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Twin-Twin Transfusion Syndrome
Anne M. Kennedy, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Twin-twin transfusion syndrome (TTTS)
          • Synonyms

            • Twin oligohydramnios polyhydramnios sequence (TOPS)
          • Definitions

            • Monochorionic twin complication caused by intrauterine transfusion of blood from donor twin (D) to recipient twin (R) via arteriovenous (AV) placental anastomoses
            • Staging based on fluid volume, bladder size, Doppler findings, presence of hydrops

          IMAGING

          • Diagnostic Criteria

            • Ultrasonographic Findings

              • Imaging Recommendations

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    • Staging, Grading, & Classification

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              Selected References

                              1. Behrendt N et al: Twin-twin transfusion and laser therapy. Curr Opin Obstet Gynecol. 28(2):79-85, 2016
                              2. Chmait RH et al: Fetal brain-sparing after laser surgery for twin-twin transfusion syndrome appears associated with two-year neurodevelopmental outcomes. Prenat Diagn. 36(1):63-7, 2016
                              3. Duryea EL et al: The natural history of twin-twin transfusion syndrome stratified by Quintero stage. J Matern Fetal Neonatal Med. 1-5, 2016
                              4. Van Winden KR et al: Decreased Total Placental Mass Found in Twin-Twin Transfusion Syndrome Gestations with Selective Growth Restriction. Fetal Diagn Ther. ePub, 2016
                              5. Ashwal E et al: Twin Anemia-Polycythemia Sequence: Perinatal Management and Outcome. Fetal Diagn Ther. ePub, 2015
                              6. Ben-Ami I et al: Monochorionic diamniotic in vitro fertilization twins have a decreased incidence of twin-to-twin transfusion syndrome. Fertil Steril. ePub, 2015
                              7. Boyle M et al: Postnatal MRI Brain in Infants Treated for Twin-Twin Transfusion Syndrome. Ir Med J. 108(8):240-3, 2015
                              8. Dhillon RK et al: Comparison of Solomon technique with selective laser ablation for twin-twin transfusion syndrome: a systematic review. Ultrasound Obstet Gynecol. 46(5):526-33, 2015
                              9. Emery SP et al: The North American Fetal Therapy Network Consensus Statement: prenatal management of uncomplicated monochorionic gestations. Obstet Gynecol. 125(5):1236-43, 2015
                              10. Persico N et al: Postnatal survival after endoscopic equatorial laser for the treatment of twin-to-twin transfusion syndrome. Am J Obstet Gynecol. ePub, 2015
                              11. Benoit RM et al: Twin-to-twin transfusion syndrome: prenatal diagnosis and treatment. Am J Perinatol. 31(7):583-94, 2014
                              12. Diehl W et al: Twin-twin transfusion syndrome: treatment and outcome. Best Pract Res Clin Obstet Gynaecol. 28(2):227-38, 2014
                              13. Moon-Grady AJ: Fetal echocardiography in twin-twin transfusion syndrome. Am J Perinatol. 31 Suppl 1:S31-8, 2014
                              14. Roberts D et al: Interventions for the treatment of twin-twin transfusion syndrome. Cochrane Database Syst Rev. 1:CD002073, 2014
                              15. Oostveen MP et al: Congenital anomalies presenting in utero as TTTS: a case series report and review of literature. J Obstet Gynaecol. 33(8):901-3, 2013
                              16. Gray PH et al: Cardiac outcomes of hydrops as a result of twin-twin transfusion syndrome treated with laser surgery. J Paediatr Child Health. 45(1-2):48-52, 2009
                              17. Habli M et al: Twin-to-twin transfusion syndrome: a comprehensive update. Clin Perinatol. 36(2):391-416, x, 2009
                              18. Kontopoulos EV et al: Assessment of the peak systolic velocity of the middle cerebral artery in twin-twin transfusion syndrome. Part I: preoperative assessment. Am J Obstet Gynecol. 200(1):61, 2009
                              19. Lopriore E et al: Risk factors for neurodevelopment impairment in twin-twin transfusion syndrome treated with fetoscopic laser surgery. Obstet Gynecol. 113(2 Pt 1):361-6, 2009
                              20. Rossi AC et al: Comparison of donor and recipient outcomes following laser therapy performed for twin-twin transfusion syndrome: a meta-analysis and review of literature. Am J Perinatol. 26(1):27-32, 2009
                              21. Rossi AC et al: Twin-twin transfusion syndrome. Minerva Ginecol. 61(2):153-65, 2009
                              22. Chmait RH et al: Fetal growth after laser therapy for twin-twin transfusion syndrome. Am J Obstet Gynecol. 199(1):47, 2008
                              23. Gungor S et al: Umbilical venous volume flow in twin-twin transfusion syndrome. Ultrasound Obstet Gynecol. 32(6):800-6, 2008
                              24. Habli M et al: Acute effects of selective fetoscopic laser photocoagulation on recipient cardiac function in twin-twin transfusion syndrome. Am J Obstet Gynecol. 199(4):412, 2008
                              25. Habli M et al: The outcome of twin-twin transfusion syndrome complicated with placental insufficiency. Am J Obstet Gynecol. 199(4):424, 2008
