Zusammenfassung
Die Fortschritte in der pränatalen Ultraschalldiagnostik der letzten 40 Jahre haben einerseits zu einer deutlichen Steigerung der Entdeckungsrate vorgeburtlicher Erkrankungen geführt, andererseits aber auch die Grundlage für die intrauterine Therapie einiger ausgewählter Erkrankungen geschaffen. Da die meisten vorgeburtlichen Interventionen mit Risiken sowohl für den Fetus als auch für die werdende Mutter verbunden sind, bleiben sie Erkrankungen vorbehalten, die unbehandelt eine tödliche Prognose haben oder im Laufe der intrauterinen Entwicklung eine deutliche Verschlechterung der Prognose erfahren. Bisher haben nur wenige dieser therapeutischen Konzepte ihren Wert im Rahmen kontrollierter Studien bewiesen. Dies sind insbesondere die Lasertherapie des fetofetalen Transfusionssyndroms, der intrauterine Verschluss der Spina bifida und die Trachealokklusion bei Zwerchfellhernien mit schwerer pulmonaler Hypoplasie. Andere intrauterine Therapieansätze sind weniger gut belegt, entweder aufgrund der Seltenheit der Erkrankung oder der mangelnden Verfügbarkeit des Instrumentariums und der entsprechend erfahrenen Therapeuten. Der Beitrag soll dem primär beratenden Gynäkologen einen Überblick über die etablierten Verfahren und die neueren Entwicklungen geben.
Abstract
Advances in prenatal ultrasound over the past 40 years have led to a significant increase in the detection rate of prenatal diseases but have also created the basis for intrauterine treatment in a subset of cases. As most prenatal interventions are associated with risks for both the fetus and the mother, they are reserved for diseases that have a fatal prognosis if left untreated, or face a significant deterioration during intrauterine development. To date, only a few of these therapeutic concepts have proven their value in controlled studies. These are, in particular, laser therapy for twin-to-twin transfusion syndrome, intrauterine closure of spina bifida, and tracheal occlusion for diaphragmatic hernia associated with severe pulmonary hypoplasia. Other intrauterine therapeutic approaches are less well documented, either due to the rarity of the condition or the lack of availability of instruments and appropriately experienced therapists. This article is intended to provide the primary consultant gynecologist with an overview of established procedures and recent developments.
Literatur
WAPM Consensus Group on Twin-to-Twin Transfusion, Baschat A, Chmait RH et al (2011) Twin-to-twin transfusion syndrome (TTTS). J Perinat Med 39:107–112. https://doi.org/10.1515/JPM.2010.147
Bamberg C, Hecher K (2022) Twin-to-twin transfusion syndrome: Controversies in the diagnosis and management. Best Pract Res Clin Obstet Gynaecol 84:143–154. https://doi.org/10.1016/j.bpobgyn.2022.03.013
Senat M‑V, Deprest J, Boulvain M et al (2004) Endoscopic laser surgery versus serial amnioreduction for severe twin-to-twin transfusion syndrome. N Engl J Med 351:136–144. https://doi.org/10.1056/NEJMoa032597
Baschat AA, Barber J, Pedersen N et al (2013) Outcome after fetoscopic selective laser ablation of placental anastomoses vs equatorial laser dichorionization for the treatment of twin-to-twin transfusion syndrome. Am J Obstet Gynecol 209(234):e1–8. https://doi.org/10.1016/j.ajog.2013.05.034
Diehl W, Diemert A, Grasso D et al (2017) Fetoscopic laser coagulation in 1020 pregnancies with twin–twin transfusion syndrome demonstrates improvement in double-twin survival rate. Ultrasound Obstet Gynecol 50:728–735. https://doi.org/10.1002/uog.17520
Slaghekke F, Lopriore E, Lewi L, et al (2014) Fetoscopic laser coagulation of the vascular equator versus selective coagulation for twin-to-twin transfusion syndrome: an open-label randomised controlled trial. Lancet 383:2144–2151. https://doi.org/10.1016/S0140-6736(13)62419-8
