SUB BAGIAN FETOMATERNAL FK-USU RS. PIRNGADI MEDANKEHAMILAN KEMBAR= MULTIPLE PREGNANCIES =(GEMELLI)

PendahuluanTwo for the price of one atau instant familyHigh Complication RiskMorbiditas & mortalitas 50% 32-38 minggu, 10% dibawahnyaPe Malpresentasi:- kedua janin sungsang 41%- Janin kembar I sungsang 17%- Locked twins (jarang)Persalinan operatif & resiko persalinan preterm

Definisi & KlasifikasiKehamilan 2 janin atau lebihKembar dizigotik (66%) Binovular-fraternal twins1. fertilisasi 2 ovum oleh 2 sperma2. Dikorionik: Amnion terpisahKembar monozigotik (33%) Mono ovular-identical twins - Pembelahan 1 ovum, fertilisasi oleh sperma sperma yang sama- Pembelahan

Mono ovular-identical twins, diamniotik monokorionik

- Pembelahan 8-13 hari: Monokorionik, Monoamniotik - Pembelahan >13 hari: Conjoined twins Fetus Papyraceous - Salah satu janin kembar tidak berkembang - Tak berbentuk, mengkerut & rata Perbandingan Mono/Dizigotik 1:2 Faktor resiko untuk kembar dizigotik: - tua - Multiparitas - Riwayat keluarga kehamilan kembar dizigotik

Fetus Papyraceous, salah satu fetus yang tidak berkembang

Insiden1% dari kehamilan, 2/3 dizigot & 1/3 monozigotEtnik (1:50 Afrika, 1:80 Causasia, 1:50 Asia)Usia (2% > 35 thn) Paritas (2% setelah kehamilan ke-4)Metode konsepsi (20% induksi ovulasi)Riwayat keluargaInsidensi menurut hukum Hellin adalah 1 dalam 80n-1 kehamilan

EtiologiBangsa, hereditas, umur & paritas binovular fraternal-twinsObat klomid & gonadotropin hormon dizigotik Fertilisasi in vitro & transfer embrio (IVF&ET)

PatofisiologiFertilisasi ovum&sperma di tuba falopii Ovum yang telah dibuahi turun uterus nidasi dan Pertumbuhan fetus

Selama proses ini kembar dapat terbentuk

Kehamilan berasal dari satu telur terjadi :Akibat adanya kerja faktor penghambat (inhibiting factor) pada masa awal pertumbuhan embrio intrauterin, mempengaruhi segmentasi selanjutnya pada berbagai tingkatan.

Tipe PresentasiJanin kembar I presentasi vertex 75%Kedua janin presentasi vertex 45%Salah satu janin vertex, lainnya bokong 37%Kedua janin presentasi bokong 10%

tipe-tipe presentasi

Distribusi dari letak dan posisi janin kembar (dalam %) antara lain:

Early DiagnosisAnamnesa Ultrasonografi

Gemelli

Pemeriksaan klinis Radiologi

Diagnosis Awal TwinsDIZYGOTICMONOZYGOTIC

Ultrasonografi kehamilan kembar pada usia kehamilan 38-40 hari

Diagnosa dini gagal - P PJT & persalinan prematur - P mortalitas & morbiditas perintal - P komplikasi

Berdasarkan observasi

36-37 mgg +++ Amnion

Differential Diagnosis

Kehamilan lewat waktu Polihidramnion Tumor fibroid uterus Kista Mola hidatiforma

Anemia Atonia uteri Hidramnion

PPH Abortus Komplikasi maternal

Retensio plasenta Partus prematur

Inersia uteri Pre-eklampsia

SolusioplasentaMalpresentasiPlasenta PreviaKPDKomplikasi fetalPrematuritasBBLRInsufisiensi plasentaKelainan kongenitalProlapsus tali pusat

Komplikasi Intrapartum

Plasenta Insufisiensi plasenta kebutuhan nutrisi>> Polihidramnion

Kondisi lain

Prolapsus tali pusat Malpresentasi

LockedPPH Komplikasi Peripartum Twins

Solusio Plasenta Transfusion Syndrom

PenatalaksanaanTindakan umum- Diet & Pola makan yang baik- Besi & Asam folat- Aktivitas

