Hydrops fetalis, Anämie und Thrombozytopenie

  • Ulrich Gembruch


Als Hydrops fetalis werden fetale Wassereinlagerungen (Aszites, Hydrothorax, Perikarderguss und Hautödem) bezeichnet, die in mindestens zwei fetalen Kompartimenten nachweisbar sind (Abb. 23.1, Abb. 23.2, Abb. 23.3, Abb. 23.4).


  1. Adzick NS (2009) Management of fetal lung lesions. Clin Perinatol 36:363–376CrossRefPubMedGoogle Scholar
  2. Adzick NS (2010) Open fetal surgery for life–threatening fetal anomalies. Semin Fetal Neonatal Med 15:1–8CrossRefPubMedGoogle Scholar
  3. Altarescu G, Geva TE, Grisaru-Granovsky S, Bonstein L, Miskin H, Varshver I, Margalioth EJ, Levy-Lahad E, Renbaum P (2012) Preimplantation genetic diagnosis for fetal neonatal alloimmune thrombocytopenia due to antihuman platelet antigen maternal antibodies. Obstet Gynecol 119:338–343CrossRefPubMedGoogle Scholar
  4. Amann C, Geipel A, Müller A, Heep A, Ritgen J, Stressig R, Kozlowski P, Gembruch U, Berg C (2011) Fetal anemia of unknown cause – a diagnostic challenge. Ultraschall Med 32(Suppl 2):E115–E121Google Scholar
  5. Arinsburg SA, Shaz BH, Westhoff C, Cushing MM (2012) Determination of human platelet antigen typing by molecular methods: Importance in diagnosis and early treatment of neonatal alloimmune thrombocytopenia. Am J Hematol 87:525–528CrossRefPubMedGoogle Scholar
  6. Bellini C, Hennekam RC, Fulcheri E, Rutigliani M, Morcaldi G, Boccardo F, Bonioli E (2009) Etiology of nonimmune hydrops featlis: a systematic review. Am J Med Genet A 149(A):844–851CrossRefGoogle Scholar
  7. Berkowitz RL, Kolb EA, McFarland JG, Wissert M, Primani A, Lesser M, Bussel JB (2006) Parallel randomized trials of risk–based therapy for fetal alloimmune thrombocytopenia. Obstet Gynecol 107:91–96CrossRefPubMedGoogle Scholar
  8. Bertrand G, Drame M, Martageix C, Kaplan C (2011) Prediction of the fetal status in noninvasive management of alloimmune thrombocytopenia. Blood 117:3209–3213CrossRefPubMedGoogle Scholar
  9. Bianchi S, Lista G, Castoldi F, Rustico M (2010) Congenital primary hydrothorax: effect of thoracoamniotic shunting on neonatal clinical outcome. J Matern Fetal Neonatal Med 23:1225–1229CrossRefPubMedGoogle Scholar
  10. Boulot P, Cattaneo A, Taib J, Peray P, Lefort G, Hedon B, Laffargue F, Viala JL (1993) Hematologic values of fetal blood obtained by means of cordocentesis. Fetal Diagn Ther 8:309–316CrossRefPubMedGoogle Scholar
  11. Braun T, Brauer M, Fuchs I, Czernik C, Dudenhausen JW, Henrich W, Sarioglu N (2010) Mirror syndrome: a systematic review of fetal associated conditions, maternal presentation and perinatal outcome. Fetal Diagn Ther 27:191–203CrossRefPubMedGoogle Scholar
  12. Bussel JB, Berkowitz RL, Hung C, Kolb EA, Wissert M, Primiani A, Tsaur FW, Macfarland JG (2010) Intracranial hemorrhage in alloimmune thrombocytopenia: stratified management to prevent recurrence in the subsequent affected fetus. Am J Obstet Gynecol 203:135.e1–135.e14CrossRefGoogle Scholar
  13. Bussel JB, Berkowitz RL, Lynch L, Lesser ML, Paidas MJ, Huang CL, McFarland JG (1996) Antenatal management of alloimmune thrombocytopenia with intravenous gamma-globulin: a randomized trial of the addition of low-dose steroid to intravenous gamma-globulin. Am J Obstet Gynecol 174:1414–1423CrossRefPubMedGoogle Scholar
