These fluidfilled spaces can occur in different fetal body locations, including. Hydrops fetalis has been a wellrecognized fetal and neonatal condition throughout history. We report a newborn infant with nonimmune hydrops fetalis nihf and congenital. 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. Rh compatibility causes massive red blood cell destruction, which leads to several problems, including. This is the story of our son, who was prenatally diagnosed with congenital chylous ascites and severe hydrops fetalis severe nonimmune hydrops. Nonimmune fetal hydrops is a condition easily diagnosed by antenatal ultrasound.
Congenital syphilis can lead to spontaneous abortion, intrauterine growth retardation, nonimmune hydrops fetalis, stillbirth, prematurity, and perinatal death as well as severe sequelae and even mortality in some liveborn infants 1, 3, 8. Severe fetal anemia and cardiac compromise are important causes of nonimmune hydrops fetalis, and. Etiology and outcome of nonimmune hydrops fetalis in southern. The incidence of fetal hydrops is reported to be 3 to 24 per 10,000 live births.
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. The incidence of nonimmune hydrops fetalis nihf is estimated at 1 in 3000 pregnancies 3. This form of hydrops is uncommon today because of medication available to prevent the condition. Google scholar windebank kp, bridges na, ostmansmith i, stevens je. Alloimmunization and nonimmune hydrops fetalis chapter. 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. Some authors define nihf as edema of the skin plus fluid accumulation in two other compartments of the fetus. Nonimmune hydrops fetalis nonimmune hydrops fetalis diagnosis definition. Hydrops fetalis is the presence of excessive fluid in two or more fetal compartments including skin edema, pleural effusion, pericardial effusion, ascites, and polyhydramnios. Here we report the etiology and outcome of 1004 fetuses with nihf, in a large single maternal and. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization eg, rhd, kell. Nonimmune hydrops can be the symptom of a number of congenital conditions. This allows correct diagnosis of congenital diaphragmatic hernia and evaluation of the consequences on pulmonary growth. Antenatal evaluation and management in nonimmune hydrops fet.
Diagnosis and management of nonimmune hydrops in the newborn. Nonimmune definition of nonimmune by medical dictionary. There are two types of hydrops fetalis, immune and nonimmune. Aetiology, diagnosis and treatment of hydrops foetalis. Pdf on jul 1, 2017, n gayatri and others published nonimmune fetal. Hydrops fetalis erythroblastosis fetalis what is hydrops fetalis. Of note, prenatal cellfree fetal dna testing otherwise termed. Nonimmune hydrops nonimmune hydrops makes up about 75 to 90 percent of all cases of hydrops seen today. Outcomes of infants with nonimmune hydrops fetalis born after 22 weeks gestation our experience between 1982 and 2000. Is the ultrasound definition of fluid collections in non immune hydrops fetalis helpful in defining the underlying cause or predicting outcome. We describe two newborns with noonans syndrome who presented with nonimmune hydrops fetalis that was most likely secondary to a generalized lymphatic vessel dysplasia.
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 nihf is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic techniques, early detection, and individualized management. 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. Fetal thyrotoxicosis manifestation is observed with the transition of tsh receptor stimulating antibodies to the fetus from the 17th20th weeks of pregnancy and with the fetal tsh receptors becoming responsive after 20 weeks. 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. There is no consensus on etiological categories used to classify nihf. Congenital hypothyroidism and nonimmune hydrops fetalis. 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. It is not a specific disease but the end result of many fetal conditions. The subject of this study was 38 cases of nonimmune hydrops fetalis and 11 cases of fetal hydrothorax and or ascites fha, a syndrome characterized by the accumulation of pleural effusion and. Hydrops fetalis, s a severe fetal condition described as the abnormal accumulation of fluid in 2 or more fetal compartments. Nonimmune hydrops fetalis journal of pediatric and neonatal.
Nonimmune hydrops fetalis nihf refers to hydrops in the absence. Hydrops fetalis is a serious condition which indicates poor prognosis for the affected fetus. It may develop because of rh disease in the mother. Hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. Before ultrasound evaluation, all mothers had a complete blood count, titers for toxoplasmosis, rubella, cytomegalovirus, and herpes torch screen, serum albumin, and a kleihauerbeke stain of a peripheral blood smear. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments. The pathophysiology of nihf is complex, and the causes are numerous. Accordingly, transcapillary fluid loss actually seems to be limited by.
