Hydrops fetalis is estimated to occur in 1 per every 1700 pregnancies and is traditionally defined as an abnormal accumulation of fluid in 2 or more fetal serous cavities, including ascites, pleural effusion, pericardial effusion, and skin edema. This is a dangerous situation that causes blood group mismatch between the baby and the mother. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments. Anemia in this setting is often immune mediated owing to rh incompatibility between fetus and mother socalled immune hydrops. Nonimmune hydrops fetalis american academy of pediatrics. Epidemiology of live born infants with nonimmune hydrops fetalis insights from a populationbased dataset. 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 is a serious condition in which abnormal amounts of fluid build up in two or more body areas of a fetus or newborn. Immune hydrops fetalis is no longer very common due to advances in the prevention of hemolytic disease of the newborn. It is a condition where abnormal amounts of fluid build up in two or more body areas causing swelling. The incidence of idiopathic nihf is estimated to be about 18%. Evaluation of hydrops begins with an antibody screen indirect coombs test to verify that it is non immune, 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. Diagnosis and management nonimmune hydrops inthe newborn.
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. An external file that holds a picture, illustration, etc. This enzyme deficiency is the cause of the lysosomal storage disease called mucopolysaccharidosis type vii. Hydrops fetalis is an excessive fluid accumulation within the fetal extra vascular compartments and body cavities. The etiology of immune hydrops is alloimmunization. Hydrops fetalis hf is defined as the presence of excessive fetal fluid in two or more of the following spaces. In another series, this combined approach of a thorough antenatal assessment and necropsy when indicated was able to determine the cause of non immune hydrops in over 90% of cases 10. Non immune hydrops fetalis occurs in approximately one of every 1,000 births. Hydrops fetalis, lysosomal storage disorders, non immune, thalassaemia, ultrasonography introduction the incidence of nonimmune hydrops nih has been.
Etiology and outcome of nonimmune hydrops fetalis in southern. 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. Appropriate investigation and management of non immune fetal hydrops education of readers about the causes of non immune fetal hydrops and its prenatal counselling and management provision of a standardized approach to non immune fetal hydrops, emphasizing the search for prenatally treatable conditions and recurrent genetic etiologies. Provision of a standardized approach to non immune fetal hydrops, emphasizing the search for prenatally treatable conditions and recurrent genetic etiologies. Non immune hydrops is universal edema unassociated with erythroblastosis. Symptoms of hydrops fetalis can occur during pregnancy or after the baby is born. The clinical presentations include ascites, scalp edema, pleural or pericardial effusions. Rh alloimmunization, once the most common cause of hydrops, is now more rare due to routine immunization of rhesus negative mothers. The incidence of fetal hydrops is reported to be 3 to 24 per 10,000 live births. For information on our recommended workup, download the non immune hydrops fetalis workup pdf.
Hydrops fetalis is a serious condition indicating a bad prognosis of affected fetuses. 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 fetalis refers to abnormal fluid collections in fetal soft tissues and serous cavities. Lysosomal storage diseases in non immune hydrops fetalis pregnancies. About half of unborn babies with hydrops fetalis do not survive. Nonimmune hydrops fetalis is a heterogeneous disorder, caused by a large number of underlying pathologic processes. Results for hydrops fetalis 1 10 of 73 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. Non immune hydrops fetalis nihf is a complex condition with a high mortality and morbidity rate. Non immune fetal hydrops is a condition easily diagnosed by antenatal ultrasound. Maternal care for hydrops fetalis, unsp trimester, unsp the 2020 edition of icd10cm o36. Erythroblastosis fetalis is a complicated process that takes place between the babys blood and the mothers blood. The mother was observed with weekly sonograms and nonstress tests three times weekly.
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. The incidence of nonimmune hydrops fetalis nihf is estimated at 1 in 3000 pregnancies 3. Hydrops fetalis is usually separated into immune and nonimmune hydrops nih based on the presence or absence of fetal anemia secondary to red cell alloimmunization, respectively. A wide range of associations have been reported in. This condition is now extremely rare in our experience, less than 1. American journal of medical genetics, 1675, 10821088.
Hydrops fetalis is found in about 1 per 2,000 births and is categorized as immune or nonimmune hydrops. Although first described in association with immune hydrops, many subsequent case descriptions have been described in the literature secondary to nonimmune hydrops fetalis nihf due to a variety of etiologies. This website uses cookies to ensure you get the best experience. Prevalence hydrops fetalis is found in about 1 per 2,000 births. The type depends on the cause of the abnormal fluid. Hydrops is due to several factors leading to accumulation of edema. 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.
