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Fetal Hydrops

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Abstract

Hydrops fetalis, meaning “edema of the fetus,” is a clinicopathological term describing an end-stage status of fetal fluid imbalance, and the diagnostic criteria include generalized skin edema with one or more serous cavity effusions. The underlying causes include various maternal, fetal, and placental conditions. Even if the underlying forces to edema formation are the same as has been described in adults (Starling forces), the unique features of fetal physiology, including characteristics of fetal microcirculation and immaturity of the developing organs, predispose to fetal edema formation. This chapter aims to provide some insight into the underlying pathophysiological mechanisms and should help to rationalize and categories the myriad conditions that may give rise to fetal hydrops. A better understanding of the various processes at work in the generation of hydrops provides more information for parental counseling and for more targeted antepartum/perinatal treatments. The importance of postmortem examination, including ancillary investigations, cannot be emphasized enough. Recent publications highlight the usefulness of immunohistochemical assessment of lymphatic vessels in idiopathic cases. It has been also shown that, when postmortem examinations are completed along with clinical assessments, the underlying causes can be found in very high percentage of the cases (>90 %).

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References

  1. Bellini C, Hennekam RCM, Fulcheri E, Rutigliani M, Morcaldi G, Boccardo F, Bonioli E. Etiology of nonimmune hydrops fetalis: a systemic review. Am J Med Genet A. 2009;149A:844–51.

    PubMed  Google Scholar 

  2. Bellini C, Hennekam RC, Boccardo F, Campisi C, Serra G, Bonioli E. Nonimmune idiopathic hydrops fetalis and congenital lymphatic dysplasia. Am J Med Genet A. 2006;140:678–84.

    PubMed  Google Scholar 

  3. Bellini C, Fulcheri E, Rutigliani M, Calevo MG, Boccardo F, Campisi C, Bonioli E, Bellini T, Hennekam RC. Immunohistochemistry in non-immune hydrops fetalis: a single center experience in 79 fetuses. Am J Med Genet A. 2010;152A:1189–96.

    PubMed  Google Scholar 

  4. Rodriguez MM, Chaves F, Romaguera RL, Ferrer PL, de la Guardia C, Bruce JH. Value of autopsy in nonimmune hydrops fetalis: series of 51 stillborn fetuses. Pediatr Dev Pathol. 2002;5:365–74.

    PubMed  Google Scholar 

  5. Sohan K, Carrol SG, Sergio DLF, Soothill P, Kyle P. Analysis of outcome in hydrops fetalis in relation to gestational age at diagnosis, cause and treatment. Acta Obstet Gynecol Scand. 2001;80:726–30.

    CAS  PubMed  Google Scholar 

  6. Yang YH, Teng RJ, Tang JR, Yau KI, Huang LH, Hsieh FJ. Etiology and outcome of hydrops fetalis. J Formos Med Assoc. 1998;97:16–20.

    CAS  PubMed  Google Scholar 

  7. Suwanrath-Kengpol C, Kor-anantakul O, Suntharasaj T, Leetanaporn R. Etiology and outcome of non-immune hydrops fetalis in southern Thailand. Gynecol Obstet Invest. 2005;59:134–7.

    PubMed  Google Scholar 

  8. Liao C, Wei J, Li Q, Li J, Li L, Li D. Nonimmune hydrops fetalis diagnosed during the second half of pregnancy in Southern China. Fetal Diagn Ther. 2007;22:302–5.

    PubMed  Google Scholar 

  9. Taweevisit M, Thorner PS. Hydrops fetalis in the stillborn: a series from the central region of Thailand. Pediatr Dev Pathol. 2010;13:369–74.

    PubMed  Google Scholar 

  10. Moreno CA, Kanazawa T, Barini R, Nomura ML, Andrade KC, Gomes CP, et al. Non-immune hydrops fetalis: a prospective study of 53 cases. Am J Med Genet A. 2013;161A:3078–86.

    PubMed  Google Scholar 

  11. Hutchinson AA, Drew JH, Yu VYH, Williams ML, Fortune DW, Beischer NA. Nonimmunologic hydrops fetalis: a review of 61 cases. Obstet Gynecol. 1982;59:347–52.

    Google Scholar 

  12. Trainor B, Tubman R. The emerging pattern of hydrops fetalis – incidence, aetiology and management. Ulster Med J. 2006;75:185–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  13. Takci S, Gharibzadeh M, Yurdakok M, Ozyuncu O, Korkmaz A, Akcoren A, et al. Etiology and outcome of hydrops fetalis: report of 62 cases. Pediatr Neonatol. 2014;55:108–13.

    PubMed  Google Scholar 

  14. Heinonen S, Ryynanen M, Kirkinen P. Etiology and outcome of second trimester non-immunologic fetal hydrops. Acta Obstet Gynecol Scand. 2000;79:15–8.

    CAS  PubMed  Google Scholar 

  15. Fukushima K, Morokuma S, Fujita Y, Tsukimori K, Satoh S, Ochiai M, et al. Short – term and long – term outcome of 214 cases of non-immune hydrops fetalis. Early Hum Dev. 2011;87:571–5.

    PubMed  Google Scholar 

  16. Santo S, Mansour S, Thilaganathan B, Homfray T, Papageorgiou A, Calvert S, et al. Prenatal diagnosis of non-immune hydrops fetalis: what do we tell the patients? Prenat Diagn. 2011;31:186–95.

    PubMed  Google Scholar 

  17. Ng ZM, Seet MJ, Erng MN, Buendia F, Chang AS, Sriram B. Nonimmune hydrops fetalis in a children’s hospital: a six-year series. Singapore Med J. 2013;54:487–90.

    PubMed  Google Scholar 

  18. Abrams ME, Meredith KS, Kinnard P, Clark RH. Hydrops fetalis: a retrospective review of cases reported to a large national database and identification of risk factors associated with death. Pediatrics. 2007;120:84–9.

    PubMed  Google Scholar 

  19. Haverkamp F, Noeker M, Gerresheim G, Fahnenstich H. Good prognosis for psychomotor development in survivors with nonimmune hydrops fetalis. BJOG. 2000;107:282–4.

    CAS  PubMed  Google Scholar 

  20. Shulman LP, Phillips OP, Emerson DS, Felker RE, Tharapel AT. Fetal space – suit hydrops in the first trimester: differentiating risk for chromosomal abnormalities by delineating characteristics of nuchal translucency. Prenat Diagn. 2000;20:30–2.

    CAS  PubMed  Google Scholar 

  21. Bauer K, Brace RA, Stonestreet BS. Fluid and electrolyte metabolism. In: Polin RA, Fox WW, Abam SH, editors. Fetal and neonatal physiology. Philadelphia: Elsevier Saunders; 2011. p. 1436–42.

    Google Scholar 

  22. Désilets V, Audibert F, Society of Obstetrician and Gynaecologists of Canada. Investigation and management of non-immune fetal hydrops. J Obstet Gynecol Can. 2013;35:923–38.

    PubMed  Google Scholar 

  23. Apkon M. Pathophysiology of hydrops fetalis. Semin Perinatol. 1995;19:437–46.

    CAS  PubMed  Google Scholar 

  24. Bellini C, Hennekam RCM. Non-immune hydrops fetalis: a short review of etiology and pathophysiology. Am J Med Genet A. 2012;158A:597–605.

    PubMed  Google Scholar 

  25. Brace RA. Effects of outflow pressure on fetal lymph flow. Am J Obstet Gynecol. 1989;160:494–7.

    CAS  PubMed  Google Scholar 

  26. Brace RA, Wolf EJ. Normal amniotic fluid volume changes throughout pregnancy. Am J Obstet Gynecol. 1989;161:382–8.

    CAS  PubMed  Google Scholar 

  27. Sha X, Xiong Z, Liu H, Di X, Ma T. Maternal-fetal fluid balance and aquaporins: from molecule to physiology. Acta Pharmacol Sin. 2011;32:716–20.

    CAS  PubMed  PubMed Central  Google Scholar 

  28. Guan J, Mao C, Feng X, Zhang H, Xu F, Geng C, et al. Fetal development of regulatory mechanisms for body fluid homeostasis. Braz J Med Biol Res. 2008;41:446–56.