Nonimmune hydrops fetalis the foundation for exxcellence in. About half of unborn babies with hydrops fetalis do not survive. Thirtyeight cases of nonimmune hydrops fetalis were evaluated by detailed realtime ultrasonography and twodimensional echocardiography. The classification of immune and nonimmune hydrops foetalis describes the difference between rhesus haemolytic. 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. Nhf accounts for almost 90 percent of current hydropic cases in neonates. Aug 22, 2019 hydrops fetalis is latin for edema of the fetus. 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. Unfortunately, fetal intervention in patients with nonimmune hydrops fetalis does not appear to offer a survival advantage. Aug 07, 2019 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. Nonimmune hydrops fetalis nhf is associated with numerous disorders that include cardiac, pulmonary, infectious, and genetic etiologies. 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.
Nonimmune hydrops fetalis accounts for 76 to 87% of all described cases of hydrops fetalis bellini et al. Ballantyne first described hydrops fetalis in, although this condition had been recognized. Jul 25, 2017 outcomes in fetuses with antenatally diagnosed idiopathic nonimmune hydrops fetalis are similarly dependent on the underlying etiology, with the presence of ascites a prognostic factor for perinatal mortality. Erythroblastosis fetalis and hydrops fetalis springerlink. Just a few of the many known causes of hydrops include. 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. During pregnancy, symptoms of hydrops fetalis may include. Nonimmune hydrops fetalis is a heterogeneous disorder, caused by a large number of underlying pathologic processes. It occurs when another condition or disease interferes with the babys ability to regulate fluid. These result in about 3% of perinatal mortality and 50% diagnosed in utero will result in. In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to rh iso.
Hydrops fetalis is found in about 1 per 2,000 births and is categorized as immune or nonimmune hydrops. Hydrops fetalis is severe swelling edema in an unborn baby or a newborn baby. It is defined as an abnormal collection of fluid in at least two different fetal organ spaces. Congenital chylous ascites and severe nonimmune hydrops. Apr 01, 2016 hydrops is traditionally classified into either immune particularly rhesus rh blood group isoimmunisation or nonimmune hydrops fetalis nihf. A pathologists approach to nonimmune hydrops springerlink. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively. 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. Nonimmune hydrops fetalis is now the most common type of hydrops fetalis. Case report, clinical report by journal of evolution of medical and dental sciences. Provision of a standardized approach to nonimmune fetal hydrops, emphasizing the search for prenatally treatable conditions and recurrent genetic etiologies. Hydrops fetalis is not itself a disease, but an ultrasound marker of other fetal complications. Hydrops is final common path of many fetal complications fetal anemia dx with doppler of mca psv immune hf most often due to maternal alloimm against rhd nonimmune hf with anemia results from fetal blood loss or iu infection nonimmune hf with no anemia may be from fetal chromosomal, structural or rhythm abnormality iu tx sometimes. Hydrops fetalis hf refers to a condition in which an excess of fluid accumulates in fetal soft tissues and serous cavities.
Immune hydrops fetalis is a complication of a severe form of rh incompatibility. The main causes of nonimmune hydrops fetalis are heart or lung problems, severe anemia or genetic or developmental problems. Etiology and outcome of nonimmune hydrops fetalis in. F, ascites in upper abdomen, at level of fetal liver and stomach. 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. Erythroblastosis fetalis, or hemolytic disease of the newborn, is a condition caused by specific antibodies of the mother, directed against red cell antigens of the fetus. Overall, 60% of the cases of nihf are diagnosed prenatally bellini et al av channel, 2015. Test invitae metabolic nonimmune fetal hydrops panel. The rest of this section deals only with nonimmune hydrops fetalis.
Nonimmune hydrops is the presence of two or more abnormal fetal fluid collections in the absence of red cell alloimmunization. With rhogam prophylaxis, the rate of sensitization has been reduced to 1%, according to a worldwide cooperative study. 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. We report a case of a preterm infant with congenital syphilis who presented with nonimmune hydrops fetalis nihf and hepatic dysfunction and was successfully managed with intravenous penicillin g and supportive care.
