Z Geburtshilfe Neonatol 2014; 218(01): 27-33
DOI: 10.1055/s-0034-1367052
Übersicht
© Georg Thieme Verlag KG Stuttgart · New York

Geburtsplanung bei fetalen Fehlbildungen aus neonatologischer Sicht

Planning Delivery in Cases of Congenital Malformations from the Neonatologist’s Point of View
A. Werlein
1   Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Marburg
,
P. Goebert
1   Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Marburg
,
R. F. Maier
1   Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Marburg
› Institutsangaben
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Publikationsverlauf

eingereicht 12. November 2013

angenommen nach Überarbeitung 16. Januar 2014

Publikationsdatum:
04. März 2014 (online)

Zusammenfassung

Mithilfe der pränatalen Sonografie ist heute ein Großteil schwerer angeborener Fehlbildungen lange vor dem Geburtstermin bekannt. Das eröffnet die Möglichkeit, die Geburt des betroffenen Kindes im Voraus zu planen und vorzubereiten. In der vorliegenden Übersicht wird für einige typische angeborene Fehlbildungen zur Wahl von Geburtsort, Geburtszeitpunkt und Geburtsmodus Stellung aus neonatologischer Sicht bezogen. Da diesbezüglich nur wenig Evidenz vorliegt, besteht dringender Bedarf an prospek­tiven Studien. Die Antwort auf die Frage nach dem Geburtsort bei einer fetalen Fehlbildung ist von mehreren wissenschaftlichen Fachgesellschaften in einer AWMF-Leitlinie konsentiert. Danach soll bei Diagnose einer schwerwiegenden fetalen Fehlbildungen die Schwangere in ein Perinatalzentrum eingewiesen werden. Die Geburt außerhalb eines Perinatalzentrums verschlechtert die Prognose hinsichtlich Mortalität bei der Zwerchellhernie und hinsichtlich Komplikationen bei der Gastroschisis. Bei der Wahl des Geburtszeitpunktes gilt es, die Risiken einer iatrogenen Frühgeburt gegen den potentiellen Nutzen für das Neugeborene abzuwägen. Nur bei wenigen Fehlbildungen gibt es Hinweise auf einen Vorteil für das betroffene Kind durch eine vorzeitige Beendigung der Schwangerschaft. Ein geplanter Kaiserschnitt hat bei einer fetalen Fehlbildung in der Regel vor allem organisatorische Gründe, ohne dass medizinische Vorteile für das betroffene Kind belegt sind. Zusammenfassend sollte bei bekannter fetaler Fehlbildung die Planung der Geburt immer im interdisziplinären Gespräch zwischen den prä- und postnatal beteiligten Spezialisten und unter Einbeziehung der werdenden Eltern erfolgen.

Abstract

Nowadays, most major congenital malformations are detected by prenatal ultrasound long before birth. This offers the opportunity to plan and organise the birth of such a baby in the most favourable setting. This paper gives an overview about how to decide on place, timing, and mode of delivery in some typical congenital malformations from the neonatologist’s point of view. As, in most cases scientific evidence is lacking, there is an urgent need for prospective clinical trials. The question concerning the best place of birth is answered in a national guideline, which has been consented by several scientific societies. In this guideline, the transfer of the pregnant woman to a high level perinatal centre is recommended in the case of a known foetal malformation. Delivery outside a perinatal centre has been shown to increase mortality in infants with congenital diaphragmatic hernia and complications in infants born with gastroschisis. With respect to the timing of the delivery, the risks of an iatrogenic preterm birth must be considered in relation to the potential benefit for the newborn baby. ­There seems to be some advantage of a birth before the regular date only in few malformations. In most cases of congenital malformation, only a Caesarean section has a remarkable advantage with respect to the organisation of the delivery room management, but has no medical necessity for the baby. In summary, the delivery of a baby with a congenital malformation should be planned and organised by an interdisciplinary team of prenatal and postnatal specialists in close contact and consent with the parents.

