INTRODUCTION: The aim of this study was to present our current practice of counseling patients and families with the most severe congenital malformations in the 3rd trimester of pregnancy and to develop practical guidelines for our team and involved healthcare/ socialcare professionals. MATERIAL & METHODS: It was a retrospective evaluation of a series of fetal cases in 2017 from single tertiary center. Maternal obstetrical medical history, time of prenatal detection of the anomaly (1st, 2nd or 3rd trimester), time between last fetal echocardiography and delivery, type of delivery, neonatal birth weight and time of neonatal demise. The total study group was subdived into early demise (during the 1st day after delivery) or late demise > 1st day after delivery. RESULTS: Mean maternal age was 30,4 +/- 5,6 years, and varied between 26 and 38 years. No chronic maternal diseases were found in medical history and no congenital malformations were present in previous children. All women had 1st trimester ultrasound, in 9 cases, it was reported as normal (with NT measurement < 2 mm), in 2 cases extracardiac abnormalities were detected: diaphragmatic hernia and omphalocele ( in both fetal karyotype 46,XY). In nine cases, the abnormalities were detected in midgestation and with maternal wish to continue the pregnancies. There were 8 neonatal deaths within 60 minutes after delivery, including one intrapartum death and 3 “late” neonatal deaths in the intensive care unit (on 12th, 21st and 22nd day). We stress upon the prenatal team approach and counseling of future parents, in order to prepare them for poor neonatal outcome. CONCLUSIONS: 1. In the most severe cases when fetal or neonatal demise was suspected, the two different opinions of specialists might not be enough and a third opinion should be recommended before final decision. 2. A Fetal Team of specialists is necessary in cases of expected fetal/neonatal demise in order to prepare a written report of recommended perinatal management for all sides involved in this difficult problem.
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1. Parravicini E, Lorenz JM Neonatal outcomes of fetuses diagnosed with life-limiting conditions when individualized comfort measures are proposed. J Perinatol. 2014;34(6):483-487
2. Respondek-Liberska M, Czichosz E, Nowak S, Sobantka S, Maroszyńska I, Gulczyńska E, Janiak K, Lukaszek S, Krasomski G, Szpakowski M. Analysis of perinatal death at the Institute of the Health Center of the Polish mother in 1995, 1996 and 1997. The reason for making changes in the accountability for perinatal death]. Ginekol Pol. 1999;70(9):581-587.
3. Arias E, MacDorman MF, Strobino DM, Guyer B. Annual summary of vital statistics 2002. Pediatrics. 2003;112(6):1215-1230.
4. Lakhoo K. Fetal counselling for surgical conditions. Early Hum Dev. 2012;88(1):9-13.
5. Więckowska K, Dudarewicz L, Moczulska H, Słodki M, Pietrzak Z, Respondek-Liberska M.: Postnatal outcomes of children with prenatally diagnosed congenital heart disease combined with congenital diaphragmatic hernia. Prenat Cardio. 2014 Dec;4(4):23-27
6. Respondek-Liberska M, Foryś S, Janiszewska-Skorupa J, Szaflik K, Wilczyński J, Oszukowski P, Krasomski G, Maroszyńska I, Biegański T, Kulig A, Jakubowski L, Chilarski A. Diaphragmatic hernia in reference hospital ICZMP-diagnostic problems and outcome. Ginekol Pol. 2008 ;79(1):23-30.
7. Gallot D, Coste K, Francannet C, Laurichesse H, Boda C, Ughetto S, Vanlieferinghen P, Scheye T, Vendittelli F, Labbe A, Dechelotte PJ, Sapin V, Lemery D. Antenatal detection and impact on outcome of congenital diaphragmatic hernia: a 12-year experience in Auvergne, France. Eur J Obstet Gynecol Reprod Biol. 2006;125(2):202-205.
8. Gratacós E, Carreras E, Becker J, Lewi L, Enríquez G, Perapoch J, Higueras T, Cabero L, Deprest J. Prevalence of neurological damage in monochorionic twins with selective intrauterine growth restriction and intermittent absent or reversed end-diastolic umbilical artery flow. Ultrasound Obstet Gynecol. 2004;24(2):159-163.
