Hydorp fetalis complete002

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Hydorp fetalis:

Hydorp fetalis นพ. ปวริศร์ วาณิชยเศรษฐ กุล

Hydrop fetalis:

Hydrop fetalis Hydrops fetalis is a condition in the fetus characterized by an abnormal collection of fluid with at least  two of the following: Edema (fluid beneath the skin, more than 5 mm). Ascites (fluid in abdomen) Pleural effusion (fluid in the pleural cavity, the fluid-filled space that surrounds the lungs) Pericardial effusion (fluid in the pericardial sac, covering  that surrounds the heart)

Hydrop fetalis:

Hydrop fetalis Hydrops fetalis is typically diagnosed during ultrasound evaluation for other  complaints such as : Polyhydramnios Size greater than dates Fetal tachycardia Decreased fetal movement Abnormal serum screening Antenatal hemorrhage

Etiology:

Etiology Hydrops fetalis is found in about 1 per 2,000 births and is categorized as : Immune hydrops Nonimmune hydrops

Immune hydrops:

Immune hydrops Accounts for 10-20%of cases Maternal antibodies against red-cells of the fetus cross the placenta and coat fetal red cells which are then destroyed (hemolysis) in the fetal spleen. The severe anemia leads to High-output congestive heart failure. Increased red blood cell production by the spleen and liver leads to hepatic circulatory obstruction (portal hypertension)

Immune hydrops:

Immune hydrops Anti-D, anti-E, and antibodies directed against other Rh antigens comprise the majority of antibodies responsible for hemolytic disease of the newborn . However, there are numerous, less commonly encountered, antibodies such as anti-K (Kell), anti-Fya (Duffy) , and anti-Jka (Kidd)  that may also cause hemolytic disease of the newborn.

Non-immune hydrops:

Non-immune hydrops Accounts for 80 -90% of cases Any other cause besides immune. In general nonimmune hydrops (NIH) is caused by a failure of the interstitial fluid (the liquid between the cells of the body) to return into the venous system .

Non-immune hydrops:

Non-immune hydrops This may due to: Cardiac failure (High output failure from anemia, sacrococcygeal teratoma , fetal adrenal neuroblastoma , etc.) Impaired venous return (Metabolic disorders) Obstruction to normal lymphatic flow (Thoracic malformations) Increased capillary permeability Decreased colloidal osmotic pressure ( Congential nephrosis )

Causes :

Causes Causes can be grouped in 6 broad categories: Cardiovascular genetic abnormalities intrathoracic malformations hematological disorders infectious conditions idiopathic forms

Cardiac causes:

Cardiac causes Structural anomalies Abnormalities of left ventricular outflow Aortic valvular stenosis Aortic valvular atresia Coarctation of the aorta Aortico-left ventricular tunnel Atrioventricular canal Left ventricular aneurysm Truncus arteriosus Hypoplastic left heart Spongiosum heart Endocardial fibroelastosis

Cardiac causes:

Cardiac causes Structural anomalies (cont.) Abnormalities of right ventricular outflow Pulmonary valvular atresia or insufficiency Ebstein anomaly

Cardiac causes:

Cardiac causes Structural anomalies (cont.) Other vascular malformations Arteriovenous malformations Diffuse hemangiomatosis Placental hemangioma Umbilical cord hemangioma Hepatic hemangioendothelioma Abdominal hemangioma Pulmonary arteriovenous fistula Cervical hemangioendothelioma Paratracheal hemangioma Cutaneous cavernous hemangioma Arteriovenous malformations of the brain

Cardiac causes:

Cardiac causes Nonstructural anomalies Obstruction of venous return Superior or inferior vena cava occlusion Absent ductus venosus Umbilical cord torsion or varix Intrathoracic or abdominal tumors or masses Disorders of lymphatic drainage

Cardiac causes:

Cardiac causes Nonstructural anomalies (cont.) Supraventricular tachycardia Congenital heart block Prenatal closure of the foramen ovale or ductus arteriosus Myocarditis Idiopathic arterial calcification or hypercalcemia Intrapericardial teratoma

Hematologic causes :