 
  • Literatur

  • 1 Arbeitsgemeinschaft der wissenschaftlichen Fachgesellschaften. S1-Leitlinie: Indikationen zur Einweisung von Schwangeren in Krankenhäuser der adäquaten Versorgungsstufe. 01.06.2008 http://www.awmf.org/uploads/tx_szleitlinien/024-001_S1_Indikationen_zur_Einweisung_von_Schwangeren_in_Krankenhaeuser_der_adaequaten_Versorgungsstufe.pdf
  • 2 Boutsikou T, Malamitsi-Puchner A. Caesarean section: impact on mother and child. Acta Paediatr 2011; 100: 1518-1522
  • 3 Lindinger A, Schwedler G, Hense H-W. Prevalence of congenital heart defects in newborns in Germany: Results of the first registration year of the PAN Study (July 2006–June 2007). Klin Paediatr 2010; 222: 321-326
  • 4 Tanner K, Sabrine N, Wren C. Cardiovascular malformations among preterm infants. Pediatrics 2005; 116: e833-e838
  • 5 Roy DL, McIntyre L, Human DG et al. Trends in the prevalence of congenital heart disease: comprehensive observations over a 24-year period in a defined region of Canada. Can J Cardiol 1994; 10: 821-826
  • 6 Lally KP. Congenital diaphragmatic hernia. Curr Opin Pediatr 2002; 14: 486-490
  • 7 DiFiore JW, Fauza DO, Slavin R et al. Experimental fetal tracheal ligation and congenital diaphragmatic hernia: A pulmonary vascular morphometric analysis. J Pediatr Surg 1995; 30: 917-924
  • 8 Bloss RS, Aranda JV, Beardmore HE. Congenital diaphragmatic hernia: pathophysiology and pharmacologic support. Surgery 1981; 89: 518-524
  • 9 Yamataka T, Puri P. Pulmonary artery structural changes in pulmonary hypertension complicating congenital diaphragmatic hernia. J Pediatr Surg 1997; 32: 387-390
  • 10 Schnitzer JJ. Control and regulation of pulmonary hypoplasia associated with congenital diaphragmatic hernia. Semin Pediatr Surg 2004; 13: 37-43
  • 11 Nasr A, Langer JC. Canadian Pediatric Surgery Network . Influence of location of delivery on outcome in neonates with congenital diaphragmatic hernia. J Pediatr Surg 2011; 46: 814-816
  • 12 Logan JW, Rice HE, Goldberg RN et al. Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies. J Perinatol 2007; 27: 535-549
  • 13 Fisher JC, Jefferson RA, Arkovitz MS et al. Redefining outcomes in right congenital diaphragmatic hernia. J Pediatr Surg 2008; 43: 373-379
  • 14 Stevens TP, Wijngaarden E, van Ackerman KG et al. Timing of delivery and survival rates for infants with prenatal diagnoses of congenital diaphragmatic hernia. Pediatrics 2009; 123: 494-502
  • 15 Frenckner BP, Lally PA, Hintz SR et al. Congenital Diaphragmatic Hernia Study Group . Prenatal diagnosis of congenital diaphragmatic hernia: how should the babies be delivered?. J Pediatr Surg 2007; 42: 1533-1538
  • 16 Reiss I, Schaible T, van den Hout L et al. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO Consortium consensus. Neonatology 2010; 98: 354-364
  • 17 Boloker J, Bateman DA, Wung J-T et al. Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnea/spontaneous respiration/elective repair. J Pediatr Surg 2002; 37: 357-366
  • 18 Wung JT, Sahni R, Moffitt ST et al. Congenital diaphragmatic hernia: survival treated with very delayed surgery, spontaneous respiration, and no chest tube. J Pediatr Surg 1995; 30: 406-409
  • 19 Kays DW, Langham Jr MR, Ledbetter DJ et al. Detrimental effects of standard medical therapy in congenital diaphragmatic hernia. Ann Surg 1999; 230: 340-348
  • 20 Frenckner B, Ehrén H, Granholm T et al. Improved results in patients who have congenital diaphragmatic hernia using preoperative stabilization, extracorporeal membrane oxygenation, and delayed surgery. J Pediatr Surg 1997; 32: 1185-1189
