All other causes are described as nonimmune hydrops fetalis nihf. Multiple fetal anatomic and functional disorders can cause nihf. In 1943 potter 1 differentiated nonimmune hydrops nih from immune hydrops due to fetalmaternal blood incompatibilities. Nonimmune fetal hydrops has a reported incidence of 1 in 2000 to 3000 pregnancies but accounts for a disproportionate share 3% of overall mortality in the perinatal period. The most ideal approach would be in utero stem cell transplantation before fetal immunological maturation 10. 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. May 11, 2019 treatment with various drugs has generally been unsuccessful, as has fetal surgery for pacing.
Gross fetal hydrops is demonstrated with associated bilateral pleural effusions and ascites. Full text get a printable copy pdf file of the complete article 1. Genetic heterogeneity of hydrops fetalis in southeast asia, alphathalassemia is the most common cause of hydrops fetalis, accounting for 60 to 90% of cases. We here present a case of a fetus of 14 weeks gestational age with diagnosis of large cystic hygroma complicating to fetal hydrops. 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. Consultant obstetrician and fetal medicine consultant, fellow in fetal medicine. Treatment with various drugs has generally been unsuccessful, as has fetal surgery for pacing.
Consultant in fetal medicine and high risk pregnancy care. Fetal ctnt was analyzed on 7 occasions in 5 cases of nonimmune fetal hydrops. Extracorporeal life support for nonimmune hydrops fetalis. Nonimmune hydrops fetalis nihf encompasses all causes of fetal hydrops that are not caused by the passage of maternal antibodies into the fetal compartment. 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. Sonographic evaluation of nuchal edema a finding associated with a variety of fetal abnormalities was first described in the axial plane in the second trimester. Idiopathic hydrops fetalis may represent about half of all cases of hydrops fetalis of nonimmunologic origin i. These fluidfilled spaces can occur in different fetal body locations, including. F, ascites in upper abdomen, at level of fetal liver and stomach. However, over 90% of hydrops in the western world is of nonimmune origin. This clinical practice guideline prepared by the authors, and approved by the society of obstetricians and. Hydrops fetalis treatment danafarberboston childrens.
Immune hydrops fetalis is most often a complication of a severe form of rh incompatibility, which can be prevented. It may be manifestation of a variety of underlying disorders. A list of the more common causes is shown in table 1. Incidence of fetal hydrops is approximately 1 in 2500 to 3500 neonates with incidence coexistence of hydrops and cystic hygroma even rarer.
About 50% of unborn babies with hydrops dont survive. It may develop because of rh disease in the mother. Since the incorporation of rhimmune globulin into antepartum care for rhnegative women, 90% of hydropic cases are nonimmune in origin 2. It is defined as an abnormal collection of fluid in at least two different fetal organ spaces.
Ultrasound diagnosis, management and prognosis in a consecutive series of 27 cases of fetal hydrops following maternal parvovirus b19 infection. Cystic hygroma associated with nonimmune fetal hydrops. Extracorporeal life support for nonimmune hydrops fetalis by john f bealer, p cameron mantor, ladonna wehling, william p tunell, and david w. Hydrops fetalis has been a wellrecognized fetal and neonatal condition throughout history. 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. Fetal hydrops fetal and neonatal pathology, 2nd ed. Depending on the severity and cause of hydrops, there may be edema of fetus and placenta, ascites, pleural effusions andor pericardial effusions. Feb 05, 2014 hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. Jun 24, 2019 we included patients with nonimmune hydrops fetalis. 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. Nonimmune hydrops fetalis occurs in approximately one of every 1,000 births.
Aetiology, diagnosis and treatment of hydrops foetalis. Although access to this website is not restricted, the information found here is intended for use by medical providers. Hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. 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 is a condition in the fetus characterized by an accumulation of fluid, or edemain at least two fetal compartments. The incidence of fetal hydrops is reported to be 3 to 24 per 10,000 live births. 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. These guidelines educate readers about the causes of nonimmune fetal hydrops and its prenatal counselling and management. It occurs when another condition or disease interferes with the babys ability to regulate fluid.
This is a condition in which mother who has rh negative blood type makes antibodies to her babys rh positive blood. The type depends on the cause of the abnormal fluid. Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic techniques. The classification of immune and nonimmune hydrops foetalis describes the difference between rhesus haemolytic disease of the newborn and other aetiologies.
In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to rh iso. The aim of this study was to outline the disease etiology in a cohort of fetuses prenatally diagnosed with nonimmune hydrops fetalis nihf. Noncompaction of the ventricular myocardium, frequently referred to as the left ventricular noncompaction lvnc. Ii2a to evaluate the risk of fetal anemia, doppler measurement of the middle cerebral artery peak systolic velocity should be performed in all hydropic fetuses. Nonimmune hydrops fetalis is now the most common type of hydrops fetalis.
The fetal heart weakens, leading to progressive heart failure with decreased pump efficiency. Since the incorporation of rhimmune globulin into antepartum care for rhnegative women, 90% of hydropic cases are nonimmune in origin 2 definition. Saul snowise, anthony johnson, in obstetric imaging. In 1943 potter 1 differentiated nonimmune hydrops nih from immune hydrops due to fetal maternal blood incompatibilities. Hydrops fetalis, characterized by fluid accumulation in fetal tissues and body cavities, is a nonspecific finding appeared in many different ways. Jun 28, 2019 citescore measures average citations received per document published. Prenatal diagnosis of cystic hygroma can be made within 2nd trimester of pregnancy.