                              26. Murakoshi T et al: Validation of Quintero stage III sub-classification for twin-twin transfusion syndrome based on visibility of donor bladder: characteristic differences in pathophysiology and prognosis. Ultrasound Obstet Gynecol. 32(6):813-8, 2008
                              27. Roberts D et al: Interventions for twin-twin transfusion syndrome: a Cochrane review. Ultrasound Obstet Gynecol. 31(6):701-11, 2008
                              28. Rossi AC et al: Laser therapy and serial amnioreduction as treatment for twin-twin transfusion syndrome: a metaanalysis and review of literature. Am J Obstet Gynecol. 198(2):147-52, 2008
                              29. Russell Z et al: What is the definition of pulsatile umbilical venous flow in twin-twin transfusion syndrome? Am J Obstet Gynecol. 199(6):634, 2008
                              30. Shah AD et al: Initial fetal cardiovascular profile score predicts recipient twin outcome in twin-twin transfusion syndrome. J Am Soc Echocardiogr. 21(10):1105-8, 2008
                              31. Crombleholme TM et al: A prospective, randomized, multicenter trial of amnioreduction vs selective fetoscopic laser photocoagulation for the treatment of severe twin-twin transfusion syndrome. Am J Obstet Gynecol. 197(4):396, 2007
                              32. Kagan KO et al: Discordance in nuchal translucency thickness in the prediction of severe twin-to-twin transfusion syndrome. Ultrasound Obstet Gynecol. 29(5):527-32, 2007
                              33. Kline-Fath BM et al: Twin-twin transfusion syndrome: cerebral ischemia is not the only fetal MR imaging finding. Pediatr Radiol. 37(1):47-56, 2007
                              34. Kontopoulos EV et al: Percent absent end-diastolic velocity in the umbilical artery waveform as a predictor of intrauterine fetal demise of the donor twin after selective laser photocoagulation of communicating vessels in twin-twin transfusion syndrome. Ultrasound Obstet Gynecol. 30(1):35-9, 2007
                              35. Lopriore E et al: Neurodevelopmental outcome after laser therapy for twin-twin transfusion syndrome. Am J Obstet Gynecol. 196(1):e20; author reply e20-1, 2007
                              36. Michelfelder E et al: Early manifestations and spectrum of recipient twin cardiomyopathy in twin-twin transfusion syndrome: relation to Quintero stage. Ultrasound Obstet Gynecol. 30(7):965-71, 2007
                              37. Norton ME: Evaluation and management of twin-twin transfusion syndrome: still a challenge. Am J Obstet Gynecol. 196(5):419-20, 2007
                              38. Quintero RA et al: Sequential selective laser photocoagulation of communicating vessels in twin-twin transfusion syndrome. J Matern Fetal Neonatal Med. 20(10):763-8, 2007
                              39. Rychik J 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. 197(4):392, 2007
                              40. Graef C et al: Long-term neurodevelopmental outcome of 167 children after intrauterine laser treatment for severe twin-twin transfusion syndrome. Am J Obstet Gynecol. 194(2):303-8, 2006
                              41. Huber A et al: Stage-related outcome in twin-twin transfusion syndrome treated by fetoscopic laser coagulation. Obstet Gynecol. 108(2):333-7, 2006
                              42. Moise KJ Jr: Neurodevelopmental outcome after laser therapy for twin-twin transfusion syndrome. Am J Obstet Gynecol. 194(5):1208-10, 2006
                              43. Ozcan T et al: Use of middle cerebral artery peak systolic velocity and intrauterine transfusion for management of twin-twin transfusion and single fetal intrauterine demise. J Matern Fetal Neonatal Med. 19(12):807-9, 2006
                              44. Beck M et al: Long-term outcome of kidney function after twin-twin transfusion syndrome treated by intrauterine laser coagulation. Pediatr Nephrol. 20(11):1657-9, 2005
                              45. Cromi A et al: Sonographic umbilical cord morphometry and coiling patterns in twin-twin transfusion syndrome. Prenat Diagn. 25(9):851-5, 2005
                              46. Matias A et al: Search for hemodynamic compromise at 11-14 weeks in monochorionic twin pregnancy: is abnormal flow in the ductus venosus predictive of twin-twin transfusion syndrome? J Matern Fetal Neonatal Med. 18(2):79-86, 2005
                              47. Moise KJ Jr et al: A randomized trial of amnioreduction versus septostomy in the treatment of twin-twin transfusion syndrome. Am J Obstet Gynecol. 2005 Sep;193(3 Pt 1):701-7. Erratum in: Am J Obstet Gynecol. 193(6):2183, 2005
                              48. Senat MV et al: Endoscopic laser surgery versus serial amnioreduction for severe twin-to-twin transfusion syndrome. N Engl J Med. 351(2):136-44, 2004
                              49. Lougheed J et al: Acquired right ventricular outflow tract obstruction in the recipient twin in twin-twin transfusion syndrome. J Am Coll Cardiol. 38(5):1533-8, 2001
                              Related Anatomy
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                              Related Differential Diagnoses
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                              References
                              Tables

