Evaluation Of Hydrops Fetalis in pregnancy. ASW.pptx

Hoe to diagnosed anomaly conegnital like hidrops fetalis

1. Background and Definition Fetal hydrops is defined as the pathological accumulation of excess fluid in two or more fetal compartments including ascites, pleural effusion, pericardial effusion Skin edema of the head and body wall (defined as skin thickness greater than 5 mm), polyhydramnios, and placental thickening (≥4 cm in the second trimester and ≥6 cm in the third trimester).

2. FIG 17-1 Scalp edema noted (between arrows) at 32 weeks’ gestation. Cause: idiopathic. ( FIG 17-3 Bilateral pleural effusions ( arrows) at 32 weeks’ gestation. HT, midline fetal heart. Cause: idiopathic FIG 17-2 Cross-sectional view of the fetal thoracic cavity at 19 weeks’ gestation. Note pericardial effusion ( arrow). Also note the cardiomegaly. FIG 17-7 Longitudinal section of the fetal abdomen (fetal head on left) at 19 weeks’ gestation

3. INCIDENCE The reported incidence ranges from 1 : 424 to 1 : 2000 live births admitted to a neonatal intensive care unit (NICU) The widespread use of anti-D immune globulin has led to a decrease in the overall incidence of immune hydrops over the past few decades. The incidence of nonimmune hydrops has been reported to range from 1 : 1500 to 1 : 3800 births

4. Immune Hydrops: Fetal anemia develops from alloimmunization of red blood cells (RBCs) — an Rh-negative mother forms antibodies against Rh-positive red blood cells. The transplacental release of these antibodies can lead to erythroblastosis fetalis. The therapy involves administration of anti-D gamma globulin. FIG 17-10 Relationship between fetal hemoglobin and gestational age. (From Mari G, Deter RL, Carpenter RL, et al: Noninvasive diagnosis by Doppler ultrasonography of fetal anemia due to maternal red-cell alloimmunization. CollaborativeGroupfor Doppler Assessment of the Blood Velocity in Anemic Fetuses

5. IMMUNE HYDROPS Mechanism: Alloimmunization occurs due to exposure to foreign bodies like RhD -positive cells, which ultimately triggers an immunological response. Common causes of this phenomenon include the transplacental entry of fetal cells into the maternal circulation, such as during miscarriage, amniocentesis, trauma, or childbirth. The risk of fetal-maternal hemorrhage increases with pregnancy age: 0.01 mL of fetal blood can be detected in 3% of first-trimester pregnancies and 46% in the third trimester. The likelihood of alloimmunization is directly proportional to the amount of fetal blood crossing the placental barrier. An estimated 0.25 mL of fetal blood is the minimum amount required to cause alloimmunization.

6. After the first exposure to antigen D, a weak immunological response starts during 6 weeks to one year. The dominant immunoglobulin produced in the first interaction is IgM. This large protein does not cross the placenta, and therefore, the first pregnancy is usually not affected. However, a second exposure to the antigen can cause B lymphocytes to differentiate into plasma cells and proliferate. This amnestic response in the immune system is marked by the production of IgG, almost all of which can cross the placental barrier.

7. Process of Fetal Erythropoiesis and Rh Antigen Response: Fetal erythropoiesis begins in the yolk sac on day 21. Rh antigens can be detected as early as day 30 of pregnancy. After the second exposure, there is an increase in the production of IgG antibodies, leading to an elevated maternal antibody titer. These antibodies recognize and bind to foreign antigens on the surface of fetal red blood cells. The ability of IgG antibodies to bind complement is very low, so when they bind, the antibody coats the fetal red blood cells, which are then isolated by macrophages and destroyed by the reticuloendothelial system extracellularly, leading to fetal anemia.

8. As the lifespan of fetal red blood cells shortens, a greater burden arises on the hematopoietic system. Medullary hematopoiesis is stimulated in mild anemia, and recruitment of extramedullary sites occurs only after the anemia progresses to severe. This is evidenced by the fact that in severe disease, both the liver and spleen become enlarged due to congestion and increased extramedullary hematopoiesis, resulting in the release of nucleated red blood cell precursors (erythroblasts) berat .

9. Severe anemia leads to decreased oxygen delivery and hypoxia in terminal organs, causing an increase in cardiac output as a compensatory mechanism. However, this mechanism may not be adequate to maintain sufficient perfusion. Increased biventricular heart diameter and umbilical vein pressure lead to lymphatic flow obstruction  increased extravasation of fluid  resulting in hydrops

10. Management and Prevention Prevention is key. Specific obstetric history and previous transfusions are considered. Initial laboratory tests include ABO and RhD status, antibody screening (Coombs test). Antibody titer levels are used to assess the candidate for prophylaxis and the potential risk to the fetus. Repeat antibody screening is performed between 24 to 28 weeks. If the screening is negative, the candidate is suitable for anti-D prophylaxis.

11. If an RhD negative mother gives birth to an RhD positive baby, a quantitative test ( Kleihauer-Betke staining) is performed to determine the exact amount of anti-D immunoglobulin that needs to be administered. The dose is given within 72 hours after delivery up to 28 days postpartum. Women at risk of RhD alloimmunization should also receive a prophylactic dose of immunoglobulin after any obstetric event or procedure that places them at risk of maternal-fetal hemorrhage.