  14. Bussel JB, Sola-Visner M (2009) Current approaches to the evaluation and management of the fetus and neonate with immune thrombocytopenia. Semin Perinatol 33:35–42CrossRefPubMedGoogle Scholar
  15. Cosmi E, Mari G, Delle Chiaie L, Detti L, Akiyama M, Murphy J, Stefos T, Ferguson 2nd JE, Hunter D, Hsu CD, Abuhamad A, Bahado-Singh R (2002) Noninvasive diagnosis by Doppler ultrasonography of fetal anemia resulting from parvovirus infection. Am J Obstet Gynecol 187:1290–1293CrossRefPubMedGoogle Scholar
  16. Courtier J, Schauer GM, Parer JT, Regenstein AC, Callen PW, Glenn OA (2012) Polymicrogyria in a fetus with human parvovirus B19 infection: a case with radiologic–pathologic correlation. Ultrasound Obstet Gynecol :CrossRefPubMedGoogle Scholar
  17. de Haan TR, van den Akker ES, Porcelijn L, Oepkes D, Kroes AC, Walther FJ (2008) Thrombocytopenia in hydropic fetuses with parvovirus B19 infection: incidence, treatment and correlation with fetal B19 viral load. BJOG 115:76–81CrossRefPubMedGoogle Scholar
  18. de Jong EP, Lindenburg IT, van Klink JM, Oepkes D, van Kamp IL, Walther FJ, Lopriore E (2012) Intrauterine transfusion for parvovirus B19 infection: long-term neurodevelopmental outcome. Am J Obstet Gynecol 206:204.e1–204.e5CrossRefGoogle Scholar
  19. de Jong EP, Walther FJ, Kroes AC, Oepkes D (2011) Parvovirus B19 infection in pregnancy: new insights and management. Prenat Diagn 31:419–425CrossRefPubMedGoogle Scholar
  20. Detti L, Mari G (2003) Noninvasive diagnosis of fetal anemia. Clin Obstet Gynecol 46:923–930CrossRefPubMedGoogle Scholar
  21. Detti L, Mari G, Akiyama M, Cosmi E, Moise Jr KJ, Stefor T, Conaway M, Deter R (2002) Longitudinal assessment of the middle cerebral artery peak systolic velocity in healthy fetuses and in fetuses at risk for anemia. Am J Obstet Gynecol 187:937–939CrossRefPubMedGoogle Scholar
  22. Detti L, Oz U, Guney I, Ferguson JE, Bahado-Singh RO, Mari G (2001) Collaborative Group for Doppler Assessment of the Blood Velocity in Anemic Fetuses: Doppler ultrasound velocimetry for timing the second intrauterine transfusion in fetuses with anemia from red cell alloimmunization. Am J Obstet Gynecol 185:1048–1051CrossRefPubMedGoogle Scholar
  23. Dijkmans AC, de Jong EP, Dijkmans BA, Lopriore E, Vossen A, Walther FJ, Oepkes D (2012) Parvovirus B19 in pregnancy: prenatal diagnosis and management of fetal complications. Curr Opin Obstet Gynecol 24:95–101CrossRefPubMedGoogle Scholar
  24. Gembruch U (2009) Fetal tachyarrhythmia. In: Yagel S, Silverman NH, Gembruch U (Hrsg) Fetal cardiology, 2. Aufl. Informa Healthcare, New York, S. 461–481Google Scholar
  25. Gembruch U, Holzgreve W (2000) The fetus with nonimmune hydrops. In: Harrison MR, Evans MI, Adzick NS, Holzgreve W (Hrsg) The unborn patient: the art and science of fetal therapy, 3. Aufl. WB Saunders, Philadelphia–London–Toronto–Montreal–Sydney–Tokyo, S. 525–582Google Scholar
  26. Gembruch U, Holzgreve W (2009) Cardiac diseases in association with hydrops fetalis. In: Yagel S, Silverman NH, Gembruch U (Hrsg) Fetal cardiology,2. Aufl. Informa Healthcare, New York, S. 483–514Google Scholar