Nonimmune fetal hydrops now accounts for 85%90% of cases 24. Investigation and management of nonimmune fetal hydrops. Education of readers about the causes of non immune fetal hydrops and its prenatal counselling and management. All other causes are described as nonimmune hydrops fetalis nihf. Cystic hygromas are single or multiple cysts found mostly in the neck region. Nonimmune hydrops fetalis yurdakok journal of pediatric and. Ultrasound evaluation of hydrops fetalis radiology key. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively.
Prognosis depends on the underlying etiology and chromosomal abnormalities generally tend to have a poor outcome. Nonimmune hydrops fetalis nihf is a complex condition with a high mortality and morbidity rate. Etiology hydrops is a non specific finding in a wide variety of fetal and maternal disorders, including. 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. Rh compatibility causes massive red blood cell destruction, which leads to several problems, including total body swelling.
The documents contained in this web site are presented for information purposes only. Apr 01, 2016 hydrops is traditionally classified into either immune particularly rhesus rh blood group isoimmunisation or non immune hydrops fetalis nihf. Nonimmune fetal hydrops is a condition easily diagnosed by antenatal ultrasound. Causes include rh incompatibility, congenital heart defects, and chromosomal abnormalities. Nonimmune hydrops fetalis directory of open access journals.
Characterization of newborns with nonimmune hydrops fetalis admitted to a neonatal intensive care unit. It may be manifestation of a variety of underlying disorders. Is the ultrasound definition of fluid collections in non immune hydrops fetalis helpful in defining the underlying cause or predicting outcome. Nonimmune hydrops fetalis accounts for 76 to 87% of all described cases of hydrops fetalis bellini et al. Peer comments on this answer and responses from the answerer. 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 chromosomal microarray analysis cma, regardless of whether a structural fetal anomaly.
The fetal non stress test was markedly improved after the procedure. Lysosomal storage disorders lsd are a rare cause of non immunological hydrops fetalis nihf and congenital ascites. Nonimmune hydrops fetalis in a childrens hospital singapore. Hydrops fetalis means generalized edema of the fetus or neonate and most often results from severe anemia during gestation. Incidence of immune hydropsfetalis is significantly decreasing, whereas more and more non immune. Severe swelling can interfere with how the body organs work and often results in death of the infant shortly before or after delivery. In this syndrome, maternal edema, rapid weight gain, mild proteinuria, and hypertension are noted. There are two types of hydrops fetalis, immune and nonimmune. Accounts for a minority 10% of cases in presentday pregnancies 8, although historically accounted for a larger proportion of cases in the early to mid 20 th century. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization eg, rhd, kell.
Hydrops fetalis fetal hydrops is a pathologic condition of excessive accumulation of fluid in at least two extravascular compartments, including fetal soft tissues and body cavities. In southeast asia, alphathalassemia is the most common cause of hydrops fetalis, accounting for 60 to 90% of cases. Non immune hydrops is the presence of two or more abnormal fetal fluid collections in the absence of red cell alloimmunization. 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. Nonimmune hydrops fetalis nihf, a form of hf, is a severe fetal condition defined as the excessive. Pdf lysosomal storage diseases in nonimmune hydrops. A condition characterized by fluid accumulation in two or more anatomic compartments in the fetus. Appropriate investigation and management of non immune fetal hydrops. Investigation and management of non immune fetal hydrops. Non immune hydrops fetalis nihf, due to causes other than rh alloimmunization. In 1943 potter 1 differentiated nonimmune hydrops nih from immune hydrops due to fetalmaternal blood incompatibilities.
Hydrops fetalis or hydrops was first described by ballantyne in 1892. Hydrops fetalis hydrops fetalis hydrops is defined by abnormal accumulation of serous fluid in skin edema and body cavities pericardial, pleural, or ascitic effusions. Underlying diseases associated with development of hydrops are diverse and range from chest occupying lesions that compress the mediastinum to highly vascularized tumors that increase. Multiple fetal anatomic and functional disorders can cause nihf. With the marked decrease in this condition due to prophylaxis with immune globulin, most cases of hydrops fetalis are now caused by other conditions and are known as non immune hydrops. This type is a complication of rh incompatibility, a condition in which the immune system of a mother with rh negative blood attacks her babys rh positive blood cells. The advent of rho d immune globulin resulted in a decline in the incidence of isoimmune fetal hydrops and increasing prominence of nonimmune causes of this severe and highly lethal fetal and.
Most cases of hydrops fetalis are now non immunological. Feb 27, 2016 the lymphatic system is a network of vessels that maintains fluids in the blood, as well as transports fats and immune system cells. Aug 03, 2019 hidrops fetal no inmune pdf pdf files. The incidence of nonimmunological fetal hydrops is between 1 in 1500 and 1 in 4000. It occurs when abnormal amounts of fluid build up in two or more body areas of a fetus or newborn. Onethird of hydropic fetuses are discovered incidentally during prenatal sonography. Rare causes of fetal hydrops need to be excluded in cases of recurrent nonimmune hydrops with no obvious aetiology following routine investigations. Etiology and outcome of nonimmune hydrops fetalis in. With the marked decrease in this condition due to prophylaxis with immune globulin, most cases of hydrops fetalis are now caused by other conditions and are known as nonimmune hydrops.