    CAS  PubMed  Google Scholar 

  29. Faber J, Anderson D. The placenta in the integrated physiology of fetal volume control. Int J Dev Biol. 2010;54:391–6.

    PubMed  PubMed Central  Google Scholar 

  30. Miner LK, Brace A, Cheung CY. Temporal relationship among fetal urine flow, ANF and AVP responses to hypertonic infusions. Am J Physiol. 1990;258:469–75.

    Google Scholar 

  31. Page KR, Abramovich D, Garden AS, Jandial L. Solute levels in uterine fluids of patients with normal values of amniotic fluid and with hydramnios. Eur J Obstet Gynecol Reprod Biol. 1978;8:287–93.

    CAS  Google Scholar 

  32. Underwood MA, Gilbert WM, Sherman MP. Amniotic fluid: not just fetal urine anymore. J Perinatol. 2005;25:341–8.

    PubMed  Google Scholar 

  33. Harding R. Fetal lung fluid. In: Brace RA, Ross MG, Robillard JE, editors. Fetal and neonatal body fluids: the scientific basis for clinical practice. Ithaca: Perinatology Press; 1989. p. p42–64.

    Google Scholar 

  34. Brace RA, Vermin ML, Huijssoon E. Regulation of amniotic fluid volume: intramembranous solute and volume fluxes in late gestation fetal sheep. Am J Obstet Gynecol. 2004;191:837–46.

    PubMed  Google Scholar 

  35. Brace RA. Current topics: progress toward understanding the regulation of amniotic fluid volume: water and solute fluxes in and through the fetal membranes. Placenta. 1995;16:1–18.

    CAS  PubMed  Google Scholar 

  36. Sherer DM. A review of amniotic fluid dynamics and the enigma of isolated oligohydramnios. Am J Perinatol. 2002;19:253–66.

    PubMed  Google Scholar 

  37. Cheung CT. Vascular endothelial growth factor activation of intramembranous absorption: a critical pathway for amniotic fluid volume regulation. J Soc Gynecol Investig. 2004;11:63–74.

    CAS  PubMed  Google Scholar 

  38. Liu H, Zheng Z, Wintour EM. Aquaporins and fetal fluid balance. Placenta. 2008;29:840–7.

    CAS  PubMed  Google Scholar 

  39. Damiano AE. Review: water channel proteins in the human placenta and fetal membranes. Placenta. 2011;32:S207–11. Supplement B. trophoblast research, 25.

    PubMed  Google Scholar 

  40. Kleinman CS, Donnerstein RL, de Vore GR, et al. Fetal echocardiography for evaluation of in utero congestive heart failure: a technique for study of nonimmune fetal hydrops. N Engl J Med. 1982;306:568–75.

    CAS  PubMed  Google Scholar 

  41. Keeling JW, Gough DJ, Iliff P. The pathology of non-rhesus hydrops. Diagn Histopathol. 1983;6:89–111.

    CAS  PubMed  Google Scholar 

  42. Holzgreve W, Curry CJR, Golbus MS, Callen PW, Filly RA, Smith JC. Investigation of nonimmune hydrops fetalis. Am J Obstet Gynecol. 1984;150:805–12.

    CAS  PubMed  Google Scholar 

  43. Im SS, Rizos N, Joutsi P, Shime J, Benzie JR. Nonimmunologic hydrops fetalis. Am J Obstet Gynecol. 1984;148:566–9.

    CAS  PubMed  Google Scholar 

  44. Allan LD, Crawford DC, Sheridan R, Chapman MG. Aetiology of nonimmune hydrops: the value of echocardiography. Br J Obstet Gynaecol. 1986;93:223–5.

    CAS  PubMed  Google Scholar 

  45. Schmid G, Fahnenstich H, Redel D, et al. Nicht-immunologischer hydrops fetalis – eine ubersicht uber 31 faille. Klin Padiatr. 1988;200:287–93.

    CAS  PubMed  Google Scholar 

  46. Villaespesa AR, Mier MPS, Ferrer PL, Baleriola IA, Gonzalez JIR. Nonimmunologic hydrops fetalis: an etiopathogenetic approach through the postmortem study of 59 patients. Am J Med Genet. 1990;35:274–9.

    Google Scholar 

  47. Ito T, Engle MA, Holswade GR. Congenital insufficiency of the pulmonic valve. Pediatrics. 1961;28:712–8.

    Google Scholar 

  48. Moller JH, Lynch RP, Edwards JE. Fetal cardiac failure resulting from congenital anomalies of the heart. J Pediatr. 1966;68:699–703.

    CAS  PubMed  Google Scholar 

  49. Moerman P, Fryns J-P, Goddeeris P, Lauweryns JM. Nonimmunological hydrops fetalis. A study of ten cases. Arch Pathol Lab Med. 1982;106:635–40.

    CAS  PubMed  Google Scholar 

  50. Wilkin P, Parmentier R. Hydrops foeto – placentaire et fibroelastose sousendocardique. Bull Soc R Belg Gynecol Obstet. 1961;31:35–44.

    CAS  Google Scholar 

  51. Etches PC, Lemons JA. Nonimmune hydrops fetalis: report of 22 cases including three siblings. Pediatrics. 1979;64:326–32.

    CAS  PubMed  Google Scholar 

  52. Katz VL, Kort B, Watson WJ. Progression of nonimmune hydrops in a fetus with Noonan syndrome. Am J Perinatol. 1993;10:417–8.

    CAS  PubMed  Google Scholar 

  53. Boyd PA, Keeling JW. Fetal hydrops. J Clin Genet. 1992;29:91–7.

    CAS  Google Scholar 

  54. Terry J, Tapas M, Nowaczyk MJM. Prenatal and pathologic features of aorto-left ventricular tunnel causing fetal hydrops and intrauterine demise. Pediatr Dev Pathol. 2013;16:97–101.

    PubMed  Google Scholar 

  55. Radford DJ, Izukawa T, Rowe RD. Congenital paroxysmal atrial tachycardia. Arch Dis Child. 1976;51:613–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  56. Benito Bartolome F, Jimenez CS. Hydrops fetalis secondary to junctional ectopic tachycardia. Rev Esp Cardiol. 2000;53:1529–30.

    CAS  PubMed  Google Scholar 

  57. Anandakumar C, Biswas A, Chew SS, Chia D, Wong YC, Ratnam SS. Direct fetal therapy for hydrops secondary to congenital atrioventricular heart block. Obstet Gynecol. 1996;87:835–7.

    CAS  PubMed  Google Scholar 

  58. Cooke RWJ, Mettau JW, van Coppelle AW, de Villeneuve VH. Familial congenital heart block and hydrops fetalis. Arch Dis Child. 1980;55:479–80.

    CAS  PubMed  PubMed Central  Google Scholar 

  59. McCue CM, Mantakes ME, Tinglestad JB, Ruddy S. Congenital heart block in newborns of mothers with connective tissue disease. Circulation. 1977;56:82–90.

    CAS  PubMed  Google Scholar 

  60. Hardy JD, Solomon S, Banwell GS, Beach R, Wright V, Howard FM. Congenital complete heart block in the newborn associated with maternal systemic lupus erythematosus and other connective tissue disorders. Arch Dis Child. 1979;54:7–13.

    CAS  PubMed  PubMed Central  Google Scholar 

  61. Cowan RH, Waldo HB, Harris G, et al. Neonatal paroxysmal supraventricular tachycardia with hydrops. Pediatrics. 1975;55:248–30.

    Google Scholar 

  62. Esscher E, Scott JS. Congenital heart block and maternal systemic lupus erythematosus. Br Med J. 1979;I:1235–8.

    Google Scholar 

  63. Ho SY, Mortimer G, Anderson RH, Pomerance A, Keeling JW. Conduction system defects in three perinatal patients with arrhythmia. Br Heart J. 1985;3:158–63.

    Google Scholar 

  64. Ranucci-Weiss D, Uerpairojkit B, Bowles N, Towbin JA, Chan L. Intrauterine adenoviral infection associated with fetal non-immune hydrops. Prenat Diagn. 1998;18:182–5.

    CAS  PubMed  Google Scholar 

  65. Lauener P-A, Payot M, Micheli J-L. Congenital hydrops and WPW Syndrome. Pediatr Cardiol. 1985;6:113–6.

    CAS  PubMed  Google Scholar 

  66. Miller TE, Estrella E, Myerburg RJ, Garcia de Viera J, Moreno N, Rusconi P, et al. Recurrent third-trimester fetal loss and maternal mosaicism for long-QT syndrome. Circulation. 2004;109:3029–34.

