Frühgeborene an der Grenze der Lebensfähigkeit
                    (Entwicklungsstufe S2k, AWMF-Leitlinien-Register Nr. 024/019, Juni
                    2020)

Christoph Bührer; Ursula Felderhoff-Müser; Ulrich Gembruch; Kurt Hecher; Franz Kainer; Sven Kehl; André Kidszun; Angela Kribs; Tanja Krones; Volker Lipp; Rolf F. Maier; Barbara Mitschdörfer; Tatjana Nicin; Claudia Roll; Monika Schindler

doi:10.1055/a-1230-0810

Zeitschrift für Geburtshilfe und Neonatologie, Table of Contents

Z Geburtshilfe Neonatol 2020; 224(05): 244-254
DOI: 10.1055/a-1230-0810

Leitlinie

Frühgeborene an der Grenze der Lebensfähigkeit (Entwicklungsstufe S2k, AWMF-Leitlinien-Register Nr. 024/019, Juni 2020)

Christoph Bührer

¹Klinik für Neonatologie, Charité – Universitätsmedizin Berlin, Berlin

,

Ursula Felderhoff-Müser

²Zentrum für Kinder- und Jugendmedizin, Universitätsklinikum Essen, Essen

,

Ulrich Gembruch

³Zentrum für Geburtshilfe und Frauenheilkunde, Universitätsklinikum Bonn, Bonn

,

Kurt Hecher

⁴Klinik für Geburtshilfe und Pränatalmedizin, Universitätskrankenhaus Eppendorf, Hamburg

,

Franz Kainer

⁵Abteilung für Geburtshilfe und Pränatalmedizin, Klinik Hallerwiese, Nürnberg

,

Sven Kehl

⁶Frauenklinik, Universitätsklinikum Erlangen, Erlangen

,

André Kidszun

⁷Zentrum für Kinder- und Jugendmedizin, Universitätsmedizin Mainz, Mainz

,

Angela Kribs

⁸Universitätskinderklinik Köln, Köln

,

Tanja Krones

⁹Klinische Ethik, Universitätsspital Zürich, Zürich

,

Volker Lipp

¹⁰Lehrstuhl für Bürgerliches Recht, Zivilprozessrecht, Medizinrecht und Rechtsvergleichung, Juristische Fakultät / Institut für Notarrecht / Zentrum für Medizinrecht, Universität Göttingen, Göttingen

,

Rolf F. Maier

¹¹Zentrum für Kinder- und Jugendmedizin, Universitätsklinikum Marburg, Marburg

,

Barbara Mitschdörfer

¹²Bundesverband „Das frühgeborene Kind“ e.V. Frankfurt

,

Tatjana Nicin

¹³Klinik für Gynäkologie und Geburtshilfe, Klinikum Hanau, Hanau

,

Claudia Roll

¹⁴Abteilung Neonatologie, Pädiatrische Intensivmedizin, Schlafmedizin, Vestische Kinder- und Jugendklinik Datteln, Universität Witten/Herdecke, Datteln

,

Monika Schindler

¹⁵Klinik für Neonatologie, Universitätsmedizin Mannheim, Mannheim

› Author Affiliations

Abstract

Leitlinienreport

Redaktionskomitee
Federführende Fachgesellschaft

Gesellschaft für Neonatologie und Pädiatrische Intensivmedizin
- mandatiert wurden: Christoph Bührer, Ursula Felderhoff-Müser, Angela Kribs, Volker Lipp, Claudia Roll