                              Tables

                              KEY FACTS

                              • Terminology

                                • Imaging

                                  • Pathology

                                    • Clinical Issues

                                      TERMINOLOGY

                                      • Abbreviations

                                        • Twin-twin transfusion syndrome (TTTS)
                                      • Synonyms

                                        • Twin oligohydramnios polyhydramnios sequence (TOPS)
                                      • Definitions

                                        • Monochorionic twin complication caused by intrauterine transfusion of blood from donor twin (D) to recipient twin (R) via arteriovenous (AV) placental anastomoses
                                        • Staging based on fluid volume, bladder size, Doppler findings, presence of hydrops

                                      IMAGING

                                      • Diagnostic Criteria

                                        • Ultrasonographic Findings

                                          • Imaging Recommendations

                                            DIFFERENTIAL DIAGNOSIS

                                              PATHOLOGY

                                              • General Features

                                                • Staging, Grading, & Classification

                                                  CLINICAL ISSUES

                                                  • Presentation

                                                    • Demographics

                                                      • Natural History & Prognosis

                                                        • Treatment

                                                          Selected References

                                                          1. Behrendt N et al: Twin-twin transfusion and laser therapy. Curr Opin Obstet Gynecol. 28(2):79-85, 2016
                                                          2. Chmait RH et al: Fetal brain-sparing after laser surgery for twin-twin transfusion syndrome appears associated with two-year neurodevelopmental outcomes. Prenat Diagn. 36(1):63-7, 2016
                                                          3. Duryea EL et al: The natural history of twin-twin transfusion syndrome stratified by Quintero stage. J Matern Fetal Neonatal Med. 1-5, 2016
                                                          4. Van Winden KR et al: Decreased Total Placental Mass Found in Twin-Twin Transfusion Syndrome Gestations with Selective Growth Restriction. Fetal Diagn Ther. ePub, 2016
                                                          5. Ashwal E et al: Twin Anemia-Polycythemia Sequence: Perinatal Management and Outcome. Fetal Diagn Ther. ePub, 2015
                                                          6. Ben-Ami I et al: Monochorionic diamniotic in vitro fertilization twins have a decreased incidence of twin-to-twin transfusion syndrome. Fertil Steril. ePub, 2015
                                                          7. Boyle M et al: Postnatal MRI Brain in Infants Treated for Twin-Twin Transfusion Syndrome. Ir Med J. 108(8):240-3, 2015
                                                          8. Dhillon RK et al: Comparison of Solomon technique with selective laser ablation for twin-twin transfusion syndrome: a systematic review. Ultrasound Obstet Gynecol. 46(5):526-33, 2015
                                                          9. Emery SP et al: The North American Fetal Therapy Network Consensus Statement: prenatal management of uncomplicated monochorionic gestations. Obstet Gynecol. 125(5):1236-43, 2015
                                                          10. Persico N et al: Postnatal survival after endoscopic equatorial laser for the treatment of twin-to-twin transfusion syndrome. Am J Obstet Gynecol. ePub, 2015
                                                          11. Benoit RM et al: Twin-to-twin transfusion syndrome: prenatal diagnosis and treatment. Am J Perinatol. 31(7):583-94, 2014