  • 21 Van Meurs K. Congenital Diaphragmatic Hernia Study Group . Is surfactant therapy beneficial in the treatment of the term newborn infant with congenital diaphragmatic hernia?. J Pediatr 2004; 145: 312-316
  • 22 Lally KP, Lally PA, Langham MR et al. Surfactant does not improve survival rate in preterm infants with congenital diaphragmatic hernia. J Pediatr Surg 2004; 39: 829-833
  • 23 Christison-Lagay ER, Kelleher CM, Langer JC. Neonatal abdominal wall defects. Semin Fetal Neonatal Med 2011; 16: 164-172
  • 24 Castilla EE, Mastroiacovo P, Orioli IM. Gastroschisis: international epidemiology and public health perspectives. Am J Med Genet C Semin Med Genet 2008; 148C: 162-179
  • 25 Loane M, Dolk H, Bradbury I. EUROCAT Working Group . Increasing prevalence of gastroschisis in Europe 1980-2002: a phenomenon restricted to younger mothers?. Paediatr Perinat Epidemiol 2007; 21: 363-369
  • 26 Fillingham A, Rankin J. Prevalence, prenatal diagnosis and survival of gastroschisis. Prenat Diagn 2008; 28: 1232-1237
  • 27 Abdullah F, Arnold MA, Nabaweesi R et al. Gastroschisis in the United States 1988-2003: analysis and risk categorization of 4344 patients. J Perinatol 2007; 27: 50-55
  • 28 Nasr A, Langer JC. Canadian Paediatric Surgery Network . Influence of location of delivery on outcome in neonates with gastroschisis. J Pediatr Surg 2012; 47: 2022-2025
  • 29 Langer JC, Bell JG, Castillo RO et al. Etiology of intestinal damage in gastroschisis, II. Timing and reversibility of histological changes, mucosal function, and contractility. J Pediatr Surg 1990; 25: 1122-1126
  • 30 Langer JC, Longaker MT, Crombleholme TM et al. Etiology of intestinal damage in gastroschisis. I: Effects of amniotic fluid exposure and bowel constriction in a fetal lamb model. J Pediatr Surg 1989; 24: 992-997
  • 31 Garcia L, Brizot M, Liao A et al. Bowel dilation as a predictor of adverse outcome in isolated fetal gastroschisis. Prenat Diagn 2010; 30: 964-969
  • 32 Alfaraj MA, Ryan G, Langer JC et al. Does gastric dilation predict adverse perinatal or surgical outcome in fetuses with gastroschisis?. Ultrasound Obstet Gynecol 2011; 37: 202-206
  • 33 Wilson MS, Carroll MA, Braun SA et al. Is preterm delivery indicated in fetuses with gastroschisis and antenatally detected bowel dilation?. Fetal Diagn Ther 2012; 32: 262-266
  • 34 Piper HG, Jaksic T. The impact of prenatal bowel dilation on clinical outcomes in neonates with gastroschisis. J Pediatr Surg 2006; 41: 897-900
  • 35 Mears AL, Sadiq JM, Impey L et al. Antenatal bowel dilatation in gastroschisis: a bad sign?. Pediatr Surg Int 2010; 26: 581-588
  • 36 Charlesworth P, Njere I, Allotey J et al. Postnatal outcome in gastroschisis: effect of birth weight and gestational age. J Pediatr Surg 2007; 42: 815-818
  • 37 Gelas T, Gorduza D, Devonec S et al. Scheduled preterm delivery for gastroschisis improves postoperative outcome. Pediatr Surg Int 2008; 24: 1023-1029
  • 38 Serra A, Fitze G, Kamin G et al. Preliminary report on elective preterm delivery at 34 weeks and primary abdominal closure for the management of gastroschisis. Eur J Pediatr Surg 2008; 18: 32-37
  • 39 Moir CR, Ramsey PS, Ogburn PL et al. A prospective trial of elective preterm delivery for fetal gastroschisis. Am J Perinatol 2004; 21: 289-294
  • 40 Logghe HL, Mason GC, Thornton JG et al. randomized controlled trial of elective preterm delivery of fetuses with gastroschisis. J Pediatr Surg 2005; 40: 1726-1731
  • 41 Grant NH, Dorling J, Thornton JG. Elective preterm birth for fetal gastroschisis. Cochrane Database Syst Rev 2013; 6: CD009394
  • 42 Holland AJA, Walker K, Badawi N. Gastroschisis: an update. Pediatr Surg Int 2010; 26: 871-878