Full Text

References

Literatur
1 UN-Kinderrechtskonvention. Übereinkommen über die Rechte des Kindes BGBl 1992; II: 121–144
2 Grundgesetz für die Bundesrepublik Deutschland. Art. 1. BGBl 1949; I: 1
3 Grundgesetz für die Bundesrepublik Deutschland. Art. 2. BGBl 1949; I: 1
4 Bundesärztekammer. Grundsätze der Bundesärztekammer zur ärztlichen Sterbebegleitung. Deutsches Ärzteblatt 2011;108: A 346–A 348
5 Lipp V, Brauer D. Behandlungsbegrenzung und „futility” aus rechtlicher Sicht. Z Palliativmed 2013; 14: 121-126
6 Bürgerliches Gesetzbuch. § 630d Abs. 1 S. 1 und 2; eingefügt durch das Gesetz zur Verbesserung der Rechte von Patientinnen und Patienten vom 20. Februar 2013. BGBl 2013; I: 277
7 §1666 Bürgerliches G. geändert durch das Gesetz zur Erleichterung familiengerichtlicherMaßnahmen bei Gefährdung des Kindeswohls. BGBl 2008; I: 1188
8 Schneiderman LJ, Jecker NS, Jonsen AR. Medical futility: its meaning and ethical implications. Ann Intern Med 1990; 112: 949-954
9 Brody BA, Halevy A. Is futility a futile concept?. J Med Philos 1995; 20: 123-144
10 Caplan AL. Odds and ends: trust and the debate over medical futility. Ann Intern Med 1996; 125: 688-689
11 Schneiderman LJ. Defining Medical Futility and Improving Medical Care. J Bioeth Inq 2011; 8: 123-131
12 Garten L, Globisch M, von der Hude K. et al. Leitsätze für Palliativversorgung und Trauerbegleitung in der Peri- und Neonatologie (PaluTiN). Bundesverband „Das frühgeborene Kind” eV. 2018
13 Truog RD. Is it always wrong to perform futile CPR?. N Engl J Med 2010; 362: 477-479
14 Elwyn G, O'Connor A, Stacey D. et al. Developing a quality criteria framework for patient decision aids: online international Delphi consensus process. BMJ 2006; 333: 417
15 Stacey D, Bennett CL, Barry MJ. et al. Decision aids for people facing health treatment or screening decisions. Cochrane Database Syst Rev. 2011: CD001431
16 Vlemmix F, Warendorf JK, Rosman AN. et al. Decision aids to improve informed decision-making in pregnancy care: a systematic review. BJOG 2013; 120: 257-266
17 Alfaleh KM, Al Luwaimi E, Alkharfi TM. et al. A decision aid for considering indomethacin prophylaxis vs. symptomatic treatment of PDA for extreme low birth weight infants. BMC Pediatr 2011; 11: 78
18 Guillén U, Suh S, Munson D. et al. Development and pretesting of a decision-aid to use when counseling parents facing imminent extreme premature delivery. J Pediatr 2012; 160: 382-387
19 Muthusamy AD, Leuthner S, Gaebler-Uhing C. et al. Supplemental written information improves prenatal counseling: a randomized trial. Pediatrics 2012; 129: e1269-e1274
20 Guillén U, Mackley A, Laventhal N. et al. Evaluating the use of a decision aid for parents facing extremely premature delivery: a randomized trial. J Pediatr 2019; 209: 52-60
21 Haward MF, Gaucher N, Payot A. et al. Personalized Decision Making: Practical Recommendations for Antenatal Counseling for Fragile Neonates. Clin Perinatol 2017; 44: 429-445
22 Charles C, Gafni A, Whelan T. Shared decision-making in the medical encounter: what does it mean? (or it takes at least two to tango). Soc Sci Med 1997; 44: 681-692
23 Charles CA, Whelan T, Gafni A. et al. Shared treatment decision making: what does it mean to physicians?. J Clin Oncol 2003; 21: 932-936
24 Kaempf JW, Tomlinson MW, Campbell B. et al. Counseling pregnant women who may deliver extremely premature infants: medical care guidelines, family choices, and neonatal outcomes. Pediatrics 2009; 123: 1509-1515