    PubMed  Google Scholar 

  67. Mostoufi-Zadeh M, Weiss LM, Discoll SG. Nonimmune hydrops fetalis: a challenge in perinatal pathology. Hum Pathol. 1985;16:785–9.

    CAS  PubMed  Google Scholar 

  68. Rungan S, Wright J. Noncompaction cardiomyopathy in a neonate presenting with hydrops fetalis and pulmonary hypertension. Pediatr Cardiol. 2013;34:2003–5.

    PubMed  Google Scholar 

  69. Naeye RL, Blanc WA. Prenatal narrowing or closure of the foramen ovale. Circulation. 1964;30:736–42.

    CAS  PubMed  Google Scholar 

  70. Personen E, Haavistu H, Anumala P, Teramu IC. Intrauterine hydrops caused by premature closure of the foramen ovale. Arch Dis Child. 1983;58:1015–6.

    Google Scholar 

  71. Hagen A, Albig M, Schmitz L, Hopp H, van Baalen A, Becker R, et al. Prenatal diagnosis of isolated foramen ovale obstruction – a report of two cases. Fetal Diagn Ther. 2005;20:70–3.

    CAS  PubMed  Google Scholar 

  72. Becker AE, Becker MJ, Wagenvoort CA. Premature contraction of the ductus arteriosus: a cause of fetal death. J Pathol. 1977;121:187–91.

    CAS  PubMed  Google Scholar 

  73. Kohler HG. Premature closure of the ductus arteriosus (PCDA): a possible cause of intrauterine circulatory failure. Early Hum Dev. 1978;2:15–23.

    CAS  PubMed  Google Scholar 

  74. Downing GJ, Thibeault DW. Pulmonary vasculature changes associated with idiopathic closure of the ductus arteriosus and hydrops fetalis. Pediatr Cardiol. 1994;15:71–5.

    CAS  PubMed  Google Scholar 

  75. Wyatt–Ashmead J. Antenatal closure of the ductus arteriosus and hydrops fetalis. Pediatr Dev Pathol. 2011;14:469–74.

    PubMed  Google Scholar 

  76. Spahr RC, Botti JJ, MacDonald HM, Holzman IR. Nonimmunologic hydrops fetalis: a review of 19 cases. Int J Gynaecol Obstet. 1980;18:303–7.

    CAS  PubMed  Google Scholar 

  77. Barberato MF, Barberato SH, Gomes CC, Costa KA. Right atrial aneurysm associated to fetal hydrops: diagnosis through fetal echocardiography. Arq Bras Cardiol. 2005;85:65–7.

    PubMed  Google Scholar 

  78. Prefumo F, Bhide A, Thilaganathan B, Carvalho JS. Fetal congenital cardiac diverticulum with pericardial effusion: two cases with different presentations in the first trimester of pregnancy. Ultrasound Obstet Gynecol. 2005;25:405–8.

    CAS  PubMed  Google Scholar 

  79. Larroche JC. Nonimmunologic hydrops fetalis. Developmental pathology of the neonate. Amsterdam: Excerpta Medica; 1977. p. 179–81.

    Google Scholar 

  80. Shturman-Ellstein R, Greco MA, Myrie C, Goldman EK. Hydrops fetalis, hydramnios and hepatic vascular malformation associated with cutaneous haemangioma and chorioangioma. Acta Paediatr Scand. 1978;67:239–43.

    CAS  PubMed  Google Scholar 

  81. Platt LD, Geirmann CA, Turkel SB, Young G, Keegan KA. Atrial hemangioma and hydrops fetalis. Am J Obstet Gynecol. 1981;141:107–9.

    CAS  PubMed  Google Scholar 

  82. Ivemark B, Lagergren C, Ljungqvist A. Generalised arterial calcification associated with hydramnios in two stillborn infants. Acta Paediatr Scand Suppl. 1962;135:103–10.

    Google Scholar 

  83. Nissen T, Silverman NH, Ursell PC. Aortic vasculopathy with aneurysm: a rare cause of fetal hydrops. Cardiol Young. 2000;10:153–5.

    CAS  PubMed  Google Scholar 

  84. Zeltser I, Parness IA, Ko H, Holtzman IR, Kemnir SA. Midaortic syndrome in the fetus and premature newborn: a new etiology of nonimmune hydrops fetalis and reversible fetal cardiomyopathy. Pediatrics. 2003;111:1437–42.

    PubMed  Google Scholar 

  85. Swarr TD, Khalek N, Treat J, Horton MA, Mirzaa GM, Riviere JB, et al. Expanding the differential diagnosis of fetal hydrops: an unusual prenatal presentation of megalencephaly – capillary malformation syndrome. Prenat Diagn. 2013;33:1010–2.

    PubMed  PubMed Central  Google Scholar 

  86. Rudolph N, Levin EJ. Hydrops fetalis with venae cavae thrombosis in utero. N Y State J Med. 1977;3:421–3.

    Google Scholar 

  87. Adiotomre PN, Burns JE, McIntosh N. Hydrops foetalis and chylothorax associated with superior caval vein obstruction and resolution following balloon dilatation. Acta Paediatr. 1994;83:983–5.

    CAS  PubMed  Google Scholar 

  88. Siven M, Ley D, Hagerstrand I, Svenningsen N. Agenesis of the ductus venosus and its correlation to hydrops fetalis and the fetal hepatic circulation: case reports and review of the literature. Pediatr Pathol Lab Med. 1995;15:39–50.

    CAS  PubMed  Google Scholar 

  89. Durakovic N, Azancot A, Eurun D, Menez F, Vuillard E, Garel C, et al. Ductus venosus agenesis. Arch Mal Coeur Vaiss. 2005;98:542–8.

    CAS  PubMed  Google Scholar 

  90. Takeuchi M, Nakayama M, Tamura A, Kitajima H. Hydrops fetalis due to agenesis of the ductus venosus: new hepatic histological features. Pediatr Dev Pathol. 2009;12:239–43.

    PubMed  Google Scholar 

  91. Hofstaetter C, Plath H, Hansmann M. Prenatal diagnosis of abnormalities of the fetal venous system. Ultrasound Obstet Gynecol. 2000;15:231–41.

    CAS  PubMed  Google Scholar 

  92. Laurence KM. Congenital pulmonary cystic lymphangiectasis. J Pathol Bacteriol. 1955;70:325–33.

    CAS  PubMed  Google Scholar 

  93. Windebank KP, Bridges NA, Ostman-Smith I, Stevens JE. Hydrops fetalis due to abnormal lymphatics. Arch Dis Child. 1987;62:198–200.

    CAS  PubMed  PubMed Central  Google Scholar 

  94. Thibeault DW, Black P, Taboada E. Fetal hydrops and familial pulmonary lymphatic hypoplasia. Am J Perinatol. 2002;19:323–31.

    PubMed  Google Scholar 

  95. Mahoney BS, Filly RA, Callen PW, Chinn DH, Golbus MS. Severe nonimmune hydrops fetalis: sonographic evaluation. Radiology. 1984;151:757–61.

    Google Scholar 

  96. Syhez JM, Dubner M, de Diaz SL, Vilaseco G. A fetus with a 90, XX karyotype. Prenat Diagn. 1999;19:178–9.

    Google Scholar 

  97. Macafee CAJ, Fortune DW, Beischer NA. Nonimmunological hydrops fetalis. J Obstet Gynecol Br Cmwlth. 1970;77:226–37.

    CAS  Google Scholar 

  98. Sahn DJ, Shenker L, Reed KL, Valdes-Cruz LM, Sobonya R, Anderson C. Prenatal ultrasound diagnosis of hypoplastic left heart syndrome in utero associated with hydrops fetalis. Am Heart J. 1982;104:1368–72.

    CAS  PubMed  Google Scholar 

  99. Kuller JA, Laifer SA. Trisomy 15 associated with nonimmune hydrops. Am J Perinatol. 1991;8:39–40.

    CAS  PubMed  Google Scholar 

  100. Knoblauch H, Sommer D, Zimmer C, Tennstedt C, Heling K, Bollmann R, et al. Fetal trisomy 10 mosaicism: ultrasound, cytogenetic and morphologic findings in early pregnancy. Prenat Diagn. 1999;19:379–82.

