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  • 1. PROF. M.C.BANSAL . MBBS. MS . MICOG. FICOG. FOUNDR PRINCIPAL & CONTROLLER; JHALAWAR MEDICAL COLLEGE AND HOSPITAL, JHALWAR. EX . PRINCIPAL & CONTROLLER; MAHATMA GANDHI MEDICLA COLLEGE AND HOSPITAL, SITAPURA , JAIPUR.
  • 2. Hydrops Fetalis  It is defined as generalized fetal edema or anasarca , which can be detected by an Antenatal USG.  It may be manifestation of a variety of underlying disorders.  The condition is subdivided in to (a) Immune hydrops . ( b) non immune hydrops. immune variety is more common in developing countries as Rh grouping is not an universal phenomenon . Non immune variety is more common in the developed countries; ratio being reported to be 9: 1 of non immune to immune cases. Rh –Ve persions ---India 5- 10 % 0f population –it is least in Japan < 2% and 15 % in Europe and USA.
  • 3. Development of Rh- D antibodies When Rh +Ve RBCs enters the Rh-Ve mother’s blood circulation; they stimulate the maternal immune response producing RBC antigen – specific antibodies –isoimmunization (sensitization ) . After few weeks , IgM antibodies ( Saline antibodies ) are produced. IgM do not cross the placenta barrier Hence can not cause fetal damage .  On subsequent exposure of fetal Rh D antigen , her previously primed B cells act swiftly to produce IgG ( albumin antibodies ) which can cross placenta ---and bounds to antigen of fetal RBCs causing sequestration and destruction of fetal RBCs.  Antibodies are formed in 16% of the mothers significantly after 6months of larger amount of fetomaternal bleeding and once formed remain in circulation through the life. And each subsequent pregnancy will have booster effect especially when fetus is Rh +Ve fetus is in utero.
  • 4. Schematic Diagram of antibody---Showing heavy , light chains and antigen binding sites
  • 5. Maternal IgG Fetal
  • 6. Immune Hydrops  The commonest cause of immune hydrops fetalis is pregnancy in Rh negetive women with a Rh positive fetus .  Rh negetive mother who is already sensitized by either by a previous pregnancy or blood transfusion, is capable of producing Immunogloblin G.  These Immunoglobin G (IgG) antibodies cross the placenta and destroy (haemolysed ) the Rh positive RBCs of fetus.  This phenomenon was reported by Levine in 1941.
  • 7. Immune Hydrops Fetalis------ First pregnancy proceeds normally as Rh -ve mother is usually not sensitized.  Feto -placental maternal transfusion occurs at the time of placental separation and the Rh +Ve fetal RBCs sensitize t6he maternal immune system.  Other procedures during pregnancy (even in first pregnancy ) can sensitize the mother are feto- maternal blood transfusion--- occuring in amnioscentesis , threatened abortion ,Ectopic pregnancy, MTP,D&E, abruptio placenta, APH , External podalic version, IPV, LSCS, MRP, Chorionic Villus Sampling , PIH , etc.
  • 8. Immune Hydrops--- In subsequent pregnancy the fetal RBCs entering in     maternal circulation may be destroyed /or some of them may have booster effect for maternal sensitization. Maternal Immunoglobins G cross the placenta and destroy fetal RBCs . In mild cases, the fetus has haemolytic disease resulting in anaemia with mild icterus. In severe cases fetus develops hydrops fetalis. If IgG titers are high the disease process may start even in early period of gestation --- resulting in IUFD.
  • 9. Degree of Risk of Developing isoimmunisation according to period of gestation Gestational period % After 1st trimester abortion 2% After MTP ( mid trimester ) 4-5% At term after Delivery—ABO Rh competability ABO incompetable ABO incompatibility confer substantial protection against developing Rh (D) issoimmunisation 16%----2% at the time of delivery , 7% willdevelopanti D antibodie by the 6 months of delivery , another 7% get sensibilized e.g. thy develop anti D slowly and very low titre which becomes detactable and measurable in next prgnancy. 1.5 -2 %
  • 10. Why all babies are not affected in Rh –Ve mother  Volume of fetomaternal trnsfusion may be too low to sensitise.  Immunological non responders ---pregnancy induced suppression of immune system –30%.  ABO Major group incompetability.  Rh set up of the fetal blood may change the Rh – antigenicity ( stimulus )
  • 11. Feto- maternal haemorrhage During Antenatal Period  O.1mlof fetal RBCs are found in 5-15 % of women’s      circulation by 8 weeks of gestation. 75% cases it is always < 1ml . 1% show atleast 5ml fetal RBCs. 0.25% have more than 30ml of fetal RBCs hence only 1.5 % women get sensitised in Antenatal period . It can be prevented by anti D therapy in antenatal period ---at 28 and 36 weeks of gestation and repating it after delivery. If any factor precipitating feto-maternal hemorrhage need immediate anti d therapy in appropriate dose.
