International Fetal Medicine and Surgery Society · Twin Pregnancies Discordant for Fetal Anomaly....

Twin Pregnancies Discordant for Fetal Anomaly.

Aiello H, Gimenez ML, Meller C, Izbizky G, Wojakows ki A, Otaño L

Servicio de Obstetricia, Unidad de Medicina FetalHospital Italiano de Buenos Aires

International Fetal Medicine and Surgery SocietySedona, ArizonaMay 13 - 18, 2011

Introduction

• An excess of structural anomalies is observed in twins compared to

singletons (Mastroiacovo, 1999), especially in monozygotic twins.

• Usually, one fetus is affected and the other is normal. Therefore,

approximately 1–2% of twin pregnancies may face the dilemma of

different management options: expectant management, selective

termination of the affected fetus, or termination of the entire pregnancy.

• Since legal abortion is not an option in our country, we have the

opportunity to asses the natural history of these conditions in most cases.

Mastroiacovo P, et al. 1999. Congenital malformatio ns in twins: an international study. Am J Med Genet 83: 117–124.

Objective

The aim of this presentation is to describe perinatal

outcomes of a series with prenatal diagnosis of twin

pregnancies discordant for fetal anomalies managed

expectantly

Materials and Methods

•Twin pregnancies discordant for fetal anomalies assessed between 2003 and 2010 were included.

•Twin pregnancies with both fetuses affected with the same type of anomaly were excluded.

•Pregnancies were divided according to placentation in monochorionic (MC) and dichorionic (DC).

•Main outcomes were type of anomaly, gestational age (GA) at referral and delivery, and perinatal survival rate for pregnancies managed expectantly.

Results

•24 Dichorionic-diamniotic pregnancies (DC)

• 23 DC

• 1 triplet TCTA

•22 Monochorionic pregnancies (MC)

• 19 MCDA

• 2 MCMA

• 1 triplet MCDA

• During 2003-2010, 46 twin pregnancies discordant fo r

a fetal anomaly were assessed:

Results

Type of congenital anomaly n DC (n) MC (n)

Central Nervous System 8 2 6

TRAP sequence 7 - 7

Genitourinary system anomalies 6 3 3

Chromosomal anomalies 5 5 -

Complete Hydatiform Mole 4 4 -

Cardiovascular Defects 4 3 1

Abdominal- Wall defects 3 1 2

Neck: Cystic hygroma 3 2 1

Thoracic lesions 2 2 -

Intra-abdominal bronchopulmonary sequestration 1 - 1

Face: Cleft lip 1 - 1

VACTERL association 1 1 -

Extremities: Arthrogryposis 1 1 -

Total 46 24 22

Types of defects for monochorionic (MC) and dichori onic (DC) twins by major anomaly group.

Results



TRAP sequence 7 - 7












Total 46 24 22


Results



TRAP sequence 7 - 7












Total 46 24 22


Dichorionic pregnancies. Fetal anomaly, gestational age at referral, management, gestational age at delivery, and perinatal outcome of twin pregnancies discordant for a major fetal anomaly

Type of anomaly Anomaly GA Ref.

ManagementGA at

deliveryOutcome

Malf Normal

Chromosomal anomalies

Trisomy 13 (Onphalocele + Polydactyly ) 16 Expectant 26 IUFD Alive

Monosomy X (Cystic hygroma + pleural effusion) 13 Expectant 37 IUFD 14s Alive

Trisomy 21 13 Expectant 37 IUFD 37s Alive

Triploidy 18 Expectant 37 IUFD 18s Alive


Complete Hydatiform Mole

Complete Hydatiform Mole 14TOP + MTX

17 _ TOP

Complete Hydatiform Mole 12 Expectant 36 _ Alive

Complete Hydatiform Mole 17 Lost of follow-up

Complete Hydatiform Mole 14 Expectant 24 _ NND

Cardiovascular

Hypoplastic left heart syndrome 27 Expectant 34 NND Alive


Univentricular Heart (left morphology) TGV + Coarctation of Aorta 27 Expectant 36 Alive Alive

Genito-urinary system

Bilateral renal agenesis + iniencephaly: caudal regresión? 21 Expectant 34 IUFD 33s Alive

Multicistic Dysplastic Kidney 33 Expectant 37 Alive Alive

Multicistic Dysplastic Kidney (TCTA pregnancy) 26 Expectant 34 Alive Alive x 2

Central Nervous SystemAnencephaly 19 Expectant 38 NND Alive

Hydrocephaly 25 Expectant 29 NND Alive

Neck: Cystic hygromaCystic hygroma + Omphalocele + Megacystis 13 Lost of follow-up

