Severe swelling can interfere with how the body organs work and often results. Evaluation of hydrops begins with an antibody screen indirect coombs test to verify that it is nonimmune, detailed sonography of the fetuses and placenta, including echocardiography and assessment for fetal arrhythmia, and middle cerebral artery doppler evaluation for anemia, as well as fetal karyotype andor. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization eg, rhd, kell. Immune hydrops fetalis is most often a complication of a severe form of rh incompatibility, which can be prevented. Hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. Hydrops fetalis is associated with a large number of pathologies see table 1 that are usuallydividedinto.
Castillo ra, devore ld, hadi ha, martin s, geist p. Mar, 2014 da silva op, ramanan r, romano w, evans m. Nonimmune hydrops fetalis nihf is used to describe fetuses and newborns with generalized edema and cavity effusions. The identification of hydrops by ultrasound is very frustrating to the family and physicians because it requires extensive search for the etiology, which include a.
All other causes are described as nonimmune hydrops fetalis nihf. It may be manifestation of a variety of underlying disorders. Hydrops fetalis medical definition merriamwebster medical. Immune hydrops fetalis is no longer very common due to advances in the prevention of hemolytic disease of the newborn. Hydrops fetalis is defined as the abnormal accumulation of fluid in at least two different fetal compartments 1. Hydrops fetalis is a condition in which a fetus or newborn has an abnormal amount of fluid buildup in the tissue surrounding the lungs pleural space, the tissue surrounding the heart pericardial sac, the abdomen a condition known as ascites, as well as under the skin a condition known as edema. Fetal death in utero, hydrops fetalis, infant mortality. Nonimmune hydrops fetalis is a heterogeneous disorder, caused by a large number of underlying pathologic processes. Rare causes of fetal hydrops need to be excluded in cases of recurrent nonimmune hydrops with no obvious aetiology following routine investigations.
Investigation and management of nonimmune fetal hydrops. This is a page to support awareness of hydrops fetalis. Hydrops fetalis is the most obvious and serious manifestation of an intrauterine parvovirus b19 infection, which is seen in approximately 4% of all affected pregnancies fig. Depending on the severity and cause of hydrops, there may be edema of fetus and placenta, ascites, pleural effusions andor pericardial effusions. Diagnosis and management nonimmune hydrops inthe newborn. These are largely rhd antigens, but rare cases of sensitization against other antigens e. These fluidfilled spaces can occur in different fetal body locations, including. Rh incompatibility causes a large number of red blood cells in the. In some patients, it may also be associated with polyhydramnios and placental edema. Managing nonimmune hydrops fetalis linkedin slideshare. In approximately 14 of all cases, the cause is not. Pulmonary defects the survival rates in hydrops and pleural effusion treated conservatively is 12%, however survival rate in cases treated with pleuroamniotic shunt is 50%.
Nonimmune hydrops fetalis is a serious fetal condition in which abnormal fluid accumulates in at least two different fetal compartments, and in which circulating antibodies against redcell antigens are absent in the mother. Made because when the doctor tells you that your baby has. Clinical characteristics and perinatal outcome of fetal hydrops. Hydrops fetalis is severe swelling edema in an unborn baby or a newborn baby. Multiple fetal anatomic and functional disorders can cause nihf. Nonimmune hydrops fetalis caused by herpes simplex virus type 2 in the setting of recurrent maternal infection. Hydrops is a symptom of a wide range of conditions which have resulted in an imbalance in fetal fluid between the vascular and interstitial space. Hydrops fetalis refers to abnormal fluid collections in fetal soft tissues and serous cavities. However, over 90% of hydrops in the western world is of nonimmune origin. It is defined as an abnormal collection of fluid in at least two different fetal organ spaces. Jul 25, 2017 hydrops fetalis fetal hydrops is a serious fetal condition defined as abnormal accumulation of fluid in 2 or more fetal compartments, including ascites, pleural effusion, pericardial effusion, and skin edema. Immune hydrops fetalis is a complication of a severe form of rh incompatibility.
