İkizden İkize Transfüzyon Sendromu (TTTS)
Özet
İkizden İkize Transfüzyon Sendromu (TTTS), monokoryonik ikiz gebeliklerde plasentadaki derin arteriovenöz anastomozlar nedeniyle bir fetüsten diğerine dengesiz kan akışı sonucu gelişen ciddi bir komplikasyondur. Bu durum verici ikizde oligohidramniyos ve büyüme geriliğine yol açarken, alıcı ikizde hipervolemi, polihidramniyos ve kardiyak anomalilere neden olur. Tedavi edilmediğinde %80-100 mortalite riski taşıyan bu sendrom, genellikle 15-26. gebelik haftalarında ortaya çıkar. Antenatal tanısı ultrasonografi ile konulan hastalık için en yaygın kullanılan sınıflandırma Quintero evreleme sistemidir. Tedavi ve yönetim seçenekleri arasında rutin gebelik takibi, selektif terminasyon, septostomi, seri amniyoredüksiyon ve fetoskopik lazer fotokoagülasyonu yer almaktadır. Özellikle 26. gebelik haftasından önce tanı alan Evre 2 ve üzeri şiddetli vakalarda, patofizyolojiyi düzelten ve vasküler anastomozları ortadan kaldıran fetoskopik lazer ablasyon tedavisi altın standart olarak kabul edilmektedir. Lazer tedavisi, seri amniyoredüksiyona kıyasla bebeklerin sağ kalım oranını ve neonatal dönemdeki nörolojik prognozu anlamlı derecede iyileştirir. Ancak her türlü optimal tedaviye rağmen TTTS, yüksek perinatal mortalite ve uzun dönemde nörolojik morbidite riski taşımaya devam eden yüksek riskli bir klinik tablodur.
Twin-to-Twin Transfusion Syndrome (TTTS) is a severe complication occurring in monochorionic twin pregnancies, characterized by an imbalanced blood flow from one fetus to another due to deep arteriovenous anastomoses in the placenta. This condition leads to oligohydramnios and growth restriction in the donor twin, while causing hypervolemia, polyhydramnios, and cardiac anomalies in the recipient. Carrying an 80-100% mortality risk if left untreated, the syndrome typically manifests between the 15th and 26th gestational weeks. Calculated through ultrasonography for antenatal diagnosis, the Quintero staging system remains the most widely accepted classification method. Management and treatment options include routine surveillance, selective termination, septostomy, serial amnioreduction, and fetoscopic laser photocoagulation. Particularly for severe cases at Stage 2 or beyond diagnosed before the 26th week of gestation, fetoscopic laser ablation, which corrects the underlying pathophysiology by coagulating vascular connections, is considered the gold standard approach. Compared to serial amnioreduction, laser therapy significantly improves the survival rate of at least one twin and enhances neurological outcomes in newborns. Nevertheless, despite optimal laser interventions, TTTS remains a highly critical condition with a perinatal mortality rate between 20% and 50% and potential long-term neurological complications.
Referanslar
Campbell S. Twin-to-twin transfusion syndrome-debates on the etiology, natural history and management. OPİNİON.Ultrasound Obstet Gynecol. 2000;16(3):210-213.
Miller L Jena. Twin to twin transfusion syndrome. Transl Pediatr.2021 May;10(5).1518-1529.
Hitoshi Y. Fetal and neonatal circulatory disorders in twin to twin transfusion syndrome. J Nippon Med Sch.2019;86(4):192-200.
Van den Wijngaard J, Umur A, Ross M et al. Twin-twin transfusion mathematical modelling. Prenat Diagn. 2008 Apr; 28(4):280-91.
Resnik R, Lockwood C, Moore T et al. (2018). İnvasive fetal therapy. Obican S, Odibo A (eds).Creasy and Resnik’s maternal-fetal medicine principles and practice.(8th ed.,pp 594-631).Philadelphia: Elsevier Health Sciences.
Kagan K, Gazzoni A, Sotiriadis A et al. Discordance in nuchal translucency thickness in the prediction of severe twin-to-twin transfusion syndrome. Ultrasound Obstet Gynecol.2007 May;29(5):527-32.
Society for Maternal-Fetal Medicine, Simpson LL. Twin-twin transfusion syndrome.Am J Obstet Gynecol.2013;208(1):3-18.
Rodis JF, Mcılveen PF, Egan JFX, et al. Monoamniotic twins :improved perinatal survival with accurate prenatal diagnosis and antenatal fetal surveillance. Am J Obstet Gynecol .1997;177(5):1046-1049.
