sin este tratamiento. (AU)

La transfusión intrauterina por cordocentesis: Una alternativa terapéutica con expectativa de vida / Intrauterine transfusion by cordocentesis: A therapuetic alternative with life expectancy

La enfermedad hemolítica por anti D es una grave problemática de nuestro medio que es reconocido como causa de muerte perinatal. Conociendo que las pacientes con enfermedad hemolítica severa, las posibilidades de viabilidad fetal son nulas a pocas semanas de gestación donde otros tratamientos no fueron suficientes, nuestro trabajo demuestra que la transfusión intrauterina por cordocentesis (TIUPC) es una técnica que logra alta expectativa de vida ya que permite no solo reabsorber el hidrops fetal alcanzando embarazos a término con fetos viables, sino también evolución de los recién nacidos sin necesidad de internaciones prolongadas, con menor costo, en relación a los gastos ocasionados por el manejo multidisciplinario que requerían los neonatos sin este tratamiento.

Pulmonary sequestration presenting as fetal hydrops.

Pulmonary sequestration is a rare developmental anomaly of the lung characterized by nonfunctional pulmonary tissue without communication with the tracheobronchial tree and receiving an aberrant systemic arterial blood supply. Few cases of non-immune hydrops fetalis associated with this entity have been reported. A 2 day old male baby born by cesarean section at 31 weeks gestational age due to fetal hydrops is presented. Autopsy revealed a hydropic baby with extralobar pulmonary sequestration and bilateral pulmonary hypoplasia. The clinicopathologic presentation of this unusual pulmonary developmental anomaly is discussed.

Pulmonary sequestration presenting as fetal hydrops

Pulmonary sequestration is a rare developmental anomaly of the lung characterized by nonfunctional pulmonary tissue without communication with the tracheobronchial tree and receiving an aberrant systemic arterial blood supply. Few cases of non-immune hydrops fetalis associated with this entity have been reported. A 2 day old male baby born by cesarean section at 31 weeks gestational age due to fetal hydrops is presented. Autopsy revealed a hydropic baby with extralobar pulmonary sequestration and bilateral pulmonary hypoplasia. The clinicopathologic presentation of this unusual pulmonary developmental anomaly is discussed.

[Non-immunologic hydrops. Report of 2 cases]. / Hidrops no inmunológico. Informe de dos casos.

UNLABELLED: Non immune hydrops is an uncommon entity, it is observed in one per 2,000 to 3,500 alive birth. It can be idiopathic or secondary to different pathologies, the most important examples are: congenital malformations, cromosompathies or viral infections. OBJECTIVE: To show our therapeutic and diagnostic experience in two cases of non immune hydrops. STUDY DESIGN: To present a case of spontaneous reversion of non immune hydrops secondary to parvovirus B 19 infection and other case secondary to congenital cardiopathy. RESULTS: We found spontaneous reversion of a case of non immune hydrops due to a parvovirus B 19 infection and infant was normal but the infant with congenital cardiopathy died ten minutes after birth. CONCLUSIONS: Two of the most important causes of non immune hydrops are cardiopathies and infections, specially parvovirus which is responsible of poor perinatal outcome but there is a remote possibility of spontaneous reversion because it is important to make a specific diagnose and to make the expected management.

Hidrops no inmunológico: informe de dos casos / Non immune hydrops: two cases report

El hidrops no inmunológico es una entidad rara que se observa en uno por cada 2,000 a 3,500 nacidos vivos y puede ser idiopático o secundario a diversas patologías entre las que destacan: malformaciones congénitas, cromosomopatías e infecciones especialmente de tipo viral. Objetivo. Mostrar nuestra experiencia diagnóstico y terapéutica en esta patología. Diseño del estudio. Se presentan dos casos de hidrops no inmunológico, uno secundario a cardiopatía congénita y el otro debido a infección por parvovirus B 19. Resultados. Obtuvimos muerte neonatal inmediata en el caso de cardiopatía congénita y comparativamente reversión espontánea del hidrops causado por parvovirus B 19. Conclusiones. Entre las causas más frecuentes del hidrops no inmunológico destacan las cardiopatías y las infecciones en especial por parvovirus que aunque son responsables de pérdidas perinatales, también existe la posibilidad de reversión espontánea o por establecimiento de la terapéutica específica, por lo que es muy importante realizar el diagnóstico preciso y el manejo apropiado.

Transfusión intravascular en el manejo de la enfermedad hemolítica perinatal: reevaluación de nuestra experiencia / Intravascular transfusion in the treatment of perinatal hemolytic disease: reevaluation of our experience

Presentamos la reevaluación de nuestra experiencia en transfusión intravascular, experiencia acumulada desde 1989 a la fecha. Los resultados alcanzados en 82 procedimientos en 26 pacientes, con sobrevida de 8 de 10 fetos hidrópicos y 13 de los 16 no hidrópicos, son comparables a los presentados pr otros centros cuyos datos están disponibles para su análisis en la literatura de la especialidad. Compartimos además, lo que hemos aprendido en la práctica de este procedimiento durante los últimos años

Hidrops fetal no inmune asociado a talasemia familiar: transfusión intravascular / Non-immune fetal hidrops associated to familiar thalasemia: intravascular transfussion

Se presenta un caso de hidrops fetal no inmune provocado por una anemia hemolítica severa secundaria a una talasemia familiar pesquisada a las 28 semanas y que revierte luego de transfusión intravascular, TIV, practicada en nuestro servicio. Se analiza el diagnóstico, manejo prenatal y posterior evolución

[Usefulness of intraperitoneal transfusion under direct ultrasound guidance]. / Utilidad de la transfusión intraperitoneal bajo guía ultrasonográfica directa.

Experience with intraperitoneal blood transfusion, using ultrasonographic guide, in 24 patients with severe isoimmunization to Rh factor, is reported. A total of 57 procedures, were done from 24 weeks of gestation. In 66.6% two or more transfusions were done in the same product. There were 71% of alive newborns and in 29% of the cases it was not possible to improve perinatal survival. The analysis of these results were correlated with antecedents: presence of hydrops, gestational age at the time of resolving pregnancy, and main disease status. The usefulness of intraperitoneal transfusion, is commented upon, and new perspectives, as intravascular transfusion in cases with severe hydrops, are considered.