  27. Hellmund A, Berg C, Rösing B, Gembruch U, Geipel A (2012) Masked anemia due to cardiac tamponade in a hydropic fetus caused by placental chorioangioma. Ultrasound Obstet Gynecol 39:479–480CrossRefPubMedGoogle Scholar
  28. Huhta JC (2009) Congestive heart failure in the fetus. In: Yagel S, Silverman NH, Gembruch U (Hrsg) Fetal cardiology,2. Aufl. Informa Healthcare, New York, S. 561–578Google Scholar
  29. Jauniaux E, Pahal G, Gervy C, Gulbis B (2000) Blood biochemistry and endocrinology in the human fetus between 11 and 17 weeks of gestation. Reprod Biomed Online 1:38–44CrossRefPubMedGoogle Scholar
  30. Kamphuis MM, Oepkes D (2011) Fetal and neonatal alloimmune thrombocytopenia: prenatal interventions. Prenat Diagn 31:712–719CrossRefPubMedGoogle Scholar
  31. Kamphuis MM, Paridaans N, Porcelijn L, De Haas M, Van Der Schoot CE, Brand A, Bonsel GJ, Oepkes D (2010) Screening in pregnancy for fetal or neonatal alloimmune thrombocytopenia: systematic review. BJOG 117:1335–1343CrossRefPubMedGoogle Scholar
  32. Kempe A, Rösing B, Berg C, Kamil D, Heep A, Gembruch U, Geipel A (2007) First-trimester treatment of fetal anemia secondary to parvovirus B19 infection. Ultrasound Obstet Gynecol 29:226–228CrossRefPubMedGoogle Scholar
  33. Kohl T, Van de Vondel P, Stressig R, Wartenberg HC, Heep A, Keiner S, Müller A, Franz A, Fröhlich S, Willinek W, Gembruch U (2009) Percutaneous fetoscopic laser decompression of congenital high airway obstruction syndrome (CHAOS) from laryngeal atresia via a single trocar – current technical constraints and potential solutions for future interventions. Fetal Diagn Ther 25:67–71CrossRefPubMedGoogle Scholar
  34. Krapp M, Kohl T, Simpson JM, Sharland GK, Katalinic A, Gembruch U (2003) Review of diagnosis, treatment, and outcome of fetal atrial flutter compared with supraventricular tachycardia. Heart 89:913–917CrossRefPubMedPubMedCentralGoogle Scholar
  35. Lam YH, Tang MH (2002) Middle cerebral artery Doppler study in fetuses with homozygous alpha-thalassaemia-1 at 12–13 weeks of gestation. Prenat Diagn 22:56–58CrossRefPubMedGoogle Scholar
  36. Lamont RF, Sobel JD, Vaisbuch E, Kusanovic JP, Mazaki-Tovi S, Kim SK, Uldbjerg N, Romero R (2011) Parvovirus B19 infection in human pregnancy. BJOG 118:175–186CrossRefPubMedGoogle Scholar
  37. Lee FL, Said N, Grikscheit TC, Shin CE, Llanes A, Chmait RH (2012) Treatment of congenital pulmonary airway malformation induced hydrops fetalis via percutaneous sclerotherapy. Fetal Diagn Ther 31:264–268CrossRefPubMedGoogle Scholar
  38. Leung KY, Cheong KB, Lee CP, Chan V, Lam YH, Tang M (2010) Ultrasonographic prediction of homozygous α0–thalassemia using placental thickness, fetal cardiothoracic ratio and middle cerebral artery Doppler: alone or in combination? Ultrasound Obstet Gynecol 35:149–154CrossRefPubMedGoogle Scholar
  39. Leung TY, Lao TT (2012) Thalassaemia in pregnancy. Best Pract Res. Clin Obstet Gynecol 26:37–51Google Scholar
