Nonimmune hydrops fetalis american academy of pediatrics. Hydrops fetalis occurs when the rate of interstitial fluid production by capillary ultrafiltration exceeds the rate of interstitial fluid return to the circulation via lymphatic vessels. 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. Hydrops fetalis refers to abnormal fluid collections in fetal soft tissues and serous cavities. Hydrops fetalis survival rate, prognosis, pictures, causes. Aetiology, diagnosis and treatment of hydrops foetalis. Depending on the severity and cause of hydrops, there may be edema of fetus. Hydrops fetalis is an excess accumulation of fluid in the fetus. Hydrops fetalis caused by a blood group antibody usually.
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. Seventy percent of all cases of ahf associated with parvo b19 infection occur between 20 and 24 weeks of gestation 11. In approximately 14 of all cases, the cause is not. Nonimmune hydrops fetalis nonimmune hydrops fetalis nihf accounts for approximately 90% of all cases. Nonimmune hydrops fetalis is a heterogeneous disorder, caused by a large number of underlying pathologic processes. Hydrops fetalis has been a wellrecognized fetal and neonatal condition throughout history. Hydrops is traditionally classified into either immune or nonimmune hydrops nihf, but in practice, nowadays in the western world 90. Get a printable copy pdf file of the complete article 272k, or click on a page image below to browse page by page.
Among 27 fetuses exhibiting severe sonographic features of nonimmune hydrops fetalis, 33% survived the neonatal period. Clinical characteristics and perinatal outcome of fetal hydrops. Hydrops can be divided into two major categories or types. Hydrops fetalis is not itself a disease, but an ultrasound marker of other fetal complications. Nonimmune fetal hydrops is a clinical phenotype and not a diagnosis. Nonimmune hydrops fetalis occurs in approximately one of every 1,000 births. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments, including the subcutaneous tissue, pleura, pericardium, or in the abdomen, which is also known as ascites. 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. In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to rh iso. Hydrops fetalis genetic and rare diseases information. Nonimmune hydrops fetalis accounts for 76 to 87% of all described cases of hydrops fetalis bellini et al. Hydrops fetalis is found in about 1 per 2,000 births and is categorized as immune or nonimmune hydrops. Outcomes of infants with nonimmune hydrops fetalis born after 22 weeks gestation our experience between 1982 and 2000.
Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization eg, rhd, kell. Nonimmune hydrops fetalis swiss society of neonatology. Nonimmune hydrops fetalis nonimmune hydrops fetalis diagnosisdefinition. Immune hydrops fetalis is most often a complication of a severe form of rh incompatibility, which can be prevented. Hydrops fetalis is excessive extravasation of fluid into the third space in a fetus which could be due to heart failure, volume overload, decreased oncotic pressure, or increased vascular permeability. Fetal hydrops is further defined as the accumulation of fluid in at least two of the following fetal serous compartments. About half of unborn babies with hydrops fetalis do not survive.
Magann hydrops fetalis hf is defined as the presence of excessive fetal fluid in two or more of the following spaces. All other causes are described as nonimmune hydrops fetalis nihf. Overall, 60% of the cases of nihf are diagnosed prenatally bellini et al av channel, 2015. Immune hydrops fetalis is no longer very common due to advances in the prevention of hemolytic disease of the newborn. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments.
Reported survival rates in nonimmune hydrops fetalis vary considerably. Hf can be mainly categorized as of immune and nonimmune causes, but with the decline of rhesus isoimmunization, most cases have nonimmune etiology. The diagnosis is made by ultrasound imaging evaluating body cavities, the. In southeast asia, alphathalassemia is the most common cause of hydrops fetalis, accounting for 60 to 90% of cases. Ng zm, seet mj, erng mn, buendia f, chang a, sriram b correspondence. The antenatal and postnatal aspects ofinvestigation andmanagement ofimmunehydrops are well covered in standard obstetric and. 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. Nonimmune hydrops is the presence of two or more abnormal fetal fluid collections in the absence of red cell alloimmunization. Nonimmune hydrops fetalis nihf refers to hydrops in the absence of maternal circulating redcell antibodies. Anaemia results from maternal isoimmunisation against rhesus or other red cell antigens. Respiratory distress or breathing problems failure of the heart bruising spots on the skin total swelling of the body severe jaundice severe anemia.
Nonimmune hydrops fetalis occurs when a disease or medical condition disrupts the bodys ability to manage fluid. Lysosomal storage disorder in nonimmunological hydrops. Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic techniques. Us confirmed absent fetal cardiac motion and the presence of diffuse skin edema, ascites and pleural.
Non immune hydrops is universal edema unassociated with erythroblastosis. Hydrops fetalis, lysosomal storage disorders, nonimmune, thalassemia, ultrasonography introduction hydrops fetalis is latin for edema of the fetus. Diagnosis and management nonimmune hydrops inthe newborn. 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. Until the latter half of the 20th century, it was believed to be due to rhesus blood group isoimmunization of the fetus, although potter described infants who had nonimmune causes of fetal hydrops.
Multiple fetal anatomic and functional disorders can cause nihf. An external file that holds a picture, illustration, etc. Hydrops fetalis is associated with a large number of pathologies see table 1 that are usuallydividedinto. 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. Hydrops occurring in patients with rh incompatibilityinfected rh is called immune hydrops fetalis,however hydrops arising out of fetal or maternal causes is called nonimmune hydrops fetalis. The rest of this section deals only with nonimmune hydrops fetalis. Various structural abnormalities in the fetus may subsequently lead to hydrops and hence require follow up scan in all cases. 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. Depending on the severity and cause of hydrops, there may be edema of fetus and placenta, ascites, pleural effusions andor pericardial effusions.
Managing nonimmune hydrops fetalis linkedin slideshare. For five of the nine survivors, the prognosis remains uncertain or poor. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively. In the past, immune hydrops fetalis was caused by rhesus iso. Hydrops fetalis hf is excessive fluid accumulation in fetal extravascular compartments and body cavities leading to edema, ascites, pleural and pericardial effusions, and anasarca. Nonimmune hydrops fetalis nihf, due to causes other than rh alloimmunization, is the cause in 85% of all affected individuals. Hydrops fetalis is a clinical condition characterized by an abnormal.
The incidence of nonimmune hydrops fetalis nihf is estimated at 1 in 3000 pregnancies 3. Nonimmune hydrops fetalis can be caused by a wide variety of factors. Hydrops is defined by abnormal accumulation of serous fluid in skin edema and body cavities pericardial, pleural, or ascitic effusions. Unfortunately, in 85% of cases causation cannot be determined. Castillo ra, devore ld, hadi ha, martin s, geist p. Ballantyne first described hydrops fetalis in 1892, although this condition had been recognized for almost 200 years and 50 years later potter described nonimmune hydrops. Nonimmune hydrops fetalis nihf comprises the subgroup of cases. In the management of nonimmune hydrops, measurement of fetal mca psv can help identify the subgroup with fetal anaemia. A total of cases of fetal hydrops registered during eightyear study period were. The type depends on the cause of the abnormal fluid. These fluidfilled spaces can occur in different fetal body locations, including.
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