C. USG setiap 4-6 mgg setelah dignosis- kemungkinan plasenta previa- kemungkinan gangguan pertumbuhan janin- presentasi janin

D. Nonstress test setelah setelah 32mgg- keadaan janin- penekanan taki pusat

E. Konsultasi perinatologi

Kembar discordant: janin resepient lebih besar dari pada janin donor abnormalitas arteriovenous tampak pada permukaan plasenta, darah arteri kaya O2 donor bercampur dengan darah resepient

PENANGANAN PERSALINANKALAU ANAK I SUNGSANG ATAU LINTANG SEBAIKNYA S.CESAR.KALAU ANAK I P.KEPALA DIUPAYAKAN DENGAN P/ VAGINAL ANAK KE DUA DENGAN V.EKSTRAKSI.SELAMA DJJ NORMAL TIDAK ADA ALASAN UNTUK MEMPERCAPAT KELAHIRAN ANAK KEDUAPENGAWASAN YANG KETAT MENENTUKAN OUTCOME PERSALINAN

anak pertama lintang atau sungsang dan anak kedua memanjang (terjadi posisi saling mengunci interlocking)

Panduan penanganan persalinan spontan pada kehamilan kembarJanin pertamaSiapkan peralatan resusitasi & perawatan bayiPasang infus & cairan intravenaPantau keadaan janin, djjPeriksa presentasi janin- vertex PSP, monitor persalinan- bokong indikasi SC- lintang SCTinggalkan klem pada ujung maternal tali pusat

Janin kedua atau berikiutnyaSegera setelah bayi pertama lahir:- Palpasi abdomen letak janin- lakukan versi luar - Periksa djjPeriksa dalam- Presentasi janin kedua- keutuhan selaput ketuban- Prolapsus tali pusat

Monoamniotic twins mortality2 to 5% loss every 2 weeks from 15 to 32 weeks

9% at 33 wks 29% at 36-38 wks

95% cord entanglement (prenatal diagnosis 28%)

Comparison of rates of complications in singleton and multiple gestationsComplicationsRate for twins (increase)

Chorioamnionitis4-foldPremature rupture of membranes4-foldFetal asphyxia5-foldTwin-twin transfusion1 of 9 monoamniotic twinsCongenital malformations3-foldHydramnios1 of 12 twinsAbruptio placentae2-foldPlacenta previa2-foldCompression of cord2-foldBirth injury10-foldPrematurity10-foldUmbilical cord knots2-fold

Maternal morbidity and obstetric complications of quadruplet pregnancy (No. 22)VARIABLEINCIDENCE (%)

Antepartum hospitalization100Hyperemesis gravidarum9.4Hyperemesis gravidarum, total parenteral nutrition required3.1Gestational diabetes mellitus, A118.8Gestational diabetes mellitus, A23.1Anemia (Hct < 30%), no antepartum transfusion required25.0Anemia (Hct < 30%), antepartum transfusion required15.6Antepartum bleeding3.1Placenta previa0.0Preeclampsia71.9HELLP syndrome2.5PPROM18.8PTL100Twin-twin transfusion syndrome3.1Chorioamnionitis6.3

I. Psychological Support and Clinical CounselingAll parents should be aware that pathologies such as fetal growth retardation, congenital anomalies, abnormal placentation, abruptio placentae, fetal malpresentation and preterm delivery, occur more commonly in multiple than in singleton pregnancyThese aspects result in higher maternal and perinatal mortality and morbidity.Antenatal complications are three to five times higher in multiple pregnancy than in singleton pregnancy.From the first trimester onwards is required to help parents to cope with possible negative outcome and also with the socio-economic problems related to multiple birth.