The rest of this section deals only with nonimmune hydrops fetalis. Prenatal diagnosis and management of nonimmunologic hydrops fetalis. For 150 years, families have come from around the corner and across the world, looking to boston childrens for answers. The antenatal and postnatal aspects ofinvestigation andmanagement ofimmunehydrops are well covered in standard obstetric and. Ultrasound evaluation of hydrops fetalis radiology key. Therefore, hydrops fetalis should not be used to describe these conditions. Erythroblastosis fetalis, or hemolytic disease of the newborn, is a condition caused by specific antibodies of the mother, directed against red cell antigens of the fetus. Hydrops fetalis is sometimes used as a synonym for homozygous alpha thalassemia, a lethal or lifethreatening disease of mid and late fetal development caused by the inability to make red.
In this case no cause for hydrops was found on either clinical evaluation, imaging or autopsy. Hydrops fetalis, lysosomal storage disorders, nonimmune, thalassemia, ultrasonography introduction hydrops fetalis is latin for edema of the fetus. The identification of hydrops by ultrasound is very frustrating to the family and physicians because it requires extensive search for the etiology, which include a wide variety of diseases and pathologies. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively. The condition is subdivided in to a immune hydrops. Gross fetal hydrops is demonstrated with associated bilateral pleural effusions and ascites. Hydrops fetalis is an excessive fluid accumulation within the fetal extra vascular compartments and body cavities. If infection develops during the first 12 weeks of gestation, the risk of. Overall, 60% of the cases of nihf are diagnosed prenatally bellini et al av channel, 2015. Hydrops can be divided into two major categories or types. Nonimmune hydrops fetalis swiss society of neonatology.
Immune hydrops is defined as the diagnosis of fetal hydrops in the presence of a maternal immunologic response to a paternally derived red blood cell antigen in the fetus. Hydrops fetalis fetal hydrops is a serious fetal condition defined as abnormal accumulation of fluid in 2 or more fetal compartments, including ascites, pleural effusion, pericardial effusion, and skin edema. Immune hydrops accounts for 1020%of cases maternal antibodies against redcells of the fetus cross the placenta and coat fetal red cells which are then destroyed hemolysis in the. It occurs when abnormal amounts of fluid build up in two or more body areas of a fetus or newborn. The incidence of fetal hydrops in our retrospective study was 24. Etiology and perinatal outcome of nonimmune hydrops. Nonimmune hydrops fetalis accounts for 76 to 87% of all described cases of hydrops fetalis bellini et al. Anaemia results from maternal isoimmunisation against rhesus or other red cell antigens. Fetal hydrops is further defined as the accumulation of fluid in at least two of the following fetal serous compartments.
Hydrops fetalis genetic and rare diseases information. It is helpful to alert physicians about the presence of anemia, heart failure, andor hypoproteinemia, but this diagnosis is frequently overlooked. It should be noted, however, that in those fetal conditions in which hydrops is a known outcome of progressive disease, a single accumulation of fluid in a body cavity may be enough to warrant referring to. The number of fluid collection sites was the significant antenatal risk factor to predict neonatal death. May 17, 2019 hydrops fetalis is a serious condition of the fetus or newborn. Erythroblastosis fetalis and hydrops fetalis springerlink. The incidence of fetal hydrops is reported to be 3 to 24 per 10,000 live births. What are the treatment options for a baby with a cpam who develops hydrops. Jan 25, 2012 hydrops fetalis is a serious condition in which abnormal amounts of fluid build up in two or more body areas of a fetus or newborn. Hydrops develops when too much fluid leaves the babys bloodstream and goes into the tissues. Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic techniques. Results for hydrops fetalis 1 10 of 71 sorted by relevance date click export csv or ris to download the entire page or use the checkboxes to select a subset of records to download export csv export ris 10 per page 50 per page 100 per page 250 per page. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments. This is the place where the most difficult challenges are faced head on, where the impossible becomes possible, and where families in search of answers find them.
