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Judi

Januadi Endjun

Gatot Soebroto Army Central Hospital/ Medical Faculty, University of Indonesia ISUOG Meeting, Bali, 2009

MATERI AJAR INI HANYA UNTUK DIPERGUNAKAN DALAM KEGIATAN PENDIDIKAN DAN KESEHATAN
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AGENDA
Denitions Introduction Etiology of twins Diagnosis of twins Vanishing twins Perinatal loss in twins Placentation Complications and Abnormality in twins pregnancy Conclusion Take home messages References
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DEFINITIONS
Monozygotic twins : a pregnancy of 2 fetuses derived

from the same zygote that divided between the day of fertilization and the 14th day. They can have phenotypic and genotypic dierences and can have either a dichorionic or a monochorionic placentation.

Dizygotic twins : a pregnancy of 2 fetuses derived from

2 dierent zygotes, resulting from the fertilization of 2 oocytes from the same cycle. They always have a dichorionic placentation.
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Matias A, Jeanty P, Toy EC. Sonography in multiple gestation. In: Sonography in Obstetrics & Gynecology, 7th Ed, 2011:337-375

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DEFINITIONS
Chorionicity : type of placentation in cases of multiple

pregnancy dened by the number of chorions.

Lambda sign : sonographic sign resulting from the

juxtaposition of 2 layers of amnion and 2 layers of chorion in a dichorionic twin pregnancy.

Matias A, Jeanty P, Toy EC. Sonography in multiple gestation. In: Sonography in Obstetrics & Gynecology, 7th Ed, 2011:337-375 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

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INTRODUCTION
Denition: any pregnancy in which 2 embryos or fetuses occupy the uterus simultaneously Epidemic of twins: ART, delayed childbearing, and ovulation induction USA (2003): 67% twins; 500% triplets and high-order The most profound implication: preterm delivery infant death
Maryam Tarsa et al. Multifetal gestation and malpresentation. In: Essentials of obstetrics and gynecology, 5th Ed, 2010 Young Mi Lee et al. Multiple pregnancy. In: Management of High-Risk Pregnancy. An Evidence-based Approach, 2007,304-315 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

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INTRODUCTION
3.2% of all live births (US 2003) (Natality Data Set, CDC, 1997 2002) 14 25% are IUGR and 25% require NICU (Mauldin J et al, 1998; Ettner SL et

Cerebral palsy: 4x (gemelli), 17x (triplet) (Elliott JP et al, 1992; Grether JK et al,
al, 1997) 1993)

IUFD: 4x (ACOG, 2004) The likelihood of not surviving the 1st year of life: 7x (Luke B et al,
1994; Kiely JL et al, 1992)

Twin-specic problems: TTTS, MCMA, conjoined Maternal complications: preeclampsia, DM: 2 - 3x (Roach VJ et al, 1998; Sibai
BM et al, 2000) JJE-20091119 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

ETIOLOGY OF TWINS
Depending on the number of eggs fertilized at conception

monozygotic or dizygotic

Monozygotic: identical, same genetic make up, the rate is constant throughout the world (1/250 pregnancies), type of placentation (DCDA, MCDA, and MCMA) and the likelihood of complications. ART: monozygotic twins: alter the zona pellucida around the time
of fertilization or delayed blastocyst implantation
Egan JFX et al. Ultrasound evaluation of multiple pregnancies. In Ultrasonography in obstetrics and gynecology. Callen, 5th Ed,2008;266-296) JJE-20091119 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

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MONOCHORIONIC TWIN

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TWINNING RATE
Twinning rate (per 1000 pregnancies) in England and Wales, 19601990 for all twins (diamond markers), dizygotic twins (square markers) and monozygotic twins (triangle markers; adapted from Derom et al. 1995)

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GROWTH RESTRICTION

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Frequency and mortality according to the types of placenta6on

DA-DC Separate placentae

DA-DC Fused placentae

DA-MC Single placentae

MA-MC Single placentae

Frequency Mortality
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35% 13%

27% 11%

36% 32%

2% 44%

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DIAGNOSIS OF TWINS
Anamnesis: risk factors Physical examination: dicult ULTRASOUND: should begin with a complete imaging sweep of the uterus FIRST TRIMESTER ULTRASOUND: number of GS and embryo, location of placenta, dividing membrane, AF, YS, and FHR determine chorionicity potential complications
Egan JFX et al. Ultrasound evaluation of multiple pregnancies. In Ultrasonography in obstetrics and gynecology. Callen, 5th Ed, 2008;266-296) JJE-20091119 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

