A list of the more common causes is shown in table 1. Nhf accounts for almost 90 percent of current hydropic cases in neonates. Hydrops fetalis is typically defined as the presence of extracellular fluid in at least two fetal body compartments. Hydrops fetalis treatment danafarberboston childrens. Aetiology, diagnosis and treatment of hydrops foetalis. Onethird of hydropic fetuses are discovered incidentally during prenatal sonography in the first or second trimester of gestation. Nonimmune hydrops fetalis nonimmune hydrops fetalis nihf accounts for approximately 90% of all cases.
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. Miscarriage perinatal mortality stillbirth infant mortality neonatal. Hydrops fetalis genetic and rare diseases information. These fluid collections include scalp and body wall edema, pericardial effusion, pleural effusions, and ascites figs. These fluidfilled spaces can occur in different fetal body locations, including. It may develop because of rh disease in the mother. Jul 25, 2017 hydrops fetalis fetal hydrops is a serious fetal condition defined as abnormal accumulation of fluid in 2 or more fetal compartments, including ascites, pleural effusion, pericardial effusion, and skin edema.
Incidence of fetal hydrops is approximately 1 in 2500 to 3500 neonates with incidence coexistence of hydrops and cystic hygroma even rarer. 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. Outcomes of infants with nonimmune hydrops fetalis born after 22 weeks gestation our experience between 1982 and 2000. The diagnosis is made by ultrasound imaging evaluating body cavities, the. Hydrops fetalis an ultrasound showing a fetus with hydrops fetalis hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edemain at least two fetal compartments. Immune hydrops is defined as the diagnosis of fetal hydrops in the presence of a maternal immunologic response to a paternally derived red blood cell antigen in the fetus. Hydrops was diagnosed more frequently while the fetus was alive, before 20 weeks gestation, and associated with chromosome anomaly than found previously. Published on aug view download carlo wa, ambalavanan n.
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. Hydrops fetalis is associated with a large number of pathologies see table 1 that are usuallydividedinto. Nonimmune hydrops is the presence of two or more abnormal fetal fluid collections in the absence of red cell alloimmunization. Nonimmune hydrops fetalis nhf is associated with numerous disorders that include cardiac, pulmonary, infectious, and genetic etiologies. Hydrops fetalis is not itself a disease, but an ultrasound marker of other fetal complications. A cardiac malformation was present in three of these infants. Hydrops fetalisor hydropsis a condition in which large amounts of fluid build up in a babys tissues and organs, causing extensive swelling edema. For all other comments, please send your remarks via fdtalis us. Describe the common causes of nonimmune hydrops fetalis nihf.
Although the incidence of isoimmune hydrops fetalis. Nihf is the result of an increase in interstitial fluid production or, in turn, of an obstruction of lymphatic return. Feb 05, 2014 hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. Although the incidence of isoimmune hydrops fetalis has decreased markedly, nonimmune hydrops fetalis cases have been detalis frequently reported. The condition is subdivided in to a immune hydrops. Hydrops fetalis is an excess accumulation of fluid in the fetus. 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. 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 is excessive extravasation of fluid into the third space in a fetus which could be due to heart failure, volume overload, decreased oncotic pressure. Hydrops fetalis is an excessive fluid accumulation within the fetal extra vascular compartments and body cavities. Prenatal diagnosis of cystic hygroma can be made within 2nd trimester of pregnancy.
Hydrops fetalis is a serious condition which indicates poor prognosis for the affected. Nonimmune hydrops includes all other diseases or complications that may interfere with the babys ability to manage fluid. There are many causes, including both hereditary and acquired diseases. Nonimmune hydrops fetalis nonimmune hydrops fetalis diagnosisdefinition. By comparison, hydrops allantois or hydrops amnion is an accumulation of excessive fluid in the allantoic or amniotic space, respectively. The type depends on the cause of the abnormal fluid. It is defined as an abnormal collection of fluid in at least two different fetal organ spaces. Hydrops fetalis has been a wellrecognized fetal and neonatal condition throughout history. Nihf is the result of an increase in interstitial fluid production or, in. Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic. Investigation and management of nonimmune fetal hydrops. Hydrops fetalis, also known as hydrops, manifests as the accumulation of extravascular fluid within two or more body cavities in utero.
