Twin-to-twin transfusion syndrome
|Twin-to-twin transfusion syndrome|
|Classification and external resources|
A pair of newborn twins affected by TTTS. Both the recipient (left) and donor (right) survived.
|ICD-10||O43.0, P02.3, P50.3|
|ICD-9||762.3 , 772.0|
Twin-to-twin transfusion syndrome (TTTS, also known as Feto-Fetal Transfusion Syndrome (FFTS) and Twin Oligohydramnios-Polyhydramnios Sequence (TOPS)) is a complication of aberrant placental blood vessel growth in monochorionic multiples, that is, multiple pregnancies where two or more fetuses share a chorion and hence a single placenta.
Twin-twin transfusion syndrome (TTTS) is diagnosed prenatally by ultrasound imaging. The diagnosis requires that certain symptoms be present in an identical twin, or higher-order multiple pregnancy in which 2 or more babies share a single placenta. They are, for the "Monochorionic-Diamniotic" pregnancies, with Individual Amnions: The presence of oligohydramnios (defined as a maximal vertical pocket [MVP] of <2 cm) in one sac, and of polyhydramnios (a MVP of >8 cm) in the other sac. MVP of 2 cm and 8 cm represent the 5th and 95th percentiles for amniotic fluid measurements, respectively, and the presence of both is used to define stage I TTTS. If there is a subjective difference in amniotic fluid in the 2 sacs that fails to meet these criteria, progression to TTTS occurs in <15% of cases. Although growth discordance (usually defined as >20%) and intrauterine growth restriction (IUGR) (estimated fetal weight <10% for gestational age) often complicate TTTS, growth discordance itself or IUGR itself are not diagnostic criteria. The differential diagnosis may include selective IUGR, or possibly an anomaly in 1 twin causing amniotic fluid abnormality. Twin anemia-polycythemia sequence (TAPS) has been recently described in MCDA gestations, and is defined as the presence of anemia in the donor and polycythemia in the recipient, diagnosed antenatally by middle cerebral artery (MCA)–peak systolic velocity (PSV) >1.5 multiples of median in the donor and MCA PSV <1.0 multiples of median in the recipient, in the absence of oligohydramnios-polyhydramnios. Further studies are required to determine the natural history and possible management of TAPS. TTTS can occur in a MCDA twin pair in triplet or higher-order pregnancies.
The most commonly used TTTS staging system was developed by Quintero et al in 1999, and is based on sonographic findings. The TTTS Quintero staging system includes 5 stages, ranging from mild disease with isolated discordant amniotic fluid volume to severe disease with demise of one or both twins. This system has some prognostic significance and provides a method to compare outcome data using different therapeutic interventions. Although the stages do not correlate perfectly with perinatal survival, it is relatively straightforward to apply, may improve communication between patients and providers, and identifies the subset of cases most likely to benefit from treatment.
The Quintero staging of twin-twin transfusion syndrome 
|Stage||Ultrasound parameter||Categorical criteria|
|I||MVP of amniotic fluid||MVP <2 cm in donor sac; MVP >8 cm in recipient sac|
|II||Fetal bladder||Symptoms of Stage I except Donor has no measurable fluid, Nonvisualization of fetal bladder in donor twin over 60 min of observation|
|III||Symptoms of Stage II with Doppler anomalies in the Umbilical artery, ductus venosus, and umbilical vein||Absent or reversed umbilical artery diastolic flow, reversed ductus venosus a-wave flow, pulsatile umbilical vein flow|
|IV||Symptoms of Stage III with Fetal hydrops||Hydrops Fetalis in one or both twins|
|V||Single or Double Loss||Fetal demise of one or both twins|
MVP = maximal vertical pocket
Since the development of the Quintero staging system, much has been learned about the changes in fetal cardiovascular physiology that accompany disease progression (discussed below). Myocardial performance abnormalities have been described, particularly in recipient twins, including those with only stage I or II TTTS. Several groups of investigators have attempted to use assessment of fetal cardiac function to either modify the Quintero TTTS stage or develop a new scoring system. While this approach has some benefits, the models have not yet been prospectively validated. As a result, a recent expert panel concluded that there were insufficient data to recommend modifying the Quintero staging system or adopting a new system. Thus, despite debate over the merits of the Quintero system, at this time it appears to be a useful tool for the diagnosis of TTTS, as well as for describing its severity, in a standardized fashion.
There are several second- and even first-trimester sonographic findings that have been associated with TTTS.
- Crown-rump length discordance
- Nuchal translucency >95th percentile or discordance >20% between twins
- Reversal or absence of ductus venosus A-wave
- Abdominal circumference discordance
- Membrane folding
- Velamentous placental cord insertion (donor twin)
- Placental echogenicity (donor portion hyperechoic)
TTTS was first described by a German obstetrician, Friedrich Schatz, in 1875. Once defined by neonatal parameters—differences in birth weight and cord hemoglobin at the time of delivery—TTTS is now defined differently. Today, it is known that discordant fetal weights will most likely be a late manifestation, and fetal hemoglobin through cordocentesis is often equivalent in the twin pair even in severe TTTS.
TTTS in art
A painting known as the De Wikkellkinderen (The Swaddled Children), from 1617, is thought to represent a depiction of TTTS. The drawing shows twins that appear to be identical, but one is pale (possibly anemic), while the other is red (possibly polycythemic). Analysis of the family histories of the owners of the painting suggests that the twins did not survive to adulthood, although whether that is due to TTTS is uncertain.
Although somewhat of a stretch, due to the detail of "magical birthgiving" in the folklore, an example of TTTS might have been noted ages before Schatz classified it or the painting "De Wikkellkinderen" illustrated, as an old Norse fairy tale, "Tatterhood", seemed to explain it, with one of the two girls being lovely but weak (pale skin and delicacy of anemia has often associated with how girls were most expected to be at the time) and the other one, the title heroine, was considered hideous and too strong (polycythemia has a higher blood cell count and often includes unsightly blemishes). Of course, with both twins growing up to be healthy adults, the debate of truth in fiction is still a mystery.
||This article's use of external links may not follow Wikipedia's policies or guidelines. (October 2013)|
- FREE Twin to Twin Transfusion Syndrome Patient Guides from Twins and Multiple Births Association, Tamba. The largest patient support group of its kind in the world
- The Twin to Twin Transfusion Syndrome Foundation
- Fetal Health Foundation
- Dr. Rubén Quintero at Jackson Memorial Hospital Miami, FL
- Texas Fetal Center (UTHealth Medical School and Children's Memorial Hermann Hospital) Twin to Twin Transfusion Syndrome
- The International Institute for the Treatment of TTTS
- Texas Children's Fetal Center, Twin-Twin Transfusion Syndrome
- TTTS Educational Video – Texas Children's Fetal Center
- Los Angeles Fetal Therapy, Children's Hospital Los Angeles - University of Southern California Fetal Therapy Program-TTTS
- The Center for Prenatal Pediatrics at Columbia University Medical Center- TTTS Evaluation & Laser Photocoagulation Therapy
- Twin-Twin Transfusion Syndrome – Children's Hospital Boston
- The Children's Hospital of Philadelphia
- The Brown Fetal Treatment Program – What is TTTS?
- Ohio State's Maternal Fetal Medicine
- St. Louis Fetal Care Institute TTTS Survivor Stories
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