SONOGRAPHY IN TWINS
Determination of the number of fetuses, amnionicity,

chorionicity, placental location, fetal presentation

Detection of complication such as : Growth discrepancy Abnormal vascular anastomosis Amniotic uid volume imbalance Fetal malformations Cord entanglement
Matias A, Jeanty P, Toy EC. Sonography in multiple gestation. In: Sonography in Obstetrics & Gynecology, 7th Ed, 2011:337-375 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

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ULTRASOUND IN TWIN
There is good evidence that the diagnosis of

twin gestation is improved by the routine use of ultrasound.

There is consensus that serial

ultrasonographic evaluation every three to four weeks is indicated in twin gestations. (I B)


SOGC, Management of twin pregnancy (Part 1), July, 2000 JJE-20091119 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

ULTRASOUND IN TWIN
Fetal growth diers slightly in twin gestations and

twin specic charts may be used to dene the normal growth rate. race specic charts.

Precision may also be obtained by using sex and

In clinical practice, however, these dierences are

small and singleton growth curves may be used.


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SOGC, Management of twin pregnancy (Part 1), July, 2000 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

ULTRASOUND IN TWIN
Patterns of fetal growth are more important

than absolute measurements.

Both must be interpreted in the light of the

clinical history, together with all the genetic and environmental factors that may aect fetal growth. (III B)
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ULTRASOUND IN TWIN
The diagnosis of discordance has been based

on the following:

AC dierence of 20 mm (sensitivity of 80%,

specicity 85%, PPV 62%)

EFW based on BPD and AC or AC and FL > 20 %

(sensitivity 25-55%) (II-2 B)


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SOGC, Management of twin pregnancy (Part 1), July, 2000 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

ST TRIMESTER ULTRASOUND 1
u Every eort should be made to determine chorionicity at

the time of diagnosis. (II-3 C) weeks. (II-3 C)

u The optimal time to determine chorionicity is 10-14 u While these recommendations apply to diagnosis of twin

pregnancy with regard to prenatal diagnosis and counseling, there have been no studies relating the establishment of prenatal chorionicity to pregnancy outcome.
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SOGC, Management of twin pregnancy (Part 1), July, 2000

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VANISHING TWINS
EARLY (< 8 weeks) Delivery < 32 W NICU > 28 days
Neurodevelopment disorders

INTERMEDIATE (> 8 and < 22 weeks)

(> 22 weeks)

LATE

1.9% 8.7% 3.3%


Comparable with singletons

5.3% 15.7% 8.0%

21.4% 43.8% 9.7%

Pregnancy outcome

Egan JFX et al. Ultrasound evaluation of multiple pregnancies. In Ultrasonography in obstetrics and gynecology. Callen, 5th Ed,2008;266-296) JJE-20091119 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

PERINATAL LOSS IN TWINS


IMR:

> 5 x = 32.9/1000 live-born twins (USA, 1999) Survival depends on chorionicity: anomalies, growth
problems & prematurity

Cumulative loss rate: 3% dichorionic & 15% monochorionic (Sabire et al, 1997) Losses are more likely to occur between 16 22 W

ultrasound examination every 1 2 W to screen TTTS Fetal demise of one twin, cerebral palsy Maternal complications: preeclampsia, GDM
Egan JFX et al. Ultrasound evaluation of multiple pregnancies. In Ultrasonography in obstetrics and gynecology. Callen, 5th Ed,2008;266-296) JJE-20091119 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

TWINS DISCORDANT
In twins discordant for abnormality, the option

of selective reduction should be oered. level center. be covered.


