Year 2023,
, 185 - 190, 02.10.2023
Merve Öztürk Ağaoğlu
,
Atakan Tanaçan
,
Zahid Ağaoğlu
,
Özgür Kara
,
Dilek Sahin
References
- 1. Sparks TN, Lianoglou BR, Adami RR, et al. Exome Sequencing for Prenatal Diagnosis in Nonimmune Hydrops Fetalis. N Engl J Med. 2020;383(18):1746-1756.
- 2. Norton ME, Chauhan SP, Dashe JS, (SMFM)
SfM-FM. Society for maternal-fetal medicine
(SMFM) clinical guideline #7: nonimmune hydrops
fetalis. Am J Obstet Gynecol. 2015;212(2):127-139.
- 3. Sparks TN, Thao K, Lianoglou BR, et al. Nonimmune hydrops fetalis: identifying the underlying
genetic etiology. Genet Med. 2019;21(6):1339-1344.
- 4. Lallemand AV, Doco-Fenzy M, Gaillard DA.
Investigation of nonimmune hydrops fetalis: multidisciplinary studies are necessary for diagnosis--review
of 94 cases. Pediatr Dev Pathol. 1999;2(5):432-439.
- 5. Bellini C, Hennekam RC. Non-immune hyd-
190
rops fetalis: a short review of etiology and pathophysiology. Am J Med Genet A. 2012;158A(3):597-605.
- 6. Bellini C, Hennekam RC, Fulcheri E, et al.
Etiology of nonimmune hydrops fetalis: a systematic
review. Am J Med Genet A. 2009;149A(5):844-851.
- 7. Beksaç MS, Saygan-Karamürsel B, Ustaçelebi
S, et al. Prenatal diagnosis of intrauterine cytomegalovirus infection in a fetus with non-immune hydrops fetalis. Acta Obstet Gynecol Scand. 2001;80(8):762-765.
- 8. 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(9):487-490.
- 9. Turgal M, Ozyuncu O, Boyraz G, Yazicioglu A, Sinan Beksac M. Non-immune hydrops fetalis
as a diagnostic and survival problems: what do we
tell the parents? J Perinat Med. 2015;43(3):353-358.
- 10. Kaykı G, Güçer Ş, Akçören Z, et al.
Non-immune hydrops fetalis: A retrospective
analysis of 151 autopsies performed at a single center. Turk J Pediatr. 2018;60(5):471-477.
- 11. Galindo A, Nieto O, Villagrá S, Grañeras
A, Herraiz I, Mendoza A. Hypoplastic left heart syndrome diagnosed in fetal life: associated findings,
pregnancy outcome and results of palliative surgery.
Ultrasound Obstet Gynecol. 2009;33(5):560-566.
- 12. Allen RH, Benson CB, Haug LW. Pregnancy outcome of fetuses with a diagnosis of
hypoplastic left ventricle on prenatal sonography. J Ultrasound Med. 2005;24(9):1199-1203.
- 13. Schmider A, Henrich W, Dähnert I, Dudenhausen JW. Prenatal therapy of non-immunologic
hydrops fetalis caused by severe aortic stenosis.
Ultrasound Obstet Gynecol. 2000;16(3):275-278.
- 14. Knilans TK. Cardiac abnormalities associated with hydrops fetalis. Semin Perinatol. 1995;19(6):483-492.
- 15. Strasburger JF, Kugler JD, Cheatham JP,
McManus BM. Nonimmunologic hydrops fetalis associated with congenital aortic valvular
stenosis. Am Heart J. 1984;108(5):1380-1382.
- 16. Robertson MA, Byrne PJ, Penkoske PA. Perinatal management of critical aortic valve stenosis diagnosed by fetal echocardiography. Br Heart J. 1989;61(4):365-367.
- 17. Divanović A, Witte D, Michelfelder E.
A fetus with hypoplastic left heart syndrome
and tricuspid stenosis with evolving hydrops fetalis. Pediatr Cardiol. 2012;33(7):1210-1212.
- 18. Huhta JC. Guidelines for the evaluation of heart failure in the fetus with or without
hydrops. Pediatr Cardiol. 2004;25(3):274-286.
- 19. Gest AL, Martin CG, Moïse AA, Hansen TN. Reversal of venous blood flow
with atrial tachycardia and hydrops in fetal sheep. Pediatr Res. 1990;28(3):223-226.