It should be noted, however, that in those fetal conditions in which hydrops is a known outcome of progressive disease, a single accumulation of fluid in a body cavity may be enough to warrant referring to the condition as early hydrops. Feb 05, 2014 hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. A list of the more common causes is shown in table 1. Appropriate investigation and management of nonimmune fetal hydrops education of readers about the causes of nonimmune fetal hydrops and its prenatal counselling and management provision of a standardized approach to nonimmune fetal hydrops, emphasizing the search for prenatally treatable conditions and recurrent genetic etiologies. Nonimmune hydrops fetalis can be caused by a wide variety of factors. Clinical characteristics and perinatal outcome of fetal hydrops ncbi.
Severe swelling can interfere with how the body organs work and often results in death of the infant shortly before or after delivery. Hydrops fetalis comes from a greek term meaning the pathological accumulation of fluid in the fetal soft tis sues and body cavities. Hydrops fetalis is a serious condition in which abnormal amounts of fluid build up in two or more body areas of a fetus or newborn. Nonimmune hydrops fetalis nonimmune hydrops fetalis nihf accounts for approximately 90% of all cases. To improve our services and products, we use cookies own or third parties authorized to show advertising related to client preferences through the analyses of navigation customer behavior. Nonimmune hydrops fetalis nihf, due to causes other than rh alloimmunization. Seventy percent of all cases of ahf associated with parvo b19 infection occur between 20 and 24 weeks of gestation 11. Also, in many other fetal diseases, the presence or absence of hydrops has a major in. In southeast asia, alphathalassemia is the most common cause of hydrops fetalis, accounting for 60 to 90% of cases. Depending on the severity and cause of hydrops, there may be edema of fetus.
It is a condition where abnormal amounts of fluid build up in two or more body areas causing swelling. Hydrops fetalis is an excess accumulation of fluid in the fetus. Hydrops fetalis, lysosomal storage disorders, nonimmune, thalassaemia, ultrasonography introduction the incidence of nonimmune hydrops nih has been. Hydrops fetalisor hydropsis a condition in which large amounts of fluid build up in a babys tissues and organs, causing extensive swelling edema. About half of unborn babies with hydrops fetalis do not survive. Table 1 etiologies and perinatal outcomes of nonimmune hydrops fetalis in southern china etiologies number top iufd nd 1 y without complications 1ywith complications hb barts hydrops 298 61. The overall prognosis of fetal hydrops is poor, with a perinatal mortality rate between 50% and 98% 1.
Unfortunately, in 85% of cases causation cannot be determined. In fetuses with severe alloimmune anemia, the severity of hydrops is a major determinant for the prognosis 2. Diagnostic criteria nonimmune hydrops fetalis nihf is as pathologic accumulation of fluid in the skin and one or more other body compartments of the fetus, including the pleural space, peritoneal cavity, pericardial sac, or placenta. Hydrops fetalis is not itself a disease, but an ultrasound marker of other fetal complications. Hidrops fetalis adalah pdf hydrops fetalis is a severe, lifethreatening problem of severe edema swelling in the fetus and newborn. Ucsf hydrops fetalis clinical trials san francisco bay area. About 50% of unborn babies with hydrops dont survive. Nonimmune hydrops fetalis american academy of pediatrics. Nonimmune hydrops is the presence of two or more abnormal fetal fluid collections in the absence of red cell alloimmunization. This is a condition in which mother who has rh negative blood type makes antibodies to her babys rh positive blood cells, and the antibodies cross the placenta. Nonimmune hydrops is the presence of two or more abnormal fetal fluid collections in the absence of red cell. Hydrops fetalis is a serious condition of the fetus or newborn. Google scholar maidman je, yeager c, anderson v, makabali g, ogrady jp, arce j, tishler dm.
Nonimmune fetal hydrops is a clinical phenotype and not a diagnosis. Fetal hydrops was defined as the presence of fluid collection in. Hydrops fetalis treatment danafarberboston childrens. Immune hydrops accounts for 1020%of cases maternal antibodies against redcells of the fetus cross the placenta and coat fetal red cells which are then destroyed hemolysis in the fetal spleen. Outcomes of infants with nonimmune hydrops fetalis born after 22 weeks gestation our experience between 1982 and 2000. In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to rh iso. Immune hydrops fetalis ihf was initially recognized as the combination of neonatal hydrops, anemia, and jaundice along with erythroblastosis and red blood cell hemolysis by diamond and coinvestigators in 1932.