Twin-To-Twin Transfusion Syndrome (TTTS)

Twin-to-twin
transfusion syndrome
(TTTS)
報告 : 黃貞瑜醫師
指導 : 洪正修主任、楊明智主任
 > 20% discordance in birthweight, and >
5 g/dL discordance in cord haemoglobin
levels –insufficient
 ultrasound-based criteria, with particular
attention to amniotic fluid discordance,
bladder volumes, and fetal Doppler
studies.
Pathophysiology
1. Placental architecture
 Almost all monochorionic twins have
intertwin vascular anastomoses.
 Arterio-arterial (AA) anastomoses and
veno-venous (VV) anastomoses are
superficial anastomoses, travelling
across the surface of the placenta
without interruption between the two cord
insertions.
 Arterio-venous (AV) anastomoses are deep
anastomoses, where an unpaired artery
and vein pierce the chorionic plate in close
adjacency to supply a shared placental
cotyledon. --provide unidirectional flow of
blood from the donor to the recipient.
 TTTS results from intertwin transfusion
across shared placental vascular
anastomoses
 TTTS occurred uncommonly (15%) despite
the high frequency of occurrence of cross-
placental vascular communication.
 TTTS is more likely to develop when there is a
paucity of bi-directional AAs and VV
anastomoses that can assist with regulation of
intertwin circulatory imbalances.
 The larger the number and type of intertwin
anastomoses, the less frequently clinical TTTS
is observed.
 the antenatal detection of AA anastomoses
predicts higher perinatal survival in
pregnancies complicated by TTTS.
Pathophysiology
2. The fetal response
 Transfusion through the unidirectional AV
anastomoses creates hydrostatic
differences between the twins.
 Atrial natriuretic peptide (ANP) and
vasopressin levels in the twins diverge;
the donor responds with oliguria, and the
recipient with polyuria and
polyhydramnios (Quintero stage 1).
 The resultant haemoconcentration in
the recipient creates an osmotic
gradient from the maternal compartment,
worsening the polyhydramnios
 As donor perfusion pressure continues to
fall urine production finally ceases
(Quintero stage 2), resulting in a ‘stuck
twin’.
 The resultant inability to swallow
aggravates the donor twin's hypotension, and
vasoconstrictor peptides, such as the
renin-angiotensin system (RAS)
mediators, increase dramatically increased
arterial resistance in the donor's placental
territory growth restriction.
 Absent or reversed end-diastolic flow
(A/REDF) in the donor umbilical artery
(UA) may be seen (Quintero stage 3: donor).
 These RAS mediators are also transfused to the
recipient via placental anastomoses （ similar
cord levels of renin and aldosterone despite
discordant renal expression of renin between
donors and recipients. ）
 Systemic hypertension in the recipient fetus,
initiated by the increase in cardiac output, now
worsens.
 [endothelin and fetal natriuretic peptides]: higher
in recipient twins--these mediators likely work
synergistically to induce pressure-overload
cardiomyopathy.

 Fetal echocardiography in recipient ： the
presence of cardiomegaly secondary to
biventricular hypertrophy, with the
majority exhibiting right ventricular
systolic and biventricular diastolic
dysfunction.
 Right ventricular outflow tract obstruction
may evolve （ 10% of recipient fetuses ）
 Venous Dopplers – ductus venosus (DV)
and umbilical vein (UV) – may now
deteriorate (Quintero stage 3: recipient).
 Continuing feto–fetal transfusion in the face of
such cardiac dysfunction  progression to fetal
hydrops (Quintero stage 4).
 Whether by terminal hypoperfusion in the donor, or
by cardiac failure in the recipient, single fetal
demise may ensue (Quintero stage 5).
 At or around the time of this death, acute feto–fetal
haemorrhage from the survivor into the placental
and fetal vascular compartment of the dead
twin can occur through the patent intertwin
vascular anastomoses.  profound hypotension
 high risk of death or severe neurological injury
(approximately 30% for each) in the co-twin.
Disease staging
Screening for TTTS
1. nuchal translucency
 Discordant crown–rump length in the first trimester
does not identify those pregnancies destined to
develop TTTS, but increased nuchal
translucency (NT) and/or NT discordance in
monochorionic twins is associated with an
increased risk for subsequent development of
TTTS.
 Increased NT (> 95th centile) at 10–14 weeks a/w
a likelihood ratio of 3.5 (95% CI, 1.9–6.2) for the
development of severe TTTS. （ Sebire et al.
Hum Reprod 2000 ）
 This transient finding probably reflects
impaired ventricular function of the
immature fetal heart in the hypervolaemic
recipient twin;
 the improvement with advancing
gestation is likely because of improved
ventricular compliance and the
establishment of diuresis.
Screening for TTTS
2. First trimester ductus venosus (DV)
 Among twin with discordant NT,
discordant reversal of the ‘a’ wave in the
DV was useful in identifying those twins
that went on to develop TTTS. （ Matias
et al. J Matern Fetal Med 2005 ）
Screening for TTTS
3. Intertwin membrane folding
 an early ultrasound marker of amniotic
fluid discordance
 the likelihood ratio of membrane folding
on ultrasound at between 15 and 17
weeks gestation for the subsequent
development of TTTS was 4.2 (95% CI
3.0–6.0). （ Sebire et al. Hum Reprod
2000 ）
Surveillance for TTTS
 BIW after NT assessment for monochorionic

