The document discusses various fetal interventions including amniocentesis, transfusions, selective feticide, radiofrequency ablation, laser therapy, fetoscopic surgery, and open fetal surgery. It provides details on indications and techniques for interventions addressing conditions like twin-twin transfusion syndrome, cardiac arrhythmias, fetal hydrops, congenital diaphragmatic hernia, and more. The success and risks of interventions for various conditions are presented along with guidelines for determining when in utero surgery may be appropriate.

1. Doç. Dr. Gökhan YILDIRIM
Kanuni Sultan Süleyman EAH
Perinatoloji Kliniği

2.  Fetus ve eklerindeki sorunu çözmek için medikal yada cerrahi müdahale
etmektir.
 Son 15 yıldır ise giderek artan oranda fetal tedavi girişimleri olmaktadır.
 Gelecekte çok daha yaygın ve sık olması beklenmektedir.
 İlk deneyimler Rh izoimmünizasyon intrauterin transfüzyon ve amniyodrenaj

3. USG
Anomalilerin tanımlanması
Tanı
Perinatal sonuçlar
Fetal tedavi

4. Amaç:
Perinatal morbidite ve mortalite’yi önlemek /
iyileştirmek
Kalıcı organ hasarlarını/Nöromorbidite’yi
önlemek / azaltmak
Fetal tedavi

5.  Medikal
Yöntemler
◦ Amniodrenaj
◦ Transfüzyon
◦ Selektif fetosid
◦ Radiofrekans
◦ Bipolar
◦ Laser
◦ Fetoskopik cerrahi
◦ Girişimsel-Cerrahi
 Kapalı
 Açık
◦ Laparotomi / histerotomi

6.  MSS
 MMC
 Pulmoner sistem
 Hidrotoraks
 Bronkopulmoner sekestrasyon
 CCAM
 Diafragm hernisi
 Kalp
 Aritmi
 Ao, Pa stenozu, Fo kapanması
 Üriner sistem
 Megasist
 Hidronefroz
 Hematolojik sistem
 Anemi
 Trombositopeni
 Endokrin sistem
 Guatır
 KAH
Fetal tedaviler: Türkiye
 İkiz gebelikler
◦ TTTS
◦ TRAP
◦ Selektif anomali, IUGR
 Plasenta-amniotik sıvı-kordon
◦ Polihidramnios drenajı
◦ Vasa previa
◦ Amniotik band
◦ Koranjioma
 Enfeksiyonlar
◦ Toxoplazma
 Fetal Tümör
◦ Sakral teratom
 Gen tedavisi
◦ Genetik geçişli hastalıklar

7.  Fetal ve Neonatal Alloimmün Trombositopeni (FNAİT)
 Fetal Kardiyak Disritmi
 Fetal Guatr
 Konjenital Adrenal Hiperplazi

8. • İnsidans; 1/350-1/1000
• PLT <50.000 ciddi FNAİT (%10 İKK)
• Olguların %85’inde HPA-1a Ag sorumlu
• HPA-1a-pozitif fetusa sahip HPA-1a-negatif kadınların yalnızca %10’unda
antikor üretilir.
• HLA DRB3*0101 yokluğunda immünüzasyon nadir (NPP %99,6)

9. Tedavi
 İntra uterin trombosit tranfüzyonu
 IVIG, 1.0 g/kg/hafta (20 – 32. hafta arasında)
 Prednisone 0.5 mg/kg/gün

10.  İntermittent ekstrasistol
 Supraventriküler taşikardi
 Atrial flutter
 Komplet AV blok
 Ventriküler taşikardi (nadir)
 Tanı; M-mode, pulsed-wave ve doku
Doppleri
 Tedavi;
 SVT/atrial flutter
 Yakın izlem
 Anti-aritmik tedavi
 Digoxin
 Flecainide
 Sotalol
 Amiodarone
 Adenosine
 Atrioventiküler blok
 Deksametazon/betametazon
 Beta-agoistler (terbutalin, salbutamol)
 Plazmaferez
 Fetal pacing

11. • Flekainid ve digoksin SVT normal ritme çevirmede ve SVT/AF’ı daha iyi tolare edilen ventriküler hıza
yavaşlatmada sotalol’dan daha etkili
• İlk basamak tedavide flekainid ve digoksin düşünülebilir
SVT (114)/AF (45) 159 olgu, retrospektif , 3 merkez

12.  Fetal Guatr
 Propsil kullanan anne
 %9.5 fetal hipotiroidi
 %10 fetal hipertiroidi
 Grave’s hastalığı
 İodine eksikliği
 Fetal tiroid disgenezisi, enzim defekti
 İntra-amniotik 200-500 mik.gr tiroksin tedavisi, 1-2 hf ara ile
 Guatr boyutu azalmakta