    CAS  PubMed  Google Scholar 

  101. Langford K, Hodgson S, Seller M, Maxwell D. Pallister-Killian syndrome presenting through nuchal translucency screening for trisomy 21. Prenat Diagn. 2000;20:670–2.

    CAS  PubMed  Google Scholar 

  102. Tercanli S, Hösli I, Berlinger A, Beyer R, Achermann J, Holzgreve W. Prenatal diagnosis of a partial trisomy 19q. Prenat Diagn. 2000;20:663–5.

    Google Scholar 

  103. Chen CP, Wang LK, Chern SR, Wu PS, Kuo YT, Chen WL, Lee MS, Wnag W. Mosaic tetrasomy 9p at amniocentesis: prenatal diagnosis, molecular cytogenetic characterization and literature review. Taiwan J Obstet Gynecol. 2014;53:79–85.

    PubMed  Google Scholar 

  104. Willekes C, Roumen FJ, van Elsacker-Niele AM, Weiland HT, Vermey-Keers C, van Krieken JH, et al. Human parvovirus B19 infection and unbalanced translocation in a case of hydrops fetalis. Prenat Diagn. 1994;14:181–5.

    CAS  PubMed  Google Scholar 

  105. Fert-Ferrier S, Guichet A, Tantau J, Delezoide AL, Ozilou C, Romana SP, et al. Subtle familial unbalanced translocation t(8;11)(p23.2;p15.5) in two fetuses with Beckwith-Wiedemann features. Prenat Diagn. 2000;20:511–5.

    Google Scholar 

  106. Faivre L, Morichon-Delvallez N, Viot G, Martinovic J, Pinson MP, Aubry JP, et al. Prenatal detection of a 1p36 deletion in a fetus with multiple malformations and a review of the literature. Prenat Diagn. 1999;19:49–53.

    CAS  PubMed  Google Scholar 

  107. Suwanrath-Kengpol C, Limprasert P, Miltarnun W. Prenatal diagnosis of deletion of chromosome 6p presenting with fetal hydrops. Prenat Diagn. 2004;24:887–9.

    PubMed  Google Scholar 

  108. Jain R, Jones C. Hydrops fetalis in an interstitial deletion of chromosome 10. Am J Med Genet. 2001;98:320–3.

    CAS  PubMed  Google Scholar 

  109. Machlitt A, Tennstedt C, Karner H, Bommer C, Chaoui R. Prenatal diagnosis of 22q11 microdeletion in an early second-trimester fetus with conotruncal anomaly presenting with increased nuchal translucency and bilateral intracardiac echogenic foci. Ultrasound Obstet Gynecol. 2002;19:510–3.

    CAS  PubMed  Google Scholar 

  110. Witters I, Van Buggenhout G, Moerman P, Fryns JP. Prenatal diagnosis of de novo distal 5q duplication associated with hygroma colli, fetal oedema and complex cardiopathy. Prenat Diagn. 1998;18:1304–7.

    CAS  PubMed  Google Scholar 

  111. Gargano G, Guidotti I, Balestri E, Vagnarelli F, Rosato S, Comitini G, et al. Hydrops fetalis in a preterm newborn heterozygous for the c.4A>G SHOC2 mutation. Am J Med Genet A. 2014;164A:1015–20.

    PubMed  Google Scholar 

  112. Ghalamkarpour A, Debauche C, Haan E, Van Regemorter N, Sznajer Y, Thomas D, et al. Sporadic in utero generalized edema caused by mutations in the lymphangiogenic genes VEGFR3 and FOXC2. J Pediatr. 2009;155:90–3.

    CAS  PubMed  Google Scholar 

  113. de Bruyn G, Casear A, Devolder K, Van Acker G, Logghe H, Devriendt K, et al. Hydrops fetalis and pulmonary lymphangiectasia due to FOXC2 mutation: an autosomal dominant hereditary lymphedema syndrome with variable expression. Eur J Pediatr. 2012;171:447–50.

    CAS  PubMed  Google Scholar 

  114. Nelson J, Kenny B, O’Hara D, Harper A, Broadhead D. Foamy changes of placental cells in probable beta glucuronidase deficiency associated with hydrops fetalis. J Clin Pathol. 1993;46:370–1.

    CAS  PubMed  PubMed Central  Google Scholar 

  115. Molyneux AJ, Blair E, Coleman N, Daish P. Mucopolysaccharidosis type VII associated with hydrops fetalis: histopathological and ultrastructural features with genetic implications. J Clin Pathol. 1997;50:252–4.

    CAS  PubMed  PubMed Central  Google Scholar 

  116. Johnson WG, Thomas GH, Miranda AF, et al. Congenital sialidosis: biochemical studies, clinical spectrum in four sibs; two successful prenatal diagnoses. Am J Hum Genet. 1980;32:43A.

    Google Scholar 

  117. Beck AM, Bender SW, Reither H-L, et al. Neuraminidase deficiency presenting as nonimmune hydrops fetalis. Eur J Pediatr. 1984;43:135–9.

    Google Scholar 

  118. Michelakakis H, Dimitriou E, Labadaridis I. The expanding spectrum of disorders with elevated plasma chitotriosidase activity: an update. J Inherit Metab Dis. 2004;27:705–6.

    CAS  PubMed  Google Scholar 

  119. Rossier A, Caldera R, Sarrut S. Sur un cas de maladie de Niemann–Pick chez un nouveau-né. Presse Med. 1958;66:535–7.

    CAS  PubMed  Google Scholar 

  120. Turski DM, Shahidi N, Viseskul C, Gilbert E. Nonimmunologic hydrops fetalis. Am J Obstet Gynecol. 1978;131:586–7.

    CAS  PubMed  Google Scholar 

  121. Tercanli S, Uyanik G, Hasli I, Cadgas A, Holzgreve W. Increased nuchal translucency in a case of long- chain 3-hydroxyacyl-coenzyme a dehydrogenase deficiency. Fetal Diagn Ther. 2000;15:322–5.

    CAS  PubMed  Google Scholar 

  122. Giuffra B, Parinii R, Rizzuti T, Morandi L, van Diggelen OP, Bruno C, et al. Severe neonatal onset of glycogenosis type IV: clinical and laboratory findings leading to diagnosis in two siblings. J Inherit Metab Dis. 2004;27:609–19.

    Google Scholar 

  123. Steenhout P, Elmer C, Clercx A, et al. Carnitine deficiency with cardiomyopathy presenting as neonatal hydrops: a successful response to carnitine therapy. J Inherit Metab Dis. 1990;13:69–73.

    CAS  PubMed  Google Scholar 

  124. Wisser J, Schreiner M, Diem H, Roithmeier A. Neonatal hemochromatosis: a rare cause of nonimmune hydrops fetalis and fetal anemia. Fetal Diagn Ther. 1993;8:273–8.

    CAS  PubMed  Google Scholar 

  125. Verstraeten L, van Regemorter N, Pardou A, de Verneuil H, da Silva V, Rodesch F, et al. Biochemical diagnosis of a fatal case of Gunther’s disease in a newborn with hydrops. Eur J Clin Chem Clin Biochem. 1993;31:121–8.

    CAS  PubMed  Google Scholar 

  126. Noelle V, Kneupfer M, Pulzer F, Schuster V, Siekmeyer W, Matthijs G, et al. Unusual presentation of congenital disorder of glycosylation type 1a: congenital persistent thrombocytopenia, hypertrophic cardiomyopathy and hydrops-like aspect due to marked peripheral oedema. Eur J Pediatr. 2005;164:223–6.

    PubMed  Google Scholar 

  127. Kepron C, Blumenthal A, Chitayat D, Cutz E, Superti-Furga E, Keating S. An autophagic vacuolar myopathy-like disorder presenting as nonimmune hydrops in a female fetus. Pediatr Dev Pathol. 2009;12:53–8.

    PubMed  Google Scholar 

  128. Whitfield CR. Prediction of rhesus haemolytic disease. In: Barson AJ, editor. Laboratory investigation of fetal disease. Bristol: Wright; 1981. p. 299–319.