The most important:EARLY DIAGNOSIS WHY?MULTIPLE PREGNANCY HIGH-RISK PREGNANCY COMPLICATIONS DURING PREGNANCY SPECIFIC MALFORMATION SEQUENCES HIGHER PERINATAL MORBIDITIY AND MORTALITY INTRAPARTAL COMPLICATIONS

=

DIAGNOSIS OF MULTIFETAL PREGNANCY:SIMULTANEOUS VISUALIZATION two or more embryos

or corresponding body parts of two or more fetuses

EARLY DIAGNOSIS OF TWINS2 GESTATIONAL SACS2 YOLK SAC ( BC / BA ) 1 GESTATIONAL SAC 2 YOLK SACS ( MC / BA )The first visible structures:DIZYGOTICMONOZYGOTICYOLK SACS fusedseparated

A firm diagnosis ofthe number of embryosafter 7th week !EARLY DIAGNOSIS OF TWINSEMBRYOS AND AMNIOTICMEMBRANES

MONOCHORIONICMONOAMNIOTICTWINS

HIGH-ORDER MULTIPLE PREGNANCYPregnancy with three or more fetuses

three amnioticthree chorionic

2D multiplanar imaging 3D reconstruction volume scanning volume rendering spatial reconstruction plastic imagingTRIPLETS

FRONTBACK

QUADRUPLETSHIGH ORDER PREGNANCY

HIGH ORDER PREGNANCY

HIGH ORDER PREGNANCYSEPTUPLETS

12 EMBRYOS HIGH ORDER PREGNANCY

II. Correct Diagnosis and Characterization of ChorionicityMultiple gestation should be suspected when the uterus is larger than predicted by menstrual history.Approximately one fifth of multiple gestations are monochorionic and four fifths are dichorionic.Type of placentation and chorionicity is helpful in the following three clinical situations: 1) The differentiation of twin to twin transfusion syndrome (TTS) from a twin gestation in which one fetus shows growth retardation; 2) the management of twins with congenital malformations, in which selective feticide may be considered as an option if the gestation is dichorionic and 3) the management of single fetal death in a multiple gestation.The thickness of dividing membrane is in 85% of monochorionic twins ~ 2 mm, in DC/DA the membrane is ~ 4 mmThe lambda sign is an indicator of dichorionic pregnancy

II. Correct Diagnosis and Characterization of ChorionicityThe following criteria must be fulfilled to diagnose monoamniotic twins:no dividing amniotic membrane is presentonly one placenta is seenboth fetuses are of the same sex the fetuses must have adequate amniotic fluid surrounding themboth fetuses must move freely within the uterine cavity.

Zigosity of spontaneus vs. ART tripletsadapted from Derom, 2000

ACCURATE PRENATAL DIAGNOSISOF CHORIONICITY IS OF PREDOMINANTIMPORTANCE FOR THE CLINICAL MANAGEMENT OF MULTIPLE PREGNANCIES

EARLY DIAGNOSIS OF CHORIONICITYNUMBER OF GESTATIONAL SACS1st TRIMESTER

EARLY DIAGNOSIS OF AMNIONICITY6 weeks NUMBER OF YOLK SACS

OR

NUMBER OF VISIBLE AMNIONS7 weeks

ALARM !MONOCHORIONICAND / ORMONOAMNIOTIC TWINS FETAL COMPLICATIONSEARLY DIAGNOSIS OF AMNIONICITYWhy is it important?

Twin embolisation syndrome ( vanishing-twin )

Twin-to-twin transfusion syndrome ( TTS )

Twin reversed arterial perfusion ( TRAP )

Cord entanglement

Conjoined twins

PECULIAR COMPLICATIONS

NUMBER OF PLACENTASSECOND ANDTHIRD TRIMESTER

TWO SEPARATED PLACENTASPLACENTA 2PLACENTA 1DETERMINATION OF THE CHORIONICITYIN SECOND TRIMESTERSonographic counting of separated placentas is an accurate method of determining the chorionicity in the second trimester

MONOCHORIONICBIAMNIOTIC TWINSBICHORIONICBIAMNIOTIC TWINS

LAMBDA SIGNBICHORIONIC BIAMNIOTIC TWINS

BIAMNIOTICBICHORIONICTWINS

MONOAMNIOTIC MONOCHORIONIC TWINS

THE Y-SHAPED JUNCTIONY-SIGNTRICHORIONICTRIAMNIOTICTRIPLETS MERCEDES SIGN

III. Close Evaluation of Fetal Anatomy

Fetal Malformations and Prenatal Genetic Diagnosis

The incidence of malformation in monozygotic twin pregnancies is twice that in dizygotics.Chromosomal anomalies are no more common in twins than singletonsAnomalies not unique to twins but believed to be increased in frequency because of mechanical factors are positional defects (such as clubfoot and congenital dislocation of the hip) due to intrauterine crowding.Additional anomalies due to vascular consequences of fetal death are congenital skin defects, microcephaly, hydrancephaly, porencephaly, multicystic encephalomalacia, hydrocephalus, intestinal atresia and limb amputation.