9. Pakkasjärvi N, Ritvanen A, Herva R, Peltonen L, Kestilä M, Ignatius J. Lethal congenital contracture syndrome (LCCS) and other lethal arthrogryposes in Finland-an epidemiological study. Am J Med Genet A. 2006;140(17):1834-1839.
10. Milner R, Adzick NS. Perinatal management of fetal malformations amenable to surgical correction. Curr Opin Obstet Gynecol. 1999;11(2):177-183.
11. Stevens GH, Schoot BC, Smets MJ, Kremer B, Manni JJ, Gavilanes AW, Wilmink JT, van Heurn LW, Hasaart TH. The ex utero intrapartum treatment (EXIT) procedure in fetal neck masses: a case report and review of the literature. Eur J Obstet Gynecol Reprod Biol. 2002 10;100(2):246-250.
12. Matte GS, Connor KR, Toutenel NA, Gottlieb D, Fynn-Thompson F.Modified EXIT-to-ECMO with Optional Reservoir Circuit for Use during an EXIT Procedure Requiring Thoracic Surgery. JECT. 2016;48:35-38
13. 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(6):940-945.
14. Escobar-Diaz MC, Sunderji S, Tworetzky W, Moon-Grady AJ.The Fetus with Ectopia Cordis: Experience and Expectations from Two Centers. Pediatr Cardiol. 2017;38(3):531-538.
15. Respondek-Liberska M, Janiak K, Kaczmarek P, Borowski D, Czichos E The significance of cardiography in the diagnosis of cardiac ectopia of the fetus. Ginekol Pol. 1998; 69(3):139-144.
16. Sadłecki P, Krekora M, Krasomski G, Walentowicz-Sadłecka M, Grabiec M, Moll J, Respondek-Liberska M. Prenatally evolving ectopia cordis with successful surgical treatment. Fetal Diagn Ther. 2011;30(1):70-72.
17. Pośpiech-Gąsior K, Słodki M, Respondek-Liberska M. What is the survival rate in prenatally detected Cantrell’s pentalogy ? Prenat Cardio 2016; 6(1):31-36.
18. Sharma S, Bhanot R, Deka D, Bajpai M, Gupta DK. Impact of fetal counseling on outcome of antenatal congenital surgical anomalies. Pediatr Surg Int. 2017;33(2):203-212.
19. Munson D, Leuthner SR. Palliative care for the family carrying a fetus with a life-limiting diagnosis. Pediatr Clin North Am. 2007;54(5):787-798.
20. Frank F, Maurer F, Pehlke-Milde J, Fleming V. Dying at Life’s Beginning. Gesundheitswesen. 2017;27.
21. Bhatia J. Palliative care in the fetus and newborn. J Perinatol. 2006; 26(1):24-26.
22. Catlin A, Carter B. Creation of a neonatal end-of-life palliative care protocol. J Perinatol. 2002;22(3):184-195.
23. Thiele P. He was my son, not a dying baby. J Med Ethics. 2010;36(11):646- 647.
24. Leuthner S, Jones EL. Fetal Concerns Program: a model for perinatal palliative care. MCN Am J Matern Child Nurs. 2007;32(5):272-278.
25. Wilkinson D, de Crespigny L, Xafis V. Ethical language and decision-making for prenatally diagnosed lethal malformations. Semin Fetal Neonatal Med. 2014;19(5):306-311.
26. Sumner LH, Kavanaugh K, Moro T. Extending palliative care into pregnancy and the immediate newborn period: state of the practice of perinatal palliative care. J Perinat Neonatal Nurs. 2006; 20(1):113-116.
27. Kumar P. Care of an infant with lethal malformation: where do we draw the line? Pediatrics. 2011;128(6):1642-1644.
1. Krekora M, Słodki M, Gulczyńska E, Maroszyńska I, Respondek-Liberska M. Prenatal 3rd trimester expectation of fetal or neonatal demise and perinatal team approach Prenat Cardio 2017 Jan; 7(1):19-25
2. Chervenak FA, McCullough LB. The Professional Responsibility Model of Perinatal Ethics. Berlin: Walter de Gruyter, 2014.