    Google Scholar 

  129. Huchet J, Soulié JC. Anasarque foeto-placentaire et incompatabilité ABO. Rev Fr Transfus Immuno-hémat. 1979;22:191–3.

    CAS  Google Scholar 

  130. Miser A, Geraci TK, Wennberg RP. Fetal erythroblastosis fetalis due to anti-Kell isoimmune disease. J Pediatr. 1975;86:567–9.

    CAS  PubMed  Google Scholar 

  131. Scanton JW, Muirhead DM. Hydrops fetalis due to anti-Kell isoimmune disease: survival with optimal long-term outcome. J Pediatr. 1976;88:484–5.

    Google Scholar 

  132. Thumasathit B, Nondasuta A, Silpisornkosol S, Lousuebsakul B, Unchalipongse P, Mangkornkanok M. Hydrops fetalis associated with Bart’s haemoglobin in Northern Thailand. J Pediatr. 1968;73:132–8.

    CAS  PubMed  Google Scholar 

  133. Weatherall DJ, Clegg JB, Wong HB. The haemoglobin constitution of infants with the haemoglobin Bart’s hydrops fetalis syndrome. Br J Haematol. 1970;18:357–67.

    CAS  PubMed  Google Scholar 

  134. Boer HR, Anido G. Hydrops fetalis caused by Bart’s haemoglobin. South Med J. 1979;72:1623–4.

    CAS  PubMed  Google Scholar 

  135. Lie ILE, Lie HG. Abnormal haemoglobin production as a probable cause of erythroblastosis and hydrops foetalis in uniovular twins. Acta Haematol. 1961;25:192–9.

    PubMed  Google Scholar 

  136. Hennekam RC, Beemer FA, Cats BP, Jansen G, Staal GE. Hydrops fetalis associated with red cell pyruvate kinase deficiency. Genet Couns. 1990;1:75–9.

    CAS  PubMed  Google Scholar 

  137. Ferreira P, Morais L, Costa R, Resende C, Dias CP, Arajo F, et al. Hydrops fetalis associated with erythrocyte pyruvate kinase deficiency. Eur J Pediatr. 2000;159:481–2.

    CAS  PubMed  Google Scholar 

  138. Perkins RP. Hydrops fetalis and stillbirth in a male glucose-6-phosphate dehydrogenase deficient fetus possibly due to maternal ingestion of sulfisoxazole. Am J Obstet Gynecol. 1971;111:379–81.

    CAS  PubMed  Google Scholar 

  139. Mentzer WC, Collier E. Hydrops fetalis associated with erythrocyte G-6-PD deficiency and maternal ingestion of fava beans and ascorbic acid. J Pediatr. 1975;86:565–9.

    CAS  PubMed  Google Scholar 

  140. Scimeca PG, Weinblatt ME, Slepowitz G, Harper RG, Kohen JA. Diamond-Blackfan syndrome: an unusual cause of hydrops fetalis. Am J Pediatr Hematol Oncol. 1988;10:241–3.

    CAS  PubMed  Google Scholar 

  141. McLennan AC, Chitty LS, Rissik J, Maxwell DJ. Prenatal diagnosis of Blackfan-Diamond syndrome: case report and review of the literature. Prenat Diagn. 1996;16:349–53.

    CAS  PubMed  Google Scholar 

  142. Carter C, Darbyshire PJ, Wickramasinghe SN. A congenital dyserythropoietic anaemia variant presenting as hydrops fetalis. Br J Haematol. 1989;72:289–90.

    CAS  PubMed  Google Scholar 

  143. Cantu-Rajnoldi A, Zanella A, Conter U, et al. A severe transfusion-dependent congenital dyserythropoietic anaemia presenting as hydrops fetalis. Br J Haematol. 1997;96:530–3.

    CAS  PubMed  Google Scholar 

  144. Vincente-Gutierrez MP, Castella-Almazyl I, Salva-Roiges MD, Rodriguez-Migualez JM, Vives-Corrons JL, Figueras-Aloy J, et al. Nonimmune hydrops due to congenital xerocytosis. J Perinatol. 2005;25:63–5.

    Google Scholar 

  145. Smrcek JM, Bashatt AA, Germer U, Gloeckner-Hofmann K, Gembruch U. Fetal hydrops and hepatosplenomegaly in the second half of pregnancy: a sign of myeloproliferative disorder in fetuses with trisomy 21. Ultrasound Obstet Gynecol. 2001;17:403–9.

    CAS  PubMed  Google Scholar 

  146. Bechara E, Dijoud F, de Saint BG, Bertrand Y, Pondarre C. Hemophagocytic lymphohistiocytosis with Munc13-4 mutation: a cause of recurrent fatal hydrops fetalis. Pediatrics. 2011;128:e251–4.

    PubMed  Google Scholar 

  147. Vermeulen MJ, de Haas V, Mulder MF, Flohil C, Fetter WP, van de Kamp JM. Hydrops fetalis and early neonatal multiple organ failure in familial hemophagocytic lymphohistiocytosis. Eur J Med Genet. 2009;52:417–20.

    PubMed  Google Scholar 

  148. Debelle GD, Gillam GL, Tauro GP. A case of hydrops fetalis due to maternal haemorrhage. Aust Paediatr J. 1977;13:131–3.

    CAS  PubMed  Google Scholar 

  149. Blackburn GK. Massive fetomaternal hemorrhage due to choriocarcinoma of the uterus. J Pediatr. 1976;89:680–1.

    CAS  PubMed  Google Scholar 

  150. Nieuwenhuijzen Kruseman AC, van Lent M, Blom AH, Lauw GP. Choriocarcinoma in mother and child, identified by immunoenzyme histochemistry. Am J Clin Pathol. 1977;67:279–83.

    Google Scholar 

  151. Bose C. Hydrops fetalis and in utero intracranial haemorrhage. J Pediatr. 1978;93:1023–4.

    CAS  PubMed  Google Scholar 

  152. Coulson CC, Kuller JA, Sweeney WJ. Nonimmune hydrops and hydrocephalus secondary to fetal intracranial hemorrhage. Am J Perinatol. 1994;4:253–4.

    Google Scholar 

  153. Seward JF, Zusman J. Hydrops fetalis associated with small bowel volvulus. Lancet. 1978;ii:52–3.

    Google Scholar 

  154. Burton PA. Intranuclear inclusions in marrow of hydropic fetus due to parvovirus infection. Lancet. 1986;ii:1155.

    Google Scholar 

  155. Porter HJ, Khong TY, Evans MF, Chan VT-W, Fleming KA. Parvovirus as a cause of hydrops fetalis: detection by in situ hybridisation. J Clin Pathol. 1988;41:381–3.

    CAS  PubMed  PubMed Central  Google Scholar 

  156. Morey AL, Keeling JW, Porter HJ, Fleming KA. Clinical and histopathological features of parvovirus B19 infection in the human fetus. Br J Obstet Gynaecol. 1992;99:566–74.

    CAS  PubMed  Google Scholar 

  157. Bain AD, Bowie JH, Flint WF, Beverley JKA, Beattie CP. Congenital toxoplasmosis simulating haemolytic disease of the newborn. J Obstet Gynaecol Br Cmwlth. 1956;63:826–32.

    CAS  Google Scholar 

  158. Bulova SI, Schwartz E, Harrer WV. Hydrops fetalis and congenital syphilis. Pediatrics. 1972;49:285–7.

    CAS  PubMed  Google Scholar 

  159. Bryan EM, Nicholson E. Hydrops fetalis in South Korea. Ann Trop Paediatr. 1981;1:181–7.

    CAS  PubMed  Google Scholar 

  160. Bates HR. Coxsackie virus B3 calcific pancarditis and hydrops fetalis. Am J Obstet Gynecol. 1970;106:629–30.

    PubMed  Google Scholar 

  161. Towbin JA, Griffin LD, Martin AB, et al. Intrauterine adenoviral myocarditis presenting as non-immune hydrops fetalis: diagnosis by polymerase chain reaction. Pediatr Infect Dis J. 1994;13:144–50.

    CAS  PubMed  Google Scholar 

  162. Silver DA, Ashworth MT, Andrews HS. Prenatal ultrasound diagnosis of fetal Candida infections: a complication of a retained intrauterine contraceptive device. Ultrasound Obstet Gynecol. 1994;4:154–7.