25 Österreichische Gesellschaft für Kinder und Jugendheilkunde. Erstversorgung von Frühgeborenen an der Grenze der Lebensfähigkeit. Leitlinie der Arbeitsgruppe Neonatologie und pädiatrische Intensivmedizin und der Arbeitsgruppe Ethik. Monatsschr Kinderheilk 2005; 153: 711-715
26 Myrhaug HT, Brurberg KG, Hov L. et al. Survival and impairment of extremely premature infants: a meta-analysis. Pediatrics. 2019 143. e20180933
27 Jeschke E, Biermann A, Günster C. et al. Mortality and major morbidity of very-low-birth-weight infants in Germany 2008–2012: a report based on administrative data. Front Pediatr 2016; 4: 23
28 Anderson JG, Baer RJ, Partridge JC. et al. Survival and major morbidity of extremely preterm infants: a population-based study. Pediatrics. 2016: 138
29 Cheong JLY, Lee KJ, Boland RA. et al. Changes in long-term prognosis with increasing postnatal survival and the occurrence of postnatal morbidities in extremely preterm infants offered intensive care: a prospective observational study. Lancet Child Adolesc Health 2018; 2: 872-879
30 Deshmukh M, Patole S. Antenatal corticosteroids in impending preterm deliveries before 25 weeksʼ gestation. Arch Dis Child Fetal Neonatal Ed 2018; 103: F173-F176
31 Travers CP, Clark RH, Spitzer AR. et al. Exposure to any antenatal corticosteroids and outcomes in preterm infants by gestational age: prospective cohort study. BMJ 2017; 356: j1039
32 Ehret DEY, Edwards EM, Greenberg LT. et al. Association of antenatal steroid exposure with survival among infants receiving postnatal life support at 22 to 25 weeksʼ gestation. JAMA Netw Open 2018; 1: e183235
33 Rysavy MA, Bell EF, Iams JD. et al. Discordance in antenatal corticosteroid use and resuscitation following extremely preterm birth. J Pediatr 2019; 208: 156-62 e5
34 Wei JC, Catalano R, Profit J. et al. Impact of antenatal steroids on intraventricular hemorrhage in very-low-birth weight infants. J Perinatol 2016; 36: 352-356
35 Boghossian NS, McDonald SA, Bell EF. et al. Association of antenatal corticosteroids with mortality, morbidity, and neurodevelopmental outcomes in extremely preterm multiple gestation infants. JAMA Pediatr 2016; 170: 593-601
36 Kiechl-Kohlendorfer U, Simma B, Urlesberger B. et al. Low mortality and short-term morbidity in very preterm infants in Austria 2011–2016. Acta Pædiatr 2019; 108: 1419-1426
37 Trotter A, Pohlandt F. Aktuelle Ergebnisqualität der Versorgung von Frühgeborenen <1500 g Geburtsgewicht als Grundlage für eine Regionalisierung der Risikogeburten. Z Geburtsh Neonatol 2010; 214: 55-61
38 Hentschel R, Guenther K, Vach W. et al. Risk-adjusted mortality of VLBW infants in high-volume versus low-volume NICUs. Arch Dis Child Fetal Neonatal Ed 2019; 104: F390-F395
39 Boland RA, Davis PG, Dawson JA. et al. Predicting death or major neurodevelopmental disability in extremely preterm infants born in Australia. Arch Dis Child Fetal Neonatal Ed 2013; 98: F201-F204
40 Hüseman D, Metze B, Walch E. et al. Laboratory markers of perinatal acidosis are poor predictors of neurodevelopmental impairment in very low birth weight infants. Early Hum Dev 2011; 87: 677-681
41 Genzel-Boroviczény O, Hempelman J, Zoppelli L. et al. Predictive value of the 1-min Apgar score for survival at 23–26 weeks gestational age. Acta Pædiatr 2010; 99: 1790-1794
42 Singh J, Fanaroff J, Andrews B. et al. Resuscitation in the “gray zone” of viability: determining physician preferences and predicting infant outcomes. Pediatrics 2007; 120: 519-526
43 Manley BJ, Dawson JA, Kamlin CO. et al. Clinical assessment of extremely premature infants in the delivery room is a poor predictor of survival. Pediatrics 2010; 125: e559-e564
