Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Article
  • Published:

Hydrops fetalis—trends in associated diagnoses and mortality from 1997–2018

Abstract

Objectives

To describe and evaluate trends in the etiology and mortality risk in neonates admitted for neonatal intensive care with hydrops fetalis.

Study design

A retrospective review of de-identified patient data in the Pediatrix Clinical Data Warehouse from 1997 to 2018.

Results

We identified 2144 infants diagnosed with hydrops fetalis. The most common diagnoses were congenital heart disease (n = 325, 15.2%), genetic diagnoses (n = 269, 12.5%) and cardiac arrhythmia (n = 176, 8.2%). Of 2144 neonates, 988 (46%) survived to hospital discharge and 775 (36%) died prior to discharge. Mortality rate was highly variable across diagnoses, ranging from 90% in infants with congenital diaphragmatic hernia to 0% in infants with atrial flutter. Over the study period, more infants were diagnosed with trisomies and fewer with twin-to-twin transfusion. Mortality decreased by 5% from 1997–2007 to 2008–2018.

Conclusions

The risk of death among neonates with hydrops fetalis is highly dependent on the underlying cause, with increasing risk of mortality at lower gestational ages.

This is a preview of subscription content, access via your institution

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Fig. 1: Mortalty by diagnosis: mortality rate by diagnosis and diagnostic category for infants with hydrops fetalis (infants transferred prior to discharge excluded).
Fig. 2: Mortality by gestational age: mortality rate by estimated gestational age at birth (infants transferred prior to discharge excluded).

Similar content being viewed by others

References

  1. Sparks TN, Lianoglou BR, Adami RR, Pluym ID, Holliman K, Duffy J, et al. Exome sequencing for prenatal diagnosis in nonimmune hydrops fetalis. N Engl J Med. 2020;383:1746–1756.

    Article  CAS  Google Scholar 

  2. Huang HR, Tsay PK, Chiang MC, Lien R, Chou YH. Prognostic factors and clinical features in liveborn neonates with hydrops fetalis. Am J Perinatol. 2007;24:33–38.

    Article  Google Scholar 

  3. Favre R, Dreux S, Dommergues M, Dumez Y, Luton D, Oury JF, et al. Nonimmune fetal ascites: a series of 79 cases. Am J Obstet Gynecol. 2004;190:407–412.

    Article  Google Scholar 

  4. Bukowski R, Saade GR. Hydrops fetalis. Clin Perinatol. 2000;27:1007–1031.

    Article  CAS  Google Scholar 

  5. Chen HY, Zheng JQ, Zhang HP. A case report of Turner syndrome associated with fetal nuchal cystic hygroma and bilateral syndactyly of the hands and feet. Ital J Pediatr. 2019;45:85.

    Article  Google Scholar 

  6. Jomoui W, Tepakhan W, Karnpean R. Strong Linkage of the Single Nucleotide Polymorphism rs77308790 with an α0-Thalassemia (—SEA deletion) Allele and Application for Double-Check Diagnosis of Hb Bart’s Hydrops Fetalis Syndrome in Thailand. Hemoglobin. 2019;43:236–240. https://doi.org/10.1080/03630269.2019.1666720.

  7. Kang Y, Ma Y, Jiang X, Lin X, Zhao F. Fetal giant right cervical cyst causing severe tracheal compression: a case report. Med (Baltim). 2019;98:e16670.

    Article  Google Scholar 

  8. Makhamreh MM, Cottingham N, Ferreira CR, Berger S, Al-Kouatly HB. Nonimmune hydrops fetalis and congenital disorders of glycosylation: A systematic literature review. J Inherit Metab Dis. 2020;43:223–233. https://doi.org/10.1002/jimd.12162.

  9. Mardy AH, Chetty SP, Norton ME, Sparks TN. A system-based approach to the genetic etiologies of non-immune hydrops fetalis. Prenat Diagn. 2019;39:732–750.

    Article  Google Scholar 

  10. McPherson E. Hydrops fetalis in a cohort of 3,137 stillbirths and second trimester miscarriages. Am J Med Genet A. 2019;179:2338–2342. https://doi.org/10.1002/ajmg.a.61340.

  11. Paladini D, Donarini G, Conti A, Constanza De AL, Witte MH, Genesio R, et al. Early fetal hydropic changes are associated with moderate dilatation of the brain ventricular system: a clue to a possible link between cervical lymphatic engorgement and ventricular dilatation? Lymphology. 2019;52:11–17.

    CAS  PubMed  Google Scholar 

  12. Sparks TN, Thao K, Lianoglou BR, Boe NM, Bruce KG, Datkhaeva I, et al. Nonimmune hydrops fetalis: identifying the underlying genetic etiology. Genet Med. 2019;21:1339–1344.

    Article  Google Scholar 

  13. Trainor B, Tubman R. The emerging pattern of hydrops fetalis—incidence, aetiology and management. Ulst Med J. 2006;75:185–186.

    CAS  Google Scholar 

  14. Burin MG, Scholz AP, Gus R, Sanseverino MT, Fritsh A, Magalhaes JA, et al. Investigation of lysosomal storage diseases in nonimmune hydrops fetalis. Prenat Diagn. 2004;24:653–657.

    Article  Google Scholar 

  15. Deng Q, Fu F, Yu Q, Li R, Li F, Wang D, et al. Nonimmune hydrops fetalis: Genetic analysis and clinical outcome. Prenat Diagn. 2020;40:803–812.

    Article  CAS  Google Scholar 

  16. Kilby MD. Nonimmune Hydrops Fetalis - More Than Meets the Eye? N Engl J Med. 2020;383:1785–1786. https://doi.org/10.1056/NEJMe2029914.

  17. Bruwer Z, Al RN, Al DT, Al MF, Al SA, Al KA, et al. Inborn errors of metabolism in a cohort of pregnancies with non-immune hydrops fetalis: a single center experience. J Perinat Med. 2018;46:968–974.

    Article  Google Scholar 

  18. Brock WW, Bradshaw WT. Congenital chylothorax: a unique presentation of nonimmune hydrops fetalis in a preterm infant. Adv Neonatal Care. 2016;16:114–123.

    Article  Google Scholar 

  19. Calongos G, Hori M, Ogino M, Sawai H. A case of thanatophoric dysplasia type I with fetal hydrops in the first trimester. Case Rep Obstet Gynecol. 2016;2016:1821230.

    PubMed  PubMed Central  Google Scholar 

  20. Singla S, Kumar S, Roy KK, Sharma JB, Kachhawa G. Severe hydrops in the infant of a Rhesus D-positive mother due to anti-c antibodies diagnosed antenatally: a case report. J Med Case Rep. 2010;4:57.

    Article  Google Scholar 

  21. 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. 2007120:84–9.

    Article  Google Scholar 

  22. Has R. Non-immune hydrops fetalis in the first trimester: a review of 30 cases. Clin Exp Obstet Gynecol. 2001;28:187–190.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Veeral N. Tolia.

Ethics declarations

Competing interests

The authors declare no competing interests.

Additional information

Publisher’s note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Tolia, V.N., Hunter Clark, R., Perelmuter, B. et al. Hydrops fetalis—trends in associated diagnoses and mortality from 1997–2018. J Perinatol 41, 2537–2543 (2021). https://doi.org/10.1038/s41372-021-01179-3

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/s41372-021-01179-3

Search

Quick links