Fetal Therapy Fetal Transfusion Fetal Medical Therapy Fetal

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태아 치료(Fetal Therapy) • 태아 수혈(Fetal Transfusion) • 태아 약물치료(Fetal Medical Therapy) • 태아

태아 치료(Fetal Therapy) • 태아 수혈(Fetal Transfusion) • 태아 약물치료(Fetal Medical Therapy) • 태아 줄기세포 이식(Fetal Stem Cell Transplantation) • 태아 유전자치료(Fetal Gene Therapy) • 태아 수술(Fetal Surgery)

144 centers (U. S. : 17 centers)

144 centers (U. S. : 17 centers)

Some Abnormalities Amenable to Fetal Surgery • Open Fetal Surgery – – Cystic adenomatoid

Some Abnormalities Amenable to Fetal Surgery • Open Fetal Surgery – – Cystic adenomatoid malformation Extralobar pulmonary sequestration Sacrococcygeal teratoma Spina bifida

Some Abnormalities Amenable to Fetal Surgery • Fetoscopic Surgery – Twin-Twin transfusion: laser of

Some Abnormalities Amenable to Fetal Surgery • Fetoscopic Surgery – Twin-Twin transfusion: laser of placental anastamoses – Diaphragmatic hernia: fetal endoscopic tracheal occlusion (FETO) – Posterior urethral valves: cystoscopic laser – Congenital high airway obstruction: vocal cord laser – Amnionic band release

Some Abnormalities Amenable to Fetal Surgery • Percutaneous Procedures – Shunt therapy • Posterior-urethral

Some Abnormalities Amenable to Fetal Surgery • Percutaneous Procedures – Shunt therapy • Posterior-urethral valves/bladder outlet obstruction • Pleural effusion: chylothorax or sequestration • Dominant cyst in congenital cystic adenomatoid malformation (CCAM) – Radiofrequency ablation • Twin-reversed arterial perfusion (TRAP) sequence • Monochorionic twins with severe anomaly(ies) of 1 twin • Chorioangioma – Fetal intracardiac catheter procedures • Aortic or pulmonic valvuloplasty for stenosis • Atrial septostomy for hypoplastic left heart with restrictive atrial septum

Some Abnormalities Amenable to Fetal Surgery • Ex-utero-intrapartum-treatment (EXIT) procedures – Congenital diaphragmatic hernia

Some Abnormalities Amenable to Fetal Surgery • Ex-utero-intrapartum-treatment (EXIT) procedures – Congenital diaphragmatic hernia after FETO – Congenital high airway obstruction sequence (CHAOS) – Severe micrognathia – EXIT-to-resection • Resection of fetal thoracic or mediastinal mass • Tumors involving airway/neck – EXIT-to-extracorporeal membrane oxygenation (ECMO) • Congenital diaphragmatic hernia(if no fetal surgery)

Fetoscopic Surgery

Fetoscopic Surgery

History of Fetoscopy First attempt : Westin, 1954 A 10 mm hysteroscope Through transcervical

History of Fetoscopy First attempt : Westin, 1954 A 10 mm hysteroscope Through transcervical approach Transabdominal approach Mandelbaum, 1967 Valenti, 1972: skin sample Scrimgeour, 1973 : ‘Fetoscope’, Dx. of spina bifida

History of Fetoscopy 1970 s: Fetoscopic blood sampling 1980 s: Fetoscopic organ biopsy 1990

History of Fetoscopy 1970 s: Fetoscopic blood sampling 1980 s: Fetoscopic organ biopsy 1990 s: Fetoscopic fetal surgery

History of Fetoscopic Surgery De Lia (1990) Fetoscopic laser occlusion of chorioangiopagous vessels Treatment

History of Fetoscopic Surgery De Lia (1990) Fetoscopic laser occlusion of chorioangiopagous vessels Treatment of TTTS

Fetoscopic Instruments (I) (Klaritsch et al. )

Fetoscopic Instruments (I) (Klaritsch et al. )

Fetoscopic Instrument (II) (Klaritsch et al. )

Fetoscopic Instrument (II) (Klaritsch et al. )

Fetoscopic Surgery Fetoscopic fetal surgery CDH Neural tube defect Cardiac malformations Lower urinary tract