Shortterm and longterm outcomes of 214 cases of non immune hydrops fetalis. The kudoz network provides a framework for translators and others to assist each other with translations or explanations of terms and short phrases. Efforts from multidisciplinary team including obstetricians, geneticists and neonatologists are required to achieve a. Rare causes of fetal hydrops need to be excluded in cases of recurrent non immune hydrops with no obvious aetiology following routine investigations. Paroxysmal supraventricular tachycardia resulting in heart failure. However, immune hydrops fetalis is not spread in society due to the. Information about the openaccess article non immune hydrops fetalis in doaj. Hydrops fetalis, lysosomal storage disorders, non immune, thalassaemia, ultrasonography introduction the incidence of non immune hydrops nih has been.
Non immune hydrops fetalis is now the most common type of hydrops fetalis. Immune hydrops erythroblastosis fetalis this occurs when the mothers immune system attacks the blood cells of the baby. Congenital chylous ascites and severe non immune hydrops. Since the incorporation of rh immune globulin into antepartum care for rhnegative women, 90% of hydropic cases are non immune in origin 2. Home fetal research hydrops hydrops our research contributes novel information about genetic disorders that cause nonimmune hydrops fetalis. May 17, 2019 hydrops fetalis is a serious condition of the fetus or newborn. Hydrops is classified into two broad categories that include immune and nonimmune hydrops fetalis.
Alloimmunization and nonimmune hydrops fetalis chapter 18. The nonimmune form of hydrops fetalis has many causes including. The condition is subdivided in to a immune hydrops. In the fetus, a cystic hygroma can progress to hydrops an excess. At 30 weeks gestation, a sonogram showed resolving hydrops fetalis, and at 31 weeks gestation, no sign of fetal hydrops remained. Rh compatibility causes massive red blood cell destruction, which leads to several problems, including.
Hydrops can be divided into two major categories or types. Perinatal and oneyear outcomes of non immune hydrops fetalis by etiology and age at diagnosis. In one series the determination of an etiology for non immune hydrops was increased from 50% without to 80% with an autopsy 10. Information for parents your baby has been diagnosed with a condition referred to as hydrops fetalis meaning the abnormal accumulation of fluid in the babys soft tissues, organs, and cavities. Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic. Hydrops fetalis genetic and rare diseases information. This is the story of our son, who was prenatally diagnosed with congenital chylous ascites and severe hydrops fetalis severe non immune hydrops. Pdf on jul 1, 2017, n gayatri and others published nonimmune fetal hydrops. Various structural abnormalities in the fetus may subsequently lead to hydrops and hence require follow up scan in all cases. Lysosomal storage disorder in nonimmunological hydrops. Douglas wilson, in fetal medicine third edition, 2020. Fetal cystic hygroma genetic and rare diseases information. It occurs when another condition or disease interferes with the babys ability to regulate fluid. A systembased approach to the genetic etiologies of non immune hydrops fetalis.
In 1943 potter 1 differentiated non immune hydrops nih from immune hydrops due to fetalmaternal blood incompatibilities. Nonimmune hydrops fetalis nihf refers specifically to cases of hydrops not caused by red cell alloimmunization. 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. Learn about hydrops fetalis on our designated hydrops fetalis page. 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. Non immune hydrops fetalis is defined as the excessive accumulation of fluid in two or more compartments of the fetus in the absence of any maternalfetal blood incompatibility. Feb 05, 2014 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. Non immune hydrops fetalis is a rare clinical entity with varied etiology. Hydrops fetalis secondary to parvovirus b19 infections. 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. Cameronseverity of non immune hydrops fetalis at birth continues to predict survival despite advances in perinatal care fetal diagn ther, 21 2006, pp.
A unique presentation of nonimmune hydrops fetalis in a preterm infant. Immune hydrops fetalis is most often a complication of a severe form of rh incompatibility, which can be prevented. 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. Non immune 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. The main causes of nonimmune hydrops fetalis are heart or lung problems, severe anemia or genetic or developmental problems. During pregnancy, symptoms of hydrops fetalis may include. Cameronseverity of nonimmune hydrops fetalis at birth continues to. The rest of this section deals only with nonimmune hydrops fetalis. Ota s, sahara j, mabuchi a, yamamoto r, ishii k, and mitsuda n. The mothers immune system causes the red blood cells in the fetus to suffer damage. This enables more accurate counseling, individualized antenatal care, and anticipation of neonatal needs. 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. It is the physiological endstage process in a number of fetal conditions and placental pathologies.