44 Edstedt Bonamy AK, Zeitlin J, Piedvache A. et al. Wide variation in severe neonatal morbidity among very preterm infants in European regions. Arch Dis Child Fetal Neonatal Ed 2019; 104: F36-F45
45 Moore T, Hennessy EM, Myles J. et al. Neurological and developmental outcome in extremely preterm children born in England in 1995 and 2006: the EPICure studies. BMJ 2012; 345: e7961
46 Patel RM, Kandefer S, Walsh MC. et al. Causes and timing of death in extremely premature infants from 2000 through 2011. N Engl J Med 2015; 372: 331-340
47 Younge N, Goldstein RF, Cotten CM. Eunice Kennedy Shriver National Institute of Child H, Research Network HDN. Survival and neurodevelopment of periviable infants. N Engl J Med 2017; 376: 1890 1
48 Zegers MJ, Hukkelhoven CW, Uiterwaal CS. et al. Changing Dutch approach and trends in short-term outcome of periviable preterms. Arch Dis Child Fetal Neonatal Ed 2016; 101: F391-F396
49 Chen F, Bajwa NM, Rimensberger PC. et al. Swiss Neonatal N. Thirteen-year mortality and morbidity in preterm infants in Switzerland. Arch Dis Child Fetal Neonatal Ed 2016; 101: F377 83
50 Norman M, Hallberg B, Abrahamsson T. et al. Association between year of birth and 1-year survival among extremely preterm infants in Sweden during 2004-2007 and 2014–2016. JAMA 2019; 321: 1188-1199
51 Salas AA, Carlo WA, Ambalavanan N. et al. Gestational age and birthweight for risk assessment of neurodevelopmental impairment or death in extremely preterm infants. Arch Dis Child Fetal Neonatal Ed 2016; 101: F494-F501
52 Brumbaugh JE, Hansen NI, Bell EF. et al. Outcomes of extremely preterm infants with birth weight less than 400 g. JAMA Pediatr 2019; 173: 434-445
53 Inoue H, Ochiai M, Yasuoka K. et al. Early mortality and morbidity in infants with birth weight of 500 grams or less in Japan. J Pediatr 2017; 190: 112-7 e3
54 Inoue H, Ochiai M, Sakai Y. et al. Neurodevelopmental outcomes in infants with birth weight ≤500 g at 3 years of age. Pediatrics 2018; 142: e20174286
55 Ding S, Lemyre B, Daboval T. et al. A meta-analysis of neurodevelopmental outcomes at 4–10 years in children born at 22–25 weeks gestation. Acta Pædiatr 2018; 108: 1237-1244
56 Hafström M, Kallén K, Serenius F. et al. Cerebral palsy in extremely preterm infants. Pediatrics. 2018 141. e20171433
57 Serenius F, Ewald U, Farooqi A. et al. Neurodevelopmental outcomes among extremely preterm infants 6.5 years after active perinatal care in Sweden. JAMA Pediatr 2016; 170: 954-963
58 Cheong JLY, Anderson PJ, Burnett AC. et al. Changing neurodevelopment at 8 years in children born extremely preterm since the 1990s. Pediatrics. 2017 139. e20164086
59 Kono Y, Yonemoto N, Nakanishi H. et al. Changes in survival and neurodevelopmental outcomes of infants born at <25 weeksʼ gestation: a retrospective observational study in tertiary centres in Japan. BMJ Paediatr Open 2018; 2: e000211
60 Twilhaar ES, Wade RM, de Kieviet JF. et al. Cognitive outcomes of children born extremely or very preterm since the 1990s and associated risk factors: a meta-analysis and meta-regression. JAMA Pediatr 2018; 172: 361-367
61 Spittle AJ, Cameron K, Doyle LW. et al. Victorian Infant Collaborative Study G. Motor impairment trends in extremely preterm children: 1991–2005. Pediatrics 2018; 141
62 Bolk J, Farooqi A, Hafström M. et al. Developmental coordination disorder and its association with developmental comorbidities at 6.5 years in apparently healthy children born extremely preterm. JAMA Pediatr 2018; 172: 765-774
