TWIN-TWIN TRANSFUSION SYNDROME

Shannon Barringer, MS, CGC AR Reproductive Genetics/ANGELS Department of OB/GYN--UAMS

Twin Pregnancies g
MZ TWINS Identical g 1:250 pregnancies One fertilized ovum splits after conception Increased risk for many complications Rarely familial DZ TWINS Fraternal, NonIdentical More common Two separate ova Some genetic component? Inc. risks with inc. maternal age.

Monozygotic Twins yg
Risk for complications dependent on timing of split Monochorionic/Monoamniotic Monchorionic/Diamniotic Dichorionic/Diamniotic j Conjoined Twins

Monochorionic/Monoamniotic
Division of Ovum occurs between Day 9-12 Chorion and Amnion already forming y g Most risky type of twin gestation

Monochorionic/Diamniotic
Division occurs between Day 4-8 y post-conception Chorion begun to form, not amnion More common than Mono/Mono

Sonography Assessment of Multiple Gestation

Is there a membrane separating fetuses?
Membrane = Diamniotic No membrane Monoamniotic Thick membrane = Di/Di, Thin = Mono/Di

Are there two placentas?

T Two = Dichorionic, O M Di h i i One Monochorionic h i i

Sex of fetuses?

Twin-Twin Transfusion Syndrome (TTTS)

M Monochorionic t i h i i twins only (? Mono/Di) Approximately 1020% of these twins affected Mortality rates can approach 80-100% for at least one baby Worst outcome if present < 20 weeks

TTTS
Blood is disproportionately passed to monochorionic babies Passed through shared placental blood vessels: one baby gets too much, other baby gets too little. TRAP

Twin-Twin Transfusion Syndrome (TTTS)

Monochorionic/Diamniotic gestations (4 35% of all MC) (4-35% Recipient Twin: poly, cardiac enlargement/failure, hydrops Donor Twin: oligo, growth restriction Overall mortality:50-100% depending on severity/treatment. Abnormal vascular connections, but more than simple transfusion process; hemodynamic changes, renin shunting from donor donor. Neurologic morbidity risks can also be high: related to polycythemia/venous stasis (recipient) and anemia/hypotension (d i /h t i (donor). )

Recipient Twin p
Gets t G t too much h Fluid overload Big twin Polyhydramnios Edema/hydrops Macrosomia Organomegaly Arterial HTN Higher risk for IUFD

Donor Twin
Gets too little Anemic Oligohydramnios O No edema typical Microsomia Mi i Small viscera Arterial Hypotension Stuck twin

Staging Criteria for TTTS

Quintero staging most widely accepted
Stage I Polyhydramnios-recipient Oligohydramnios-donor Urine in bladder-donor Stage III Poly/Oligo W/ or W/O urine Abnormal Dopplers Stage II Polyhydramnios-recipient Stuck donor No urine in bladder-donor Stage IV Ascites/Hydrops Stage V Demise of either fetus

TTTS Treatment Options

Serial Amnioreduction (AR)
Oldest, most widely used therapy Less costly, less invasive Can be done here Stage I, or later onset Stage II 39-56% survival of both or one twin 18-26% survivors with neurologic impairment 18 26% or imaging changes (likely related to prematurity) Average GA at delivery: 30.9 wks Morbidity continues Inadvertent septostomy? Eurofetus Trial (200+ cases), partial NIH trial (40 cases) (2001-2005)

Laser Photocoagulation g
Purpose is to shut off the anastomoses p Need better randomized-controlled trials Only in true diagnoses Deep versus superficial Arteries are followed (fetoscopy) to make sure vein drains back to same twin
If not, laser coagulation Average of 3-4 deep anastomoses per twins

TTTS-SFLP
To arrest shunting of blood and transfer of vasoactive mediators A A V V or unpaired A-A, V-V, artery/vein Earlier onset Stage II/III/IV are target g p groups Fetoscopic method of surgery.

TTTSSFLP

Few centers in US (Texas Childrens and Cincinnati Childrens closest to AR) ? Insurance coverage

Eurofetus Trial Overall survival both twins36-50% Survival of one Twin77-90% (to birth) Decreased risk of neurologic/CNS comps versus AR at 6months of life. Average GA at birth: 32.1 weeks NIH trial No sig. difference between AR and sig SFLP overall survival of both twins. Study halted; Recipient Twin mortality seemed disprop high in disprop. SFLP, as did # of AR failures causing cardiac changes in RTs.

TTTSOther treatments
Microseptostomy---jury out, but some p p y j y , promise from Eurofetus if stage II/III >24 weeks gestation. Prelim. Data: 65-71% survival of one twin in these groups groups. Nonselective FLP---higher death rate for donor twin, twin but easier procedure procedure. Cord Coagulation---In twin with hydrops (usually recipient), usually reserved for imminent demise. Identification of CV risk indicators: might be helpful in identifying twins who would most likely benefit from SFLP SFLP.

Morbidity in TTTS y
Loss of one twin increases risk of neurologic handicap in surviving twin Mesenteric ischemia Bowel necrosis Skin necrosis ? Limb abnormalities P l Polycythemia th i Hydrocephaly CP Preterm birth