Cystic hygroma 14 Expectant 24 NND NND

ThoracicDiaphragmatic hernia 28 Expectant 34 Alive Alive

Cystic Adenomatoid Malformation 20 Expectant 35 Alive Alive

Abdominal- Wall defects Omphalocele 15 Expectant 28 NND Alive

Extremities Arthrogryposis + Micrognatia + Enlarged cisterna magna 18 Lost of follow-up

Other VACTER Cardiopathy + Bilateral renal anomaly + Anhidramnios 19 Expectant 22 Miscarriage Miscarriage

Dichorionic pregnancies. Fetal anomaly, gestational age at referral, management, gestational age at delivery, and perinatal outcome of twin pregnancies discordant for a major fetal anomaly

Type of anomaly Anomaly GA Ref.

ManagementGA at

deliveryOutcome

Malf Normal

Chromosomal anomalies

Trisomy 13 (Onphalocele + Polydactyly ) 16 Expectant 26 IUFD Alive

Monosomy X (Cystic hygroma + pleural effusion) 13 Expectant 37 IUFD 14s Alive

Trisomy 21 13 Expectant 37 IUFD 37s Alive



Complete Hydatiform Mole

Complete Hydatiform Mole 14TOP + MTX

17 _ TOP

Complete Hydatiform Mole 12 Expectant 36 _ Alive

Complete Hydatiform Mole 17 Lost of follow-up

Complete Hydatiform Mole 14 Expectant 24 _ NND

Cardiovascular



Univentricular Heart (left morphology) TGV + Coarctation of Aorta 27 Expectant 36 Alive Alive


Bilateral renal agenesis + iniencephaly: caudal regresión? 21 Expectant 34 IUFD 33s Alive

Multicistic Dysplastic Kidney 33 Expectant 37 Alive Alive

Multicistic Dysplastic Kidney (TCTA pregnancy) 26 Expectant 34 Alive Alive x 2

Central Nervous SystemAnencephaly 19 Expectant 38 NND Alive

Hydrocephaly 25 Expectant 29 NND Alive

Neck: Cystic hygromaCystic hygroma + Omphalocele + Megacystis 13 Lost of follow-up

Cystic hygroma 14 Expectant 24 NND NND

ThoracicDiaphragmatic hernia 28 Expectant 34 Alive Alive

Cystic Adenomatoid Malformation 20 Expectant 35 Alive Alive

Abdominal- Wall defects Omphalocele 15 Expectant 28 NND Alive

Extremities Arthrogryposis + Micrognatia + Enlarged cisterna magna 18 Lost of follow-up

Other VACTER Cardiopathy + Bilateral renal anomaly + Anhidramnios 19 Expectant 22 Miscarriage Miscarriage

Monochorionic Pregnancies. Fetal anomaly, gestation al age at referral, management, gestational age at delivery, and perinatal outcome of twin pregnancies discordant for a major fetal anomaly

Type of anomaly Anomaly GA Ref. Management GA at delivery Outcome

Malf Normal

Anomalies associated with twinning

TRAP sequence 13 Expectant 34 - Alive


TRAP sequence 23 Radiofrequency + amniodrainage 37 - Alive

TRAP sequence 16 Expectant Lost of follow-up

TRAP sequence 28 Expectant On going

TRAP sequence + T21 16 TOP TOP TOP

F-1: TRAP; F-2: VSD + cardiomegaly (Triplet MCDA) 25 Expectant Lost of follow-up

Central Nervous System

Anencephaly 14 Expectant 32 NND Alive

Anencephaly (MCMA) 16 Radiofrequency 23 w 35 - Alive

Anencephaly + Fetal death 26 Expectant 36 IUFD NND

Dandy Walker Malformation 31 Expectant On going

Cephalocele 21 Expectant 36 Alive Alive

Spina bifida 19 Expectant 36 Alive Alive


Hydronephrosis 30 Expectant 35 Alive Alive

Megacystis (47,XY,+21) 14 TOP 14 TOP TOP

Multicistic Dysplastic Kidney 30 Expectant On going

Abdominal- wall defects

Gastroschisis 15 TOP 15 TOP TOP

Body-Stalk anomaly (MCMA) 20 Expectant 32 NND Alive

Neck Cystic hygroma + Pleural effusion + Onphalocele + Discordant sex 12 Expectant 17 Miscarriage Miscarriage

