Twin Embolization Syndrome
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Transcript of Twin Embolization Syndrome
INTERESTING CASE PRESENTATIONDr. A. JEYARAMAN, D.M.R.D., TUTOR,
Dr. S. THIYAGARAJAN, II YR D.M.R.D. – PG
DEPARTMENT OF RADIOLOGYGOVERNMENT RAJAJI HOSPITAL
MADURAI
• A 21 year old previous LSCS lady • G2P1L1A0• LMP-GA 30 weeks 5 days• Intrauterine Monochorionic twin
pregnancy which is confirmed by ultrasound at 11th week of pregnancy
• No family history of twin pregnancy
• Previous child was normal.
US findings• Intrauterine monochorionic twin gestation• Twin 1• Gross ventriculomegaly with thinned out
cerebral mantle• Multiple dilated small bowel loops s/o small
bowel atresia • Non visualisation of left kidney• No e/o spinal anomaly• No e/o cardiac anomaly
US findings…
• Twin 2• Fetus papyraceous• Side to side compressed head• Compressed body
Diagnosis
• Twin embolisation syndrome / vanishing twin syndrome
Discussion
Multiple gestation
Zygosity• Zygosity
• Genetic makeup of the pregnancy• Is determined by type of fertilization,
i.e. monozygotic or dizygotic• Zygosity can only be determined
by genetic analysis of both fetuses• USG can be used to determine the
likelihood of zygosity
Chorionicity
• Chorionicity• Membrane complement of the
pregnancy• Is determined by the occurrence and
timing• Determined non-invasively by
ultrasound
Dizygotic Pregnancy
• Twins resulting from 2 ova fertilized by 2 sperm
• These are ALWAYS dichorionic• Fused or separated
Monozygotic Pregnancy
• One ovum fertilized by one sperm that subsequently splits
Chorionicity
Chorionicity - USG
• Ultrasound detection of two separate placentas. • Confirms a dichorionic pregnancy• The detection of different-sex fetuses.
Chorionicity
• Ultrasound detection of a single placenta
• Either monochorionic or dichorionic (fused placenta)
Chorionicity
• Thick inter-twin membrane • Often taken as > 2 mm• All membranes look thin in third
trimester• Count layers with high resolution
transducer, if ≥ 2 must be DC
"Twin peak" or Lambda sign
• Chorionic tissue extends into inter-twin membrane at placenta
• Chorion forms echogenic triangle• Triangle base on placental surface,
apex fades into inter-twin membrane
• Reliable indicator of dichorionicity
Chorionicity
"T" sign
• Absent "twin-peak"• Membrane abuts placental surface
without triangle of chorionic tissue• Does NOT exclude dichorionicity• Monochorionic pregnancies have a
thin wispy membrane between the sacs made up of two layers of amnion and generally less than 1 mm in thickness
Chorionicity
Dichorionic diamniotic pregnancy
Visualization of two placentas and a dividing membrane ("half twin peak" sign) orVisualization of one placenta and a dividing membrane, plus a lambda sign
Monochorionic diamniotic pregnancy
Visualization of one placenta and a dividing membrane, plus a T sign
Monochorionic monoamniotic pregnancy
Visualization of one placenta; dividing membrane and T sign are not visualized
Zygosity - USG
• Twins are definitely dizygotic if they are of different sexes.
Monozygotic twins
• Single gestational sac with only one placenta
• Intertwined two umbilical cords• Conjoined twin
Complication of multiple pregnancy
• DICHORIONIC DIAMNIOTIC TWINS• Maternal complications> singleton
pregnancyo Hypertensiono Preeclampsiao Antenatal hemorrhage
• Placenta previa• Placental abruption• Other causes
o Postpartum hemorrhage• Perinatal mortality reported 10%
o Preterm delivery• Median gestational age (GA) twins at delivery 36 weeks
o Intrauterine growth restrictiono Anomalies
MONOCHORIONIC TWIN
• MONOCHORIONIC DIAMNIOTIC TWIN Vanishing twin Discordant twin growth TTTS Twin embolisation syndrome Parasitic twin (acardiac
acranius)/(TRAP sequence)
MONOCHORIONIC TWIN
• MONOCHORIONIC MONOAMNIOTIC TWINDiscordant twin growthTTTSConjoined twinsTangled umblical cords
Discordant twin growth
• May occur in monochorionic or dichorionic pregnancies
• Monochorionic more common• Discordant growth
One twin with intrauterine growth restriction • EFW < 10th percentile• AC difference > 20mm• EFW difference > 20%
USG
• Crown rump length disparity in first trimester predictor for discordant birth weight
• Series dichorionic pregnancies with demise/anomalies excluded
• CRL difference> 3 days at 11-14 weeks gestational age
USG
• Oligohydramnios about smaller twin• Sign of placental insufficiency• May also occur with anomaly or
aneuploidy• Twin-twin transfusion syndrome
unlikely unless monochorionic twins and polyhydramnios around other fetus
Color Doppler
• UmA • Significant difference in SD ratio>
15% between twins• SD ratio difference > 0.4 between
twins has also been used to predict discordance
Vascular anastomoses betweenfetus
• Present only in monochorionic twin placentas.