twins
 amniotic fluid discordance, intertwin
membrane folding, bladder volumes, and
Doppler studies.
Diagnosis and assessment
of TTTS
 Minimum sonographic criteria ：
(i) monochorionic twins, that is, single
placenta, same sex twins, and absence of
intervening chorion (‘twin peak’ sign);
(ii) oligohydramnios (maximal vertical pocket
(MVP) ≤ 2 cm) in the donor sac; and
(iii) polyhydramnios (MVP ≥ 8 cm) in the
recipient sac.
Differential diagnoses
1. selective intrauterine growth restriction
(IUGR), which also affects 15% of
monochorionic twins, and may result in
oligohydramnios, delayed growth and
abnormal umbilical Dopplers in one twin.
2. monochorionic twins discordant for anomaly
(particularly renal agenesis) may result in
anhydramnios around one twin.
-- neither of these conditions is associated
with polyhydramnios in the other twin
Treatment options
 untreated perinatal mortality for severe

midtrimester TTTS is up to 90%.
1. Selective laser photocoagulation
(SLPC) of intertwin vascular
anastomoses
2. Amnioreduction and septostomy
3. cord occlusion in TTTS
TTTS in monochorionic
monoamniotic (MCMA)
 much less common ∵nearly all MCMA placentas
have AA anastomoses, and a decreased number
of AV anastomoses, when compared to MCDA
placentas.
 may still occur --will lack the classic sign of
discordant sac size.
--The combination of polyhydramnios in the single
amniotic cavity with discordant bladder size is
usually sufficient to make the diagnosis,
particularly where there are discordant cord
diameters and abnormal Doppler waveforms.
-- Stage 1 disease, however, may escape detection.
TTTS in
Dichorionic diamnion?
Dizygotic twins?
1. monochorionic dizygotic
twins seems increase after
pregnancy by ART(?)
 Monochorionic (MC) dizygotic twins (DZT) are
extremely rare in natural pregnancy
1. Unusual monochorionic placentation with
heterosexual twins. （ ObstetGynecol 1970;36:621-
5. ）
2. sex-discordant monochorionic twins conceived by in
vitro fertilization （ The New England Journal of
Medicine. 2003. 349(2): 154-159 ）
3. DZ monochorionic twins conceived by ART, of which
one has both Klinefelter syndrome and Beckwith-
Wiedemann syndrome (BWS). （
Journal of Pediatrics.2005, 146(4):565-567 ）
--J Hum Genet. 2005;50(1):1-6.
2. Twins with two separate placental
masses can still be monochorionic and
therefore have vascular anastomoses
pathogenesis
of bipartite
placentation
in MC
twinning ：
not clear
American Journal of Obstetrics and Gynecology 2006

True DADC?
 The combination of the lambda sign or 2 separate
placentas on sono in twin pregnancies predicts
dichorionicity with a sensitivity of 97% and a
specificity of 100% ; “T” sign -- the most useful sign
in predicting monochorionicity with a sensitivity of
100% and a specificity of 98%. （ GA 10-14 wks ）
 2 separate placental masses are not per se DC
 Microscopic examination of the intertwin membrane
after delivery -- the gold standard for chorionicity
If true DADC
 Anastomotic communication was found
almost universally in monochorionic
placentation and very rarely with
dichorionic placentas. （ Placental injection
studies in twin gestation. Robertson EG. Am J Obstet
Gynecol 1983; 147(2): 170-174 ）
 Vascular Anastomoses in Dichorionic
Diamniotic-Fused Placentas side-to-side
：
connections between small subchorionic

vessels. （ International Journal of Gynecological
Pathology. 22(4):359-361, October 2003 ）
Non-immune hydrops fetalis
1. Cardiac failure
2. Anemia
3. Arteriovenous shunts
4. Mediastinal compression
5. Metabolic disorder
6. Fetal infection/tumor
7. Congenital renal/pulmonary/GI/skeletal defect
8. Chromosomal anomalies

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Twin-to-twin

 BIW after NT assessment for monochorionic

 untreated perinatal mortality for severe

American Journal of Obstetrics and Gynecology 2006

connections between small subchorionic

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