  40. Lindenburg IT, Smits-Wintjens VE, van Klink JM, Verduin E, van Kamp IL, Walther FJ, Schonewille H, Doxiadis II, Kanhai HH, van Lith JM, van Zwet EW, Oepkes D, Brand A, Lopriore E (2012) LOTUS study group: Long-term neurodevelopmental outcome after intrauterine transfusion for hemolytic disease of the fetus/newborn: the LOTUS study. Am J Obstet Gynecol 206:141.e1–141.e8CrossRefGoogle Scholar
  41. Lindenburg IT, Wolterbeek R, Oepkes D, Klumper FJ, Vandenbussche FP, van Kamp IL (2011) Quality control for intravascular intrauterine transfusion using cumulative sum (CUSUM) analysis for the monitoring of individual performance. Fetal Diagn Ther 29:307–314CrossRefPubMedGoogle Scholar
  42. Loh KC, Jelin E, Hirose S, Feldstein V, Goldstein R, Lee H (2012) Microcystic congenital pulmonary airway malformation with hydrops fetalis: steroids vs open fetal resection. J Pediatr Surg 47:36–39CrossRefPubMedGoogle Scholar
  43. Mari G, Deter RL, Carpenter RL, Rahman F, Zimmerman R, Moise Jr KJ, Dorman KF, Ludomirsky A, Gonzalez R, Gomez R, Oz U, Detti L, Copel JA, Bahado-Singh R, Berry S, Martinez-Poyer J, Blackwell SC (2000) Noninvasive diagnosis by Doppler ultrasonography of fetal anemia due to maternal red-cell alloimmunization. Collaborative Group for Doppler Assessment of the Blood Velocity in Anemic Fetuses. N Engl J Med 342:9–14CrossRefPubMedGoogle Scholar
  44. Mari G, Hanif F, Kruger M, Cosmi E, Santolaya-Forgas J, Treadwell MC (2007) Middle cerebral artery peak systolic velocity: a new Doppler parameter in the assessment of growth-restricted fetuses. Ultrasound Obstet Gynecol 29:310–316CrossRefPubMedGoogle Scholar
  45. Mari G, Zimmermann R, Moise Jr KJ, Deter RL (2005) Correlation between middle cerebral artery peak systolic velocity and fetal hemoglobin after 2 previous intrauterine transfusions. Am J Obstet Gynecol 193:1117–1120CrossRefPubMedGoogle Scholar
  46. Martí-Carvajal AJ, Peña-Martí GE, Comunián-Carrasco G (2009) Medical treatments for idiopathic thrombocytopenic purpura during pregnancy. Cochrane Database Syst Rev 4:CD007722Google Scholar
  47. Merz WM, Kübler K, Fimmers R, Stoffel-Wagner B, Geipel A, Gembruch U (2012) Circulating n-terminal pro-B-type natriuretic peptide in fetal anemia before and after treatment. Pediatr ResGoogle Scholar
  48. Merz WM, Patzwaldt F, Fimmers R, Stoffel-Wagner B, Gembruch U (2012) Dual-colour flow cytometry for the analysis of fetomaternal haemorrhage during delivery. J Clin Pathol 65:186–187CrossRefPubMedGoogle Scholar
  49. Moise Jr KJ (2008) Management of rhesus alloimmunization in pregnancy. Obstet Gynecol 112:164–176CrossRefPubMedGoogle Scholar
  50. Nicolaides KH, Thilaganathan B, Mibashan RS (1989) Cordocentesis in the investigation of fetal erythropoiesis. Am J Obstet Gynecol 161:1197–1200CrossRefPubMedGoogle Scholar
  51. Oepkes D, Seaward PG, Vandenbussche FP, Windrim R, Kingdom J, Beyene J, Kanhai HH, Ohlsson A, Ryan G (2006) DIAMOND Study Group: Doppler ultrasonography versus amniocentesis to predict fetal anemia. N Engl J Med 355:156–164CrossRefPubMedGoogle Scholar
  52. Pacheco LD, Berkowitz RL, Moise Jr KJ, Bussel JB, McFarland JG, Saade GR (2011) Fetal and neonatal alloimmune thrombocytopenia: a management algorithm based on risk stratification. Obstet Gynecol 118:1157–1163CrossRefPubMedGoogle Scholar