    CAS  PubMed  Google Scholar 

  163. Bachmaier N, Fusch C, Stenger RD, Grabow D, Mentel R, Warzok R. Nonimmune hydrops fetalis due to enterovirus infection. Eur J Obstet Gynecol Reprod Biol. 2009;142:83–4.

    CAS  PubMed  Google Scholar 

  164. Engellenner W, Kaplan C, van de Vegte GL. Pulmonary agenesis association with nonimmune hydrops. Pediatr Pathol. 1989;9:725–30.

    CAS  PubMed  Google Scholar 

  165. Phillips RR, Batcup G, Vinall PS. Non-immunologic hydrops fetalis. Arch Dis Child. 1985;60:84.

    CAS  PubMed  PubMed Central  Google Scholar 

  166. Keswani SG, Crombleholme TM, Pawel BR, Johnson MP, Flake AW, Hedrick HL, et al. Prenatal diagnosis and management of Mainstem bronchial atresia. Fetal Diagn Ther. 2005;20:74–8.

    PubMed  Google Scholar 

  167. Khong TY, Keeling JW. Massive congenital mesenchymal malformation of the lung: another cause of non-immune hydrops. Histopathology. 1990;16:609–11.

    CAS  PubMed  Google Scholar 

  168. Priyadarshi A, Sugo E, Challis D, Bolisetty S. Accessory diaphragm associated with non-immune hydrops fetalis. BMJ Case Rep 2014. doi: 10.1136/bcr-2014-204090.

    Google Scholar 

  169. Larroche J-CI. Anasarque foeto-placentaire (hydrops) sans immunisation. Med Hyg. 1982;40:2061–73.

    Google Scholar 

  170. Potter EL. Universal edema of the fetus unassociated with erythroblastosis. Am J Obstet Gynecol. 1943;46:130–4.

    Google Scholar 

  171. Van Maldergem L, Jauniaux E, Fourneau C, Gillerot Y. Genetic causes of hydrops fetalis. Pediatrics. 1992;89:81–6.

    PubMed  Google Scholar 

  172. Gray ES. Mesoblastic nephroma and non-immunological hydrops fetalis (letter). Pediatr Pathol. 1989;9:607–9.

    CAS  PubMed  Google Scholar 

  173. Cetinkaya M, Durmaz O, Buyukkale G, Ozbek S, Acar D, Kilicaslan I, et al. Neonatal Bartter syndrome and unilateral ectopic renal cyst as new renal causes of hydrops fetalis: two case reposts and review of the literature. J Matern Fetal Neonatal Med. 2013;26:1147–50.

    PubMed  Google Scholar 

  174. Worthen HG, Vernier RL, Good RA. Infantile nephrosis. Am J Dis Child. 1959;98:731–48.

    CAS  Google Scholar 

  175. Taweevisit M, Manotaya S, Thorner PS. Cystic malformation of lower female genital tract resulting in hydrops fetalis: a case report. Pediatr Dev Pathol. 2013;16:39–44.

    PubMed  Google Scholar 

  176. Nistal M, Rodríguez J, García-Fernández E, Cajaiba MM, Reyes-Múgica M. Fetal gonadoblastoid testicular dysplasia: a focal failure of testicular development. Pediatr Dev Pathol. 2007;10:274–81.

    PubMed  Google Scholar 

  177. Golbus MS, Hall BD, Filly RA, Poskaizer LR. Prenatal diagnosis of achondrogenesis. J Pediatr. 1977;91:464–6.

    CAS  PubMed  Google Scholar 

  178. Fleischer AC, Killam AP, Boehm FH, et al. Hydrops fetalis: sonographic evaluation and clinical implications. Radiology. 1981;141:163–8.

    CAS  PubMed  Google Scholar 

  179. Richardson MM, Wagner ML, Malini S, Rosenberg HS, Lucci JA. Prenatal diagnosis of recurrence of Saldino–Noonan dwarfism. J Pediatr. 1977;91:467–71.

    CAS  PubMed  Google Scholar 

  180. Chitayat D, Gruber H, Mullen BJ, et al. Hydrops-ectopic calcification – moth-eaten skeletal dysplasia (Greenberg dysplasia): prenatal diagnosis and further delineation of a rare genetic disorder. Am J Med Genet. 1993;47:272–7.

    CAS  PubMed  Google Scholar 

  181. Laccone F, Schoner K, Krabichler B, Kluge B, Schwerdtfeger R, Schulze B, et al. Desbuquois dysplasia type I and fetal hydrops due to novel mutations in the CANT1 gene. Eur J Hum Genet. 2011;19:1133–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  182. Lecollier B, Bercau G, Gonzales M, Afriat R, Rambaud D, Mulliez N, et al. Radiographic, haematological, and biochemical findings in a fetus with Caffey disease. Prenat Diagn. 1992;12:637–41.

    Google Scholar 

  183. Stassou S, Nadroo A, Schubert R, Chin S, Gudavalli M. A new syndrome of myopathy with muscle spindle excess. J Perinat Med. 2005;33:179–82.

    PubMed  Google Scholar 

  184. Robin NH, Curtis MT, Mulla W, et al. Non-immune hydrops fetalis associated with impaired fetal movement: a case report and review. Am J Med Genet. 1994;53:251–4.

    CAS  PubMed  Google Scholar 

  185. Ostor AG, Fortune DW. Tuberous sclerosis initially seen as hydrops fetalis: report of a case and review of the literature. Arch Pathol Lab Med. 1978;102:34–9.

    CAS  PubMed  Google Scholar 

  186. Bruch SW, Adzick NS, Reiss R, Harrison MR. Prenatal therapy for pericardial teratomas. J Pediatr Surg. 1997;32:1113–5.

    CAS  PubMed  Google Scholar 

  187. Platt LD, Collea JV, Joseph DM. Transitory fetal ascites: an ultrasound diagnosis. Am J Obstet Gynecol. 1978;132:906–8.

    CAS  PubMed  Google Scholar 

  188. Huppmann AR, Coffin CM, Hoot AC, Kahwash S, Pawel BR. Congenital peribronchial myofibroblastic tumor: comparison of fetal and postnatal morphology. Pediatr Dev Pathol. 2011;14:124–9.

    PubMed  Google Scholar 

  189. Anders D, Kindermann G, Pfeifer U. Metastasising fetal neuroblastoma with involvement of the placenta simulating fetal erythroblastosis. J Pediatr. 1973;82:50–3.

    CAS  PubMed  Google Scholar 

  190. Vadeyar S, Ramsay M, James D, O’Neill D. Prenatal diagnosis of congenital Wilms’ tumour (nephroblastoma) presenting as fetal hydrops. Ultrasound Obstet Gynecol. 2000;16:80–3.

    CAS  PubMed  Google Scholar 

  191. Castellino SM, Powers R, Kalwinsky D, DeVoe M. Abdominal rhabdoid tumour presenting as fetal hydrops: a case report. J Pediatr Hematol Oncol. 2001;23:258–9.

    CAS  PubMed  Google Scholar 

  192. Fuchs IB, Henrich W, Kalache KD, Lippek F, Dudenhausen JW. Prenatal sonographic features of a rhabdoid tumour of the kidney. Ultrasound Obstet Gynecol. 2006;23:407–10.

    Google Scholar 

  193. Kazzi NJ, Chang CH, Roberts EC, Shankaran S. Fetal hepatoblastoma presenting as nonimmune hydrops. Am J Perinatol. 1989;6:278–80.

    CAS  PubMed  Google Scholar 

  194. Kamata S, Nose K, Sawai T, Hasegawa T, Kuroda S, Sasaki T, et al. Fetal mesenchymal hamartoma of the liver: report of a case. J Pediatr Surg. 2003;38:639–41.

    PubMed  Google Scholar 

  195. Ruiz-Villaespesa A, Suarez Mier MP, Lopez Ferrer P, Alvarez Baleriola I, Sabet LM. Congenital glioblastoma multiforme associated with congestive heart failure. Arch Pathol Lab Med. 1982;106:31–4.

    Google Scholar 

  196. Kohler H. Sacrococcygeal teratoma and “nonimmunological” hydrops fetalis. Br Med J. 1976;ii:422–3.

    Google Scholar 

  197. Beischer NA, Fortune DW, Macafee J. Non-immunologic hydrops fetalis and congenital abnormalities. Obstet Gynecol. 1971;38:86–95.