Overall, 60% of the cases of nihf are diagnosed prenatally bellini et al av channel, 2015. Ultrasound evaluation of hydrops fetalis radiology key. Hydrops fetalis is severe swelling edema in an unborn baby or a newborn baby. Hydrops fetalis is a serious condition that indicates poor prognosis for the affected fetus 1.
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 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. Despite improvements in postnatal care such as advanced mechanical ventilation, surfactant treatment, and inhaled nitric oxide, mortality rates are still high in. Incidence of immune hydropsfetalis is significantly decreasing, whereas more and more nonimmune. Nonimmune hydrops fetalis caused by herpes simplex virus type 2 in the setting of recurrent maternal infection. Multiple fetal anatomic and functional disorders can cause nihf. Non immune hydrops fetalis accumulation of extracellular fluid in tissues and 2 or more serous cavities without evidence of circulating antibodies against red cell antigens. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edema, in at least two fetal compartments. Hydrops fetalis it is defined as generalized fetal edema or anasarca, which can be detected by an antenatal usg. Nonimmune hydrops fetalis nihf comprises the subgroup of cases not caused by red cell alloimmunization eg, rhd, kell. 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. Jun 19, 2019 extracorporeal life support for nonimmune hydrops fetalis documents. 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.
Fetal hydrops is further defined as the accumulation of fluid in at least two of the following fetal serous compartments. Nonimmune hydrops fetalis nihf refers specifically to cases of hydrops not caused by red cell alloimmunization. See more ideas about turner syndrome, survival and will turner. Erythroblastosis fetalis and hydrops fetalis springerlink.
Pdf nonimmune hydrops fetalis nihf is a rare fetal condition with a very high mortality in spite of advances in prenatal diagnostic techniques. Hydrops fetalis is found in about 1 per 2,000 births and is categorized as immune or nonimmune hydrops. 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. Managing nonimmune hydrops fetalis linkedin slideshare. Mar, 2014 da silva op, ramanan r, romano w, evans m. 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. Six infants with nonimmune fetal hydrops nifh were found to have trisomy 21 down syndrome.
Improved diagnosis in nonimmune hydrops fetalis using a. The classification of immune and nonimmune hydrops foetalis describes the difference. The incidence of fetal hydrops is reported to be 3 to 24 per 10,000 live births. Inspeksi teknik sri widharto pdf the main differential diagnosis is hydrops fetalis see this term without alphathalassemia, which is a common nonspecific sign of numerous fetal or maternal disorders. Nonimmune hydrops fetalis can be caused by a wide variety of factors. Nonimmune hydrops fetalis american academy of pediatrics. Cystic hygroma associated with nonimmune fetal hydrops. Disease definition hb barts hydrops fetalis is the most severe form of alphathalassemia see this term and is almost always lethal. There are two types of hydrops fetalis, immune and nonimmune. However, over 90% of hydrops in the western world is of nonimmune origin. Anaemia results from maternal isoimmunisation against rhesus or other red cell antigens.
These are largely rhd antigens, but rare cases of sensitization against other antigens e. Nonimmune hydrops fetalis nihf refers to hydrops in the absence of maternal circulating redcell antibodies. 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. Ileus necrotizing enterocolitis meconium peritonitis. Nonimmune hydrops fetalis occurs in approximately one of every 1,000 births.
Delineate the procedures required for ongoing postnatal management of the neonate who has nihf. The antenatal and postnatal aspects ofinvestigation andmanagement ofimmunehydrops are well covered in standard obstetric and. May 11, 2019 hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edemain at least two fetal compartments. The rest of this section deals only with nonimmune hydrops fetalis.