Quintero RA, Morales WJ, Allen MH, et al. Staging of twin-twin transfusion syndrome. J Perinatology.1999;19(8):550-555.
Rychik J, Tian Z, Bebbington M, et al. The twin-twin transfusion syndrome:spectrum of cardiovascular abnormality and development of a cardiovascular score to assess severity of disease. Am J Obstet Gynecol.2007;197(4):392.
Thorson HL, Ramaeker DM,Emery SP. Optimal interval for ultrasound surveillance in monochorionic twin gestations. Obstet Gynecol .2011;117:131-135.
Mahieu-Caputo D, Meulemans A, Martinovic J, et al. Paradoxic activation of the renin-angiotensin system in twin-twin transfusion syndrome: an explanation for cardiovascular disturbances in the recipient.Pediatr Res. 2005;58(4):685-688.
Sueters M, Middeldorp JM, Lopriore E, et al. Timely diagnosis of twin-to-twin transfusion syndrome in monochorionic twin pregnancies by biweekly sonography combined with patient instruction to report onset of symptoms. Ultrasound Obstet Gynecol. 2006;28(5):659-664.
Lewi L, Gratacos E, Orbitus E, et al. Pregnancy and infant outcome of 80 consecutive cord coagulations in complicated monochorionic multiple pregnancies. Am J Obstet Gynecol.2006;194(3):782-789.
Rossi AC, D’Addario V. Laser therapy and serial amnioreduction as treatment for twin-twin transfusion syndrome: a meta-analysis and review of the literatüre. Am J Obstet Gynecol.2008;198(2):147-152.
Moise KJ, Dorman K, Lamvu G, et al. A randomized trial of amnioreduction versus septostomy in the treatment of twin-twin transfusion syndrome. Am J Obstet Gynecol.2005;193(3):701-707.
Wee LY, Fisk NM. The twin-twin transfusion syndrome. Semin Neonatol. 2002;7(3):187-202.
Mari G, Roberts A, Detti L. Perinatal morbidity and mortality rates in severe twin-twin transfusion syndrome:results of the international amnioreduction registry. Am J Obstet Gynecol .2001;185(3):708-715.
Senat MV, Deprest J, Boulvain M, et al. Endoscopic laser surgery versus serial amnioreduction for severe twin-to-twin transfusion syndrome. NEJM. 2004;351(2):136-144.
Ville Y, Hyett J, Hecher K, et al. Preliminary experience with endoscopic laser surgery for severe twin-twin transfusion syndrome.N Engl J Med.1995;332(4):224-227.
De Lia JE, Cruikshank DP, Keye WR. Fetoscopic neodymium:YAG laser occlusion of placental vessels in severe twin-twin transfusion syndrome. Obstet Gynecol.1990;75(6):1046-1053.
Valsky DV, Eixarch E, Martinez-Crespo JM, et al. Fetoscopic laser surgery for twin-to-twin transfusion syndrome after 26 weeks of gestation. Fetal Diagn Ther.2012;31(1):30-34.
Middeldorp J, Lopriore E, Sueters M, et al. Twin-to-twin transfusion syndrome after 26 weeks of gestations:is there a role for fetoscopic laser surgery? BJOG Int J Obstet Gynaecol .2007;114(6):694-698.
Gratacos E, Van Schoubroeck D, Carreras E, et al. Impact of laser coagulation in severe twin-twin transfusion syndrome on fetal Doppler indices and venous blood flow volüme. Ultrasound Obstet Gynecol.2002;20(2):125-130
Anand KJS, Sippel WG, Aynsley-Greeen A. Randomised trial of fentanyl anaesthesia in preterm babies undergoing surgery:effects on the stress response. The Lancet. 1987; 329:62-66.
Yamamoto M, Murr LE,Robyr R, et al.Incidence and impact of perioperative complications in 175 fetoscopy-guided laser coagulations of chorionic plate anastomoses in fetofetal transfusion syndrome before 26 weeks of gestation.Am J Obstet Gynecol.2005;193(3):1110-1116.
Taylor MJ, Govender L, Jolly M, et al. Validation of the Quintero staging system for twin-twin transfusion syndrome. Obstet gynecol.2002;100(6):1257-1265.
Huber A, Hecher K. How can we diagnose and manage twin-twin transfusion syndrome? Best Practice and Research Clinical Obstetrics and Gynaecology .2004;18(4):543-556.
Bamberg C, Hecher K et al. Update on twin-to-twin transfusion syndrome. Best Pract Res Clin Obstet Gynaecol.2019 Jul;58:55-65.