Akkermans J, De Vries SM, Zhao D et al (2017) What is the impact of placental tissue damage after laser surgery for twin-twin transfusion syndrome? A secondary analysis of the Solomon trial. Placenta 52:71–76. https://doi.org/10.1016/j.placenta.2017.02.023
Stirnemann J, Slaghekke F, Khalek N, et al (2021) Intrauterine fetoscopic laser surgery versus expectant management in stage 1 twin-to-twin transfusion syndrome: an international randomized trial. Am J Obstet Gynecol 224:528.e1–528.e12. https://doi.org/10.1016/j.ajog.2020.11.031
Berg C, Holst D, Mallmann MR et al (2014) Early vslate intervention in twin reversed arterial perfusion sequence. Ultrasound Obstet Gynecol 43:60–64. https://doi.org/10.1002/uog.12578
Lewi L, Valencia C, Gonzalez E et al (2010) The outcome of twin reversed arterial perfusion sequence diagnosed in the first trimester. Am J Obstet Gynecol 203(213):e1–4. https://doi.org/10.1016/j.ajog.2010.04.018
Weber EC, Recker F, Gottschalk I et al (2022) Outcome of TRAP sequence treated in the first trimester—a ten-year single-center experience. Ultraschall Med 43:614–618. https://doi.org/10.1055/a-1526-1775
de Sousa TM, Glosemeyer P, Diemert A et al (2020) First-trimester intervention in twin reversed arterial perfusion sequence. Ultrasound Obstet Gynecol 55:47–49. https://doi.org/10.1002/uog.20860
Weber EC, Recker F, Gottschalk I et al (2021) Outcome of monochorionic monoamniotic twin reversed arterial perfusion sequence diagnosed in the first trimester. Fetal Diagn Ther 48:778–784. https://doi.org/10.1159/000519860
Adzick NS, Thom EA, Spong CY et al (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004. https://doi.org/10.1056/NEJMoa1014379
McLone DG, Dias MS (2003) The Chiari II malformation: cause and impact. Child’s Nervous System 19:540–550. https://doi.org/10.1007/s00381-003-0792-3
Meuli M, Meuli-Simmen C, Hutchins GM, et al (1997) The spinal cord lesion in human fetuses with myelomeningocele: Implications for fetal surgery. Journal of Pediatric Surgery 32:448–452. https://doi.org/10.1016/S0022-3468(97)90603-5
Chaoui R, Benoit B, Mitkowska-Wozniak H et al (2009) Assessment of intracranial translucency (IT) in the detection of spina bifida at the 11–13-week scan. Ultrasound Obstet Gynecol 34:249–252. https://doi.org/10.1002/uog.7329
Chaoui R, Benoit B, Entezami M et al (2020) Ratio of fetal choroid plexus to head size: simple sonographic marker of open spina bifida at 11–13 weeks’ gestation. Ultrasound Obstet Gynecol 55:81–86. https://doi.org/10.1002/uog.20856
Ushakov F, Sacco A, Andreeva E et al (2019) Crash sign: new first-trimester sonographic marker of spina bifida. Ultrasound Obstet Gynecol 54:740–745. https://doi.org/10.1002/uog.20285
Maiz N, Arévalo S, García-Manau P et al (2023) Presurgery motor level assessment for prediction of motor level at birth in fetuses undergoing prenatal repair of open spina bifida: time to abandon anatomical level in counseling. Ultrasound Obstet Gynecol 61:728–733. https://doi.org/10.1002/uog.26180
Carreras E, Maroto A, Illescas T et al (2016) Prenatal ultrasound evaluation of segmental level of neurological lesion in fetuses with myelomeningocele: development of a new technique: Functional ultrasound in MMC. Ultrasound Obstet. Gynecol, Bd. 47, S 162–167 https://doi.org/10.1002/uog.15732
Trigo L, Chmait RH, Llanes A et al (2024) Revisiting MOMS criteria for prenatal repair of spina bifida: upper gestational-age limit should be raised and assessment of prenatal motor function rather than anatomical level improves prediction of postnatal function. Ultrasound in Obstet &. Gyne 63:53–59. https://doi.org/10.1002/uog.27536