This is a condition in which mother who has rh negative blood type makes antibodies to her babys rh positive blood. Hydrops fetalis is defined as accumulation of fluid in serous cavities and or edema of soft tissues in the fetus. Hydrops fetalis is a serious condition indicating a bad prognosis of affected fetuses. Nonimmune hydrops fetalis associated with angioosteohypertrophy klippeltrenaunay syndrome zeev mor, md, peter schreyer, md, zvi wainraub, md, eliahu hayman, md, and eliahu caspi, md zerifin, israel a case of nonimmune hydrops fetalis in association with angioosteohypertrophy klippeltrenaunay syndrome is reported for the first time. It occurs when abnormal amounts of fluid build up in two or more body areas of a fetus or newborn. Hydrops foetalis is defined as a state of excessive fluid accumulation in the nonimmune hydrops fetalis. About half of unborn babies with hydrops fetalis do not survive. Apr 01, 2016 hydrops is traditionally classified into either immune particularly rhesus rh blood group isoimmunisation or nonimmune hydrops fetalis nihf. Castillo ra, devore ld, hadi ha, martin s, geist p. The main causes of nonimmune hydrops fetalis are heart or lung problems, severe anemia or genetic or developmental problems. Describe the clinical interventions required for in utero management of nihf.
In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to rh iso. In the past, immune hydrops fetalis was caused by rhesus iso. There is no one mechanism to explain nonimmune hydrops. Extracorporeal life support for nonimmune hydrops fetalis documents. Immune hydrops fetalis is no longer very common due to advances in the prevention of hemolytic disease of the newborn. Managing non immune hydrops fetalis dr vidya a thobbi professor and head department of obg alameen medical college bijapur 2. Epidemiology of live born infants with nonimmune hydrops fetalis insights from a populationbased dataset. The aim of this study was to outline the disease etiology in a cohort of fetuses prenatally diagnosed with nonimmune hydrops fetalis nihf. Full text get a printable copy pdf file of the complete article 1. Nonimmune hydrops fetalis is a heterogeneous disorder, caused by a large number of underlying pathologic processes. This type occurs when the baby has a disease or medical condition that disrupts the bodys ability to manage fluids. Hydrops fetalis is a serious condition which indicates poor prognosis for the affected fetus. The manifestations of down syndrome were obscured by the generalized edema at birth.
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. Fast protocol for the diagnosis of lysosomal diseases in nonimmune hydrops fetalis. Pulmonary defects the survival rates in hydrops and pleural effusion treated conservatively is 12%, however survival rate in cases treated with pleuroamniotic shunt is 50%. Mar 29, 2017 stories of loss and survival, resources, support, awareness. Characterize the primary steps in antenatal investigation of nihf. 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. Perinatal and oneyear outcomes of nonimmune hydrops fetalis by etiology and age at diagnosis. Unfortunately, in 85% of cases causation cannot be determined. Immune hydrops fetalis is most often a complication of a severe form of rh incompatibility, which can be prevented. Hydrops may result from various underlying congenital anomalies that cause either increased central vascular pressure, decreased lymph flow, or decreased plasma oncotic pressure, leading to a net imbalance of fluid movement between the. Hydrops fetalis is a condition in the fetus characterized by an accumulation of fluid, or edemain at least two fetal compartments. Ota s, sahara j, mabuchi a, yamamoto r, ishii k, and mitsuda n.
Hydrops fetalis is a serious disorder, usually indicative of an ominous prognosis for the affected fetus. 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. Ballantyne first described hydrops fetalis in, although this condition had been recognized. Hydrops can be divided into two major categories or types. Immune hydrops fetalis is a complication of a severe form of rh incompatibility. 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 cases. The association of down syndrome and nifh emphasizes the need for chromosome analysis in the workup of infants or fetuses with nifh. In some patients, it may also be associated with polyhydramnios and placental edema. Nonimmune hydrops fetalis nihf, a form of hf, is a severe fetal condition defined as the excessive accumulation. After completing this article, readers should be able to. Therefore, severity of hydrops is the main predictor for mortality. Nonimmune hydrops fetalis yurdakok journal of pediatric. Rh compatibility causes massive red blood cell destruction, which leads to several problems, including.
369 1332 1122 993 578 1158 568 601 536 48 729 66 1380 113 685 384 1401 1420 71 373 338 138 1003 609 952 763 310 716 914 63 1253 1365 414 643 340