Cardiovascular Dilated Cardiomyopathy 22 Expectant 36 Alive Alive

Face Cleft lip 27 Expectant On going

BP sequestration Intraabdominal bronchopulmonary sequestration 25 Expectant 31 Alive Alive



Malf Normal





























Malf Normal





























Malf Normal





TRAP sequence 16 Expectant Lost of follw-up






















Monoamniotic twin pregnancy discordant for anencephaly

Radiofrequency ablation

Radiofrequency ablation

Original PaperMonoamniotic twin pregnancy discordant for lethal o pen cranial defect: management dilemmas Waldo Sepulveda, Amy Wong, Rodrigo Sandoval, Horacio Aiello, Lucas OtañoPrenatal Diagnosis . (Article first published online: 27 MAR 2011)

CONCLUSIONS: Monoamniotic twin pregnancy discordant for severe cranial vault defect is a serious condition with a high risk of fetal demise of the structurally normal fetus.

Our experience, although limited, suggests that aggressive management with selective termination of the abnormal twin and transection of its umbilical cord in these cases may prevent the demise of the structurally normal co-twin.



Malf Normal





TRAP sequence 16 Expectant - - LOF






















MRI of co-twin at 34 weeks

• Dilated ventricles• Lissencephaly• Microcephaly

MCDA pregnancy with IUFD of the anencephalic fetus at 19 weeks



Malf Normal





TRAP sequence 16 Expectant - - LOF






















Results

Dichorionic Monochorionic p

Mean GA at referral (w) 20 (± 5.9) 21 (± 6.3) 0.57

Mean GA at delivery (w) 33.1 (± 5.0) 31.7 (± 5.7) 0.48

Perinatal survival rate(IC 95%)

Fetus with anomaly 28* % (10 – 53) §55% (9 – 81) 0.32

Normal co-twin 85 % (55 – 98) 86% (54 – 98) 0.84

* Excluding pregnancies with complete hydatiform mole§ Excluding pregnancies with TRAP sequence

Mean gestational age at referral, mean gestational age at delivery and perinatal survival rate of the malformed twin and the normal co-twin.

Chorionicity, gestatioanl age at diagnosis, gestati onal age at delivery, and survival rate of non-affected twin

Cases

(n) DC MC

GA at

Diagnosis

GA at

DeliverySurvival

rate

Malone et al, 1996 14 11 3 17w6d 33w4d 93%

Gul et al, 2003 13 10 3 27w1d 32w0d 69%

Chang et al, 2004 35 25 10 21w2d 34w4d 86%

Present series 39 20 14 20w3d 32w3d 85%

total 101 66 30 21w1d 32w4d 84%

Chorionicity, gestatioanl age at diagnosis, gestati onal age at delivery, and survival rate of non-affected twin

Cases

(n) DC MC

GA at

Diagnosis

GA at

DeliverySurvival

rate

Malone et al, 1996 14 11 3 17w6d 33w4d 93%

Gul et al, 2003 13 10 3 27w1d 32w0d 69%

Chang et al, 2004 35 25 10 21w2d 34w4d 86%

Present series 39 20 14 20w3d 32w3d 85%

Total 101 66 30 21w1d 32w4d 84%

Twins

n: 300

Discordant twins

DC

n: 20

MC

n: 14

Median Gestational Age at delivery (wks)

36

(IQ 35-37)

33

(IQ 26-37)

32

(IQ 25-37)

Gestational age at delivery in twin pregnancies discordant for fetal anomalies with expectant

management

Conclusion

•The perinatal mortality rate of the normal co-twin seems

to be increased (~15%) for both MC and DC pregnancies

• The natural history of a twin pregnancy discordant for

fetal anomaly is associated to an increase risk of preterm

delivery.

Thanks for your attentionThanks for your [email protected]

Unidad de Medicina FetalHoracio Aiello Cesar MellerMaría Cohen Laura Giménez Gustavo Izbizky Pablo MarantzLucas Otaño Marcelo PietraniRaquel Sod Leandro Suárez Adriana Wojakowski

Unidad de Medicina FetalHoracio Aiello Cesar MellerMaría Cohen Laura Giménez Gustavo Izbizky Pablo MarantzLucas Otaño Marcelo PietraniRaquel Sod Leandro Suárez Adriana Wojakowski

www.hospitalitaliano.org.ar/medicinafetalwww.hospitalitaliano.org.ar/medicinafetal

Iguazu Falls, Argentina and Brasil

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