Immune hydrops fetalis is most often a complication of a severe form of rh incompatibility, which can be prevented. Results the median gestational age at diagnosis of fetal hydrops was 14 weeks. Nonimmune hydrops fetalis can be caused by a wide variety of factors. Hydrops fetalis genetic and rare diseases information. Hydrops fetalis is an excessive fluid accumulation within the fetal extra vascular compartments and body cavities. The incidence of fetal hydrops is reported to be 3 to 24 per 10,000 live births. 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. Up to 90% of all cases of hydrops today are nonimmune hydrops. These result in about 3% of perinatal mortality and 50% diagnosed in utero will result in. The rest of this section deals only with nonimmune hydrops fetalis. We report a newborn infant with nonimmune hydrops fetalis nihf and congenital.
Nonimmune hydrops is the presence of two or more abnormal fetal. 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. Nonimmune definition of nonimmune by the free dictionary. Google scholar windebank kp, bridges na, ostmansmith i, stevens je. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments. Investigation and management of nonimmune fetal hydrops. In approximately 14 of all cases, the cause is not determined.
Nonimmune hydrops fetalis american academy of pediatrics. A list of the more common causes is shown in table 1. Scans every 23 weeks to monitor the evolution of hydrops. Nonimmune hydrops fetalis occurs in approximately one of every 1,000 births. Nonimmune hydrops fetalis nonimmune hydrops fetalis diagnosis definition.
The reported incidence of nonimmune fetal hydrops nifh is 1 in 20003000 pregnancies. Hydrops fetalis treatment danafarberboston childrens. Aug 07, 2019 hydrops fetalis caused by alphathalassemia. These findings are consistent with transient myeloproliferative disorder. 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.
Presentation may be prenatal with ultrasound diagnosis on at the time of delivery. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively. Nonimmune fetal hydrops is a clinical phenotype and not a diagnosis. Hydrops can be divided into two major categories or types. Nonimmune hydrops fetalis and congenital disorders of. During pregnancy, symptoms of hydrops fetalis may include. Mar 29, 2017 stories of loss and survival, resources, support, awareness.
Hydrops fetalis is not itself a disease, but an ultrasound marker of other fetal complications. Seminars in perinatology nonimmune hydrops fetalis. Ninety percent are nonimmune not the result of red cell alloimmunization. Hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. Immune hydrops occurs when the mothers immune system attacks and destroys the babys red blood cells due to incompatible maternal and fetal blood types.
However, over 90% of hydrops in the western world is of nonimmune origin. Before the introduction of antenatal rhd rhesus d antigen immune globulin prophylaxis in the 1960s, the most frequent cause of hydrops fetalis was rh. It is not a specific disease but the end result of many fetal conditions. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization. Nonimmune hydrops fetalis nihf comprises the subgroup of cases it seems to us that you have your javascript disabled on your browser. Hydrops fetalis is the presence of excessive fluid in two or more fetal compartments including skin edema, pleural effusion, pericardial effusion, ascites, and polyhydramnios. Outcome of nonimmune hydrops fetalis diagnosed during the fi.
Hydrops fetalis or hydrops was first described by ballantyne in 1892. Javascript is required in order for our site to behave correctly. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization eg, rhd, kell. The most ideal approach would be in utero stem cell transplantation before fetal immunological maturation 10. Rh compatibility causes massive red blood cell destruction, which leads to several problems, including. According to the clinical guideline from the society for maternal fetal medicine 4, hf is defined as the presence of at least two abnormal fluid collections in the fetus, including ascites, pericardial effusion, pleural effusions, and generalized. The other type is nonimmune, which comprises the vast majority of cases today and is the type of hydrops that we are studying. Gross fetal hydrops is demonstrated with associated bilateral pleural effusions and ascites. Etiology and outcome of nonimmune hydrops fetalis in. Hydrops fetalis is an excess accumulation of fluid in the fetus. Nonimmune hydrops fetalis nonimmune hydrops fetalis nihf accounts for.
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. C, placental thickening, with placenta measuring 4 mm in thickness. Nonimmune definition of nonimmune by medical dictionary. There is a risk of maternal morbidity due to the mirror syndrome combination of fetal hydrops with generalized fluid overload and a preeclamptic state in the mother. In november, at 18 weeks pregnant, my husband and i were given the devastating news that our unborn daughter had a fatal illness known as nonimmune fetal hydrops. 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. Jan 01, 2009 hydrops fetalis is the presence of excessive fluid in two or more fetal compartments including skin edema, pleural effusion, pericardial effusion, ascites, and polyhydramnios.
Nonimmune hydrops fetalis is a heterogeneous disorder, caused by a large number of underlying pathologic processes. Pdf congenital hypothyroidism and nonimmune hydrops fetalis. Multiple fetal anatomic and functional disorders can cause nihf. 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. The condition is subdivided in to a immune hydrops. It occurs when abnormal amounts of fluid build up in two or more body areas of a fetus or newborn. 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. This form of hydrops is uncommon today because of medication available to prevent the condition. 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. In this case no cause for hydrops was found on either clinical evaluation, imaging or autopsy.
Aetiology, diagnosis and treatment of hydrops foetalis. 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. Hydrops fetalis hf refers to a condition in which an excess of fluid accumulates in fetal soft tissues and serous cavities 1 3. Investigation and management of nonimmune fetal hydrops jogc. Hydrops fetalis is found in about 1 per 2,000 births and is categorized as immune or nonimmune hydrops. 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. Test invitae metabolic nonimmune fetal hydrops panel. There are two types of hydrops fetalis, immune and nonimmune. Hydrops fetalisor hydrops is a condition in which large amounts of fluid build up in a babys tissues and organs, causing extensive swelling edema. Nonimmune hydrops fetalis nihf, due to causes other than rh alloimmunization. The classification of immune and nonimmune hydrops foetalis describes the difference between rhesus. In the management of nonimmune hydrops, measurement of fetal mca psv can help identify the subgroup with fetal anaemia. Timing and method of delivery depend on the cause of hydrops.