                                                          12. Diehl W et al: Twin-twin transfusion syndrome: treatment and outcome. Best Pract Res Clin Obstet Gynaecol. 28(2):227-38, 2014
                                                          13. Moon-Grady AJ: Fetal echocardiography in twin-twin transfusion syndrome. Am J Perinatol. 31 Suppl 1:S31-8, 2014
                                                          14. Roberts D et al: Interventions for the treatment of twin-twin transfusion syndrome. Cochrane Database Syst Rev. 1:CD002073, 2014
                                                          15. Oostveen MP et al: Congenital anomalies presenting in utero as TTTS: a case series report and review of literature. J Obstet Gynaecol. 33(8):901-3, 2013
                                                          16. Gray PH et al: Cardiac outcomes of hydrops as a result of twin-twin transfusion syndrome treated with laser surgery. J Paediatr Child Health. 45(1-2):48-52, 2009
                                                          17. Habli M et al: Twin-to-twin transfusion syndrome: a comprehensive update. Clin Perinatol. 36(2):391-416, x, 2009
                                                          18. Kontopoulos EV et al: Assessment of the peak systolic velocity of the middle cerebral artery in twin-twin transfusion syndrome. Part I: preoperative assessment. Am J Obstet Gynecol. 200(1):61, 2009
                                                          19. Lopriore E et al: Risk factors for neurodevelopment impairment in twin-twin transfusion syndrome treated with fetoscopic laser surgery. Obstet Gynecol. 113(2 Pt 1):361-6, 2009
                                                          20. Rossi AC et al: Comparison of donor and recipient outcomes following laser therapy performed for twin-twin transfusion syndrome: a meta-analysis and review of literature. Am J Perinatol. 26(1):27-32, 2009
                                                          21. Rossi AC et al: Twin-twin transfusion syndrome. Minerva Ginecol. 61(2):153-65, 2009
                                                          22. Chmait RH et al: Fetal growth after laser therapy for twin-twin transfusion syndrome. Am J Obstet Gynecol. 199(1):47, 2008
                                                          23. Gungor S et al: Umbilical venous volume flow in twin-twin transfusion syndrome. Ultrasound Obstet Gynecol. 32(6):800-6, 2008
                                                          24. Habli M et al: Acute effects of selective fetoscopic laser photocoagulation on recipient cardiac function in twin-twin transfusion syndrome. Am J Obstet Gynecol. 199(4):412, 2008
                                                          25. Habli M et al: The outcome of twin-twin transfusion syndrome complicated with placental insufficiency. Am J Obstet Gynecol. 199(4):424, 2008
                                                          26. Murakoshi T et al: Validation of Quintero stage III sub-classification for twin-twin transfusion syndrome based on visibility of donor bladder: characteristic differences in pathophysiology and prognosis. Ultrasound Obstet Gynecol. 32(6):813-8, 2008
                                                          27. Roberts D et al: Interventions for twin-twin transfusion syndrome: a Cochrane review. Ultrasound Obstet Gynecol. 31(6):701-11, 2008
                                                          28. Rossi AC et al: Laser therapy and serial amnioreduction as treatment for twin-twin transfusion syndrome: a metaanalysis and review of literature. Am J Obstet Gynecol. 198(2):147-52, 2008
                                                          29. Russell Z et al: What is the definition of pulsatile umbilical venous flow in twin-twin transfusion syndrome? Am J Obstet Gynecol. 199(6):634, 2008
                                                          30. Shah AD et al: Initial fetal cardiovascular profile score predicts recipient twin outcome in twin-twin transfusion syndrome. J Am Soc Echocardiogr. 21(10):1105-8, 2008
                                                          31. Crombleholme TM et al: A prospective, randomized, multicenter trial of amnioreduction vs selective fetoscopic laser photocoagulation for the treatment of severe twin-twin transfusion syndrome. Am J Obstet Gynecol. 197(4):396, 2007