III. Close Evaluation of Fetal AnatomyFetoplacental Markers in Twin Pregnancies Affected by Down SyndromeAround one-third of twin pregnancies are monozygous and their rate of Down syndrome is relatively independent of race and maternal age.Dizygous twins are more common in older mothers and as they arise from separate fertilisation of two simultaneously shed ova there is double the age-related risk than for a singleton pregnancy that either twin will have Down syndrome

Incidence of congenital anomalies is 2 - 3 x higher in twin than in singleton pregnancy.Anomaly rates for: singletons 2 - 4 % twins 5 - 10 %

Monozygotic twins have an anomaly rate 50% higher than dizygotic twins.EPIDEMIOLOGY OF CONGENITAL ANOMALIES IN TWINS

CONJOINED (SIAMESE) TWINS INCIDENCE 1: 50 000 BIRTHS

ULTRASOUND CRITERIA FOR DIAGNOSIS:

1) LACK OF SEPARATE VISUALISATION OF FETUSES IN SPECIFIC ANATOMICAL REGIONS

2) FIXED POSITION OF THE TWIN TOWARD EACH OTHER

3) MISSING SEPARATING MEMBRANE

SYMMETRICAL COMPLETE FORM Two fetuses share a certain amount of tissue

Surgical separation is possible in general.

PATTERNS OF PHYSICAL JOINING

PATTERNS OF PHYSICAL JOININGSYMMETRICAL INCOMPLETE FORM

Surgical separation is usually impossible

Conjoined twins:

subtotal fusionwith partial separation of fetal headsEARLY DIAGNOSIS OF CONJOINED TWINS

CONJOINED TWINS THORACO- OMPHALOPHAGUS

lack of separate visualisation of fetuses in thoraco-abdominal region

COLOR DOPPLERSINGLE SHARED UMBILICAL CORDFIVE - VESSEL CORDTHORACO-OMPHALOPHAGUS

VI. Avoidance of Most Frequent Complications

Complications of multiple pregnancies comprise:Abortion,Vanishing twin syndromeMalformationVasa previaGrowth discrepancyIntra uterine growth restriction (IUGR)PolyhydramniosPreeclampsiaPreterm-premature rupture of membranes (P-PROM)Preterm deliveryGestational diabetesIntrauterine fetal death.

VANISHING TWIN in 20% of twins single fetal demise high-risk surviving twin intrauterine hematomas better prognosis in dichorionic thromboplastine embolisation

VANISHING TWINSUBCHORIONIC HAEMATOMAdifferential diagnosis

VII. Consideration of Some Specific PathologiesTwin to Twin Transfusion Syndrome (TTS)Is associated with a high rate of mortality and, among survivors, substantial morbidity.Diagnostic criteria include: monochorionic pregnancy; same sex with growth discordance between twins; olygohydramnios of the growth retarded fetus and polyhydramnios of the larger twin; an intertwin hemoglobin difference > 5mg/dl (after cordocentesis).Antepartum management of TTS is not without controversy, because no suggested therapy is without problems.The three types of vascular anastomoses, A-A, V-V and A-V, are generally present in monochorionic placentae.

MONOCHORONIC / BIAMNIOTIC:TWIN TO TWIN TRANSFUSION SYNDROME

MONOAMNIOTIC:UMBILICAL CORD ENTAGLEMENTACARDIAC TWIN - TRAP SEQUENCE CONJOINED TWINSTTTS

5% - 20% monochorionic twinsarterio venous anastomosesdiscordant growth

DONOR RECIPIENTOLIGOHYDRAMNIOS POLYHYDRAMNIOSIUGR MACROSOMIA, HYDROPSMICROCARDIA CARDIOMEGALIAANEMIA POLYCYTHAEMIAfetal loss 80%

