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. Secondary to the decreasing prevalence of immune hydrops, nihf now comprises 90% of all hydrops cases. Nonimmune hydrops fetalis nihf encompasses all causes of fetal hydrops that are not caused by the passage of maternal antibodies into the fetal compartment. Maternal antibody screening is recommended in all pregnancies. Pdf investigation of nonimmune hydrops fetalis roy. All of the other reasons that a fetus develops nihf are included in this category. Symptoms of hydrops fetalis can occur during pregnancy or after the baby is born. Recent improvements in diagnosing parvovirus infections and the availability of intrauterine transfusion have reduced the overall rate of fetal loss after maternal exposure. Hydrops fetalis is an excessive accumulation of fetal fluid. Nonimmune hydrops fetalis is an uncommon but serious disorder associated with an overall poor prognosis, characterized by abnormal fluid accumulation in two or more fetal serous compartments, together with generalized soft tissue edema. Nonimmune hydrops fetalis nihf refers to hydrops in the absence of maternal circulating redcell antibodies, and constitutes up to 90% of all described hydrops fetalis cases. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization eg, rhd, kell.
Diagnosis and management of nonimmune hydrops in the newborn. All other causes are described as nonimmune hydrops fetalis nihf. See letter diagnosis and management of nonimmune hydrops in the newborn in volume 71 on page f149. In some patients, it also may be associated with polyhydramnios and placental edema. 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. Thorough investigations are needed to be able to identify an underlying cause. Hydrops fetalis is an excess accumulation of fluid in the fetus. Nonimmune hydrops fetalis american academy of pediatrics. 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. Nonimmune hydrops fetalis nihf, a form of hf, is a severe fetal condition defined as the excessive accumulation. In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to rh iso.
Etiology and outcome of nonimmune hydrops fetalis in. Nonimmune fetal hydrops is a condition easily diagnosed by antenatal ultrasound. Nonimmune hydrops is the presence of two or more abnormal fetal fluid collections in the absence of red cell alloimmunization. Hydrops is defined as an abnormal accumulation of serous fluid in at least two body cavities or tissues. With rhogam prophylaxis, the rate of sensitization has been reduced to 1%, according to a worldwide cooperative study. Pdf on jul 1, 2017, n gayatri and others published nonimmune fetal hydrops. See commentary diagnosis of nonimmune hydrops in the newborn.
In the era before routine immunization of rhesus rh negative mothers, most cases of hydrops were due to erythroblastosis from rh alloimmunization, but nowadays, nonimmune hydrops fetalis nihf is more frequent, representing 7687% of. Onethird of hydropic fetuses are discovered incidentally during prenatal sonography in the first or second trimester of gestation. Nonimmune hydrops fetalis nonimmune hydrops fetalis nihf accounts for approximately 90% of all cases. Nonimmune hydrops fetalis incidentally found on routine. Anaemia results from maternal isoimmunisation against rhesus or other red cell antigens. Overall, 60% of the cases of nihf are diagnosed prenatally bellini et al av channel, 2015.
Nonimmune hydrops fetalis caused by g6pd deficiency. For information on our recommended workup, download the nonimmune hydrops fetalis workup pdf fetal exome sequencing next generation sequencing ngs is one area of precision medicine the holds promise for the diagnosis and treatment of fetal congenital anomalies, genetic disorders and other complications of pregnancy. Hydrops can be divided into two major categories or types. It is defined as an abnormal collection of fluid in at least two different fetal organ spaces. A list of the more common causes is shown in table 1. The condition is subdivided in to a immune hydrops. Pubmed or medline, cinahl, and the cochrane library in. Abstract objective to investigate the genetic causes and clinical outcomes of nonimmune hydrops fetalis nihf. The type depends on the cause of the abnormal fluid. Hydrops fetalis is associated with a large number of pathologies see table 1 that are usuallydividedinto. It may develop because of rh disease in the mother.
Incidence of immune hydropsfetalis is significantly decreasing, whereas more and more nonimmune. Rarely, acute infection in pregnancy may lead to fetal loss or hydrops fetalis. Asymptomatic or mild infection occurs most often when b19 affects immunocompetent adults. Almost all observed cases of hf are of the nonimmune type, the causes of which remain undetermined in 15% of patients. Non immune is defined as absence of a detectable circulating antibodies against red blood cells in the mother. Hydrops fetalis that develop in the absence of red cell hemolysis is termed as nonimmune fetal hydrops nifh and was first described by dr.
Hydrops fetalis or fetal hydrops is defined as a generalized abnormal accumulation of serous fluid in the entire body including abnormal collection in tissues and body cavities. A long list of etiologies has been found in association with nonimmune hydrops. The causes of nihf range from diseases to structural anomalies to genetic abnormalities. Hydrops fetalis is a serious condition indicating a bad prognosis of affected fetuses.