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The procedure should be performed in a tertiary Transportation and out-of-province costs should
SOGC, Management of twin pregnancy (Part 1), July, 2000 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

PLACENTATION
The most important is the identication of

chorionicity

Ultrasound is very useful in determining

placentation (chorionicity and amnionicity) and are very important in predicting twin pregnancy complications
Egan JFX et al. Ultrasound evaluation of multiple pregnancies. In Ultrasonography in obstetrics and gynecology. Callen, 5th Ed,2008;266-296) HANYA UNTUK PENDIDIKAN DAN KESEHATAN

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PLACENTATION
Chorionicity and amnionicity First, second and third trimester Membrane insertion, twin-peak sign Membrane thickness Membrane layers Multiple sonographic markers to determine

chorionicity and amnionicity Monoamniotic twins


Egan JFX et al. Ultrasound evaluation of multiple pregnancies. In Ultrasonography in obstetrics and gynecology. Callen, 5th Ed,2008;266-296) HANYA UNTUK PENDIDIKAN DAN KESEHATAN

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Sonographic determinaFon of chorionicity and amnionicity in rst trimester twins gestaFons


Placentation Gestational Sacs Yolk Sacs Embryos / Sac Amniotic Cavities

DC, DA MC, DA MC, MA

2 1* 1*

2 2
1 or partially divided*

1 2* 2*

2 2 1

* Amnionicity cannot be determined by this nding


Egan JFX et al. Ultrasound evaluation of multiple pregnancies. In Ultrasonography in obstetrics and gynecology. Callen, 5th Ed,2008;266-296) JJE-20091119 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

MONOCHORIONIC & DICHORIONIC

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TRIPLETS

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QUADRUPLET

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Complica6ons and Abnormality in twins pregnancy


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CONJOINED TWINS
MC, MA twins
Embryo divides at 13 to 15 days from conception The two fetal poles may be attached at varying sites (Early

ultrasound nding: bid appearing fetal pole) Visualizing in the same relative position in all views Direct opposition of the twins from each other Extreme extension of the fetal spine Inseparable skin contour must be persistent Prognosis: very poor
Egan JFX et al. Ultrasound evaluation of multiple pregnancies. In Ultrasonography in obstetrics and gynecology. Callen, 5th Ed,2008;266-296) JJE-20091119 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

Sumber: Dr. dr. Adityawarman, SpOG(K)

CONJOINED TWINS

Adapted from: Romero, R., Pilu, G., Jeanty, P., Ghidini, A. and Hobbins, J.C.(1988). Prenatal Diagnosis of Congenital Anomalies, p 405. ( courtesy from Philippe Jeanty www.thefetus.net ) http://www.centrus.com.br/DiplomaFMF/SeriesFMF/11-14weeks/images-thefetus/conj-01.jpg JJE-20091119 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

ECTOPARASITIC TWINS
Ectoparasitic twins are parts of twins implanted in another fetus. In this case what appears to be an omphalocele on the left is a fetal abdomen with lower legs on the extreme left.
(Courtesy Glynis Sack, MD, www.TheFetus.net)

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TWIN TO TWIN TRANSFUSION SYNDROME


MC twin placental vascular anastamoses communication of the two fetoplacental circulations; may be arterioarterial, venovenous, or arteriovenous in nature (Benirschke K. Twin placenta in perinatal mortality. N Y St J Med
1961;61:1499508)

This phenomenon of a shared circulation between

monochorionic twins was rst described by Schatz in 1882


(Schatz F. Eine besondere Art von einseitiger Polyhydramnie mit anderseitiger Oligohydramnie bei eineiigen Zwillingen. Arch Gynakol 1882;19:329)

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TWIN TO TWIN TRANSFUSION SYNDROME

NEJM, July 2004


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TWIN TO TWIN TRANSFUSION SYNDROME


Anatomical studies

arteriovenous anastomoses are deep in the placenta but almost always proceed through the cotyledonary capillary bed (Benirschke K, Kim CK. Multiple pregnancy.
N Eng J Med 1973;288:127684)

25% of MC twins imbalance in the net ow of blood

across the placental vascular arteriovenous communications from one fetus, the donor, to the other, the recipient, twin-to-twin transfusion syndrome; 50% of these casessevere twin-to-twin transfusion syndrome acute polyhydramnios in the second trimester
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Staging of twin to twin transfusion syndrome, Quintero RA et al, 1999


Stage Amniotic Fluid D: oligo R: poly As above As above As above As above Fetal Bladder Normal D: bladder not seen As above As above As above MCA Doppler, UA or UV Normal Normal Abnormal Abnormal Abnormal Hydrops Fetal Demise No No No No Yes, either twin

I II III IV V

No No No Yes, either twin Yes, either twin

Egan JFX et al. Ultrasound evaluation of multiple pregnancies. In Ultrasonography in obstetrics and gynecology. Callen, 5th Ed,2008;266-296)

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TTTS

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STUCK TWINS

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Twin reversed arterial perfusion sequence (TRAP)


TRAP (acardiac twin): 1 : 35,000 pregnancies The most extreme manifestation of TTTS

1% of MC twin is acardiac twinning (acardius chorioangiopagus parasiticus).