Nonimmune hydrops fetalis american academy of pediatrics. 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. Almost all observed cases of hf are of the nonimmune type, the causes of which remain undetermined in 15% of patients. Secondary to the decreasing prevalence of immune hydrops, nihf now comprises 90% of all hydrops cases. These are largely rhd antigens, but rare cases of sensitization against other antigens e. The condition is subdivided in to a immune hydrops. Hydrops fetalis genetic and rare diseases information. Nonimmune hydrops fetalis has been reported to be associated with congenital malformations. Unfortunately, in 85% of cases causation cannot be determined. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization. Pdf investigation of nonimmune hydrops fetalis roy. Immune hydrops fetalis is most often a complication of a severe form of rh incompatibility, which can be prevented.
Depending on the severity and cause of hydrops, there may be edema of fetus and placenta, ascites, pleural effusions andor pericardial effusions. This type occurs when the baby has a disease or medical condition that disrupts the bodys ability to manage fluids. Erythroblastosis fetalis causes the mothers white blood cells to attack her babys red blood cells as they would any other foreign invader. Although the incidence of isoimmune hydrops fetalis. 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. It may be manifestation of a variety of underlying disorders. 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. 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. Nonimmune hydrops fetalis nonimmune hydrops fetalis nihf accounts for approximately 90% of all cases. 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 definition hydrops fetalis is a severe fetal condition described as the abnormal accumulation of fluid in 2 or more fetal compartments.
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. The type depends on the cause of the abnormal fluid. Health, general hydrops fetalis care and treatment case studies diagnosis. This is the place where the most difficult challenges are faced head on, where the impossible becomes possible, and where families in search of answers find them. In southeast asia, alphathalassemia is the most common cause of hydrops fetalis, accounting for 60 to 90% of cases. Hydrops fetalis remains an oftendevastating pregnancy complication. Nonimmune hydrops fetalis occurs in approximately one of every 1,000 births. Hydrops fetalis refers to abnormal fluid collections in fetal soft tissues and serous cavities. Examples of conditions that can interfere with the babys fluid management include. Appropriate investigation and management of nonimmune fetal hydrops. Douglas wilson, in fetal medicine third edition, 2020. Education of readers about the causes of nonimmune fetal hydrops and its prenatal counselling and management. Department of significance of 3methoxytyrosine and it.
May 11, 2019 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. 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. Immune hydrops occurs when the mothers immune system attacks and destroys the babys red blood cells due to incompatible maternal and fetal blood types. It occurs when the blood types of the mother and her. 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. For 150 years, families have come from around the corner and across the world, looking to boston childrens for answers. Nonimmune hydrops fetalis in noonans syndrome jama.
Hydrops fetalis hf is defined as the presence of excessive fetal fluid in two or more of the following spaces. 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. However, over 90% of hydrops in the western world is of nonimmune origin. This is a condition in which mother who has rh negative blood type makes antibodies to her babys rh positive blood. The reported incidence of nonimmune fetal hydrops nifh is 1 in 20003000 pregnancies 4, 5. C, placental thickening, with placenta measuring 4 mm in thickness.
Hydrops fetalis, or hydrops, is a condition that occurs when large amounts of fluid build up in a babys tissues and organs causing extreme swelling. Hydrops fetalis is an excess accumulation of fluid in the fetus. Jul 24, 2019 nonimmune hydrops fetalis nihf is a complex condition with a high mortality and morbidity rate. Castillo ra, devore ld, hadi ha, martin s, geist p. For full access to this pdf, sign in to an existing account, or purchase an annual subscription. We report a newborn infant with nonimmune hydrops fetalis nihf and congenital hypothyroidism. Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high. Up to 90% of all cases of hydrops today are nonimmune hydrops. Multiple fetal anatomic and functional disorders can cause nihf. Nonimmune hydrops fetalis and hepatic dysfunction in a. Symptoms of hydrops fetalis can occur during pregnancy or after the baby is born. Fahnenstich h, schmid g, kowalewski s, gembruch u and hansmann m 1989.
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