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. Thorough investigations are needed to be able to identify an underlying cause. 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. See letter diagnosis and management of nonimmune hydrops in the newborn in volume 71 on page f148. Aetiology, diagnosis and treatment of hydrops foetalis.
Its usually a complication of another medical condition that affects the way the body manages fluid. Pdf investigation of nonimmune hydrops fetalis roy. All other causes are described as nonimmune hydrops fetalis nihf. 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. Pubmed or medline, cinahl, and the cochrane library in. Maternal antibody screening is recommended in all pregnancies.
Depending on the severity and cause of hydrops, there may be edema of fetus and placenta, ascites, pleural effusions andor pericardial effusions. All of the other reasons that a fetus develops nihf are included in this category. Immune hydrops fetalis is a complication of a severe form of rh incompatibility. Nonimmune hydrops fetalis nihf refers specifically to cases of hydrops not caused by red cell alloimmunization. Multiple fetal anatomic and functional disorders can cause nihf. Pdf on jul 1, 2017, n gayatri and others published nonimmune fetal hydrops. As a result, nihf now accounts for almost 90% of cases of. Nonimmune hydrops fetalis with cystic hygroma colli. Overall, 60% of the cases of nihf are diagnosed prenatally bellini et al av channel, 2015. Hydrops fetalis is the presence of excessive fluid in two or more fetal compartments including skin edema, pleural effusion, pericardial effusion, ascites, and polyhydramnios. It may be manifestation of a variety of underlying disorders.
Nonimmune hydrops fetalis nonimmune hydrops fetalis diagnosis definition. 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. Congenital hypothyroidism and nonimmune hydrops fetalis. If you are rh negative and have an rh positive baby, your immune system attacks your unborn baby. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments.
Unfortunately, in 85% of cases causation cannot be determined. With rhogam prophylaxis, the rate of sensitization has been reduced to 1%, according to a worldwide cooperative study. Nonimmune hydrops is the presence of two or more abnormal fetal fluid collections in the absence of red cell alloimmunization. Presentation may be prenatal with ultrasound diagnosis on at the time of delivery. Nonimmune hydrops fetalis nihf is a complex condition with a high mortality and morbidity rate. Anaemia results from maternal isoimmunisation against rhesus or other red cell antigens. 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. Immune hydrops fetalis is most often a complication of a severe form of rh incompatibility, which can be prevented. A list of the more common causes is shown in table 1. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell antigens alloimmunization. Nonimmune hydrops fetalis, which occurs when disease or other complications interfere with a babys ability to manage fluid. The classification of immune and nonimmune hydrops foetalis describes the difference. Onethird of hydropic fetuses are discovered incidentally during prenatal sonography in the first or second trimester of gestation. Nonimmune hydrops fetalis nihf is a term that defines an edematous fetus that does not have erythroblastosis fetalis from isoimmunization.
There are two types of hydrops fetalis, immune and nonimmune. 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. 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. Abstract objective to investigate the genetic causes and clinical outcomes of nonimmune hydrops fetalis nihf. Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic. Diagnosis and management of nonimmune hydrops in the newborn. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization eg, rhd, kell. Parvovirus b19 infection during pregnancy uptodate. 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. Nonimmune hydrops fetalis american academy of pediatrics.
About half of unborn babies with hydrops fetalis do not survive. Methods cohort of cases of nihf between july 20 and december 2018. 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. Recent improvements in diagnosing parvovirus infections and the availability of intrauterine transfusion have reduced the overall rate of fetal loss after maternal exposure. Almost all observed cases of hf are of the nonimmune type, the causes of which remain undetermined in 15% of patients. It is defined as an abnormal collection of fluid in at least two different fetal organ spaces. 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. Hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. 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. The antenatal and postnatal aspects ofinvestigation andmanagement ofimmunehydrops are well covered in standard obstetric and. Hydrops fetalis refers to abnormal fluid collections in fetal soft tissues and serous cavities.
These fluidfilled spaces can occur in different fetal body locations, including. Hydrops is traditionally classified into either immune or non. Hydrops is defined as an abnormal accumulation of serous fluid in at least two body cavities or tissues. During pregnancy, symptoms of hydrops fetalis may include. Alpha thalassemia major four gene alpha thalassemia, or homozygous alpha thalassemia interferes with the fetus ability to make red blood cells. Non immune is defined as absence of a detectable circulating antibodies against red blood cells in the mother.
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. Hydrops fetalis is severe swelling edema in an unborn baby or a newborn baby. In some patients, it also may be associated with polyhydramnios and placental edema. 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 associated with a large number of pathologies see table 1 that are usuallydividedinto. 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.
Ninety percent are nonimmune not the result of red cell alloimmunization. Hydrops fetalis treatment danafarberboston childrens. Nonimmune hydrops fetalis nihf, a form of hf, is a severe fetal condition defined as the excessive accumulation. It is not a specific disease but the end result of many fetal conditions. It may develop because of rh disease in the mother. Etiology and outcome of nonimmune hydrops fetalis in. Here we report the etiology and outcome of 1004 fetuses with nihf, in a large single maternal and. A long list of etiologies has been found in association with nonimmune hydrops. Most cases of hydrops fetalis are now caused by other conditions and are known as nonimmune hydrops. Hydrops fetalis is found in about 1 per 2,000 births and is categorized as immune or nonimmune hydrops.
Rarely, acute infection in pregnancy may lead to fetal loss or hydrops fetalis. Hydrops fetalis secondary to parvovirus b19 infections. The condition is subdivided in to a immune hydrops. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively. Hydrops fetalis is a serious condition indicating a bad prognosis of affected fetuses. 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. 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. Hydrops fetalis genetic and rare diseases information. In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to rh isoimmunization. Rh compatibility causes massive red blood cell destruction, which leads to. Nonimmune hydrops fetalis nihf encompasses all causes of fetal hydrops that are not caused by the passage of maternal antibodies into the fetal compartment. Nonimmune hydrops fetalis can be caused by a wide variety of factors.
The type depends on the cause of the abnormal fluid. Nonimmune fetal hydrops is diagnosed when there is fluid accumulation in more than one extravascular space. Nonimmune hydrops fetalis incidentally found on routine. 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. Symptoms of hydrops fetalis can occur during pregnancy or after the baby is born. Nonimmune hydrops fetalis caused by g6pd deficiency. With the development and widespread use of rhd immune globulin, the prevalence of rhd alloimmunization and associated hydrops has dramatically decreased. Hydrops can be divided into two major categories or types. The causes of nihf range from diseases to structural anomalies to genetic abnormalities. Parvovirus b19 infection is a common childhood illness.
Nonimmune hydrops fetalis nonimmune hydrops fetalis nihf accounts for approximately 90% of all cases. See commentary diagnosis of nonimmune hydrops in the newborn. Hydrops fetalis is not itself a disease, but an ultrasound marker of other fetal complications. Asymptomatic or mild infection occurs most often when b19 affects immunocompetent adults. Hydrops fetalis is an excess accumulation of fluid in the fetus. In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to rh iso. Secondary to the decreasing prevalence of immune hydrops, nihf now comprises 90% of all hydrops cases.
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