63 Samuelsson M, Holsti A, Adamsson M. et al. Behavioral patterns in adolescents born at 23 to 25 weeks of gestation. Pediatrics 2017; 140: e20170199
64 Pignotti MS, Donzelli G. Perinatal care at the threshold of viability: an international comparison of practical guidelines for the treatment of extremely preterm births. Pediatrics 2008; 121: e193-e198
65 Guillén Ú, Weiss EM, Munson D. et al. Guidelines for the management of extremely premature deliveries: a systematic review. Pediatrics 2015; 136: 343-350
66 Kattwinkel J, Perlman JM, Aziz K. et al. Part 15: neonatal resuscitation: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Circulation 2010; 122: S909-S919
67 Kattwinkel J, Perlman JM, Aziz K. et al. Neonatal resuscitation: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Pediatrics 2010; 126: e1400-e1413
68 Richmond S, Wyllie J. European Resuscitation Council Guidelines for Resuscitation 2010 Section 7. Resuscitation of babies at birth. Resuscitation 2010; 81: 1389-1399
69 Moriette G, Rameix S, Azria E. et al. Naissances tres prematurées: dilemmes et propositions de prise en charge. Première partie: pronostic des naissances avant 28 semaines, identification d'une zone grise. Arch Pédiatr 2010; 17: 518-526
70 Moriette G, Rameix S, Azria E. et al. Naissances tres prematurées: dilemmes et propositions de prise en charge. Seconde partie: enjeux éthiques, principes de prise en charge et recommandations. Arch Pédiatr 2010; 17: 527-539
71 de Laat MW, Wiegerinck MM, Walther FJ. et al. Richtlijn ‘Perinataal beleid bij extreme vroeggeboorteʼ. Ned Tijdschr Geneeskd 2010; 154: A2701
72 Berger TM, Bernet V, El Alama S. et al. Perinatal care at the limit of viability between 22 and 26 completed weeks of gestation in Switzerland. 2011 revision of the Swiss recommendations. Swiss Med Wkly 2010; 141: w13280
73 Pohlandt F. Frühgeburt an der Grenze der Lebensfähigkeit des Kindes. AMWF- Leitlinien-Register. Z Geburtsh Neonatol 2008; 212: 109-113
74 Berger A, Kiechl-Kohlendorfer U, Berger J. et al. Update: Erstversorgung von Frühgeborenen an der Grenze der Lebensfähigkeit. Aktualisierte gemeinsame Leitlinie der Arbeitsgruppe Neonatologie und pädiatrische Intensivmedizin der Österreichischen Gesellschaft für Kinder- und Jugendheilkunde (ÖGKJ), der Arbeitsgruppe Ethik in der Kinder- und Jugendheilkunde der ÖGKJ sowie des Instituts für Ethik und Recht in der Medizin der UniversitätWien (IERM). Monatsschr Kinderheilkd 2019; 167: 36-45
75 Domellöf M, Pettersson K. Riktlinjer vid hotande fortidsbord ska ge battre och mer jamlik vard – Konsensusdokument for enhetligt omhandertagande av gravida och extremt for tidigt fodda barn. Lakartidningen 2017; 114: EEYI
76 Cuttini M, Nadai M, Kaminski M. et al. End-of-life decisions in neonatal intensive care: physicians' self-reported practices in seven European countries. EURONIC Study Group. Lancet 2000; 355: 2112-2118
77 Wilkinson D, Verhagen E, Johansson S. Thresholds for resuscitation of extremely preterm infants in the UK, Sweden, and Netherlands. Pediatrics 2018; 142: S574-S584
78 Schneider K, Metze B, Bührer C. et al. End-of-life decisions 20 years after EURONIC: neonatologists' self-reported practices, attitudes, and treatment choices in Germany, Switzerland, and Austria. J Pediatr 2019; 207: 154-160
79 Brown SD, Donelan K, Martins Y. et al. Differing attitudes toward fetal care by pediatric and maternal-fetal medicine specialists. Pediatrics 2012; 130: e1534-e1540