  53. Pasman SA, van den Brink CP, Kamping MA, Adama van Scheltema PN, Oepkes D, Vandenbussche FP (2009) Total blood volume is maintained in nonhydropic fetuses with severe hemolytic anemia. Fetal Diagn Ther 26:10–15CrossRefPubMedGoogle Scholar
  54. Picklesimer AH, Oepkes D, Moise Jr KJ, Kush ML, Weiner CP, Harman CR, Baschat AA (2007) Determinants of the middle cerebral artery peak systolic velocity in the human fetus. Am J Obstet Gynecol 197:526.e1–526.e4CrossRefGoogle Scholar
  55. Pistorius LR, Smal J, de Haan TR, Page-Christiaens GC, Verboon-Maciolek M, Oepkes D, de Vries LS (2008) Disturbance of cerebral neuronal migration following congenital parvovirus B19 infection. Fetal Diagn Ther 24:491–494CrossRefPubMedGoogle Scholar
  56. Porcelijn L, Van den Akker ES, Oepkes D (2008) Fetal thrombocytopenia. Semin Fetal Neonatal Med 13:223–230CrossRefPubMedGoogle Scholar
  57. Rayment R, Brunskill SJ, Soothill PW, Roberts DJ, Bussel JB, Murphy MF (2011) Antenatal interventions for fetomaternal alloimmune thrombocytopenia. Cochrane Database Syst Rev 5:CD004226Google Scholar
  58. Ruano R, Ramalho AS, Cardoso AK, Moise Jr K, Zugaib M (2011) Prenatal diagnosis and natural history of fetuses presenting with pleural effusion. Prenat Diagn 31:496–499CrossRefPubMedGoogle Scholar
  59. Scheffer PG, Ait Soussan A, Verhagen OJ, Page-Christiaens GC, Oepkes D, de Haas M, van der Schoot CE (2011) Noninvasive fetal genotyping of human platelet antigen-1a. BJOG 118:1392–1395CrossRefPubMedGoogle Scholar
  60. Scheffer PG, van der Schoot CE, Page-Christiaens GC, de Haas M (2011) Noninvasive fetal blood group genotyping of rhesus D, c, E and of K in alloimmunised pregnant women: evaluation of a 7-year clinical experience. BJOG 118:1340–1348CrossRefPubMedGoogle Scholar
  61. Scheier M, Hernandez-Andrade E, Fonseca EB, Nicolaides KH (2006) Prediction of severe fetal anemia in red blood cell alloimmunization after previous intrauterine transfusions. Am J Obstet Gynecol 195:1550–1556CrossRefPubMedGoogle Scholar
  62. Schonewille H, Klumper FJ, van de Watering LM, Kanhai HH, Brand A (2007) High additional maternal red cell alloimmunization after Rhesus- and K-matched intrauterine intravascular transfusions for hemolytic disease of the fetus. Am J Obstet Gynecol 196:143.e1–143.e6CrossRefGoogle Scholar
  63. Serrarens-Janssen VM, Semmekrot BA, Novotny VM, Porcelijn L, Lotgering FK, Delemarre FM, Steegers EA (2008) Fetal/neonatal allo-immune thrombocytopenia (FNAIT): past, present, and future. Obstet Gynecol Surv 63:239–252CrossRefPubMedGoogle Scholar
  64. Smits-Wintjens VE, Rath ME, Lindenburg IT, Oepkes D, van Zwet EW, Walther FJ, Lopriore E (2012) Cholestasis in neonates with red cell alloimmune hemolytic disease: incidence, risk factors and outcome. Neonatology 101:306–310CrossRefPubMedGoogle Scholar
  65. Swartz AE, Ruma MS, Kim E, Herring AH, Menard MK, Moise Jr. KJ (2009) The effect of fetal heart rate on the peak systolic velocity of the fetal middle cerebral artery. Obstet Gynecol 113:1225–1229CrossRefPubMedPubMedCentralGoogle Scholar
  66. Takagi K, Tanaka H, Nishijima S, Masaoka N, Miyake Y, Sakata H, Satoh K (1989) Fetal blood values by percutaneous umbilical blood sampling. Fetal Ther 4:152–160CrossRefPubMedGoogle Scholar
