ttts: physiology, signs and treatment options · ttts: physiology, signs and treatment options...

TTTS

TTTS: physiology, signs and treatment options

Laurent J SALOMON

Bologna, April 12 2008

TTTSChorionicity and prognosis

Fetal loss < 24 w. DC MC

1.8% 12.2%

Perinatal death DC MC

Sebire et al. The hidden mortality of monochorionic twin pregnancies.

Br J Obstet Gynaecol. 1997 Oct;104(10):1203-7.

467 twin

11-13+6 scan

IUGR DC MC

12% 21%

Perinatal death DC MC

1.6% 2.8%

467 twin

pregnancies

102 MC 365 DC

Follow-up

Premature birth < 32 w. DC MC

5.5% 9.2%Delivery

TTTSPlacental anastomoses

TTTSSpecific complications

• TTS

• IUFD

• Selective IUGR

EMERGENCIES

• Selective IUGR

• Oligo-polyhydramnios sequence w/o

TTS

• Malformations

TTTS

HYPOXICHYPOXICHYPOXICHYPOXICHYPOXICHYPOXICHYPOXICHYPOXIC--------ISCHEMICISCHEMICISCHEMICISCHEMICISCHEMICISCHEMICISCHEMICISCHEMICCHANGESCHANGESCHANGESCHANGESCHANGESCHANGESCHANGESCHANGES

ReninReninReninReninReninReninReninReninSecretionSecretionSecretionSecretionSecretionSecretionSecretionSecretion

Renal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular Dysgenesis

OLIGURIAOLIGURIAOLIGURIAOLIGURIA

DONORDONOR

RECIPIENTRECIPIENT

HYPOVOLEMIAHYPOVOLEMIAHYPOVOLEMIAHYPOVOLEMIAHYPOVOLEMIAHYPOVOLEMIAHYPOVOLEMIAHYPOVOLEMIA--------HYPOPERFUSIONHYPOPERFUSIONHYPOPERFUSIONHYPOPERFUSIONHYPOPERFUSIONHYPOPERFUSIONHYPOPERFUSIONHYPOPERFUSION

HYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSION

↑↑↑↑ANP↓↓↓↓ADH

Renal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular DysgenesisRenal Tubular Dysgenesis

???????? ANGIOTENSINANGIOTENSINANGIOTENSINANGIOTENSINANGIOTENSINANGIOTENSINANGIOTENSINANGIOTENSIN

MahieuMahieuMahieuMahieuMahieuMahieuMahieuMahieu CaputoCaputoCaputoCaputoCaputoCaputoCaputoCaputo 2000 2000 2000 2000 2000 2000 2000 2000

HYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERTENSIONHYPERVOLEMIAHYPERVOLEMIAHYPERVOLEMIAHYPERVOLEMIAHYPERVOLEMIAHYPERVOLEMIAHYPERVOLEMIAHYPERVOLEMIA

GlomerularGlomerularGlomerularGlomerularGlomerularGlomerularGlomerularGlomerularhypertensivehypertensivehypertensivehypertensivehypertensivehypertensivehypertensivehypertensive

lesionslesionslesionslesionslesionslesionslesionslesions

Renin Renin Renin Renin Renin Renin Renin Renin secretionsecretionsecretionsecretionsecretionsecretionsecretionsecretion

POLYURIAPOLYURIAPOLYURIAPOLYURIA

TTTS

HYPOVOLEMIAHYPOVOLEMIAHYPOVOLEMIAHYPOVOLEMIAHYPERVOLEMIAHYPERVOLEMIAHYPERVOLEMIAHYPERVOLEMIA

RECIPIENTRECIPIENTRECIPIENTRECIPIENT DONORDONORDONORDONOR

INCREASED INCREASED INCREASED INCREASED

CARDIAC OUTPUTCARDIAC OUTPUTCARDIAC OUTPUTCARDIAC OUTPUT

POLYURIAPOLYURIAPOLYURIAPOLYURIA OLIGURIAOLIGURIAOLIGURIAOLIGURIA

TTTS

http://bms.brown.edu

TTTS

30/100

15/100

TTTS

PolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramnios

> 10 cm> 10 cm> 10 cm> 10 cm> 10 cm> 10 cm> 10 cm> 10 cm

Diagnostic CriteriaDiagnostic CriteriaDiagnostic CriteriaDiagnostic CriteriaDiagnostic CriteriaDiagnostic CriteriaDiagnostic CriteriaDiagnostic Criteria

PolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramnios

Oligo/anhydramniosOligo/anhydramniosOligo/anhydramniosOligo/anhydramniosOligo/anhydramniosOligo/anhydramniosOligo/anhydramniosOligo/anhydramnios OliguriaOliguriaOliguriaOliguriaOliguriaOliguriaOliguriaOliguria HypervolemiaHypervolemiaHypervolemiaHypervolemiaHypervolemiaHypervolemiaHypervolemiaHypervolemia

PolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramniosPolyhydramnios

TTTSTTTS. Pre-operative Staging

Stage I.Stage I. Poly/Poly/OligohydramniosOligohydramnios withwith

bladderbladder of the of the DonorDonor stillstill visiblevisible

Stage IIStage II.. BladderBladder of the of the DonorDonor not visiblenot visible

Stage IIIStage III.. PresencePresence of of EitherEither aedfaedf in the UA, in the UA, ..reverse flow in the DV, or pulsatile UV reverse flow in the DV, or pulsatile UV

in in eithereither twintwin

Stage IVStage IV. . HydropsHydrops in in eithereither twintwin

Stage VStage V.. DemiseDemise of one or of one or bothboth twinstwins

Quintero et al 1999 Quintero et al 1999

TTTS

• New staging options.

• Based on detailed ultrasound cardiac examination in both twins.

• Cluster analysis allows to categorize cases

TTTS. Pre-operative Staging

• Cluster analysis allows to categorize cases based on cardiac profile in the recipient twins.

• Three main profiles with prognostic values

(Stirnemann 2008)

TTTSTreatment:

• Emergency

• Importance of initial choice

• Appropriate initial assessment

TTTSTreatment options:

• Expectant management ?:

–Not an option….!

–Overall survival rate of 27% together with a 25% rate of neurological damage in the 25% rate of neurological damage in the survivors (Berghella and Kaufmann 2001).


• Septostomy:

– Allow fluid to freely flow from the polyhydramniotic sac to the stuck-twin oligohydramniotic.

– Expected to restore a normal pressure balance between the twins.the twins.

– Overall perinatal survival ≤ to amnioreduction in several randomized studies (Johnson, Rossi et al. 2001; Moise,

Dorman et al. 2005).

– Risk: PABS


• Amnioreduction:

– Simple and accessible.

– Amniotic fluid is removed until a DVP of 5-6 cm is

reached (Senat, Deprest et al. 2004).

– Improves placental perfusion / doppler. – Improves placental perfusion / doppler. (Smith, Pesterfield et al.

1997)

– Before 26 weeks, amnioreduction should be offered

only if fetoscopic laser coagulation is not readily

available and depending on the degree of

emergency.

– Complications do exist !

– Overall survival rate in TTTS between 15% and 83%.


• Laser:– Based on high-level evidence (Senat, Deprest et al.

2004), laser surgery has been established as the

best first-line treatment for TTTS before 26 weeks.

– Complication: PPROM, infection.– Complication: PPROM, infection.

TTTSLaser vs amnioreduction

Eurofoetus RCT amnioreduction vs laser

142 TTS

France

Belgium

Netherlands

Switzerland

Italy

USA

randomization

Perinatal survival of at least one twin*

Laser Amnioreduction

76% 56%

randomization


N=72 N=70

Gestational age at delivery


33 w. 29 w.

Survival 6 months without neuro impairment


52% 31%

TTTSLaser - technique

• Emergency

• Local anesthesia

• Percutaneous

• 2 mm semi-rigid fetoscope

• Drainage < 4L• Drainage < 4L

• Cerclage if CL ≤ 15mm

TTTSNon-Selective

Coagulation: 15-36%

TTTSSelective

coagulation: 64-85%

Vein

Artery

TTTS

R

NSC

SC

Laser procedure - Selectivity

D

Quintero R.A., UOG, 2000Stirnemann J.J., AJOG, 2007

→ « Dichorionization » of a MC placenta

TTTSOverall outcome - Laser

2 alive and

well (1+/1+)

0 alive

(0/0)

2 alive and

well at 28

days

(28%)

1 alive and

well (1+/0)1+/1-

1-/0

1-/1-

At least 1 alive and

well at 28 days

(65%)


• Selective TOP:

– Whenever the prognosis of one twin is apparently

hopeless .