    CAS  PubMed  Google Scholar 

  198. Donnenfeld AE, Scott SC, Henselder-Kimmel M, Dampier CD. Prenatally diagnosed non-immune hydrops caused by congenital transient leukaemia. Prenat Diagn. 1994;14:721–4.

    CAS  PubMed  Google Scholar 

  199. Zipursky A, Rose T, Skidmore M, Thorner P, Doyle J. Hydrops fetalis and neonatal leukemia in Down syndrome. Pediatr Hematol Oncol. 1996;13:81–7.

    CAS  PubMed  Google Scholar 

  200. Martinez AE, Robinson MJ, Alexis JB. Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops. Arch Pathol Lab Med. 2004;128:678–81.

    PubMed  Google Scholar 

  201. Lam PM, Leung TN, Ng PC, Vlantis AC, Wong W, Lau TK. Congenital fibrosarcoma with hydrops fetalis. Acta Obstet Gynecol Scand. 2004;83:773–6.

    PubMed  Google Scholar 

  202. Treadwell MC, Sherer DM, Sacks AJ, Ghezzi F, Romero R. Successful treatment of recurrent non-immune hydrops secondary to fetal hyperthyroidism. Obstet Gynecol. 1996;87:838–40.

    CAS  PubMed  Google Scholar 

  203. Dufke A, Vollmer B, Kendziorra H, Mackensen-Haen S, Orth U, Orlikowsky T, et al. Hydrops fetalis in three male fetuses of a female with incontinentia pigmenti. Prenat Diagn. 2001;21:1019–21.

    CAS  PubMed  Google Scholar 

  204. Terespolsky D, Farrell SA, Siegel-Bartelt J, Weksberg R. Infantile lethal variant of Simpson–Golabi–Behmel syndrome associated with hydrops fetalis. Am J Med Genet. 1995;59:329–33.

    CAS  PubMed  Google Scholar 

  205. Jones CE, Rivers RP, Chir B, Taghizadeh A. Disseminated intravascular coagulation and fetal hydrops in a newborn infant in association with a chorioangioma of placenta. Pediatrics. 1973;50:901–7.

    Google Scholar 

  206. Knoth M, Rygaard J, Hesseldahl H. Chorioangioma with hydramnios and intrauterine fetal death. Acta Obstet Gynecol Scand. 1976;55:279–81.

    CAS  PubMed  Google Scholar 

  207. Akbarzadeh-Jahromi M, Sari Aslani F, Parvari S. Placental mesenchymal dysplasia complicated by hydrops fetalis and fetal death: a case report. Arch Iran Med. 2013;16:551–4.

    PubMed  Google Scholar 

  208. Collins JH. Prenatal observation of umbilical cord torsion with subsequent premature labor and delivery of a 31-week infant with mild nonimmune hydrops. Am J Obstet Gynecol. 1995;172:1048–9.

    CAS  PubMed  Google Scholar 

  209. Altenberger KM, Jedziniak M, Roper WL, Hernandez J. Congenital complete heart block associated with hydrops fetalis. J Pediatr. 1977;91:618–20.

    Google Scholar 

  210. Hage ML, Liu R, Marcheschi DG, Bowie JD, Allen NB, Macik BG. Fetal renal vein thrombosis, hydrops fetalis, and maternal lupus anticoagulant. A case report. Prenat Diagn. 1994;14:873–7.

    CAS  PubMed  Google Scholar 

  211. Graves GR, Baskett TF. Nonimmune hydrops fetalis: antenatal diagnosis and management. Am J Obstet Gynecol. 1984;148:563–5.

    CAS  PubMed  Google Scholar 

  212. Zarafu IW, Tseng PI, Chuachingco J. Hydrops fetalis, fetal maternal transfusion and choriocarcinoma of the placenta. Pediatr Res. 1978;12:537.

    Google Scholar 

  213. Arcasoy MO, Gallagher PG. Hematologic disorders and nonimmune hydrops fetalis. Semin Perinatol. 1995;19:502–15.

    CAS  PubMed  Google Scholar 

  214. Furdon SA. Recognizing congestive heart failure in the neonatal period. Neonatal Netw. 1997;16:5–13.

    CAS  PubMed  Google Scholar 

  215. Gardiner HM. Response of the fetal heart to changes in load: from hyperplasia to heart failure. Heart. 2005;91:871–3.

    CAS  PubMed  PubMed Central  Google Scholar 

  216. Williams IA, Kleinman CS. Is hydrops fetalis a manifestation of fetal pulmonary edema caused by impaired drainage? Ultrasound Obstet Gynecol. 2008;31:96–9.

    CAS  PubMed  Google Scholar 

  217. Chisaka H, Morita E, Yaegashi N, Sugamura K. Parvovirus B19 and the pathogenesis of anaemia. Rev Med Virol. 2003;13:347–59.

    CAS  PubMed  Google Scholar 

  218. Swain S, Cameron AD, McNay MB, Phil M, Howatson AG. Prenatal diagnosis and management of nonimmune hydrops fetalis. Aust N Z J Obstet Gynaecol. 1999;39:285–90.

    CAS  PubMed  Google Scholar 

  219. Bellini C, Hennekam RC, Bonioli E. A diagnostic flow chart for non-immune hydrops fetalis. Am J Med Genet A. 2009;149A:852–3.

    PubMed  Google Scholar 

  220. Rodriguez MM, Bruce JH, Jimenez XF, Romaguera RL, Bancalari E, Garcia OL, et al. Nonimmune hydrops fetalis in the liveborn: series of 32 autopsies. Pediatr Dev Pathol. 2005;8:368–78.

    Google Scholar 

  221. Machin GA. Diseases causing fetal and neonatal ascites. Pediatr Pathol. 1985;4:195–211.

    CAS  PubMed  Google Scholar 

  222. Taweevisit M, Thorner PS. The contribution of extramedullary hematopoiesis to hepatomegaly in anemic hydrops fetalis: a study in alpha-thalassemia hydrops fetalis. Pediatr Dev Pathol. 2012;15:206–12.

    PubMed  Google Scholar 

  223. Keeling JW. Fetal pathology. Edinburgh: Churchill Livingstone; 1994. p. 89, 104–105.

    Google Scholar 

  224. O’Malley DP. Benign extramedullary myeloid proliferation. Mod Pathol. 2007;20:405–10.

    PubMed  Google Scholar 

  225. Weeks AD, Davies NP, Sprigg A, Fairlie FM. The sequential in utero death of heterokaryotic monozygotic twins. A case report and literature review. Prenat Diagn. 1996;16:657–63.

    CAS  PubMed  Google Scholar 

  226. Taweevisit M, Shanup S, Thorner PS. Adrenal cytomegaly is a frequent pathologic finding in hemoglobin Bart hydrops fetalis. Pediatr Dev Pathol. 2012;15:187–91.

    PubMed  Google Scholar 

  227. Maeno Y, Himeno W, Saito A, Hirashi S, Hirose O, Ikuma M, et al. Clinical course of fetal congenital atrioventricular block in the Japanese population: a multicentre experience. Heart. 2005;91:1075–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  228. Berg C, Geipel A, Kohl T, Breuer J, Germer U, Krapp M, et al. Atrioventricular block detected in fetal life: associated anomalies and potential prognostic markers. Ultrasound Obstet Gynecol. 2005;26:4–15.

    CAS  PubMed  Google Scholar 

  229. Rees L, Vlies PR, Adams J. Hydrops fetalis, an unusual cause, presentation and method of diagnosis. Br J Obstet Gynaecol. 1980;87:1169–70.

    CAS  PubMed  Google Scholar 

  230. Iliff P, Nicholls JM, Keeling JW, Gough JD. Nonimmunologic hydrops fetalis: a review of 27 cases. Arch Dis Child. 1983;58:979–82.

    CAS  PubMed  PubMed Central  Google Scholar 

  231. Schmider A, Henrich W, Dahnert I, Dudenhausen JW. Prenatal therapy of non-immunologic hydrops fetalis caused by severe aortic stenosis. Ultrasound Obstet Gynecol. 2000;16:275–8.