TWIN TO TWIN TRANSFUSION SYNDROME

TWIN TO TWIN TRANSFUSION SYNDROME RECIPIENT: Fetal hydropsSCALP EDEMAASCITES

collapsed amniotic membrane DONOR:Stuck twin

TWIN TO TWIN TRANSFUSION SYNDROMEfixed twinanhydramnios

POLYHYDRAMNIOS OF RECIPIENT TWIN

TWIN TO TWIN TRANSFUSION SYNDROME

TWIN TO TWIN TRANSFUSION SYNDROME

PULSATIONS WITHREVERSE- FLOW AT THE END OF DIASTOLEUMBILICAL VEIN SONOGRAM IN RECIPIENT TWIN DUCTUS VENOSUSSONOGRAMIN RECIPIENT TWINREVERSAL OF FLOWDURING ATRIALCONTRACTIONTWIN TO TWIN TRANSFUSION SYNDROMERecipient : venous return pattern

Plethoric RECIPIENTAnaemic DONORTWIN TO TWIN TRANSFUSION SYNDROMEWeight difference > 25%Haemoglobin difference >5%

VASCULAR ANASTOMOSES IN A TWIN PLACENTA:ARTERIO VENOUSARTERIO ARTERIOUS VENO VENOUSsuperficialdeep

VISUALIZATION WITHPOWER ANGIO MODESURFACE ANASTOMOSES

VII. Consideration of Some Specific PathologiesTwin Reversed Arterial Perfusion (TRAP) SequenceThe most extreme manifestation of twin to twin transfusion syndrome, found in approximately 1% of monozygotic twin pregnancies is acardiac twinning (acardius chorioangiopagus parasiticus).The underlying mechanism is thought to be disruption of normal vascular perfusion and development of the recipient twin due to an umbilical arterial-to-arterial anastomosis with the donor or pump twin.At least 50% of donor twins die due to congestive heart failure or severe preterm delivery, the consequence of polyhydramnios.All perfused twins die due to the associated multiple malformations.

TWIN REVERSED ARTERIAL PERFUSION(TRAP)IN MONOCHORIONIC TWINS ONE TWIN ( PUMP-TWIN ) ACTIVELY PERFUSESTHE SECOND TWIN ( PERFUSED TWIN ) VIA LARGE A -A AND/OR V - V ANASTOMOSES1% of monozygotic twins are affected Incidence 1 : 35 000 births

ARTERIAL SUPPLY INTO PLACENTA BY THE PUMP TWIN IS ABLE TO OVERCOME THE BLOOD PRESSURE OF THECO-TWIN SO AS TO PERFUSE THAT TWINBY REVERSED FLOW (TOWARD CO-TWIN)IN THE UMBLICAL ARTERIES OF THE CO-TWINPATHOGENESIS

PERFUSED TWIN ACARDIUS

NORMAL( PUMP TWIN )TRAP BLOOD FLOWS FROM AN UMBILICAL ARTERY OF THE PUMP TWIN IN REVERSE DIRECTION VIA ARTERIO - ARTERIAL ANASTOMOSES INTO UMBILICAL ARTERY OF THE PERFUSED TWIN.THE UMBILICAL VEIN OF THE PARASITIC FETUS RETURNS THE BLOOD INTO THE PLACENTA ANDBACK TO PUMP TWINREVERSE FLOW NORMAL FLOW

EARLY REVERSE OF CIRCULATION

REVERSE PASSIVE PERFUSION OF TWIN

PERFUSION IN OPPOSITE DIRECTION ANDPERFUSION WITH DEOXIGENATED BLOOD

INDUCTION OF DEVELOPMENTAL DISORDERS

REDUCTION ANOMALIES ( EXTREMITIES )DEVELOPMENTAL ATROPHIES ( HEART AND BRAIN ) PATHOGENESIS OF FETAL DYSMORPHIA:

Ultrasound finding = early ultrasound detection the most bizzarre fetal malformations

PUMP - TWIN

normalmorphology

normaldirection ofblood flow PERFUSED TWIN

acardius

reduction anomalies of head and extremities

reversed blood flow

TWINS MC / MA, 15 wks

REVERSEDPERFUSION

COLORDOPPLER

ULTRASONIC CRITERIA FOR ACARDIUSAn amorphous mass with its own umbilical cord in monochorionic- monoamniotictwin pregnancy

ACARDIAC - ACEPHALICThis acardiac twin consists mainly of lower extremitiesNo heart and brain No trunkand head

VII. Consideration of Some Specific PathologiesStuck TwinRefers to the ultrasonographic finding of one of a monochorionic diamniotic twin pair in an oligohydramniotic sac fixed in a location adjacent to the uterine wall.This is frequently a manifestation of the twin-to-twin transfusion syndrome (TTS).Management may include: selective feticide; umbilical cord ligation of one twin; laser occlusion of anastomosing placental vessels; serial amniocentesis.