  67. Tiblad E, Kublickas M, Ajne G, Bui TH, Ek S, Karlsson A, Wikman A, Westgren M (2011) Procedure-related complications and perinatal outcome after intrauterine transfusions in red cell alloimmunization in Stockholm. Fetal Diagn Ther 30:266–273CrossRefPubMedGoogle Scholar
  68. van den Akker ES, de Haan TR, Lopriore E, Brand A, Kanhai HH, Oepkes D (2008) Severe fetal thrombocytopenia in Rhesus D alloimmunized pregnancies. Am J Obstet Gynecol 199:387.e1–387.e4CrossRefGoogle Scholar
  69. van der Schoot CE, Tax GH, Rijnders RJ, de Haas M, Christiaens GC (2003) Prenatal typing of Rh and Kell blood group system antigens: the edge of a watershed. Transfus Med Rev 17:31–44CrossRefPubMedGoogle Scholar
  70. van Dongen H, Klumper FJ, Sikkel E, Vandenbussche FP, Oepkes D (2005) Non-invasive tests to predict fetal anemia in Kell-alloimmunized pregnancies. Ultrasound Obstet Gynecol 25:341–315CrossRefPubMedGoogle Scholar
  71. van Kamp IL, Klumper FJ, Oepkes D, Meerman RH, Scherjon SA, Vandenbussche FP, Kanhai HH (2005) Complications of intrauterine intravascular transfusion for fetal anemia due to maternal red-cell alloimmunization. Am J Obstet Gynecol 192:171–177CrossRefPubMedGoogle Scholar
  72. Vandenbussche FPHA, Brand A, Kanhai HHH (2011) Fetal thrombocytopenia. In: James D, Steer PJ, Weiner CP, Gonik B, Crowther CA, Robson SC (Hrsg) High risk pregnancy: management options,4. Aufl. Saunders, St. Louis, S. 229–237CrossRefGoogle Scholar
  73. Vogel M, McElhinney DB, Wilkins-Haug LE, Marshall AC, Benson CB, Juraszek AL, Silva V, Lock JE, Marx GR, Tworetzky W (2011) Aortic stenosis and severe mitral regurgitation in the fetus resulting in giant left atrium and hydrops: pathophysiology, outcomes, and preliminary experience with pre-natal cardiac intervention. J Am Coll Cardiol 57:348–355CrossRefPubMedGoogle Scholar
  74. Weiner CP (2011) Fetal hemolytic disease. In: James D, Steer PJ, Weiner CP, Gonik B, Cowther CA, Robson SC (Hrsg) High risk pregnancy: management options, 4. Aufl. Saunders, St. Louis, S. 209–227CrossRefGoogle Scholar
  75. Witlox RS, Lopriore E, Oepkes D, Walther FJ (2011) Neonatal outcome after prenatal interventions for congenital lung lesions. Early Hum Dev 87:611–618CrossRefPubMedGoogle Scholar
  76. Wylie BJ, D’Alton ME (2010) Fetomaternal hemorrhage. Obstet Gynecol 115:1039–1051CrossRefPubMedGoogle Scholar
  77. Yang YS, Ma GC, Shih JC, Chen CP, Chou CH, Yeh KT, Kuo SJ, Chen TH, Hwu WL, Lee TH, Chen M (2012) Experimental treatment of bilateral fetal chylothorax using in-utero pleurodesis. Ultrasound Obstet Gynecol 39:56–62CrossRefPubMedGoogle Scholar
  78. Yinon Y, Grisaru-Granovsky S, Chaddha V, Windrim R, Seaward PG, Kelly EN, Beresovska O, Ryan G (2010) Perinatal outcome following fetal chest shunt insertion for pleural effusion. Ultrasound Obstet Gynecol 36:58–64CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Ulrich Gembruch
    • 1
  1. 1.Abt. für Geburtshilfe und Pränatale MedizinUniversitätsklinikum Bonn, Zentrum für Geburtshilfe und FrauenheilkundeBonnDeutschland

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