  • 43 Carpenter MW, Curci MR, Dibbins AW et al. Perinatal management of ventral wall defects. Obstet Gynecol 1984; 64: 646-651
  • 44 How HY, Harris BJ, Pietrantoni M et al. Is vaginal delivery preferable to elective cesarean delivery in fetuses with a known ventral wall defect?. Am J Obstet Gynecol 2000; 182: 1527-1534
  • 45 Moretti M, Khoury A, Rodriquez J et al. The effect of mode of delivery on the perinatal outcome in fetuses with abdominal wall defects. Am J Obstet Gynecol 1990; 163: 833-838
  • 46 Lewis DF, Towers CV, Garite TJ et al. Fetal gastroschisis and omphalocele: is cesarean section the best mode of delivery?. Am J Obstet Gynecol 1990; 163: 773-775
  • 47 Biard J-M, Wilson RD, Johnson MP et al. Prenatally diagnosed giant omphaloceles: short- and long-term outcomes. Prenat Diagn 2004; 24: 434-439
  • 48 Segel SY, Marder SJ, Parry S et al. Fetal abdominal wall defects and mode of delivery: a systematic review. Obstet Gynecol 2001; 98: 867-873
  • 49 Salihu HM, Emusu D, Aliyu ZY et al. Mode of delivery and neonatal survival of infants with isolated gastroschisis. Obstet Gynecol 2004; 104: 678-683
  • 50 Swamy R, Embleton N, Hale J. Sacrococcygeal teratoma over two decades: birth prevalence, prenatal diagnosis and clinical outcomes. Prenat Diagn 2008; 28: 1048-1051
  • 51 Wakhlu A, Misra S, Tandon RK et al. Sacrococcygeal teratoma. Pediatr Surg Int 2002; 18: 384-387
  • 52 Altman RP, Randolph JG, Lilly JR. Sacrococcygeal teratoma: American Academy of Pediatrics Surgical Section Survey-1973. J Pediatr Surg 1974; 9: 389-398
  • 53 Frey L, Hauser WA. Epidemiology of neural tube defects. Epilepsia 2003; 44 (Suppl. 03) 4-13
  • 54 Correia-Pinto J, Reis JL, Hutchins GM et al. In utero meconium exposure increases spinal cord necrosis in a rat model of myelomeningocele. J Pediatr Surg 2002; 37: 488-492
  • 55 Lam S, Grandhi R, Greene S. Meconium staining of the brainstem with open myelomeningocele: Case report. J Neurosurg Pediatr 2013; 11: 150-153
  • 56 Lingman G. Management of pregnancy and labour in cases diagnosed with major fetal malformation. Curr Opin Obstet Gynecol 2005; 17: 143-146
  • 57 McKechnie L, Vasudevan C, Levene M. Neonatal outcome of congenital ventriculomegaly. Semin Fetal Neonatal Med 2012; 17: 301-307
  • 58 Almog B, Gamzu R, Achiron R et al. Fetal lateral ventricular width: what should be its upper limit? A prospective cohort study and reanalysis of the current and previous data. J Ultrasound Med 2003; 22: 39-43
  • 59 Kelly EN, Allen VM, Seaward G. Mild ventriculomegaly in the fetus, natural history, associated findings and outcome of isolated mild ventriculomegaly: a literature review. Prenat Diagn 2001; 21: 697-700
  • 60 Eberhardt H, Herterich R. Management und Outcome der Vena-Galeni-Malformation im Kindesalter. Klin Paediatr 2008; 221: 6-13
  • 61 Zerah M, Garcia-Monaco R, Rodesch G et al. Hydrodynamics in vein of Galen malformations. Childs Nerv Syst ChNS Off. J Int Soc Pediatr Neurosurg 1992; 8: 111-117
  • 62 Ogita K, Suita S, Taguchi T et al. Outcome of fetal cystic hygroma and experience of intrauterine treatment. Fetal Diagn Ther 2001; 16: 105-110
  • 63 Howarth ES, Draper ES, Budd JLS et al. Population-based study of the outcome following the prenatal diagnosis of cystic hygroma. Prenat Diagn 2005; 25: 286-291
  • 64 Stefini S, Bazzana T, Smussi C et al. EXIT (Ex utero Intrapartum Treatment) in lymphatic malformations of the head and neck: Discussion of three cases and proposal of an EXIT-TTP (Team Time Procedure) list. Int J Pediatr Otorhinolaryngol 2012; 76: 20-27