Kunpalin Y, Sichitiu J, Krishnan P et al (2023) Simple prenatal imaging predictors for postnatal cerebrospinal fluid diversion surgery in fetuses undergoing in utero surgery for spina bifida. Prenat Diagn 43:1605–1613. https://doi.org/10.1002/pd.6453
Mustafa HJ, Arab K, Javinani A, et al (2023) Prenatal predictors of need for cerebrospinal fluid diversion in infants following prenatal repair of open spina bifida; systematic review and meta-analysis. Am J Obstet Gynecol MFM 5:100983. https://doi.org/10.1016/j.ajogmf.2023.100983
Agrawal S, Al-Refai A, Abbasi N et al (2021) Correlation of fetal ventricular size and need for postnatal cerebrospinal fluid diversion surgery in open spina bifida. Ultrasound Obstet Gynecol. uog, Bd. 24767. https://doi.org/10.1002/uog.24767
Kohl T (2014) Percutaneous minimally invasive fetoscopic surgery for spina bifida aperta. Part I: surgical technique and perioperative outcome. Ultrasound Obstet Gynecol 44:515–524. https://doi.org/10.1002/uog.13430
Belfort MA, Whitehead WE, Shamshirsaz AA et al (2019) Comparison of two fetoscopic open neural tube defect (ONTD) repair techniques: Single-layer vs three-layer closure. Ultrasound Obstet Gynecol. uog, Bd. 21915. https://doi.org/10.1002/uog.21915
Belfort MA, Whitehead WE, Shamshirsaz AA et al (2017) Fetoscopic open neural tube defect repair: Development and refinement of a two-port, carbon dioxide insufflation technique. Obstet Gynecol 129:734–743. https://doi.org/10.1097/AOG.0000000000001941
Pedreira DAL, Zanon N, De Sá RAM et al (2014) Fetoscopic single-layer repair of open spina bifida using a cellulose patch: preliminary clinical experience. J Matern Fetal Neonatal Med 27:1613–1619. https://doi.org/10.3109/14767058.2013.871701
Pedreira DAL, Zanon N, Nishikuni K, et al (2016) Endoscopic surgery for the antenatal treatment of myelomeningocele: the CECAM trial. Am J Obstet Gynecol 214:111.e1–111.e11. https://doi.org/10.1016/j.ajog.2015.09.065
Lapa DA, Chmait RH, Gielchinsky Y et al (2021) Percutaneous fetoscopic spina bifida repair: effect on need for postnatal cerebrospinal fluid diversion, ambulation and bladder catheterization. Ultrasound Obstet Gynecol. uog, Bd. 23658. https://doi.org/10.1002/uog.23658
Verweij EJ, de Vries MC, Oldekamp EJ et al (2021) Fetoscopic myelomeningocoele closure: is the scientific evidence enough to challenge the gold standard for prenatal surgery? Prenat Diagn. pd, Bd. 5940. https://doi.org/10.1002/pd.5940
Sanz Cortes M, Chmait RH, Lapa DA, et al (2021) Experience of 300 cases of prenatal fetoscopic open spina bifida repair: report of the International Fetoscopic Neural Tube Defect Repair Consortium. Am J Obstet Gynecol 225:678.e1–678.e11. https://doi.org/10.1016/j.ajog.2021.05.044
Deprest J, Brady P, Nicolaides K et al (2014) Prenatal management of the fetus with isolated congenital diaphragmatic hernia in the era of the TOTAL trial. Semin Fetal Neonatal Med 19:338–348. https://doi.org/10.1016/j.siny.2014.09.006
Deprest JA, Nicolaides KH, Benachi A et al (2021) Randomized trial of fetal surgery for severe left diaphragmatic hernia. N Engl J Med 385:107–118. https://doi.org/10.1056/NEJMoa2027030
Deprest JA, Benachi A, Gratacos E et al (2021) Randomized trial of fetal surgery for moderate left diaphragmatic hernia. N Engl J Med 385:119–129. https://doi.org/10.1056/NEJMoa2026983
Van Calster B, Benachi A, Nicolaides KH, et al (2022) The randomized Tracheal Occlusion To Accelerate Lung growth (TOTAL)-trials on fetal surgery for congenital diaphragmatic hernia: reanalysis using pooled data. Am J Obstet Gynecol 226:560.e1–560.e24. https://doi.org/10.1016/j.ajog.2021.11.1351