13.  KAH
◦ 21-OH eksikliği, OR
◦ ACTH-Adrenal hiperplazi-androjen artışı
◦ Female fetusda virilizasyon
◦ 1.5 mg Deksametazon <7 hf , 11-14 hf CVS: male /Female- CYP21
normal Deksametazon stop
◦ %85 virilizasyonu önlüyor

14. Fetal Cerrahi için Kriterler
 Doğru tanı ve evreleme olanaklı olmalı, diğer anomaliler dışlanmalı
 Hastalığın doğal seyri ve prognozu tanımlanmış olmalı
 Etkili postnatal tedavisi olmamalı
 Hayvan modellerinde in-utero cerrahinin yapılabilir olduğu ve sorunun olumsuz
etkilerinin geri döndürülebilir olduğu kanıtlanmalı
 Girişim katı protokolleri olan ve ebeveynler bilgilendirilerek lokal etik komitelerin
onayının alındığı özelleşmiş multidisipliner fetal tedavi merkezlerinde
yapılmalıdır.

17.  Kritik Aort Stenozu
 Restriktif Foramen Ovale (HLHS)
 Pulmoner Atrezi (İntakt Ventriküler Septum)

18.  Koroner balon kateteri, 3-4.5 mm
 18 - 19 G
 Lokal / genel anestezi
 Fetal ekokardiografi-USG eşliğinde

20. • Komplikasyonlar
 Fetal resüsitasyon gerektiren bradikardi (%17 – 38)
 Hemoperikardium (%13)
 Ventriküler tromboz (%15 – 20)
 Fetal ölüm (%8 – 13)

21. Balon valvuloplasti
Bakırköy EAH, 2009
Kr.Ao.Sten.

22. Fetal Toraks Patolojileri
 Plevral Efüzyon (Hidrotoraks/Şilotoraks)
 Kistik Konjenital Adenoid Malformasyon
 Bronkopulmoner Sekestrasyon

23. Plevral Effüzyon
 İzlem
 Torakosentez
 Plöroamniyotik şant
 Plörodezis (OK-432, Streptokokus Piyogenes)
Torakoamniotik şunt, Has R, İTF

24. Yang YS et al., 2012
 Yaşam oranları
 Hidropik : % 0-67
 Non-hidropik: % 33-100
 Şant migrasyonu/obstrüksiyonu (%10 – 20)
 Preterm erken membran rüptürü/eylem

25.  Pulmoner Parankim Lezyonları
 CCAM (makrokistik/mikrokistik)
 İzlem
 Torakosentez
 Torakoamniyotik şant
 Minimal invaziv teknikler (interstisyel lazer,
radyofrekans ablasyon, siyanoakrilat enjeksiyonu)
 Açık fetal cerrahi (mikrokistik)
 Maternal Kortikosteroid (Betametazon 12 mg, iki
kez)
 BPS
 Sklerozan enjeksiyonu
 İnterstisyel lazer koagülasyonu
CVR >1.6 ise hidrops riski yüksek
to polyhydramnios. In case of polyhydramnios, cervical
length should be measured and taken into consideration
when assessing the necessity of intervention.
Figure 1—Transverse view of the fetal chest showing a macrocystic
CCAM
Figure 2—Transverse view of the fetal chest showing a microcystic
CCAM
lun
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Copyright ã 2011 John Wiley & Sons, Ltd.
PRENATAL INTERVENTIONS FOR FETAL LUNG LESIONS 633
In addition seven single cases of hydropic fetuses
with BPS treated by thoracoamniotic shunt placement
have been described (Weiner et al., 1986; Slotnick et al.,
1990; Hernanz-Schulman et al., 1991; Favre et al., 1994;
Salomon et al., 2003; Picone et al., 2004; Odaka et al.,
2006) In one case PE reaccumulated probably because of
shunt occlusion (Weiner et al., 1986) In all other cases
hydrops resolved and fetuses survived after birth.
Laser and sclerosing agents in the
treatment of CCAM and BPS
Attempts at percutaneous ablation of a microcystic
CCAM in a hydropic fetus using Nd:YAG laser have
been described four times in the literature (Fortunato
et al., 1997; Bruner et al., 2000; Davenport et al., 2004;
Ong et al., 2006) In all cases a 600 mm laser fibre
was passed through the lumen of an 18G-needle after
which the tumour itself was photocoagulated using an
Nd:YAG laser. In one case (Ong et al., 2006) hydrops
resolved and the fetus survived needing postnatal respi-
ratory support and surgical resection the lesion. In one
case (Bruner et al., 2000) the fetus died prenatally. In
one case (Davenport et al., 2004) the fetus died 4 days
after birth. In the last case resolution of hydrops was
described but no further outcome was reported (Fortu-
nato et al., 1997)
The group of Quintero reported on three cases of
CCAM complicated by fetal hydrops and treated with
percutaneous insertion of a sclerosing agent directly into
the CCAM (Bermudez et al., 2008) In all cases hydrops
resolved and all fetuses were born alive. One neonate
died after 10 days because of nosocomial sepsis.
Figure 4—Ultrasound image of a large lung lesion with PE
Figure 5—Colour Doppler showing systemic artery, thus diagnosis of
pulmonary sequestration
Figure 1—Transverse view of the fetal chest showing a macrocystic
CCAM
Figure 2—Transverse view of the fetal chest showing a microcystic
CCAM
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A
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2
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th
si
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N
ca
ra
si
Copyright ã 2011 John Wiley & Sons, Ltd.