  • 12. Prophylaxis---- Universal ABO Rh grouping of all girls / expactant mother.  Premarrital councellig regarding The Rh factor in both life partners --- advising marriage of identical Rh factor.  Rh Immunoglobulin (Rh-IgG) therapy within 24-48 hrs to all Rh –Ve women after ectopic/MTP/ abortion/ delivery /amnioscentasis ,Version , APH bleeding and at 28th and 36th week of gestation in primigravida too..  This therapy was introduced in1966 and its world wide acceptance has remarkably reduced the incidence of immune hydrops in developed countries , but it is still common type of hydrops in developing world.
  • 13. Hydrops fetalis  Occasionally . Immune hydrops may be due to other erythrocyte antigen apart from Rh D antigen.  These include –major A.B.O. incopetability and minor blood group antigens ---C D E, Kell , Jk, S, c , and Duffy.  If issoimmunization occurs , then the titers of antibodies are monitored at appropriate intervals depending on the levels and rapidity with which they are changing ( either every 2 or 4 weeks).
  • 14. Rh Blood system  Rh complex involves genetic expression of 3 separate alleles,      namely C,D,E and small d. Genetic locus of Rh complex is situate on short arm of chromosome 1. D antigen is most potent and is responsible for almost all cases of Rh incompatibility. The potency of immune sensitivity of antigen is in the order of D>d >C> E>e>c. The commonest genotypes are Cede/ cede ,Cede / cede and cede/CDE . Capital D is dominant and express in clinical sympatomatology—whenever present the person is called Rh +Ve, while one having small dd is non effective hence taken as Rh -Ve
  • 15. Homozygous / Heterozygous Rh- Grouping.  Homozygous Rh + Ve = DD ( D on both sets of antigen--- mother and father both Have contributed D &D.  Homologous Rh –ve = dd eg. Each of both small d parents have contributed set of small d( Maternal d and paternal d)  Heterozygous means---Rh +ve when Dd( one logus has D and another logus has d) eg. D has come from father / mother and d has come from Mother / father – both parents have contributed different D and d.
  • 16. Rh +Ve Mother Homogyous Rh +Ve parents Rh +Ve Father DD All children Homogygous +Ve D D DD DD DD DD Both parents are Rh +ve ( DD) hence all 4 children will be Rh +Ve (DD)
  • 17. Rh –Ve mother Rh +Ve Father Homozygous dd Dd DD Dd dD dD All 4 Children will be Rh +Ve ( heterogygous) as D is dominant while d is recessive
  • 18. Rh –ve Mother Rh –Ve father d d d d d d d d d d all 4 children will be Rh -ve d d
  • 19. fetal Rh +ve RBCs enter in Rh-ve maternal circulation---Sensitise maternal immune system ------Mother produce saline (IgM) & albumin (IgG ) antibodies against fetal cells– IgG antibodies cross the placental blood barrier and reach to fetal circulation and haemolyse the fetal cells resulting in HDFN
  • 20. Antibody Formation ( Iso immunization of Rh -ve Mother ) Haemolysis of fetal Rh+Ve RBCs
  • 21. Characteristics of Rh -Immunoglobins  Saline antibodies----  It is IgG .  It is IgM.  Agglutinate with Rh +Ve  Agglutinate Rh +ve RBCs when suspended in saline.  It appear first –before 24 weeks– and then decline  Larger molecule.  Can not cross placenta hence can not cause Haemolytic disease of fetus and new born.     RBCs when suspended in 20% Albumin solution. It appear later the saline antibodies & continue to increase as pregnancy advances. Small Molecule. It crosses the placenta. Causes haemolytic disease of fetus and newborn .
  • 22. Manifestation Of HDFN ( Erythroblastosis Fetalis Depending upon the degree of fetal RBCs haemolysis ---It may be - 1. Hydrops Fetalis ----Fetus is severely affected . . 2. Icterus gravidorum neonatorum -----Relatively less affected. 3.Congenital Anaemia ---of the newborn ; mildly affected . Baby develops anaemia ; jaundice is not so evident or mild , prognosis is good
  • 23. Hydrops Fetalis Severe form of Rh – HDFN. 2. Sever anaemia , generalised edema , ascites, and hydrothorax. 3. High cardiac output. 4. Liver damage due to fetal anoxaemia and acidosis. 5. Liver become the site of hemopoesis to compensate anemia due to haemolysis. 6. Liver fails to produce proteins from aminoacids --hypoproteinaemia ; responsible for generalised edema. 7. Enlargement of placenta due to hyperplasia to meet the demand of o2 transfusion to fetus. 8. USG---scalp edema ,skin edema, pericardial, pleural effusion and ascites. 9. X-ray—buddha position __hallo around scalp—scalp edema .Limbs apart ---due to abdominal distension. 10. Outcome  usually IUFD , sometime live birth but immediate newborn death. 1.