Hellmund A, Berg C, Geipel A et al (2016) Prenatal diagnosis and evaluation of sonographic predictors for intervention and adverse outcome in congenital pulmonary airway malformation. PLoS ONE 11:e150474. https://doi.org/10.1371/journal.pone.0150474
Macardle CA, Ehrenberg-Buchner S, Smith EA, et al (2016) Surveillance of fetal lung lesions using the congenital pulmonary airway malformation volume ratio: natural history and outcomes: CVR-based surveillance of fetal lung malformations. Prenat Diagn 36:282–289. https://doi.org/10.1002/pd.4761
Morris LM, Lim F‑Y, Livingston JC et al (2009) High-risk fetal congenital pulmonary airway malformations have a variable response to steroids. J Pediatr Surg 44:60–65. https://doi.org/10.1016/j.jpedsurg.2008.10.012
Derderian SC, Coleman AM, Jeanty C et al (2015) Favorable outcomes in high-risk congenital pulmonary airway malformations treated with multiple courses of maternal betamethasone. J Pediatr Surg 50:515–518. https://doi.org/10.1016/j.jpedsurg.2014.08.019
Witlox RS, Lopriore E, Oepkes D (2011) Prenatal interventions for fetal lung lesions. Prenat Diagn 31:628–636. https://doi.org/10.1002/pd.2778
Peranteau WH, Adzick SN, Boelig MM et al (2015) Thoracoamniotic shunts for the management of fetal lung lesions and pleural effusions: a single-institution review and predictors of survival in 75 cases. J Pediatr Surg 50:301–305. https://doi.org/10.1016/j.jpedsurg.2014.11.019
Yong PJ, Von Dadelszen P, Carpara D et al (2012) Prediction of pediatric outcome after prenatal diagnosis and expectant antenatal management of congenital cystic adenomatoid malformation. Fetal Diagn Ther 31:94–102. https://doi.org/10.1159/000331936
Kunisaki SM, Ehrenberg-Buchner S, Dillman JR et al (2015) Vanishing fetal lung malformations: Prenatal sonographic characteristics and postnatal outcomes. J Pediatr Surg 50:978–982. https://doi.org/10.1016/j.jpedsurg.2015.03.025
Cavoretto P, Molina F, Poggi S et al (2008) Prenatal diagnosis and outcome of echogenic fetal lung lesions. Ultrasound Obstet Gynecol 32:769–783. https://doi.org/10.1002/uog.6218
Mallmann MR, Geipel A, Bludau M et al (2014) Bronchopulmonary sequestration with massive pleural effusion: pleuroamniotic shunting vs intrafetal vascular laser ablation. Ultrasound Obstet Gynecol. https://doi.org/10.1002/uog.13304
Cruz-Martinez R, Méndez A, Dueñas-Riaño J et al (2015) Fetal laser surgery prevents fetal death and avoids the need for neonatal sequestrectomy in cases with bronchopulmonary sequestration. Ultrasound Obstet Gynecol 46:627–628. https://doi.org/10.1002/uog.14921
Ruano R, de A Pimenta EJ, Marques da Silva M, et al (2007) Percutaneous intrauterine laser ablation of the abnormal vessel in pulmonary sequestration with hydrops at 29 weeks’ gestation. J Ultrasound Med 26:1235–1241
Yang W, Gao Y, Li P, Eckman MH (2023) Should asymptomatic patients with congenital lung malformations undergo surgery? A decision analysis. Pediatr Pulmonol 58:449–456. https://doi.org/10.1002/ppul.26206
Smith RP, Illanes S, Denbow ML, Soothill PW (2005) Outcome of fetal pleural effusions treated by thoracoamniotic shunting: Fetal chest shunting. Ultrasound Obstet. Gynecol, Bd. 26, S 63–66 https://doi.org/10.1002/uog.1883
Yinon Y, Grisaru-Granovsky S, Chaddha V et al (2010) Perinatal outcome following fetal chest shunt insertion for pleural effusion. Ultrasound Obstet Gynecol 36:58–64. https://doi.org/10.1002/uog.7507
Picone O, Benachi A, Mandelbrot L et al (2004) Thoracoamniotic shunting for fetal pleural effusions with hydrops. Am J Obstet Gynecol 191:2047–2050. https://doi.org/10.1016/j.ajog.2004.05.016
Aubard Y, Derouineau I, Aubard V et al (1998) Primary fetal hydrothorax: A literature review and proposed antenatal clinical strategy. Fetal Diagn Ther 13:325–333. https://doi.org/10.1159/000020863