                                                          32. Kagan KO et al: Discordance in nuchal translucency thickness in the prediction of severe twin-to-twin transfusion syndrome. Ultrasound Obstet Gynecol. 29(5):527-32, 2007
                                                          33. Kline-Fath BM et al: Twin-twin transfusion syndrome: cerebral ischemia is not the only fetal MR imaging finding. Pediatr Radiol. 37(1):47-56, 2007
                                                          34. Kontopoulos EV et al: Percent absent end-diastolic velocity in the umbilical artery waveform as a predictor of intrauterine fetal demise of the donor twin after selective laser photocoagulation of communicating vessels in twin-twin transfusion syndrome. Ultrasound Obstet Gynecol. 30(1):35-9, 2007
                                                          35. Lopriore E et al: Neurodevelopmental outcome after laser therapy for twin-twin transfusion syndrome. Am J Obstet Gynecol. 196(1):e20; author reply e20-1, 2007
                                                          36. Michelfelder E et al: Early manifestations and spectrum of recipient twin cardiomyopathy in twin-twin transfusion syndrome: relation to Quintero stage. Ultrasound Obstet Gynecol. 30(7):965-71, 2007
                                                          37. Norton ME: Evaluation and management of twin-twin transfusion syndrome: still a challenge. Am J Obstet Gynecol. 196(5):419-20, 2007
                                                          38. Quintero RA et al: Sequential selective laser photocoagulation of communicating vessels in twin-twin transfusion syndrome. J Matern Fetal Neonatal Med. 20(10):763-8, 2007
                                                          39. Rychik J 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. 197(4):392, 2007
                                                          40. Graef C et al: Long-term neurodevelopmental outcome of 167 children after intrauterine laser treatment for severe twin-twin transfusion syndrome. Am J Obstet Gynecol. 194(2):303-8, 2006
                                                          41. Huber A et al: Stage-related outcome in twin-twin transfusion syndrome treated by fetoscopic laser coagulation. Obstet Gynecol. 108(2):333-7, 2006
                                                          42. Moise KJ Jr: Neurodevelopmental outcome after laser therapy for twin-twin transfusion syndrome. Am J Obstet Gynecol. 194(5):1208-10, 2006
                                                          43. Ozcan T et al: Use of middle cerebral artery peak systolic velocity and intrauterine transfusion for management of twin-twin transfusion and single fetal intrauterine demise. J Matern Fetal Neonatal Med. 19(12):807-9, 2006
                                                          44. Beck M et al: Long-term outcome of kidney function after twin-twin transfusion syndrome treated by intrauterine laser coagulation. Pediatr Nephrol. 20(11):1657-9, 2005
                                                          45. Cromi A et al: Sonographic umbilical cord morphometry and coiling patterns in twin-twin transfusion syndrome. Prenat Diagn. 25(9):851-5, 2005
                                                          46. Matias A et al: Search for hemodynamic compromise at 11-14 weeks in monochorionic twin pregnancy: is abnormal flow in the ductus venosus predictive of twin-twin transfusion syndrome? J Matern Fetal Neonatal Med. 18(2):79-86, 2005
                                                          47. Moise KJ Jr et al: A randomized trial of amnioreduction versus septostomy in the treatment of twin-twin transfusion syndrome. Am J Obstet Gynecol. 2005 Sep;193(3 Pt 1):701-7. Erratum in: Am J Obstet Gynecol. 193(6):2183, 2005
                                                          48. Senat MV et al: Endoscopic laser surgery versus serial amnioreduction for severe twin-to-twin transfusion syndrome. N Engl J Med. 351(2):136-44, 2004
                                                          49. Lougheed J et al: Acquired right ventricular outflow tract obstruction in the recipient twin in twin-twin transfusion syndrome. J Am Coll Cardiol. 38(5):1533-8, 2001