Hematologic causes Isoimmunization (hemolytic disease of the newborn, erythroblastosis) Rh (most commonly D; also C, c, E, e) Kell (K, k, Kp, Js[B]) ABO MNSs (M, to date) Duffy (Fy b )

Hematologic causes :

Hematologic causes Other hemolytic disorders Glucose phosphate isomerase deficiency (autosomal recessive) Pyruvate kinase deficiency (autosomal recessive) G-6-PD deficiency (X-linked dominant)

Hematologic causes :

Hematologic causes Disorders of red cell production Congenital dyserythropoietic anemia types I and II (autosomal dominant) Diamond-Blackfan syndrome (autosomal dominant) Lethal hereditary spherocytosis (spectrin synthesis defects) (autosomal recessive) Congenital erythropoietic porphyria (Günther disease) (autosomal recessive) Leukemia (usually associated with Down or Noonan syndrome) Alpha-thalassemia (Bart hemoglobinopathy) Parvovirus B19 (B19V)

Hematologic causes :

Hematologic causes Fetal hemorrhage Intracranial or intraventricular Hepatic laceration or subcapsular Placental subchorial Tumors (especially sacrococcygeal teratoma) Fetomaternal hemorrhage Twin-to-twin transfusion Isoimmune fetal thrombocytopenia

Infectious causes:

Infectious causes B19V Cytomegalovirus (CMV) Syphilis Herpes simplex Toxoplasmosis Hepatitis B Adenovirus Ureaplasma urealyticum Coxsackievirus type B Listeria monocytogenes Enterovirus 10 Lymphocytic choriomeningitis virus (LCMV) 11

Inborn errors of metabolism:

Inborn errors of metabolism Glycogen-storage disease, type IV Lysosomal storage diseases Gaucher disease, type II (glucocerebroside deficiency) Morquio disease (mucopolysaccharidosis, type IV-A) Hurler syndrome (mucopolysaccharidosis, type 1H; alpha1–iduronidase deficiency) Sly syndrome (mucopolysaccharidosis, type VII; beta-glucuronidase deficiency Farber disease (disseminated lipogranulomatosis) G M1 gangliosidosis, type I (beta-galactosidase deficiency) Mucolipidosis I I-cell disease (mucolipidosis II) Niemann-Pick disease, type C

Inborn errors of metabolism:

Inborn errors of metabolism Salla disease (infantile sialic acid storage disorder [ISSD] or sialic acid storage disease, neuroaminidase deficiency) Hypothyroidism and hyperthyroidism Carnitine deficiency

Genetic syndromes :

Genetic syndromes Achondrogenesis, type IB (Parenti-Fraccaro syndrome) Achondrogenesis, type II (Langer-Saldino syndrome) Arthrogryposis multiplex congenita, Toriello-Bauserman type Arthrogryposis multiplex congenita, with congenital muscular dystrophy Beemer-Langer (familial short-rib syndrome) Blomstrand chondrodysplasia Caffey disease (infantile cortical hyperostosis; uncertain inheritance) Coffin-Lowry syndrome (X-linked dominant) Cumming syndrome Eagle-Barrett syndrome (prune-belly syndrome; since 97% males, probably X-linked)

Genetic syndromes :

Genetic syndromes Familial perinatal hemochromatosis Fraser syndrome Fryns syndrome Greenberg dysplasia Lethal congenital contracture syndrome Lethal multiple pterygium syndrome (excess of males, so probably X-linked) Lethal short-limbed dwarfism McKusick-Kaufman syndrome Myotonic dystrophy (autosomal dominant) Nemaline myopathy with fetal akinesia sequence Noonan syndrome (autosomal dominant with variable penetrance)

Genetic syndromes :

Genetic syndromes Perlman/familial nephroblastomatosis syndrome (inheritance uncertain) Simpson-Golabi-Behmel syndrome (X-linked [Xp22 or Xp26]) Sjögren syndrome A (uncertain inheritance) Smith-Lemli-Opitz syndrome Tuberous sclerosis (autosomal dominant) Yellow nail dystrophy with lymphedema syndrome (autosomal dominant

Chromosomal syndromes:

Chromosomal syndromes Beckwith-Wiedemann syndrome (trisomy 11p15) Cri-du-chat syndrome (chromosomes 4 and 5) Dehydrated hereditary stomatocytosis (16q23-qter) Opitz G syndrome (5p duplication) Pallister-Killian syndrome (isochrome 12p mosaicism) Trisomy 10, mosaic Trisomy 13 Trisomy 15 Trisomy 18 Trisomy 21 (Down syndrome) Turner syndrome (45, X)

Tumor or mass causes :

Tumor or mass causes Intrathoracic tumors or masses Pericardial teratoma Rhabdomyoma Mediastinal teratoma Cervical vascular hamartoma Pulmonary fibrosarcoma Leiomyosarcoma Pulmonary mesenchymal malformation Lymphangiectasia

Tumor or mass causes :

Tumor or mass causes Intrathoracic tumors or masses (cont.) Bronchopulmonary sequestration Cystic adenomatoid malformation of the lung Upper airway atresia or obstruction (laryngeal or tracheal) Diaphragmatic hernia Eventration of the diaphragm

Tumor or mass causes :

Tumor or mass causes Abdominal tumors or masses Metabolic nephroma Polycystic kidneys Neuroblastoma Hepatic mesenchymal hamartoma Hepatoblastoma Ovarian cyst

Tumor or mass causes :

Tumor or mass causes Other conditions Placental choriocarcinoma Placental chorangioma Cystic hygroma Intussusception Meconium peritonitis Intracranial teratoma Sacrococcygeal teratoma

Pathophysiology :

Pathophysiology In immune hydrops, excessive and prolonged hemolysis causes anemia, which in turn stimulates marked marrow erythroid hyperplasia It also stimulates extramedullary hematopoiesis in the spleen and liver with eventual hepatic dysfunction

Pathophysiology :

Pathophysiology The precise pathophysiology of hydrops remains unknown Theories includes Heart failure form profound anemia and hypoxia Portal hypertension due to hepatic parenchymal disruption caused by extramedullary hemopoiesis Decreased colloid oncotic pressure resulting from liver dysfunction and hypopreteinemia

Pathophysiology :

Pathophysiology The degree and duration of anemia is the major factor causing and influencing the severity of ascites Secondary factors include hypoproteinemia caused by liver dysfunction and capillary endothelial leakage resulting from tissue hypoxia, both of these lead to protein loss and decreased colloid oncotic pressure

Pathophysiology :

Pathophysiology Severe anemia Hepatic extramedullary hematopoeisis Decreased production of plasma proteins Decreased plasma COP

Pathophysiology :

Pathophysiology Congestive heart failure Increased central venous pressure Increased capillary hydrostatic pressure

Pathophysiology :

Pathophysiology Severe tissue hypoxia Endothelial cell damage Capillary leak of fluid & protein

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Decreased COP Increased CVP Capillary leak Increased fluid efflux from intravascular space

Pathophysiology :

Pathophysiology There may be cardiac enlargement and pulmonary hemmorrhage Fluid collects in the fetal thorax, abdominal cavity, or skin The placenta is markedly edematous, enlarge, and boggy. It contains large, prominent cotyledons and edematous villi

Pathophysiology :

Pathophysiology Pleural effusions may be so severe as to restrict lung development, which causes pulmonary compromise after birth Ascites, hepatomegaly, and splenomegaly may lead to severe labor dystocia Severe hydropic changes are easily seen with sonography

Pathophysiology :

Pathophysiology Fetuses with hydrops may die in utero from profound anemia and circulatory failure One sign of severe anemia and impending death is the sinusoidal fetal heart rate pattern Hydrops placental changes leading to placentomegaly can cause preeclampsia

Pathophysiology :

Pathophysiology The liveborn hydropic infant appears pale, edematous, and limp at birth and usually requires resuscitation The spleen and liver are enlarged, and there may be widespread ecchymosis or scattered petechiae Dyspnea and circulatory collapse are common

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Non-immune Hydrops Fetalis

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Placenta of Hydropic Pregnancy Placenta of Normal Pregnancy

Associated complication:

Associated complication Polyhydramnios Placenta abruption Uterine atony Pre-mature labour Hydropic, theickened placenta (> 6 cm) Retained placenta Preeclapsia

Associated complication:

Associated complication In an attempt to compensate for the fetal hypoxia, placenta increases in size and sometimes also penetrate deeper into the myometrium. Thus causes the morbid adherence of placenta and can cause the problems for third stage of labor necessitating the manual removal of Placenta.

