5/12/2016 Twintwin transfusion syndrome: Management and outcome UpToDate Official reprint from UpToDate® www.uptodate.com ©2016 UpToDate® Twintwin transfusion syndrome: Management and outcome Authors: Anthony Johnson, DO, Ramesha Papanna, MD, MPH Section Editors: Deborah Levine, MD, Louise WilkinsHaug, MD, PhD Deputy Editor: Vanessa A Barss, MD, FACOG All topics are updated as new evidence becomes available and our peer review process is complete Literature review current through: Nov 2016. | This topic last updated: Oct 24, 2016 INTRODUCTION — Monochorionic twin pregnancies are monitored for development of twintwin transfusion syndrome (TTTS) with ultrasound examination every two weeks, beginning at 16 weeks of gestation and continuing until the midthird trimester, although most cases present in the early second trimester. Severity of disease is staged according to the Quintero system (table 1). The stage may remain stable, regress, or progress over time, and progression can occur rapidly. (See "Twintwin transfusion syndrome and twin anemia polycythemia sequence: Pathogenesis and diagnosis", section on 'Monitoring for TTTS'.) The three primary approaches to management of TTTS are expectant management, fetoscopic laser ablation of anastomotic vessels, and amnioreduction. Selective reduction is another option, but is rarely performed in the absence of discordant malformations or severe selective fetal growth restriction. The choice of approach depends on the Quintero stage, maternal symptoms and signs, gestational age, and availability of requisite technical expertise This topic will review the management and outcome of TTTS. The pathogenesis, clinical manifestations, diagnosis, and monitoring for TTTS are discussed separately. (See "Twintwin transfusion syndrome and twin anemia polycythemia sequence: Pathogenesis and diagnosis".) MANAGEMENT OF QUINTERO STAGE I — The choice of therapy for Quintero stage I TTTS is based primarily on severity of maternal discomfort from uterine distention and on cervical length. No randomized trials have compared treatment approaches for stage I TTTS. A systematic review concluded that the optimal initial management of stage I TTTS "remains in equipoise" [1]. In this review, the pooled incidence of progression in stage I TTTS was 27 percent (95% CI 1639) Women with no or tolerable symptoms and a normal cervical length Choice of therapy — For women with Quintero stage I (table 1) TTTS and no maternal symptoms or tolerable symptoms and transvaginal cervical length >25 mm, we avoid intervention and monitor TTTS status with weekly ultrasound examinations to detect progression to more severe disease. In addition to the morbidity associated with any intervention, unnecessary intervention can affect therapeutic options later in pregnancy if intervention becomes indicated because of progressive disease. For example, amnioreduction performed as a firstline treatment of minimally symptomatic stage I disease can result in an inadvertent septostomy or bloody amniotic fluid, which would make subsequent laser treatment difficult to undertake when indicated because of worsening TTTS This approach is based on limited but reassuring data of the outcome of welldefined stage I disease in the absence of any intervention. In a 2013 systematic review of seven observational studies including 262 twin pregnancies, expectantly managed stage I TTTS resolved or remained stable in 85 percent of cases [2]. The survival rate with expectant management was 86 percent versus 85 percent with laser therapy and 77 percent https://www.uptodate.com/contents/twintwintransfusionsyndromemanagementandoutcome/print?source=search_result&search=twin%20twin%20transfu… 1/24 5/12/2016 Twintwin transfusion syndrome: Management and outcome UpToDate with amnioreduction. Outcomes were similar when laser therapy was a secondchoice rather than a firstchoice treatment, which suggests that delaying this intervention does not worsen the prognosis In contrast, a subsequent retrospective observational study by the North American Fetal Therapy Network reported poor outcomes with expectant management of stage I TTTS [3]. In the 49 expectantly managed stage I TTTS pregnancies, 8 percent remained stable, 22 percent regressed, 60 percent progressed to a more severe stage, and 10 percent resulted in a spontaneous previable preterm birth. The mean duration from diagnosis of stage I TTTS to a change in status was 11.1 days ±14.3 days; in those cases that progressed, the mean duration was 9 days. Both amnioreduction and laser therapy at stage I TTTS decreased the likelihood of no survivors (odds ratio [OR] 0.11, 95% CI 0.020.68 and OR 0.07, 95% CI 0.010.37, respectively) compared with expectant management An international randomized trial comparing expectant management with laser ablation in management of stage I TTTS is underway and should provide better data on which to base recommendations regarding the appropriate role of early intervention at the onset of TTTS [4] Prenatal followup and care — We monitor pregnancies with stage I TTTS and no maternal symptoms or tolerable symptoms and transvaginal cervical length >30 mm for disease progression with ultrasound: ● Amniotic fluid volume is assessed weekly ● Fetal growth is assessed every three to four weeks. If selective fetal growth lag is identified (ie, one fetus with estimated fetal weight 1.5 multiples of the median (MoM) in one fetus in conjunction with a value of 10 cm Data from: Quintero RA, Morales WJ, Allen MH, et al. Staging of twintwin transfusion syndrome. J Perinatol 1999; 19:550 Graphic 107708 Version 4.0 https://www.uptodate.com/contents/twintwintransfusionsyndromemanagementandoutcome/print?source=search_result&search=twin%20twin%20transf… 20/24 5/12/2016 Twintwin transfusion syndrome: Management and outcome UpToDate Arteriovenous anastomosis preablation The vessel at 11 o'clock is the recipient's vein, the vessel coming in at 5 o'clock is the donor artery, the "green" targeting light is focused on the donor vessel Courtesy of Kenneth J Moise, Jr, MD and Anthony Johnson, DO Graphic 67539 Version 4.0 https://www.uptodate.com/contents/twintwintransfusionsyndromemanagementandoutcome/print?source=search_result&search=twin%20twin%20transf… 21/24 5/12/2016 Twintwin transfusion syndrome: Management and outcome UpToDate Arteriovenous anastomosis postablation The ablated area between the recipient vein and donor artery. The laser fiber is at 12 o'clock Courtesy of Kenneth J Moise, Jr, MD and Anthony Johnson, DO Graphic 80616 Version 4.0 https://www.uptodate.com/contents/twintwintransfusionsyndromemanagementandoutcome/print?source=search_result&search=twin%20twin%20transf… 22/24 5/12/2016 Twintwin transfusion syndrome: Management and outcome UpToDate Arteriovenous anastomosis postablation Ablated (blanched) area between the recipient vein at 12 o'clock and the donor artery at 5 o'clock Courtesy of Kenneth J Moise, Jr, MD and Anthony Johnson, DO Graphic 56339 Version 5.0 https://www.uptodate.com/contents/twintwintransfusionsyndromemanagementandoutcome/print?source=search_result&search=twin%20twin%20transf… 23/24 5/12/2016 Twintwin transfusion syndrome: Management and outcome UpToDate Contributor Disclosures Anthony Johnson, DO Nothing to disclose Ramesha Papanna, MD, MPH Nothing to disclose Deborah Levine, MD Nothing to disclose Louise WilkinsHaug, MD, PhD Nothing to disclose Vanessa A Barss, MD, FACOG Nothing to disclose Contributor disclosures are reviewed for conflicts of interest by the editorial group. 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