Nihf was first described by edith potter in 1943 as universal edema unassociated with erythroblastosis. 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. In the management of nonimmune hydrops, measurement of fetal mca psv can help identify the subgroup with fetal anaemia. Review of the cases of hydrops foetalis followed up in our hospital from severe swelling can hidtopesia with how the body organs work. Aug 07, 2019 hydrops fetalis caused by alphathalassemia. 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. We included patients with nonimmune hydrops fetalis. There are two types of hydrops fetalis, immune and nonimmune. Hydrops fetalis is severe swelling edema in an unborn baby or a newborn baby.
About half of unborn babies with hydrops fetalis do not survive. Clinical characteristics and perinatal outcome of fetal hydrops. During the postnatal period, cases were followed up until the 40 th day of life, and 12 cases had intrauterine fetal death. 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. In the case of immune hydrops fetalis, a frequent cause is maternofetal incompatibility as in that related to a number of genetic anemias and. Rodriquez mm, chaves f, romaguera rl, ferrer pl, delaguardia c, bruce jh. Examples of conditions that can interfere with the babys fluid management include. Immune hydrops fetalis is a complication of a severe form of rh incompatibility.
Hydrops fetalis is a rare manifestation of severe congestive heart failure in a fetus, resulting in pathological fluid accumulation in fetal soft tissues andor serous cavities. Despite the progresses in therapy, foetal mortality is still very high. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively. The rest of this section deals only with nonimmune hydrops fetalis. Hydrops fetalis is an excess accumulation of fluid in the fetus. Apr 01, 2016 hydrops is traditionally classified into either immune particularly rhesus rh blood group isoimmunisation or nonimmune hydrops fetalis nihf. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization eg, rhd, kell.
Investigation of lysosomal storage diseases in nonimmune hydrops fetalis. Parvovirus b19 is the most common infectious agent associated with hydrops. Hydrops fetalisor hydropsis a condition in which large amounts of fluid build up in a babys tissues and organs, causing extensive swelling edema. In this case no cause for hydrops was found on either clinical evaluation, imaging or autopsy. Hydrops can result from any of diverse etiologies, although it has. The aim of this study was to report the fetal cardiac troponin t ctnt levels in cases of nonimmune fetal hydrops. Investigation and management of nonimmune fetal hydrops. Secondary to the decreasing prevalence of immune hydrops, nihf now comprises 90% of all hydrops cases. Prognostic indicators of the resolution of nonimmune hydrops fetalis and survival of the fetus. Nonimmune fetal hydrops is a condition easily diagnosed by antenatal ultrasound. Mar, 2014 fetal chromosome analysis and genetic microarray molecular testing should be offered where available in all cases of nonimmune fetal hydrops where available. Rh compatibility causes massive red blood cell destruction, which leads to. Hydrops fetalis is found in about 1 per 2,000 births and is categorized as immune or nonimmune hydrops.
Nonimmune hydrops is the presence of two or more abnormal fetal fluid collections in the absence of red cell alloimmunization. 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. Citescore measures average citations received per document published. With the use of anti d prophylaxis immunological causes account for less than 20% cases. Nonimmune hydrops fetalis can be caused by a wide variety of factors. Nonimmune hydrops fetalis is a heterogeneous disorder, caused by a large number of underlying pathologic processes.
Nonimmune hydrops fetalis nonimmune hydrops fetalis nihf accounts for. There are many recent reports indicating that nonimmune hydrops can be an extreme presentation of a number of metabolic disorders, mostly lysosomal storage. Hydrops fetalis is not itself a disease, but an ultrasound marker of other fetal complications. Managing nonimmune hydrops fetalis linkedin slideshare. 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. Mar 29, 2017 stories of loss and survival, resources, support, awareness. Nonimmune hydrops fetalis american academy of pediatrics. Shortterm and longterm outcomes of cases of nonimmune hydrops fetalis. Hydrops fetalis refers to abnormal fluid collections in fetal soft tissues and serous cavities. Nonimmunemediated hydrops can be caused by hemoglobinopathies 2. 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. Seventy percent of all cases of ahf associated with parvo b19 infection occur between 20 and 24 weeks of gestation 11. See more ideas about turner syndrome, survival and will turner.
In approximately 14 of all cases, the cause is not determined. Hydrops develops when too much fluid leaves the babys bloodstream and goes into the tissues. Fetal hydrops is a medical emergency because the hydropic fetus is usually in a precarious state and even minimal delays may prevent access to lifesaving procedures. Investigation for maternalfetal infections, and alphathalassemia in women at risk because of their ethnicity, should be performed in all cases of unexplained fetal hydrops. 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. Symptoms of hydrops fetalis can occur during pregnancy or after the baby is born.
Hydrops fetalis is a descriptive term for generalized edema of the fetus, with fluid accumulation in extravascular components and body cavities. Nonimmune mediated hydrops can be caused by hemoglobinopathies 2. Hydrops fetalis genetic and rare diseases information. This leads to progressive pressure differential between the heart and body, resulting in thirdspacing of fluid in the skin, heart, and lungs. The condition is subdivided in to a immune hydrops. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments. Improved diagnosis in nonimmune hydrops fetalis using a. New therapeutic aspects of nonimmune hydrops fetalis based on 402 prenatally diagnosed cases. Hydrops fetalis is defined as accumulation of fluid in serous cavities and or edema of soft tissues in the fetus. Fetal treatment options for nihf depend on the etiology and the gestational age at diagnosis. Non immune hydrops can result from a large num ber of causes including cardiac and. Nonimmune hydrops fetalis accounts for 76 to 87% of all described cases of hydrops fetalis bellini et al. During pregnancy, symptoms of hydrops fetalis may include. Figure 1 antenatal ultrasound ndings suggestive of fetal hydrops.