26. • Sağ kalım; Hidropik %68.2/Non-hidropik %87.5

27.  Seri vezikosentez
 Vesiko-amniotik şunt
 Piyeloamniotik şunt
 Fetal sistokopi
Renal fonksiyonlar (iyi prognoz) (güvenilirlik?)
 Na < 100 mEq/L
 Cl < 90 mEq/L
 Osmolarite <210 osm
 Beta-2 Mikroglobulin
Pyelo-amniotik şunt, Şahinoğlu Z, ZKH
Vesiko-amniotik şunt, Has R, İTF
656 J. A. DEPREST et al.
Figure 1— First description of lamb model for multiple access endoscopic in utero surgery. With permission, from Luks et al. (1994)
Figure 2— The first successful open fetal surgery at UCSF. The fetal
lower torso is exteriorised through the hysterotomy and the urinary
tract is decompressed surgically. With permission, from Harrison
(1996, chapter 5, p. 76)
treatment and frank disclosure of failures. They agreed
on ethical guidelines, such as peer review publication
prior to media exposure, and standards for fetal inter-
vention. They first met in 1981 in Santa Ynez (Califor-
nia), where Sir William Liley was a keynote lecturer.
The IFMSS was officially established 1 year later in
Aspen (Colorado) and drafted the ethical framework that
still applies today (Table 1). The society founded a jour-
nal, established a registry of interventions, and published
a first report on intra-uterine shunting shortly thereafter
(Manning et al., 1986). Whereas shunts were success-
ful for LUTO, the group agreed at that moment on a
voluntary moratorium on shunting for hydrocephalus.
LUTO can be caused by stenosis of the urethral
meatus, valves, urethral atresia, ectopic insertion of a
ureter or even (peri)vesical tumours. Bladder shunts are
effective for urine diversion, restoring amniotic fluid
and thereby preventing pulmonary hypoplasia (recently
reviewed by Mann et al., 2010). Whether shunting
effectively salvages renal function is uncertain. For

28. • Toplam 12 çalışma; 261 girişim (%87 VAS; 9 fetus açık cerrahi; 26 fetal sistoskopi ) (RKT yok)
• Antenatal girişim perinatal sağ kalımı düzeltiyor (OR= 3,82)
• İyi prognoz kriteri olan olgularda sağ kalımı düzeltiyor, ancak anlamlı değil
• Kötü prognostik kritere sahip olgularda etki daha fazla

29. • Fetal sistoskopi vs VAS; perinatal sağ kalımda anlamlı fark yok (OR=1,49)
• Fetal sistoskopi vs tedavi yok; perinatal sağ kalımda anlamlı düzelme (OR=20,51)

30. • 150 olgu planlanmış
• Yeterli hasta toplanamadığı
Için erken sonlandırıldı.
Terminasyon sayısı beklenenden
yüksek (68)
• Her iki grup belirgin uzun ve
kısa dönem morbiditeye
sahip

31. Konjenital Diyafragma Hernisi (FETO)
 3000 gebelikte 1
 Sorun pulmoner hipoplazi
 Postnatal cerrahide mortalite %30
 26-30 hf trakeal balon
 34 hf trakeal balon çıkarılıyor
 Lokal, lokorejyonel veya genel
anestezi