Fetoscopic Surgery Fetoscopic fetal surgery CDH Neural tube defect Cardiac malformations Lower urinary tract obstruction Surgery on the placenta, cord, membrane TTTS Fetal acardiacus and discordant anomalies

TTTS Transfusion from donor twin to recipient twin

TTTS Transfusion from donor twin to recipient twin

Procedure 1. 2. 3. 4. 5. 6. 7. 8. Prophylactic antibiotics and tocolytics 1

Procedure 1. 2. 3. 4. 5. 6. 7. 8. Prophylactic antibiotics and tocolytics 1 hr before Local anethesia A 2 -mm skin incision and insertion of trocar into recipient’s sac under sonographic guidance Application of endospcpy: 1. 2 -2. 0 mm Identify dividing membranes (White Line) Selective approach vs. non-selective approach Coagulation at a distance of 1 cm and at a 90°angle (3 -4 seconds) Amnioreduction slightly below normal (Kateb and Ville, 2008)

Fetoscopic Laser op.

Fetoscopic Laser op.

Twin Births in Korea 통계청 2007

Twin Births in Korea 통계청 2007

TTTS in SNUH • Between 2007. 1 -2009. 8 No GA Stage Treatment GAD

TTTS in SNUH • Between 2007. 1 -2009. 8 No GA Stage Treatment GAD Outcome 1 18. 1 III AR + septo 19. 3 Aggr. - termination 2 18. 6 IV AR + septo 19. 9 Aggr. - termination 3 25. 0 I II (30) Observation 31. 3 D: NEC, PVL; R: BPD 4 18. 7 II Serial AR 29. 1 D: death d/t pul. hmr R: mod. BPD 5 18. 4 III Serial AR 23. 4 Distress both death

TTTS in SNUH • Between 2007. 1 -2009. 8 No GA Stage Treatment GAD

TTTS in SNUH • Between 2007. 1 -2009. 8 No GA Stage Treatment GAD Outcome 21. 6 I IV AR 25. 3 D: Death; R: BPD, PDA, sepsis, club foot 7 22. 6 I III (selective IUGR>>) - 34. 6 Both: alive 8 21. 0 IV - 9 23. 3 III (selective IUGR>>) - 6 F/U Loss 26. 3 Ongoing

태아수술의 활용 (2) 선천성 횡격막탈장(Congenital Diaphragmatic Hernia; CDH) l Fetoscopic Endoscopic – Tracheal Occlusion

태아수술의 활용 (2) 선천성 횡격막탈장(Congenital Diaphragmatic Hernia; CDH) l Fetoscopic Endoscopic – Tracheal Occlusion (FETO) 이용 l 심한 폐발육부전군에서 생존률의 급격한 향상!

FETO

FETO

태아수술의 활용(3) • Amniotic band syndrome – Laser: cause collateral damage – Development of

태아수술의 활용(3) • Amniotic band syndrome – Laser: cause collateral damage – Development of ‘'optical’' scissors: pair of parrot-beaked scissors with an endoscope • Shunting and fetal cystoscopy – obstructive uropathy, cystic lesion of thorax, hydrocephalus – Double-pigtail shape: d 1. 67 mm - 3. 0 mm – Cystoscopy: still too large • Fetal intracardic catheter procedures – Aotic valvuloplasty, pulm. valvuloplasty, atrial septostomy

Development of fetal endoscopic surgery • Limitation of open fetal surgery – PTL –

Development of fetal endoscopic surgery • Limitation of open fetal surgery – PTL – Altered fetal homeostasis resulting from hysterotomy – Fetal exposure • Stimulation of fetal endoscopic surgery by limitation • Experimental and clinical experience of fetoscopic surgery – Diminution in the severity of postoperative PTL – Less impaired fetal homeostasis

Future of fetoscopic surgical intervention • Clarifying the natural history of fetal disorders •

Future of fetoscopic surgical intervention • Clarifying the natural history of fetal disorders • Improving diagnostic and imaging techniques : small diameter, high-resolution steerable scopes • Instrument refinements – robotic surgery – amniodistension – noninvasive fetal ECG(marketed) • Training and credentialing of clinicians • Better understanding of PTL and PPROM • Less invasive therapy, noninvasive energy modalities, medical alternatives