- 20. Liu CA, Huang HC, Chou YY. Retrospective analysis of 17 liveborn neonates with hydrops
fetalis. Chang Gung Med J. 2002;25(12):826-831.
- 21. Tongsong T, Wanapirak C, Piyamongkol W,
et al. Fetal ventricular shortening fraction in hydrops fetalis. Obstet Gynecol. 2011;117(1):84-91.
- 22. Czernik C, Stiller B, Hübler M, Hagen A, Henrich W. Hydrops fetalis caused
by a large intrapericardial teratoma. Ultrasound Obstet Gynecol. 2006;28(7):973-976.
- 23. Kamil D, Tepelmann J, Berg C, et al. Spectrum
and outcome of prenatally diagnosed fetal tumors.
Ultrasound Obstet Gynecol. 2008;31(3):296-302.
- 24. Fotiou E, Martin-Almedina S, Simpson MA, et
al. Novel mutations in PIEZO1 cause an autosomal recessive generalized lymphatic dysplasia with non-immune hydrops fetalis. Nat Commun. 2015;6:8085.
- 25. Santolaya J, Alley D, Jaffe R, Warsof SL. Antenatal classification of hydrops fetalis. Obstet Gynecol. 1992;79(2):256-259.
- 26. Has R. Non-immune hydrops fetalis in the first trimester: a review of 30 cases.
Clin Exp Obstet Gynecol. 2001;28(3):187-190.
A Retrospective Analysis of Cases of Non-Immune Hydrops Fetalis in A Tertiary Center
Year 2023,
, 185 - 190, 02.10.2023
Merve Öztürk Ağaoğlu
,
Atakan Tanaçan
,
Zahid Ağaoğlu
,
Özgür Kara
,
Dilek Sahin
Abstract
Introduction: Non-immune hydrops fetalis (NIHF) can occur at different gestational ages and with different etiologies. The aim of this study was to investigate the association of gestational age at diagnosis and a novel clinical scoring system with survival in NIHF cases
Methods: This retrospective study was conducted between January 2020 and January 2023 in the perinatology clinic of a tertiary care center. Maternal characteristics, causes of NIHF, and survival rate were analyzed. The hydrops score was calculated and compared for those less than and greater than 20 weeks.
Results: Of 41 NIHF, etiology was determined in 76% (87% <20 weeks vs. 70% ≥20 weeks), including cardiovascular malformations (27%), cystic hygromas (17%), and chromosomal defects (12%). Cystic hygromas were more common before 20 weeks. Cardiac malformations were the most common cause after 20 weeks. There was a negative correlation between live birth and hydrops score. The overall survival was 7.3%. The most favorable overall survival is in NIHF cases associated with fetal arrhythmias and placental pathology for the second and third trimesters, respectively.
Conclusion: Earlier gestational age at diagnosis and higher hydrops score are associated with lower survival rates in NIHF cases. Determining the cause, accompanying structural abnormalities, and the week NIHF is diagnosed will help predict prognosis and apply treatments earlier to improve care for these fetuses and newborns
Supporting Institution
Ankara Bilkent Şehir Hastanesi
References
- 1. Sparks TN, Lianoglou BR, Adami RR, et al. Exome Sequencing for Prenatal Diagnosis in Nonimmune Hydrops Fetalis. N Engl J Med. 2020;383(18):1746-1756.
- 2. Norton ME, Chauhan SP, Dashe JS, (SMFM)
SfM-FM. Society for maternal-fetal medicine
(SMFM) clinical guideline #7: nonimmune hydrops
fetalis. Am J Obstet Gynecol. 2015;212(2):127-139.
- 3. Sparks TN, Thao K, Lianoglou BR, et al. Nonimmune hydrops fetalis: identifying the underlying
genetic etiology. Genet Med. 2019;21(6):1339-1344.
- 4. Lallemand AV, Doco-Fenzy M, Gaillard DA.
Investigation of nonimmune hydrops fetalis: multidisciplinary studies are necessary for diagnosis--review
of 94 cases. Pediatr Dev Pathol. 1999;2(5):432-439.
- 5. Bellini C, Hennekam RC. Non-immune hyd-
190
rops fetalis: a short review of etiology and pathophysiology. Am J Med Genet A. 2012;158A(3):597-605.
- 6. Bellini C, Hennekam RC, Fulcheri E, et al.
