Twin-twin transfusion syndrome and twin anemia polycythemia sequence: Pathogenesis and diagnosis
- Ramesha Papanna, MD, MPH
Ramesha Papanna, MD, MPH
- Assistant Professor
- Department of Obstetrics & Gynecology
- The University of Texas McGovern Medical School at Houston
- Anthony Johnson, DO
Anthony Johnson, DO
- Departments of Obstetrics & Gynecology and Pediatric Surgery
- University of Texas McGovern Medical School at Houston
- Section Editors
- Deborah Levine, MD
Deborah Levine, MD
- Section Editor — Imaging
- Professor of Radiology
- Director of Ob/Gyn Ultrasound
- Department of Radiology
- Beth Israel Deaconess Medical Center
- Louise Wilkins-Haug, MD, PhD
Louise Wilkins-Haug, MD, PhD
- Section Editor — Prenatal Diagnosis and Genetics
- Professor of Obstetrics, Gynecology, and Reproductive Biology
- Harvard Medical School
Twin-twin transfusion syndrome (TTTS) is one of the most serious complications of monochorionic multiple gestations. The cardinal prenatal findings are monochorionic placentation with concordant gender and discordant amniotic fluid volumes (maximum vertical pocket: donor <2 cm and recipient >8 cm ). TTTS is associated with a high risk of fetal/neonatal mortality, especially in previable gestations, and fetuses who survive are at risk of severe cardiac, neurologic, and developmental disorders.
Twin anemia polycythemia sequence (TAPS) is an atypical chronic form of TTTS without oligohydramnios-polyhydramnios sequence. Prenatally, TAPS can be diagnosed when the middle cerebral artery-peak systolic velocity (MCA-PSV) is greater than 1.5 multiples of median (MoM) in one twin and less than 0.8 MoM in the other twin, although criteria are not uniform across studies. Placental discordance is typically noted on ultrasound: the anemic donor has a thickened hyperechoic placenta and the plethoric recipient has a thinner hypoechoic placenta, with clear demarcation between the donor and recipient territories.
This topic will review the pathogenesis and diagnosis of TTTS and TAPS. Management of TTTS and TAPS is discussed separately. (See "Twin-twin transfusion syndrome: Management and outcome".)
TWIN-TWIN TRANSFUSION SYNDROME
Incidence — The incidence of TTTS is not clear since some fetal losses in monochorionic multifetal gestations in the first half of pregnancy may be related to undiagnosed TTTS . For this reason, incidence based on data from live borns or sonograms in the second half of pregnancy may not be accurate. Our best estimate of the incidence of TTTS is 1:40 to 1:60 twin pregnancies [2-5], 9 to 15 percent of monochorionic diamniotic pregnancies [1,3], and 6 percent of monoamniotic pregnancies . In one report, monochorionic diamniotic twins conceived by in vitro fertilization had a lower incidence of TTTS than those conceived naturally (1/43 [2 percent] versus 36/284 [13 percent]) .
Pathophysiology — There is no animal model for study of TTTS, but computer models have been developed [8-10].
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- TWIN-TWIN TRANSFUSION SYNDROME
- - Unbalanced vascular anastomoses
- - Cardiovascular response to circulatory imbalance
- Clinical manifestations and diagnosis
- Differential diagnosis
- - Quintero stages
- - Cardiovascular score
- Monitoring for TTTS
- - Our approach
- - Diagnosis of TTTS versus selective intrauterine growth restriction
- TWIN ANEMIA POLYCYTHEMIA SEQUENCE
- Natural history
- SUMMARY AND RECOMMENDATIONS