• Nearly 100% of monochorionic twin placentas have vascular anastomoses,but there are marked variations in the number, size, and direction.
• A-A anastomoses on the chorionic surface of the placenta have been identified in up to 75%.
Type of anastomoses
TTTS (Twin to Twin Transfusion Syndrome)
• Incidence : 4 - 20% of MC twins
• It is characterised by an imbalance of blood flow between the twins
• 15 - 20% of perinatal deaths in twins
• Donor twinHypovolemia
Hypotension, decreased venous
return
Growth retardation, anemia
Heart failure
Hydrops
Intrauterine death
• Recipient twinHypervolemia
Hypertension, increased venous
return
Myocardial hypertrophy, plethora
Heart failure
Hydrops
Intrauterine death
Ultrasound signs of TTTS
• Detection of monochorionic placenta with different echogenicities
• Detection of concordant external genitalia• Growth discordance between the twins
Discrepancy in abdominal circumference > 20 mm or Weight discrepancy > 20% relative to the larger twin
• Unequal amniotic fluid volumes Donor: oligohydramnios (stuck twin) Recipient: polyhydramnios
• Unequal bladder filling Donor little or no visible bladder filling Recipient: well-distended bladder
• Unequal umbilical cord thickness Donor: thin umbilical cord, Recipient: thick umbilical cord
• Hydrops of one fetus• Marked discrepancy in Doppler findings (umbilical
artery) between the two umbilical cords S/D ratio discrepancy > 0.4
• Color Doppler: development of tricuspid insufficiency in the recipient
• Vascular anastomoses in the chorionic plate may be directly visualized with color Doppler
Staging of TTTS
• Stage 1: Donor bladder visible, normal Doppler
• Stage 2: Donor bladder empty, normal Doppler
• Stage 3: Donor bladder empty, abnormal Doppler
• Stage 4: Hydrops in recipient• Stage 5: Demise of one or both
TTTS (Twin to Twin Transfusion Syndrome)
Diamniotic Gestation with Stuck Twin
Twin Embolization Syndrome
• Uncommon• Death of One Twin In Utero• Blood Products from Dead Twin
Shunted• Results
• Disseminated Intravascular Coagulopathy
• Multifocal Tissue Infarction
Twin Embolization Syndrome
Passage of thromboplastin like material on embolic debris into the circulation of the surviving twin
A variety of ischemic or vascular disruptive defects of the central nervous system, gastrointestinal tract, on genitouninamy tract.
Twin embolization syndrome: Current theory
• Twin demise => loss of peripheral resistance• Vascular anastomoses between twins due tomonochorionic placentation• Abrupt drop in peripheral resistance secondary todemise hypotension in live twino End result is "hypoperfusion" lesions of brain andkidneys
Intraventricular hemorrhage Porencephaly Periventricular leukomalacia Renal infarction
Twin Embolization Syndrome
• CNS anomalies Fetal ventriculomegaly Porencephalic cyst Fetal cerebral atrophy Cystic encephalomalacia Microcephaly
• GI anomalies Hepatic and splenic infarcts Small bowel atresia Gastroschisis
Twin Embolization Syndrome
• Genitourinary AnomaliesFetal Renal Cortical NecrosisHorse shoe kidney
• Other findingsFetal HydrothoraxPulmonary InfarctsHemifacial microsomiaAplasia cutis congenitaTerminal Limb Defects
TRAP (Twin Reversed Arterial Pressure)
• Acardiac Monster• Extremely Rare <1:25000• Large arterio-arterial anastomosis• “Pump” & “Perfused” twins• Perinatal mortality in the pump
twin is 55%, due to polyhydramnios and cardiac failure
USGAcardiac twin• Dysmorphic with edema and cyst
formation in soft tissues• No cardiac structures or activity• Often no identifiable cranial
structures• Presence and structure of upper
extremities variable• Usually recognizable torso and
lower extremities
USG
• Lower extremities move spontaneously
• Single umbilical artery in 66% of acardiac twins
• Polyhydramnios• Strong correlation with presence of
renal tissue in acardiac twin• Increases risk for premature labor
CONJOINED TWINS
• Thoracopagus: Fused at chest• Omphalopagus: Fused xiphoid to
umbilicus• Thoraco-omphalopagus: Extensive
chest and abdominal fusion• Pygopagus: Fused at buttocks• Ischiopagus: Fused at hips• Craniopagus: Fused at cranial level
Craniopagus occipitalis. Cephalothoracopagus monosymmetros. Thoracopagus.
Pygopagus.
Ischiopagus.
CONJOINED TWINS
• Symmetrical conjoined twins ("Siamese twins")
• Asymmetrical conjoined twins (autosite and parasite)
Symmetrical conjoined twins
• Same-sex twins that are joined at certain body sites
• Often called "Siamese twins" • Forms
Complete symmetrical conjoined twins
Incomplete symmetrical conjoined twins
• Complete form• Both twins are equally well developed
and are conjoined at certain body regions.
• Incomplete form• The superior or inferior part of the
body is duplicated in varying degrees
Omphalopagus twins shows a fused liver
Common heart
Asymmetrical conjoined twins
• One is complete than the other, fully developed twin (autosite)