Disruption of normal vascular perfusion and development of

one twin (the recipient) due to an umbilical arterio arterio anastomosis with the other (donor or pump) twin

(Van Allen MI, Smith DW, Shepard TH. Twin reversed arterial perfusion (TRAP) sequence: study of 14 twin pregnancies with acardius. Semin Perinatol 1983;7:28593)

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Twin reversed arterial perfusion sequence (TRAP)


50% of donor twins die due to congestive heart

failure or severe preterm delivery, the consequence of polyhydramnios (Van Allen MI et al, 1983; Moore TR, 1990) malformations. (Nicolaides KH et al, 2001)

All perfused twins die due to the associated multiple Acardiac twins: hydrops, heart beat +/- (rudimentary
Hecher et al. 2009)

pulsatile cardiac structure), reversed perfusion via SUA (Kurt


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Twin reversed arterial perfusion sequence (TRAP)

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GENETIC TESTING
All women carrying twin pregnancies should be

referred for counseling to a centre for the consideration of invasive testing at age 32.

The counseling must be individualized and the nal

decision must be taken by the parents since the risk of amniocentesis is uncertain in twin gestation. (II-3 C)

SOGC, Management of twin pregnancy (Part 1), July, 2000

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GENETIC SCREENING
Biochemical screening for aneuploidy is not recommended

in twins.

MS-AFP is useful for detection of open neural tube and

other birth defects. (II-3 C)

Evidence is promising that NT screening is useful for

identifying twin pregnancies at high risk of aneuploidy. This requires further prospective investigation. (II-3 C)
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INVASIVE GENETIC TESTING


The fetal loss rates with invasive testing (amniocentesis

and CVS in twins are unclear. (II-3 C)


Development of a protocol for standardization of technique

(as determined by expert opinion) is recommended.


Invasive testing should be oered to twins according to

the usual standard of care.


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PRETERM BIRTH PREVENTION


Routine hospitalization for bed rest in multiple gestation is not

recommended. (I E)
There is insucient evidence to support prophylactic activity

restriction or work leave in multiple gestation. (III C)


There is moderate evidence against routine prophylactic cervical

cerclage in multiple gestation.


However, cerclage maybe indicated for the treatment of

incompetent cervix or other specic circumstances. (I;II-2 D)


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PROPHYLACTIC TOCOLYSIS
There is moderate evidence against

prophylactic tocolysis in the management of multiple gestation, but it may be indicated on other grounds. (I;II-2 D)
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ROUTINE CLINICAL CERVICAL EXAMINATION


There is good evidence that premature cervical

change by digital examination predicts preterm birth in twins. (II-2 A) available, the role of sonographic clinical cervical assessment in the prenatal period has not been determined. (C)

Since there are no well designed intervention trials

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SONOGRAPHIC CERVICAL ASSESSMENT


There is good evidence that transvaginal sonographic

measurement of cervical length predicts preterm birth in twins. (II-1 A)


There are no intervention studies that have evaluated

cervical length measurement in the prevention of preterm birth, the role of sonographic clinical cervical assessment in the prenatal period has not been determined. (C)
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Trust Your Vaginal Ultrasound

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Measurement of the cervical length

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Risk of preterm delivery using cervical length at 23 weeks


Cx 5 mm 10 mm 15 mm 20 mm 25 mm 30 mm 40 mm 50 mm 60 mm
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(Heath et al 1998)

LR 52 9,1 2,7 1,2 0,7 0,5 0,5 0,4 0,1


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FETAL FIBRONECTIN
There is good evidence that the presence of cervicovaginal

fetal bronectin in twins predicts preterm birth.