  • 24. Icterus Gravis Neonatorum Lesser form of haemolytic disease than hydrops fetalis . Newborn develops jaundice with in 24 hours after birth . In uterus, most of the unconjugated bilirubin is washed away through the placenta and thus no development of fetal jaundice in utero. After delivery excess of bilirubin is not excreted due to failure of a large amount of bilirubin load by the liver of neonate. When serum bilirubin level rise above 20mg % it crosses the blood brain barrier to deposit in basal ganglia resulting in kernicterus. This baby needs exchange transfusion.
  • 25. Investigations Blood in vials are sent to lab – (a) 3ml oxalate / EDTA blood-- for Hb% and peripheral smear.---Haemolysed RBCs, ghost cells (Maternal cells ) (b) 2ml clotted blood in another vial ---for ABO Rh grouping , direct coomb’s test and serum bilrubin level . DIRECT COOMB’S TEST  it is test to know , is baby is affected by Rh –haemolytic disease or not . If direct coomb’s test is +ve . it means baby is affected and special management is to be done acoording to serum bilirubin level . As mother is already sensitized there is no role of prophylactic anti D therapy . If direct coomb’s test is –ve ---newborn is not affected by Rh –iso immunizationi
  • 26. Investigations (a)INDIRECT COOMB’S TEST  RBC’s of specific or mixed antigen ( KNOWN) are mixed with serum of patient and anti human antiglobulin is added . If agglutination of RBC’s occur ; it means antibodies against Rh factor are present in patient’s blood. Antibodies present in patient’s serum get attached to RBCs and agglutination occurs. Titer 1: 16 means agglutination occurs up to 16 times dilution of patients serum . (b)DIRECT COOMB’S TEST  Anti human anti globulin are added directly to the rocs of baby. Agglutination means anti bodies are bound to the rocs of baby.
  • 27. Immune Hdrops Fetalis
  • 28. 3 D image of fetus with features of immune hydrops
  • 29. Rh –is0 immune –Hyderops ( aNasarca)
  • 30. Non Immune Hydrops Fetalis( USG) Trisomy case
  • 31. Immunoprophylaxis Routine antenatal Rh anti therapy is recommended to Rh negetive women married to Rh+ve partner. 300 ug ,im in deltoid muscle at 28 weeks and 36 weeks of gestation. 1 0 0 u g , i m i s g i v e n a f t e r a n y b l e e d i n g i n 1 st trimester. Higher dose to be given after any precipitating factor or procedure likely to cause feto placental hemorrhage. 300ug anti D is sufficient to neutralize 15 ml of have fetal cell/ 30ml of whole blood.
  • 32. Measurement of fetomaternal Haemorrhage KLEIHAUER- BETKE TEST - Based on acid elution technique. Fetal and maternal rocs have different response to KOH solution. Maternal cells (adult Hb ) get eluded leaving behind only cell membrane and hence appear as swollen round large ―GHOST CELLS‖ against normal fetal cells whose Hb remain unaltered hence look as red refractile round cells . If in 50 low power fields of maternal peripheral blood 80 fetal RBC’s are found- it is estimated that 4ml of fetomaternal hemorrhage has occurred. For 1ml of fetal blood 10ug of Rh anti D is needed. Thus 300ug anti D will be sufficient for 30 ml of fetal blood which has entered the maternal circulation.
  • 33. Materna l Adult Hb Get eluded with
  • 34. Fetal Hb Is resistant to KOH Maternal Adult Hb gets elude by KOH --RBCs –Swollen with fluid
  • 35. Rh –ve(dd) fetus rarely may be exposed to maternal D antigen as result of materno fetal haemorrhage and may become sensitized in their intra uterine life. When such girl at her adult age has pregnancy with Rh +ve fetus , the very first fetus may suffer from HDFN . this mechanism of isoimmunisation is called GRAND MOTHER THEORY .
  • 36. Unique complication of Hydrops Fetalis where mother develops anasarca similar to fetus & PIH.
  • 37. In a previously sensitized / sensibilized women the antibody titer may rise to high level in subsequent pregnancy. This may occur in d-negative fetus also.
  • 38. Non –IMMUNE Hydrops Fetalis causes--• INFECTIONS- Parvovirus B 19, Cytomegalovirus, Toxoplasma, Syphilis, Herpes, Rubella, Leptospirosis. • MATERNAL CAUSE -> Diabetes Mellitus, PIH, Severe Anaemia , Hypopteinaemia . • CHONDROPLASIAS  Thanatophoic dwarfism, Osteogenesis Inperfecta, Achondrogenesis, Hypophosphatasia. • TWIN PREGNANCY  Twin to Twin Transfusion, Acardia Twin . • CONGENITAL TUMORS  CNS, Renal, Genital, GIT, Pulmonary Hepatic & Placental tumors, Sacrococygeal Teratoma, Tuberus sclerosis and Neuroblastoma. • INBORN ERRORS OF METABOLISM --.>Mucopolyscharidosis, Gausher’s disease, Gangliosidosis, Neuramindase deficiency, Fetal Red cell disease -Alpha thalassaemia, Sicle cell, Spherocytosis, Elliptocytosis, and Morquio’s disease.
  • 39. Non immune --Hyudrops fetalis with large nenigo Myelocele in neck Region