Associated complication:

Associated complication Mirror syndrome The mother develops preeclampsia along with severe edema that is similar to that of the fetus Caused by vascular changes in the swollen, hydropic placenta, this likely related to antiangiopenic factors produced by hyperplacentosis

History :

History A history suggesting the presence of any of the following factors should trigger an extensive diagnostic study for hydrops fetalis: Maternal history Rh negative (d;d) blood type Known presence of isoimmune blood group antibodies Prior administration of blood products Risks of illicit drug use

History :

History Maternal history (cont.) Collagen-vascular disease Thyroid disease or diabetes Organ transplant (liver, kidney) Blunt abdominal trauma (abuse, auto accident) Coagulopathy Use of indomethacin, sodium diclofenac, or potentially teratogenic drugs during pregnancy Younger (<16 y) or older (>35 y) maternal age

History :

History Maternal history (cont.) Risk factors for sexually transmitted diseases Hemoglobinopathy (especially with Asian or Mediterranean ethnicity) Occupational exposure to infants or young children Pet cat Current or recent community epidemic of viral illness

History :

History Family history Jaundice in other family members or in previous child Family history of twinning (specifically, monozygotic) Family history of genetic disorders, chromosomal abnormalities, or metabolic diseases Congenital malformation in previous child Previous fetal death

History :

History Family history (cont.) Hydramnios in earlier pregnancies Prior hydrops fetalis Previous fetomaternal transfusion Congenital heart disease in previous child

Physical :

Physical The presence of any of the following maternal or fetal physical findings should prompt further diagnostic evaluation: Twinning Hydramnios Exanthem or other evidence of intercurrent viral illness Herpetic lesion or chancre Decrease in fetal movements

Laboratory Studies:

Laboratory Studies Diagnostic studies may be considered best by temporal grouping (ie, fetal, maternal, placental, neonatal, postmortem). Assessments generally proceed from low-risk noninvasive tests to higher-risk invasive techniques as required for precise and complete diagnosis to properly manage the individual pregnancy.

Maternal laboratory studies :

Maternal laboratory studies Assessment of maternal blood type (red cells) and antibody screen (identification, and quantitation when indicated, of maternal plasma antibodies) Qualitative and quantitative estimates of the proportion of red cells containing fetal hemoglobin in the maternal circulation

Maternal laboratory studies :

Maternal laboratory studies The search for maternal-fetal infection Syphilis serology Antibody screens for common fetal infections toxoplasmosis, other infections, rubella, CMV infection, and herpes simplex [TORCH] Hemoglobin electrophoresis for alpha-thalassemia heterozygosity

Maternal laboratory studies :

Maternal laboratory studies Maternal serum screening tests AFP levels Unconjugated estriol (uE3) Human chorionic gonadotropin levels inhibin-A levels Maternal serum IgG placental alkaline phosphatase levels

Laboratory Studies:

Laboratory Studies Ultrasound - a diagnostic imaging technique which uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.

Laboratory Studies:

Laboratory Studies Level II sonogram with Doppler measurement of the peak systolic velocity (PSV)  in the fetal middle cerebral artery (MCA) to assess for fetal anemia. If there is evidence for anemia or equivocal result obtain: Maternal blood counts and hemoglobin electrophoresis (with hemoglobin DNA analysis), Kleihauer-Betke stain, glucose 6-phosphate dehydrohgenase deficiency screen. Maternal TORCH titers, RPR, listeria, parvovirus B19, coxsackie, adenovirus, and varicella IgG and IgM, as indicated.

Laboratory Studies:

Laboratory Studies Fetal echocardiogram Consider fetal heart rate monitoring for 12 to 24 hours if fetal arrhythmia is suspected. Amniocentesis for fetal karyotype and PCR (polymerase chain reaction) for infections Fetal percutaneous blood sampling for same and in addition fetal liver function; and metabolic testing if indicated.