    CAS  PubMed  Google Scholar 

  232. Entezami M, Halis G, Waldschmidt J, Opri F, Runkel S. Congenital cystic adenomatoid malformation of the lung and fetal hydrops – a case with favourable outcome. Eur J Obstet Gynecol Reprod Biol. 1998;79:99–101.

    CAS  PubMed  Google Scholar 

  233. Strasburger JF, Cuneo BF, Michon MM, Gotteiner NL, Deal NL, McGregor SN, et al. Amiodarone therapy for drug-refractory fetal tachycardia. Circulation. 2004;109:375–9.

    CAS  PubMed  Google Scholar 

  234. Hansmann M, Gembruch U, Bald R. New therapeutic aspects in nonimmune hydrops fetalis based on 402 prenatally diagnosed cases. Fetal Ther. 1989;4:29–36.

    CAS  PubMed  Google Scholar 

  235. Petrikovsky B, Schneider E, Ovadia M. Natural history of hydrops resolution in fetuses with tachyarrhythmias diagnosed and treated in utero. Fetal Diagn Ther. 1996;11:292–5.

    CAS  PubMed  Google Scholar 

  236. Hansemann M, Gembruch U, Bald R, Manz M, Redel DA. Fetal tachyarrhythmias: transplacental and direct treatment of the fetus – a report of 60 cases. Ultrasound Obstet Gynecol. 1991;1:162–8.

    Google Scholar 

  237. Hickstein H, Kulz T, Claus R, Stange J, Schmidt R. Autoimmune-associated congenital heart block: treatment of the mother with immunoadsorption. Ther Apher Dial. 2005;9:148–53.

    CAS  PubMed  Google Scholar 

  238. Leung WC, Ngai C, Lam TP, Chan KL, Lao TT, Tang MH. Unexpected intrauterine death following resolution of hydrops fetalis after betamethasone treatment in a fetus with a large cystic adenomatoid malformation of the lung. Ultrasound Obstet Gynecol. 2005;25:610–2.

    CAS  PubMed  Google Scholar 

  239. Tsao K, Hawgood S, Vu L, Hirose S, Sydorak R, Albanese CT, Farmer DL, et al. Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy. J Pediatr Surg. 2005;38:508–10.

    Google Scholar 

  240. Yamashita A, Hidaka N, Yamamoto R, Nakayama S, Sasahara J, Ishii K, et al. In utero resolution of microcystic congenital cystic adenomatoid malformation after prenatal betamethasone therapy: a report of three cases and a literature review. J Clin Ultrasound. 2015;43:451–7.

    PubMed  Google Scholar 

  241. Scott F, Hinde F, Boogert A. Resolution of hydrops in twin-twin transfusion syndrome: could steroids have a role? Prenat Diagn. 1997;17:467–70.

    CAS  PubMed  Google Scholar 

  242. Duncan KR, Denbow ML, Fisk NM. The aetiology and management of twin-twin transfusion syndrome. Prenat Diagn. 1997;17:1227–36.

    CAS  PubMed  Google Scholar 

  243. Huhta J, Quintero RA, Suh E, Bader R. Advances in fetal cardiac intervention. Curr Opin Pediatr. 2004;16:487–93 fetalis: a review of 61 cases. Obstet Gynecol. 2004; 59:347–352.

    PubMed  Google Scholar 

  244. Brown MF, Lewis D, Brouillette RM, Hilman B, Brown EG. Successful prenatal management of hydrops, caused by congenital cystic adenomatoid malformation, using serial aspiration. J Pediatr Surg. 1995;30:1098–9.

    CAS  PubMed  Google Scholar 

  245. Ryo E, Okai T, Namba S, et al. Successful thoracoamniotic shunting using a double-flower catheter in a case of fetal cystic adenomatoid malformation associated with hydrops and polyhydramnios. Ultrasound Obstet Gynecol. 1997;10:293–6.

    CAS  PubMed  Google Scholar 

  246. Picone O, Benachi A, Mandelbrot L, Ruano R, Dumez Y, Dommergues M. Thoracoamniotic shunting for fetal pleural effusions with hydrops. Am J Obstet Gynecol. 2004;191:2047–1050.

    PubMed  Google Scholar 

  247. Favre R, Bettahar K, Christmann D, Becmeur F. Antenatal diagnosis and treatment of fetal hydrops secondary to pulmonary extralobar sequestration. Ultrasound Obstet Gynecol. 1994;4:335–8.

    CAS  PubMed  Google Scholar 

  248. Salomon LJ, Audibert F, Dommergues M, Vial M, Frydman R. Fetal thoracoamniotic shunting as the only treatment for pulmonary sequestration with hydrops: favourable long-term outcome without postnatal surgery. Ultrasound Obstet Gynecol. 2003;21:299–301.

    CAS  PubMed  Google Scholar 

  249. Sklansky M, Greenberg M, Lucas V, Gruslin-Giroux A. Intrapericardial teratoma in a twin fetus: diagnosis and management. Obstet Gynecol. 1997;89:807–9.

    CAS  PubMed  Google Scholar 

  250. Grebille AG, Mitanchez D, Benachi A, Aubry MC, Houfflin-Debrarge V, Vouhe P, et al. Pericardial teratoma complicated by hydrops: successful fetal therapy by thoracoamniotic shunting. Prenat Diagn. 2003;23:735–9.

    CAS  PubMed  Google Scholar 

  251. Adzick NS, Flake AW, Crombleholme TM. Management of congenital lung lesions. Semin Pediatr Surg. 2003;12:10–6.

    PubMed  Google Scholar 

  252. Crombleholme TM, Sylvester K, Flake AW, Adzick NS. Salvage of a fetus with congenital high airway obstruction syndrome by ex utero intrapartum treatment (EXIT) procedure. Fetal Diagn Ther. 2000;15:280–2.

    CAS  PubMed  Google Scholar 

  253. Lim FY, Crombleholme TM, Hedrick HL, Flake AW, Johnson MP, Howell LJ, Adzick NS. Congenital high airway obstruction syndrome: natural history and management. J Pediatr Surg. 2003;38:940–5.

    PubMed  Google Scholar 

  254. Hirose S, Farmer DL. Fetal surgery for sacrococcygeal teratoma. Clin Perinatol. 2003;30:493–506.

    PubMed  Google Scholar 

  255. Soothill P. Intrauterine blood transfusion for nonimmune hydrops fetalis due to parvovirus B19 infection. Lancet. 1990;336:121 (letter).

    CAS  PubMed  Google Scholar 

  256. Fairley CK, Smoleniec JS, Caul OE, Miller E. Observational study of effect of intrauterine transfusions on outcome of fetal hydrops after parvovirus B19 infection. Lancet. 1995;346:1335–7.

    CAS  PubMed  Google Scholar 

  257. Craparo FJ, Bonati F, Gementi P, Nicolini U. The effects of serial intravascular transfusions in ascitic/hydropic RhD-alloimmunized fetuses. Ultrasound Obstet Gynecol. 2005;25:144–5.

    CAS  PubMed  Google Scholar 

  258. Shimokawa H, Hara K, Maeda H, Miyamoto S, Koyanagi T, Nakano H. Intrauterine treatment of idiopathic hydrops fetalis. J Perinat Med. 1988;16:133–8.

    CAS  PubMed  Google Scholar 

  259. Hirose M, Murata A, Kita N, Aotani H, Takebayashi K, Noda Y. Successful intrauterine treatment with radiofrequency ablation in a case of acardiac twin pregnancy complicated with a hydropic pump twin. Ultrasound Obstet Gynecol. 2004;23:509–12.

    CAS  PubMed  Google Scholar 

  260. Wanapirak C, Tongsong T, Sirichotiyakul S, Chanprapaph P. Alcoholization: the choice of intrauterine treatment for chorangioma. J Obstet Gynaecol Res. 2002;28:71–5.

    PubMed  Google Scholar 

  261. Kubawara Y, Sawa R, Otsubo Y, Yoneyama Y, Asakura H, Araki T, et al. Intrauterine therapy for the acutely enlarging cystic hygroma. Fetal Diagn Ther. 2004;19:191–4.

    Google Scholar 

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Correspondence to Anita Nagy MS, FRCPath .

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Nagy, A., Malcomson, R.D.G. (2015). Fetal Hydrops. In: Khong, T.Y., Malcomson, R.D.G. (eds) Keeling’s Fetal and Neonatal Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-19207-9_12

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