12. Wanita yang telah tersensitisasi terhadap RhD  pemeriksaan titer antibodi setiap 4 minggu selama trimester pertama dan kedua , setiap 2 minggu selama trimester ketiga untuk menentukan risiko pengembangan penyakit hemolitik Ibu- janin Nilai titer yang ditentukan bervariasi dari 1 : 8 hingga 1 : 32 Tes paternal juga dapat digunakan untuk menentukan risiko perkembangan anemia janin . Ayah dengan RhD positif mempunyai kemungkinan heterozigot . Jika ayah heterozigot , janin mempunyai kemungkinan 50% memiliki RhD negatif . Jika ayah memiliki RhD negatif , janin juga akan memiliki RhD negatif dan oleh karena itu tidak akan berada dalam risiko .

13. PREDICTION OF ANEMIA Cordocentesis Amniocentesis Middle cerebral artery doppler  Pada janin dengan anemia ringan sedang  mekanisme kompensasi ( peningkatan ventrikuler output + stroke volume) + viskositas darah yang encer  peak systolic velosity (PSV) ↑ Cordocentesis, amniocentesis, and middle cerebral artery Doppler are used to assess fetal anemia. Starting from 16-18 weeks of gestation, the peak systolic velocity (PSV) in middle cerebral artery (MCA) can be checked; a higher PSV indicates a compensatory mechanism due to mild to moderate anemia.

14. FIG 17-11 Nomogram for mild anemia (1.29 MoM) and moderate-severe anemia (1.5 MoM). MCA, middle cerebral artery; MoM, multiples of median; zone A values, mild anemia; zone B values, moderate to severe anemia .

15. INTRAUTERINE TRANSFUSION Jarum ukuran 22-G, target  akses vena Jika posisi plasenta di anterior  penusukan langsung pad area insersi tali pusat dapat dilakukan Sampling darah janin untuk Analisa fetal ABO and Rh type, direct Coombs test, dan CBC termasuk reticulocyte dan total bilirubin level. Target hematokrit  40% Prognosis  86%

16. FIG 17-15 Packed red blood cells seen streaming in the fetal umbilical vein ( arrow) at the time of an intravascular transfusion at 26 weeks’ gestation. The cord insertion into the anterior placenta is located at the asterisk.

17. NONIMMUNE HYDROPS NIHF is a heterogeneous condition that can occur due to several causes including  cardiovascular disorders such as arrhythmias, structural heart defects, cardiomyopathy (16% of total cases), chromosomal abnormalities (4% to 12%), and hematological disorders.

18. MECHANISM Tergantung dengan penyakit yang mendasari . Disregulasi dari pergerakan cairan antara vascular dan ruang interstitial yagn disebabkan bisa karena peningkatan produksi cairan interstitial atau penurunan pengembalian aliran cairan limfatik Contoh : Kelainan jantung structural  peningkatan tekanan jantung kanan  peningkatan tekanan vena sentral Tumor/ massa rongga dada  Halangan aliran darah arteri /vena Pengisian diastolic ventrikel yang tidak adekuat pada aritmia Fetal hepatic fibrosis  penurunan fungsi hati dan hypoalbuminemia Nefrosis kongenital  tekanan osmotic menurun Fetal anemia berat karena produksi RBC yang sedikit , perdarahan , destruksi sel darah merah ↑ output pompa jantung meningkat  gagal jantung  gangguan keseimbangan cairan  hydrops Infeksi bakteri atau virus  fetal hepatitis/ miokarditis , Parvovirus b19  supresi sum sum tulang ( infeksi sel eritroid )

19. EVALUATION AND MANAGEMENT Anamnesis: Riwayat infeksi , terpapar toxin, hewan piaraan , perjalanan jauh , konsumsi makanan , penelusuran kekerabatan (autosomal resesif ) Detail anatomi survey Fetal echocardiogram + evaluasi doppler Maternal ABO, rhD antigen, kleihauer betke smear, maternal serologic antibody titer (parvovirus), amniosintesis untuk bacterial/virus

20. TREATMENT Tergantung dari kapan diagnosis ditegakkan dan penyebab NIHF yang disebabkan karena kelainan struktur  prognosis buruk Malformasi jalan nafas & TTTS  fetal intervensi sacrococcygeal teratomas / lesi obstruksi dada  open fetal surgery Anemia berat janin  transfuse intrauterine Parvovirus b19  shunting / paracentesis / torakocentesis

21. PROGNOSIS Bergantung dengan penyebab 31% survival rate jika terdiagnosa sebelum 24 minggu 48% survival rate jika ditegakkan lebih dari 24 minggu Kematian Neonatal: 60% MATERNAL RISKS FROM HYDROPS: komplikasi yang ditandai dengan kombinasi gejala yang menyerupai preeklampsia dengan edema (90%), hipertensi (60%), dan proteinuria (40%). Edema Paru yang dapat terjadi pada (21%)

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