– IUFD -> Adverse outcome (50%) in the surviving twin

without placental surgery without placental surgery (van Heteren, Nijhuis et al. 1998; Quarello 2008).

– Bipolar forceps coagulation (Ville 2003; Robyr, Yamamoto et al. 2005).

–++ if severe malformations, neurological anomalies

in either twin and preagonal growth-restriction or

hydrops.

– Complication: PPROM, infection.


• Delivery:

– ++ after 28 weeks’.

– ? 26-28 weeks’.

TTTS

Treatment option Comment

Percutaneousselective laser coagulation (PSLC)

Best first-line treatment.Widely available but requires specific equipment and skills

Cord coagulation (CC)

Only in specific indications if one twin displays preagonal features.Technically challenging.Technically challenging.Raises ethical problems.

Amniodrainage Recommended as first-line treatment if PSLC is not available.Easier than PSLC and CCRecommended after 26 weeks together with lung maturation and elective delivery.

Delivery In rare cases of late 3rd trimester occurrence of TTTS.An amniodrainage is recommended before delivery regardless of the delivery mode.


• Follow-up:

StillStillStillStillStillStillStillStill atatatatatatatat riskriskriskriskriskriskriskrisk

••RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 –––––––– 14 %)14 %)14 %)14 %)14 %)14 %)14 %)14 %)••RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 RECURRENCE (10 –––––––– 14 %)14 %)14 %)14 %)14 %)14 %)14 %)14 %)

••DISCORDANCE DISCORDANCE DISCORDANCE DISCORDANCE DISCORDANCE DISCORDANCE DISCORDANCE DISCORDANCE HbHbHbHbHbHbHbHb (10 (10 (10 (10 (10 (10 (10 (10 -------- 13 %)13 %)13 %)13 %)13 %)13 %)13 %)13 %)

TTTS

Lewi et al 2006

Large/Multiple: Recurrence

Small/Unique: Transfusion

Remaining vessels

Lewi et al 2006

TTTS

MARI et al.

2000

TTTS

RR22 = 0.70= 0.70p = 0.001p = 0.001

CorrelationCorrelationCorrelationCorrelationCorrelationCorrelationCorrelationCorrelation PSVPSVPSVPSVPSVPSVPSVPSV--------MCA / MCA / MCA / MCA / MCA / MCA / MCA / MCA / FetalFetalFetalFetalFetalFetalFetalFetal HbHbHbHbHbHbHbHb

BeforeBefore TransfusionTransfusion

Se =90%

SP =90%

FP = 10%

p = 0.001p = 0.001

1.50 MoM ⇔⇔⇔⇔ moderate anemia

> 1.55 MoM ⇔⇔⇔⇔ severe anemia

FP = 10%

Senat 2004

TTTSCorrelationCorrelationCorrelationCorrelationCorrelationCorrelationCorrelationCorrelation PSVPSVPSVPSVPSVPSVPSVPSV--------MCA / MCA / MCA / MCA / MCA / MCA / MCA / MCA / FetalFetalFetalFetalFetalFetalFetalFetal HbHbHbHbHbHbHbHb

AfterAfter TransfusionTransfusion

p = 0.001p = 0.001

1.50 MoM ⇔⇔⇔⇔ moderate anemia

> 1.55 MoM ⇔⇔⇔⇔ severe anemiaSenat et al 2004

TTTS

Diagnosis of TTTS

PSLC Amnioreduction Delivery

(amnioreduction should precede)

≤26 weeks >26 weeks Late pregnancyGA at diagnosis

Follow-up Sonographic follow-up

Daily during early post-operative

Weekly thereafter

Survivors 1 2

First-line treatment

1 2

Acute exsanguination of

the survivor

suspected on

PSV MCA > 1.5 MoM

FBS +/- transfusion

Recurrence

Feto-fetal

hemorrhage

•FBS + transfusion

•+/- repeat laser or

•Cord coagulation

•Repeat laser or

•Cord coagulation or

•Amnioreduction or

•Delivery

28-32 weeks: MRI

34 weeks: discuss

elective delivery

Complications

ttts: physiology, signs and treatment options · ttts: physiology, signs and treatment options...

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