In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to rh iso. The basis of the disorder is an imbalance in the regulation of fetal fluid movement between the vascular and interstitial space. The white blood cell count was normal in one but greatly elevated in the other. About half of unborn babies with hydrops fetalis do not survive. Nonimmune hydrops foetalis is mainly due to foetal causes 8 which includes sacrococcygeal teratoma, fetal adrenal neuroblastoma, thoracic malformations and the conditions associated with nihf includes cardiomyopathy, coarctation of aorta, hypoplastic left heart, heart block, noonan syndrome, turner syndrome, parvovirus, cmv infections, mediastinal tumour, diagphragmatic hernia, fetal sacrococcygeal teratoma, fetal adrenal neuroblastoma, placental tumors, cystic hygromas. Almost all observed cases of hf are of the nonimmune type, the causes of which remain undetermined in 15% of patients. We describe two newborns with noonans syndrome who presented with nonimmune hydrops fetalis that was most likely secondary to a generalized lymphatic vessel dysplasia. Health, general dropsy development and progression edema enzymes genetic disorders infants premature pregnancy premature infants. 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. Diagnosis and management of nonimmune hydrops in the newborn. Symptoms of hydrops fetalis can occur during pregnancy or after the baby is born. Nonimmune hydrops fetalis nihf can be caused by chromosomal abnormalities, single gene disorders, birth defects, infections, and other causes. Feb 05, 2014 hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. We report a newborn infant with nonimmune hydrops fetalis nihf and congenital hypothyroidism.
Objective to evaluate the etiology and outcome of fetal hydrops of nonimmune origin diagnosed in utero during the first half of pregnancy. Hydrops fetalis refers to abnormal fluid collections in fetal soft tissues and serous cavities. The condition occurs when a disease or medical condition affects the bodys ability to manage fluid. 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 hematocrit in both fetuses was markedly decreased. 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. Learn about hydrops fetalis on our designated hydrops fetalis page. Fetal medicine, department of obstetrics and gynecology, sidney kimmel medical college of thomas jefferson university, philadelphia, pennsylvania. Paras advanced center for fetal medicine 2 b, virvijay society, opp.
Oct 27, 2017 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. Nonimmune hydrops fetalis has been reported to be associated with congenital malformations. Since the incorporation of rhimmune globulin into antepartum care for rhnegative women, 90% of hydropic cases are nonimmune in origin 2. Nonimmune hydrops is the presence of two or more abnormal fetal fluid collections in the absence of red cell alloimmunization. Immune hydrops fetalis is a complication of a severe form of rh incompatibility. Introduction hydrops fetalis latin edema of the fetus defined as a collection of fluid in at least two serous compartments is of 2 types immune hydrops and nonimmune hydrops more common 2hale t. 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.
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. It is defined as an abnormal collection of fluid in at least two different fetal organ spaces. It may be manifestation of a variety of underlying disorders. Hydrops fetalis that develop in the absence of red cell hemolysis is termed as nonimmune fetal hydrops nifh and was first described by dr. This leads to progressive pressure differential between the heart and body, resulting in thirdspacing of fluid in the skin, heart, and lungs. 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.
Congenital hypothyroidism and nonimmune hydrops fetalis. See more ideas about turner syndrome, survival and will turner. In 1943 potter 1 differentiated nonimmune hydrops nih from immune hydrops due to fetal maternal blood incompatibilities. Hydrops fetalis has been a wellrecognized fetal and neonatal condition throughout history. Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic techniques. Nonimmune hydrops fetalis and congenital disorders of glycosylation. These fluidfilled spaces can occur in different fetal body locations, including. Hydrops fetalis, characterized by fluid accumulation in fetal tissues and body cavities, is a nonspecific finding appeared in many different ways.
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. The reported incidence of nonimmune fetal hydrops nifh is 1 in 20003000 pregnancies 4, 5. 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. Hydrops is traditionally classified into either immune or non. Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high. Nonimmune hydrops fetalis in noonans syndrome jama. With improved treatment and diagnosis of rhesus isoimmunization, nonimmune factors have become a more frequent cause of hydrops fetalis. Pdf on jul 1, 2017, n gayatri and others published nonimmune fetal. Nonimmune fetal hydrops is diagnosed when there is fluid accumulation in more. 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. The invitae metabolic causes of nonimmune fetal hydrops panel analyzes up to 53 genes that are associated with metabolic conditions that may include a prenatal onset presentation of fetal hydrops hydrops fetalis, edema, or ascites.
Fetal medicine, department of obstetrics, gynecology, and reproductive sciences, university of california, san francisco, san francisco, ca, us. Apr 01, 2016 hydrops is traditionally classified into either immune particularly rhesus rh blood group isoimmunisation or nonimmune hydrops fetalis nihf. Hydrops fetalisor hydropsis a condition in which large amounts of fluid build up in a babys tissues and organs, causing extensive swelling edema. This fetal condition is caused when abnormal amounts of fluid build in two or more body areas of a fetus or newborn. Depending on the severity and cause of hydrops, there may be edema of fetus and placenta, ascites, pleural effusions andor pericardial effusions. Nonimmune fetal hydrops and trisomy 21 may be associated without cardiac or anatomical anomalies.
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