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320 E.P. Gaziano, U. F. Harkness<br />

ing at < 28 weeks, a survival rate of only 21% was reported<br />

for TTS and neither decompression amniocentesis<br />

nor tocolysis altered this outcome [65].<br />

Pathology of Monochorionic Placentation<br />

When placentas from monochorionic twins are compared<br />

with the presence or absence of TTS, placentas<br />

from pregnancies with TTS had fewer anastomoses<br />

overall, and fewer anastomoses for each specific type,<br />

such as arterioarterial (AA), venovenous (VV) and<br />

arteriovenous (AV) [66, 67]. In addition, anastomoses<br />

in the TTS group tended to be indirect or AV, rather<br />

than direct AA or VV when compared to monochorionic<br />

twins without TTS. Hemodynamic models of<br />

TTS in monochorionic twins support the pathologic<br />

findings that fewer anastomoses effect greater discordance<br />

between monochorionic twins and that the discordance<br />

increases beyond fetal compensatory capacity<br />

[68]. Color Doppler insonation of placental vasculature<br />

in monochorionic twins also demonstrates an<br />

absence of functional AA anastomoses in monochorionic<br />

twins with TTS [69]. In assessment of clinical<br />

outcomes, AV anastomoses in the absence of AA and<br />

VV, especially if present with placental asymmetry,<br />

carry the worse prognosis [67]. Unidirectional AV<br />

flow in the absence of compensatory bidirectional<br />

anastomoses results in adverse fetal effects and poor<br />

outcomes for some monochorionic gestations.<br />

The placenta of the donor twin may have a small,<br />

inconspicuous vascular supply and may demonstrate<br />

a large number of villi per unit volume of placenta,<br />

whereas the recipient twin demonstrates an increase<br />

in the volume of fetal capillaries [70]. Donor twins<br />

may have a deficiency of maternally transferred immunoglobulin<br />

G (IgG), indicating impaired intervillous<br />

maternal-fetal transfer [71].<br />

Placental Symmetry and TTS<br />

Abnormalities of monochorionic placental symmetry<br />

have received less attention than the anastomoses but<br />

are of importance. Monochorionic twin placental<br />

asymmetry has been variously described as unequal<br />

sharing of venous return zones, unequal allocation of<br />

parenchyma, and discordant vascular perfusion zones,<br />

but a precise quantitative definition is lacking [6].<br />

The morphogenesis of MC placental asymmetry is<br />

also unknown, but the monochorionic monozygotic<br />

(monochorionic MZ) twin blastocyst has an intrinsic<br />

polarity defect at implantation [6]. The portions of<br />

the monochorionic twin placenta in TTS are often<br />

asymmetrical with the donor twin's placenta typically<br />

the smaller [5, 67, 72]. The threshold for significant<br />

asymmetry (e.g., 60 : 40, 70 : 30, 80:20, 90 : 10, etc.)<br />

and clinical placental insufficiency in one twin may<br />

vary in each case and depend on gestational age and<br />

type and number of anastomotic vessels. In the absence<br />

of anastomoses, unequal sharing is an important<br />

cause of growth discordance in monochorionic<br />

twins [73]. The vascular anastomoses place an monochorionic<br />

twin with an adequate placental share at<br />

risk for pathophysiologic processes of the twin with<br />

placental insufficiency. Conversely, the anastomoses<br />

may sustain a twin with a small share by supplementing<br />

nutrients, which would otherwise be deficient. In<br />

addition to quantitative differences, the asymmetric<br />

monochorionic portions may differ qualitatively in<br />

placental circulation relative to umbilical cord insertions,<br />

chorion surface vessel pattern, and villous capillaries.<br />

In monochorionic twin gestations, the relative<br />

roles of reduced placental mass, abnormal cord insertions,<br />

and intertwin vascular anastomoses are difficult<br />

to ascertain and often occur together.<br />

Umbilical Artery Doppler Velocimetry ±TTS<br />

A summary of fetal Doppler evaluation of TTS is presented<br />

in Table 20.3, including the method of assessment,<br />

principal findings, and conclusions.<br />

Umbilical artery velocimetry was first reported in<br />

5 of 76 twin pregnancies with TTS [24]. Using CW<br />

Doppler technology and a cutoff for A/B (S/D) ratio<br />

difference of 1.57 (greater than the 75th percentile),<br />

TTS pregnancies showed normal A/B ratios that were<br />

concordant. Growth restriction was present in 12 of<br />

18 monochorionic pairs, but an elevated A/B ratio<br />

was present only in 7 pairs. The authors concluded<br />

that the A/B ratios in TTS are normal and concordant,<br />

and that differences in size on ultrasonography<br />

in the presence of no S/D difference suggest TTS.<br />

Another report described TTS in two twin pregnancies<br />

± one at 20 weeks and one during labor [25].<br />

The amniotic fluid volume assay indicated hydramnios/oligohydramnios<br />

in the first case and a normal<br />

state in the second case. Using CW Doppler technology,<br />

the S/D ratio of the umbilical artery was abnormal<br />

in the SGA donor twin and normal in the recipient,<br />

and the S/D ratio difference was 1.9 (an S/D ratio<br />

of > 0.4 was the cutoff for twin abnormality).<br />

Serial Doppler and ultrasound examinations were<br />

reported in a twin pregnancy with TTS [74]. The donor<br />

twin showed abnormal velocimetry with cyclic<br />

variations thought to be due to an arterial anastomosis.<br />

This donor fetus demonstrated abnormal flow<br />

(zero and reversal) and died in utero. The recipient<br />

survived, demonstrating normal velocimetry.<br />

Pretorius et al. [75] reported fetal Doppler ultrasound<br />

findings in eight cases of TTS. Pulsed-wave<br />

duplex Doppler sonography was employed with highresolution<br />

ultrasound, and A/B ratios were determined<br />

for the umbilical artery. Significant A/B ratio<br />

differences were noted in seven of the eight cases,

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