32. THE MAKING OF FETAL SURGERY 661
e 5—Survival rates of fetuses with isolated left-sided CDH depending on measurement of the observed/expected lung to head ratio (O/E
measurements and liver position as in the antenatal CDH registry (Jani et al., 2006b); figure modified from Deprest et al. (2009)
olar type II cells (De Paepe et al., 1998) and sur-
ant (O’Toole et al., 1996). The Leuven group sug-
ed to limit the latter effects by reversal of TO before
(plug–unplug sequence) (Flageole et al., 1998).
wever, functional studies still demonstrate that the
onse was better but not yet ideal (Luks et al., 2000;
ey et al., 2003). Nearly normal lung growth and
use a combination of both variables to define poor
prognostic groups (Figure 5). In the near future, we
expect magnetic resonance imaging (MRI) volumetry
to play a more important role, because it has less
maternal limitations and it can reliably and accurately
measure total rather than unilateral lung size as well
as quantifying the amount of liver herniated into the
 O/E LHR: < % 15, sağ kalım şansı yok
 O/E LHR %15-25; sağ kalım %25
 O/E LHR > %15-25; sağ kalım %60 ve ↑
Lung area = Length 1 X Length 2
Lung area to Head circumference
Ratio (LHR) = Lung area / Head
circumference
The Lung area to Head
circumference Ratio (LHR) =
Lung area / Head circumference

33. Brezilya, 2012
 Yaşam oranları
◦ FETO: %50
◦ Kontrol: %5

34.  Yaşam oranları
 L: %54
 R: %39
210 vaka
Çok merkezli

35. Tracheal Occlusion To Accelerate Lung Growth (TOTAL)
Trial for Severe Pulmonary Hypoplasia
Primary Outcome: Survival at discharge from neonatal intensive care unit
Placebo Comparator: expectant management during pregnancy
watchful waiting during pregnancy
Experimental: fetal endoluminal tracheal occlusion
fetoscopic balloon occlusion at 27 to 30 weeks of gestation
Estimated Enrollment: 148
Study Start Date: December 2010
Estimated Study Completion Date: October 2017
Estimated Primary Completion Date: October 2015 (Final data collection
date for primary outcome measure)

36. Nöral Tüp Defektleri
 Meningomyolosel
 10.000 doğumda 3.4
 %10 fetal mortalite
 5 yıl > %15-30 mortalite
 Ağır nöromorbidite
Amaç:
 Nörolojik hasar ve komplikasyonların gelişimini önlemek
 Fetoskopik cerrahi, 1997
 Açık fetal cerrahi, 1997
◦ 400 vaka
Figure 1.
Open fetal repair for myelomeningocele. a) Myelomeningocele defect before repair. b) Fi
skin closure of a myelomeningocele defect.
Saadai and Farmer Page
Clin Perinatol. Author manuscript; available in PMC 2013 June 01.
NIH-PAAuthorManuscriptNIH-PAAuthorManuscriptNIH-PAAuthorManuscript

37. 2011
Children’s Hospital of Philadelphia
Vanderbilt University
University of California, San Francisco

38. Box 1. Inclusion and exclusion criteria for the Management of
Myelomeningocele Study
I nclusion criteria:
1. Myelomeningocele at level T1 through S1 with hindbrain herniation.
2. Maternal age 18 years or older.
3. Gestational age at randomization of 19 0 to 256 weeks.
4. Normal karyotype.
Exclusion criteria:
1. Non-resident of the United States.
2. Non-singleton pregnancy.
3. Insulin-dependent pregestational diabetes.
4. Fetal anomaly not related to MMC.
5. Kyphosis in the fetus of 30 degrees or greater.
6. Current or planned cerclage or documented history of incompetent cervix.
7. Short Cervix (<20mm).
8. Placenta previa or placental abruption.
9. Body mass index 35 or greater.
10. Previous spontaneous delivery prior to 37 weeks’ gestation.
11. Maternal-fetal Rh isoimmunization, Kell sensitization or neonatal alloimmune
thrombocytopenia
12. Maternal HIV or Hepatitis B status positive.
13. Known Hepatitis C positivity.
14. Uterine anomaly such as large or multiple fibroids or müllerian duct
abnormality
15. Other maternal medical condition which is a contraindication to surgery or
general anesthesia
16. Patient does not have a support person.
17. Inability to comply with travel and follow-up requirements.
18. Patient does not meet other psychosocial criteria to handle the implications of
the trial.
19. Participation in another intervention study that influences maternal and fetal
morbidity and mortality or participation in this trial in a previous pregnancy.
20. Maternal hypertension which would increase the risk of preeclampsia or preterm
delivery.

39.  Şant ihtiyacı azalıyor, %40 v %82
 Hindbrain herniasyon azalıyor, %64 v %96
 Bağımsız yürüme, %42 v %21

40.  Polihidramnios
Plasenta-koryon-amniyon
 Fetal ekstremite
amputasyonu, defektleri
 Amniodrenaj
 Laser
 Bipolar
 Fetal kalp yetmezliği
 Amniotik band
 Preterm doğum
 Koranjioma

41.  Sakral teratom
Fetal tümörler
 Açık cerrahi
 Laser
 Bipolar
 Radiofrekans
 Fetal kalp yetmezliği
 Anemi, hidrops fetalis

42. GELECEK