Walter A, Strizek B, Weber EC et al (2022) Intrauterine Valvuloplasty in severe aortic stenosis—a ten years single center experience. JCM 11:3058. https://doi.org/10.3390/jcm11113058
Gottschalk I, Strizek B, Menzel T et al (2019) Severe pulmonary stenosis or atresia with intact ventricular septum in the fetus: The natural history. Fetal Diagn Ther. https://doi.org/10.1159/000502178
Tulzer A, Arzt W, Gitter R et al (2018) Immediate effects and outcome of in-utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis. Ultrasound Obstet Gynecol 52:230–237. https://doi.org/10.1002/uog.19047
Chalouhi GE, Morency A, De Vlieger R et al (2017) Prenatal incision of ureterocele causing bladder outlet obstruction: a multicenter case series. Prenat Diagn 37:968–974. https://doi.org/10.1002/pd.5091
Strizek B, Gottschalk I, Recker F et al (2020) Vesicoamniotic shunting for fetal megacystis in the first trimester with a Somatex® intrauterine shunt. Arch Gynecol Obstet 302:133–140. https://doi.org/10.1007/s00404-020-05598-z
Morris RK, Malin GL, Quinlan-Jones E et al (2013) The Percutaneous shunting in Lower Urinary Tract Obstruction (PLUTO) study and randomised controlled trial: evaluation of the effectiveness, cost-effectiveness and acceptability of percutaneous vesicoamniotic shunting for lower urinary tract obstruction. Health Technol Assess 17:1–232. https://doi.org/10.3310/hta17590
Gottschalk I, Berg C, Menzel T et al (2023) Single-center outcome analysis of 46 fetuses with megacystis after intrauterine vesico-amniotic shunting with the Somatex®intrauterine shunt. Arch Gynecol Obstet. https://doi.org/10.1007/s00404-022-06905-6
Strizek B, Spicher T, Gottschalk I et al (2022) Vesicoamniotic Shunting before 17 + 0 Weeks in Fetuses with Lower Urinary Tract Obstruction (LUTO): Comparison of Somatex vs. Harrison Shunt Systems. J Clin Med 11:2359. https://doi.org/10.3390/jcm11092359
Kohaut J, Fischer-Mertens J, Cernaianu G et al (2023) Postnatal surgical treatment and complications following intrauterine vesicoamniotic shunting with the SOMATEX® intrauterine shunt. A single center experience. J Pediatr Urol 19(567):e1–567.e6. https://doi.org/10.1016/j.jpurol.2023.06.027
Ruano R, Duarte S, Bunduki V et al (2010) Fetal cystoscopy for severe lower urinary tract obstruction—initial experience of a single center. Prenat Diagn 30:30–39. https://doi.org/10.1002/pd.2418
Miller JL, Baschat AA, Rosner M, et al (2023) Neonatal survival after serial amnioinfusions for bilateral renal agenesis: The Renal Anhydramnios Fetal Therapy Trial. JAMA 330:2096. https://doi.org/10.1001/jama.2023.21153
Weber E, Recker F, Strizek B et al (2020) Fetoskopische Therapie einer kongenitalen Megalourethra. Frauenarzt: 42–43
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Interessenkonflikt
E.C. Weber, I. Gottschalk, I. Bedei und C. Berg geben an, dass kein Interessenkonflikt besteht.
Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.
Additional information
Redaktion
Andreas Schröer, Berlin
Jan Weichert, Lübeck
Ulrich Gembruch, Bonn
Hinweis des Verlags
Der Verlag bleibt in Hinblick auf geografische Zuordnungen und Gebietsbezeichnungen in veröffentlichten Karten und Institutsadressen neutral.
QR-Code scannen & Beitrag online lesen
Rights and permissions
About this article
Cite this article
Weber, E.C., Gottschalk, I., Bedei, I. et al. Intrauterine Therapie – wie ist der Stand der Dinge?. Gynäkologie (2024). https://doi.org/10.1007/s00129-024-05231-z
Accepted:
Published:
DOI: https://doi.org/10.1007/s00129-024-05231-z
Schlüsselwörter
- Fetale Intervention
- Kongenitale Zwerchfellhernie
- Intrauterine Valvuloplastie
- Meningomyelozele
- Obstruktion der unteren Harnwege