Laboratory Studies:

Laboratory Studies In the presence of a family history of an inheritable metabolic disorder or recurrent nonimmune hydrops test for : Storage disorders such as Gaucher’s, gangliosidosis, sialidosis, beta-glucuronidase deficiency, and mucopolysaccharidosis Enzyme analysis and carrier testing in parents and/or analysis of fetal or neonatal blood or urine. Histological examination of fetal tissues. Maternal thyroid antibodies

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Normal four Chamber Cardiac View

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Pericardial Effusion Heart

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Body wall edema in a hydropic fetus

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www.thefetus.net Fetal Ascites

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Fetal Ascites

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© www.thefetus.net Fetal Ascites

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© http://www.fetalmedicine.ac.uk/fmu.shtml

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© http://www.fetalmedicine.ac.uk/fmu.shtml

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© http://www.fetalmedicine.ac.uk/fmu.shtml

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Hydrocele can be an early manifestation in hydrops

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Soft Tissue shadow and pleural effusion in hydropic neonate

Treatment:

Treatment Cause Treatment Fetal anemia Fetal blood sampling followed by in utero transfusion Fetal Arrhythmia Medications such as digoxin , sotalol , propanolol , flecainide , amiodarone Intrinsic thoracic malformations Thoracentesis or thoracoamniotic shunt for pleural effusions in select cases Twin to twin transfusion Fetoscopic laser ablation of communicating vessels Syphilis Penicillin

Treatment :

Treatment Transplacental drug therapy Drugs are given to the mother and are passed to the fetus through the placenta The main conditions which respond to this approach are fetal dysrrhythmias (SVT) Once the type of dysrrhythmia is identified, anti-arrhythmic agent is given to the mother, with careful monitoring of her ECG & blood levels. Drugs: Digoxin , Verapamil , Amiadarone , Flecanide . Careful Maternal & Fetal Monitoring is Essential

Treatment :

Treatment Direct fetal drug therapy Maternal administration of drugs may be ineffective due to: Maternal Metabolism Maternal Side Effects Variable Passage Across Placenta Routes for direct fetal drug therapy: Intraperitoneal Intramuscular Intravascular

Treatment :

Treatment Invasive Procedures Blood / Albumin Transfusion to Fetus Intraperitoneal Intravenous Umbilical Vein Hepatic Vein

Treatment :

Treatment Invasive Procedures Drainage Procedures: Large Pleural Effusions Ascities All invasive procedures carry an inherent increased risk of fetal demise or pre-mature labor.

Counseling:

Counseling Long term prognosis depends on underlying cause and severity of the heart failure. If the cause of NIH cannot be determined, the perinatal mortality is approximately 50% Prognosis is much poorer if diagnosed at less than 24 weeks , pleural effusion is present,  or structural abnormalities are present . Pulmonary hypoplasia is a common cause of death in neonates with plerual effusions. Fetal hydrops associated with a structural heart defect is associated with an almost 100% mortality rate.

Counseling:

Counseling If early in pregnancy (less than 24 weeks)  with no treatable cause the option of termination may be a consideration. Recurrence is uncommon unless related to blood group incompatibility (isoimmunization) or inheritable disorder.

Antepartum:

Antepartum Follow up of the fetus will depend on the gestational age of the fetus,  and the mother's wishes regarding intervention. If treatment has been successful or hydrops is resolving spontaneously, the fetus may be followed with repeat sonograms every 1 to 2 weeks and antenatal testing. Patients treated for immune hydrops are usually  delivered at  37 weeks' or when fetal lung maturity has been confirmed.

Antepartum:

Antepartum Consultation with the neonatologist may help to decide when it is appropriate to  proceed with preterm delivery for possible postnatal treatment . The mother should be evaluated frequently for signs of "mirror" syndrome.

Delivery:

Delivery The fetus should be delivered at tertiary care center with neonatologists and other appropriate specialists. There is no evidence that delivery by cesarean section has a marked effect on outcome. Cord blood should be obtained at delivery for hemoglobin concentration and direct Coombs testing

Delivery:

Delivery A postmortem evaluation should be performed in all cases of hydrops that result in neonatal death. One study showed that a combined approach of a thorough antenatal assessment and autopsy may be more likely to determine the cause of non-immune hydrops .

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A hydropic neonate under extensive intensive care

Thank you for your attention:

Thank you for your attention

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