COMPLICATION SPECIFIC FORMONOAMNIOTIC MONOCHORIONICTWINSCORD ENTAGLEMENT

THE CLOSE INSERTION OF THE UMBILICAL CORDS INTO PLACENTA IS ASSOCIATED WITH:LARGE-CALIBER ANASTOMOSES AND HIGH PREDISPOSITION FOR ENTANGLEMENTMONOAMNIOTIC TWINNING CORD ENTANGLEMENT

POWER DOPPLERCOLOR DOPPLER CORD ENTANGLEMENT

Multiple gestations present a significantdecrease in fetal growth which is in direct relationship to the number of fetuses in high order pregnancies

TWIN-TO-TWIN TRANSFUSIONshould be considered when growth discordancy is diagnosed in monochorionic gestations

VIII. Close Monitoring of FetusesDoppler VelocimetryRecent studies have addressed the usefulness of this technique in predicting twin fetuses small for gestational age (SGA) or IUGR, twins with TTS, and those with discordant growth

VIII. Close Monitoring of FetusesCardiotocographyIs not always easy to identify the twins and it is possible to perform two NSTs on the same fetus.The best method is the simultaneous recording of FHR patterns on one tracing.

COMPLEX BODY MOVEMENTS HICCUPS HAND-FACE CONTACTS MOUTH OPENING SWALLOWING BREATHING MOVEMENTS HEAD MOVEMENTS EXTREMITY MOVEMENTS JUMPING TWISTING STRETCHING YAWNING SPONTANEOUS MOTORIC ACTIVITY

NO INTERTWIN CONTACTSFETAL ACTIVITYCOMPLEXBODY MOVEMENTS

EXTREMITY MOVEMENTSNO INTERTWIN CONTACTSFETAL ACTIVITY

FIRST REACH AND TOUCH FIRST REACTION SLOW OR FAST ARM, LEG, HEAD OR BODY CONTACT MOUTH CONTACT COMPLEX INTERACTIONSINTER-TWIN CONTACTS

JUMPINGTRIPLET ACTIVITY AND CONTACTSHEAD TO BODY CONTACT

The Ten Commandments in Multiple PregnanciesI. Psychological Support and Clinical CounselingII. Correct Diagnosis and Characterization of ChorionicityIII. Close Evaluation of Fetal AnatomyIV. Management at Referral CentersV. Individualization of CareVI. Avoidance of Most Frequent ComplicationsVII Consideration of Some Specific PathologiesVIII. Close Monitoring of FetusesIX. Planning of Time and Mode of DeliveryX. Monitoring of the Mother During Postpartum

Ultrasound assessment of multiple pregnancy:

1. EARLY DIAGNOSIS OF MULTIPLE PREGNANCY 2. DIAGNOSIS OF CHORIONICITY AND AMNIONICITY 3. COMPLICATIONS IN MONOCHORIONIC TWINS 4. FETAL CONGENITAL ANOMALIES 5. APPROPRIATE VERSUS DISCORDANT GROWTH 6. COLOR-DOPPLER OF MULTIFETAL PREGNANCY 7. PREDICTION OF PRETERM DELIVERY 8. INTRAPARTUM ULTRASONOGRAPHY

TWIN PREGNANCY(MC/MA) IN 15 TH WEEK IS DEPICTED. ONE FETUS IS NORMAL WHILE THE OTHER IS AMORPHEUS. CIRCULATION IS DETECTED BY COLOR DOPPLER TECHNIQUE, CONFIRMING THE REVERS FLOW IN UMBILICAL ARTERY OF MALFORMED TWIN. 3-D RECONSTRUCTION SHOWS THE CONTOUR OF HEAD AND TRUNK GENERALISED HYDROPS AND REDUCTION OF EXTRREMITIES.CLINICAL AND MORPHOLOGICAL SEQUENCE INCLUDE TRAP AND ACARDIAC TWIN.