Etiology of nonimmune hydrops fetalis: a systematic
review. Am J Med Genet A. 2009;149A(5):844-851.
- 7. Beksaç MS, Saygan-Karamürsel B, Ustaçelebi
S, et al. Prenatal diagnosis of intrauterine cytomegalovirus infection in a fetus with non-immune hydrops fetalis. Acta Obstet Gynecol Scand. 2001;80(8):762-765.
- 8. 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(9):487-490.
- 9. Turgal M, Ozyuncu O, Boyraz G, Yazicioglu A, Sinan Beksac M. Non-immune hydrops fetalis
as a diagnostic and survival problems: what do we
tell the parents? J Perinat Med. 2015;43(3):353-358.
- 10. Kaykı G, Güçer Ş, Akçören Z, et al.
Non-immune hydrops fetalis: A retrospective
analysis of 151 autopsies performed at a single center. Turk J Pediatr. 2018;60(5):471-477.
- 11. Galindo A, Nieto O, Villagrá S, Grañeras
A, Herraiz I, Mendoza A. Hypoplastic left heart syndrome diagnosed in fetal life: associated findings,
pregnancy outcome and results of palliative surgery.
Ultrasound Obstet Gynecol. 2009;33(5):560-566.
- 12. Allen RH, Benson CB, Haug LW. Pregnancy outcome of fetuses with a diagnosis of
hypoplastic left ventricle on prenatal sonography. J Ultrasound Med. 2005;24(9):1199-1203.
- 13. Schmider A, Henrich W, Dähnert I, Dudenhausen JW. Prenatal therapy of non-immunologic
hydrops fetalis caused by severe aortic stenosis.
Ultrasound Obstet Gynecol. 2000;16(3):275-278.
- 14. Knilans TK. Cardiac abnormalities associated with hydrops fetalis. Semin Perinatol. 1995;19(6):483-492.
- 15. Strasburger JF, Kugler JD, Cheatham JP,
McManus BM. Nonimmunologic hydrops fetalis associated with congenital aortic valvular
stenosis. Am Heart J. 1984;108(5):1380-1382.
- 16. Robertson MA, Byrne PJ, Penkoske PA. Perinatal management of critical aortic valve stenosis diagnosed by fetal echocardiography. Br Heart J. 1989;61(4):365-367.
- 17. Divanović A, Witte D, Michelfelder E.
A fetus with hypoplastic left heart syndrome
and tricuspid stenosis with evolving hydrops fetalis. Pediatr Cardiol. 2012;33(7):1210-1212.
- 18. Huhta JC. Guidelines for the evaluation of heart failure in the fetus with or without
hydrops. Pediatr Cardiol. 2004;25(3):274-286.
- 19. Gest AL, Martin CG, Moïse AA, Hansen TN. Reversal of venous blood flow
with atrial tachycardia and hydrops in fetal sheep. Pediatr Res. 1990;28(3):223-226.
- 20. Liu CA, Huang HC, Chou YY. Retrospective analysis of 17 liveborn neonates with hydrops
fetalis. Chang Gung Med J. 2002;25(12):826-831.
- 21. Tongsong T, Wanapirak C, Piyamongkol W,
et al. Fetal ventricular shortening fraction in hydrops fetalis. Obstet Gynecol. 2011;117(1):84-91.
- 22. Czernik C, Stiller B, Hübler M, Hagen A, Henrich W. Hydrops fetalis caused
by a large intrapericardial teratoma. Ultrasound Obstet Gynecol. 2006;28(7):973-976.
- 23. Kamil D, Tepelmann J, Berg C, et al. Spectrum
and outcome of prenatally diagnosed fetal tumors.
Ultrasound Obstet Gynecol. 2008;31(3):296-302.
- 24. Fotiou E, Martin-Almedina S, Simpson MA, et
al. Novel mutations in PIEZO1 cause an autosomal recessive generalized lymphatic dysplasia with non-immune hydrops fetalis. Nat Commun. 2015;6:8085.
- 25. Santolaya J, Alley D, Jaffe R, Warsof SL. Antenatal classification of hydrops fetalis. Obstet Gynecol. 1992;79(2):256-259.
- 26. Has R. Non-immune hydrops fetalis in the first trimester: a review of 30 cases.
Clin Exp Obstet Gynecol. 2001;28(3):187-190.