Nonimmune hydrops fetalis nihf is a complex condition with a high mortality and morbidity rate. 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. Ninety percent are nonimmune not the result of red cell alloimmunization. Immune hydrops fetalis is most often a complication of a severe form of rh incompatibility, which can be prevented. Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic. Alpha thalassemia major four gene alpha thalassemia, or homozygous alpha thalassemia interferes with the fetus ability to make red blood cells. Hydrops fetalis is the presence of excessive fluid in two or more fetal compartments including skin edema, pleural effusion, pericardial effusion, ascites, and polyhydramnios. Depending on the severity and cause of hydrops, there may be edema of fetus and placenta, ascites, pleural effusions andor pericardial effusions. Parvovirus b19 infection is a common childhood illness. Hydrops fetalis hf consists of an abnormal accumulation of fluid in two or more fetal compartments, including ascites, pleural effusion, pericardial effusion, and skin edema.
Multiple fetal anatomic and functional disorders can cause nihf. Presentation may be prenatal with ultrasound diagnosis on at the time of delivery. These fluidfilled spaces can occur in different fetal body locations, including. Nonimmune hydrops fetalis occurs in approximately one of every 1,000 births. Nonimmune hydrops fetalis with cystic hygroma colli. Nonimmune hydrops fetalis can be caused by a wide variety of factors. Hydrops fetalis refers to abnormal fluid collections in fetal soft tissues and serous cavities. Nonimmune hydrops fetalis nonimmune hydrops fetalis diagnosis definition. 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. Aetiology, diagnosis and treatment of hydrops foetalis. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments.
Methods cohort of cases of nihf between july 20 and december 2018. If you are rh negative and have an rh positive baby, your immune system attacks your unborn baby. See letter diagnosis and management of nonimmune hydrops in the newborn in volume 71 on page f148. The decline in the frequency of rh isoimmunization has resulted in a decrease in the total incidence of hydrops fetalis, but there has been an increase in the relative proportion of nonimmune hydrops fetalis. The classification of immune and nonimmune hydrops foetalis describes the difference.
Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell antigens alloimmunization. Managing non immune hydrops fetalis dr vidya a thobbi professor and head department of obg alameen medical college bijapur slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. About half of unborn babies with hydrops fetalis do not survive. Nonimmune hydrops fetalis, which occurs when disease or other complications interfere with a babys ability to manage fluid. It may be manifestation of a variety of underlying disorders. Hydrops is traditionally classified into either immune or non. Here is a case of an 18 week pregnancy with hydrops fetalis. Its usually a complication of another medical condition that affects the way the body manages fluid. There are two types of hydrops fetalis, immune and nonimmune. Hydrops fetalis is severe swelling edema in an unborn baby or a newborn baby. Here we report the etiology and outcome of 1004 fetuses with nihf, in a large single maternal and.
Nonimmune fetal hydrops is diagnosed when there is fluid accumulation in more than one extravascular space. Nonimmune hydrops fetalis nihf is a term that defines an edematous fetus that does not have erythroblastosis fetalis from isoimmunization. Parvovirus b19 infection during pregnancy uptodate. The diagnosis is reached when there is fluid accumulation in more than one extravascular space, this includes soft tissue edema in the skin or scalp, or fluid in body cavities such as ascites pleural effusion, pericardial effusion, or hydrocele. Hydrops fetalis treatment danafarberboston childrens.
Immune hydrops fetalis is a complication of a severe form of rh incompatibility. Most cases of hydrops fetalis are now caused by other conditions and are known as nonimmune hydrops. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively. Hydrops fetalis secondary to parvovirus b19 infections.
The reported incidence of nonimmune fetal hydrops nifh is 1 in 20003000 pregnancies 4, 5. Hydrops fetalis is found in about 1 per 2,000 births and is categorized as immune or nonimmune hydrops. Rh compatibility causes massive red blood cell destruction, which leads to. With the development and widespread use of rhd immune globulin, the prevalence of rhd alloimmunization and associated hydrops has dramatically decreased. Hydrops fetalis is not itself a disease, but an ultrasound marker of other fetal complications. Congenital hypothyroidism and nonimmune hydrops fetalis. Nonimmune hydrops fetalis nihf refers specifically to cases of hydrops not caused by red cell alloimmunization. Unfortunately, in 85% of cases causation cannot be determined. It is routine to evaluate for maternal antibodies at the first prenatal visit, and if initially negative, to retest at 28 weeks and then after delivery. As a result, nihf now accounts for almost 90% of cases of. Hydrops fetalis genetic and rare diseases information. It is not a specific disease but the end result of many fetal conditions. In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to rh isoimmunization. The antenatal and postnatal aspects ofinvestigation andmanagement ofimmunehydrops are well covered in standard obstetric and.
Hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. During pregnancy, symptoms of hydrops fetalis may include. Fetal infection by human parvovirus b19 is a common cause of fetal anemia, nonimmune hydrops fetalis, and spontaneous abortion and can result in fetal death. Immune hydrops accounts for 1020%of cases maternal antibodies against redcells of the fetus cross the placenta and coat fetal red cells. Hydrops fetalis is a serious, lifethreatening condition in which a fetus or newborn has an abnormal buildup of fluids in the tissue around the lungs, heart, or abdomen, or under the skin.
1485 1128 1569 735 571 1311 150 1199 90 911 861 1468 280 517 214 1543 290 1281 90 763 369 975 924 1390 1075 1159 1177 63 274 211 170 889 780 253 114 64 849 674 641 1312 783 88 845 1488