Without well designed intervention trials available, there is

no basis for incorporating fetal bronectin screening into routine prenatal management of multiple gestation. (C)
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MISCARRIAGE AND PERINATAL MORTALITY

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Dichorionic and DiamnioFc pregnancy at 9+2 weeks gestaFon, with a discrepancy of to embryos size

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ULTRASOUND MANAGEMENT
Performed in 1st trimester: number, amnionicity, chorionicity, and NT (10
14 W)

Detailed US examination: 18 20 W, fetal gender, number of placenta,


the thickness and number of layers in membrane, and lambda (twin peak) sign

Dichorionic pregnancy: fetal growth (FG) evaluation every 3 4 W (if FG


and AFV normal)

Monochorionic diamniotic: evaluation every 2 3 W, TTTS, fetal


echocardiography
Young Mi Lee et al. Multiple pregnancy. In: Management of High-Risk Pregnancy. An Evidence-based Approach, 2007,304-315

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ULTRASOUND MANAGEMENT
Dichorionic or monochorionic: if IUGR, discordant fetal growth, discordant AFV NST, Biophysical Prole, Doppler studies Monoamniotic: daily NST starting from 24 26 W (risk of sudden IUFD from cord entanglement) variable deceleration delivery?
Young Mi Lee et al. Multiple pregnancy. In: Management of High-Risk Pregnancy. An Evidence-based Approach, 2007,304-315

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FETAL LOSS & DELIVERY TIME

Cumulative fetal loss rates in monochorionic (solid line) and dichorionic (dashed line) twin pregnancies, from 12 weeks of gestation20

Gestational age distribution at delivery of monochorionic (solid bars) and dichorionic (open bars) twin pregnancies. The proportion of pregnancies delivering very preterm (before 32 weeks) is considerably higher in monochorionic compared to dichorionic twins20

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ELECTIVE CAESAREAN SECTION


The indications for elective Caesarean section in twin gestations are: a) Monoamniotic twins because the risk of entrapment is too great to permit elective vaginal delivery; b) Conjoined twins other than at gestations remote from term; c) Indications as for singleton pregnancies. (III C)
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CASE REPORT
Mrs I, 34 year, G1P0A0 20 weeks, dizygotic twin

pregnancy (28-03-2008) Fetus: gemelli, breech-breech presentation, boy and girl, no major anomaly seen Placenta: normal, two placenta at right and left side of the uterus Amniotic uid: normal, amniotic membrane (+) Biometry: equal to 19 weeks, EFW 1: 332 gr and EFW 2: 338 gr
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CASE REPORT
Mrs F, 33th years old, G2P1A0:

DIZYGOTIC TWINS IVF Two embryo fetal variability (2nd fetus) CS at 36 W: F 2480 grams, AS 9/9; B 2082 grams, AS 9/9. Two placenta, two amnion and two chorion. The placenta for the 2nd baby is smaller
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1. Female, 2480 gr, AS 9/9 2. Boy, 2082 gr, AS 9/9

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CONCLUSIONS
ART and delayed childbearing increase multiple

pregnancy
High perinatal morbidity and mortality rates Early diagnosis and serial ultrasound studies are

important on maternal and neonatal outcomes


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TAKE HOME MESSAGES


Early diagnose of twin pregnancy (ultrasound !) Determination of zygosity: !! Conjoined twins Screening for fetal anomaly and growth

disturbances When the best time to delivery? Condent diagnosis of zygosity may require detailed examination of the placenta after delivery
JJE-20091119 HANYA UNTUK PENDIDIKAN DAN KESEHATAN

REFERENCES
Tarsa M, Moore TR. Multifetal gestation and malpresentation. In:

Essentials of obstetrics and gynecology, 5th Ed, 2010;160-172

Hecher K, Diehl W. Multiple pregnancies. In: Ultrasound in

obstetrics and gynaecology. European practice in Gynecology and obstetrics. Edited by JW Wladimiro, SH Eik-Nes,2009,247-258 Risk Pregnancy. An Evidence-based Approach, 2007,304-315

Young Mi Lee et al. Multiple pregnancy. In: Management of High-

Egan JFX, Borgida AF. Ultrasound evaluation of multiple pregnancies. In:

Ultrasonography in obstetrics and gynecology, Callen, 5th Ed, 2008;181-224

JJE-20091119

HANYA UNTUK PENDIDIKAN DAN KESEHATAN

THANK YOU

http://www.youtube.com/watch?v=50JO-YtGshw http://www.facebook.com/judi.j.endjun?ref=prole judijanuadi@hotmail.com


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