80 Guinsburg R, Branco de Almeida MF, Dos Santos Rodrigues Sadeck L. et al. Proactive management of extreme prematurity: disagreement between obstetricians and neonatologists. J Perinatol 2012; 32: 913-919
81 Diguisto C, Goffinet F, Lorthe E. et al. Providing active antenatal care depends on the place of birth for extremely preterm births: the EPIPAGE 2 cohort study. Arch Dis Child Fetal Neonatal Ed 2017; 102: F476-F482
82 Diguisto C, Foix L'Helias L, Morgan AS. et al. Neonatal outcomes in extremely preterm newborns admitted to intensive care after no active antenatal management: a population-based cohort study. J Pediatr 2018; 203: 5
83 Barber CA, Sikes NC, Norton JD. et al. Effects of mode of delivery on mortality and severe brain injury in extremely low birth weight infants in Arkansas. J Ark Med Soc 2007; 104: 63-66
84 American College of Obstetricians and Gynecologists. Obstetric care consensus no. 6: periviable birth. Obstet Gynecol 2017; 130: e187-e199
85 Carlo WA, McDonald SA, Fanaroff AA. et al. Association of antenatal corticosteroids with mortality and neurodevelopmental outcomes among infants born at 22 to 25 weeksʼ gestation. JAMA 2011; 306: 2348-2358
86 Mori R, Kusuda S, Fujimura M. Antenatal corticosteroids promote survival of extremely preterm infants born at 22 to 23 weeks of gestation. J Pediatr 2011; 159: 110-4 e1
87 Ochiai M, Kinjo T, Takahata Y. et al. Survival and neurodevelopmental outcome of preterm infants born at 22–24 weeks of gestational age. Neonatology 2014; 105: 79-84
88 Geurtzen R, van Heijst AFJ, Draaisma JMT. et al. Development of nationwide recommendations to support prenatal counseling in extreme prematurity. Pediatrics 2019; 143
89 Cummings J, CoFa Newborn. Antenatal counseling regarding resuscitation and intensive care before 25 weeks of gestation. Pediatrics 2015; 136: 588-595
90 Humphrey JD, Hagan JL, Suresh GK. et al. Neonatologists less frequently discuss long term complications: A simulation-based study of prenatal consults beyond the threshold of viability. J Neonatal Perinatal Med 2019; 12: 87-94
91 Caeymaex L, Speranza M, Vasilescu C. et al. Living with a crucial decision: a qualitative study of parental narratives three years after the loss of their newborn in the NICU. PLoS One 2011; 6: e28633
92 Orfali K, Gordon EJ. Autonomy gone awry: a cross-cultural study of parentsʼ experiences in neonatal intensive care units. Theor Med Bioeth 2004; 25: 329-365
93 Griswold KJ, Fanaroff JM. An evidence-based overview of prenatal consultation with a focus on infants born at the limits of viability. Pediatrics 2010; 125: e931-e937
94 Garten L, von der Hude K, Rösner B. et al. Familienzentrierte Sterbe- und individuelle Trauerbegleitung an einem Perinatalzentrum. Z Geburtsh Neonatol 2013; 217: 95-102
95 Wool C, Limbo R, Denny-Koelsch EM. “I Would Do It All Over Again”: Cherishing Time and the Absence of Regret in Continuing a Pregnancy after a Life-Limiting Diagnosis. J Clin Ethics 2018; 29: 227-236
96 Baumann-Hölzle R, Maffezzoni M, Bucher HU. A framework for ethical decision making in neonatal intensive care. Acta Pædiatr 2005; 94: 1777-1783
97 Schulz-Baldes A, Hüseman D, Loui A. et al. Neonatal end-of-life practice in a German perinatal centre. Acta Pædiatr 2007; 96: 681-687
98 American College of Obstetricians and Gynecologists. Perinatal Palliative Care (ACOG committee opinion, number 786). Obstet Gynecol 2019; 134: e84-e89
99 Tyson JE, Parikh NA, Langer J. et al. Intensive care for extreme prematurity – moving beyond gestational age. N Engl J Med 2008; 358: 1672-1681