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Update in Fetal Surgery
Jimmy Espinoza, MD, MSc, FACOGAssociate Professor
Baylor College of Medicine and Texas Children’s Pavilion for WomenHouston, Texas
Texas Children’s Pavilion for Women
Disclosures
Ø I have no conflict of interests with the contents of this lecture
Objectives
ØWhat is fetal surgery?
ØWhy do fetal surgery?
ØWho needs fetal surgery?
ØWho should do fetal surgery and how should we monitor what is being done?
ØSome examples of fetal surgery
What is Fetal Surgery?• Application of established
surgical techniques to the unborn baby
– During gestation– At end of gestation
Why do Fetal Surgery?• To improve outcome in cases of congenital malformation.
• To prevent fetal death
• To prevent postnatal deathand/or reduce significant long-term morbidity
Principles of fetal surgery
ØCorrect and precise prenatal diagnosisØAbsence of associated anomalyØKnowledge of the natural historyØHigh perinatal morbidity/mortalityØAbsence of effective neonatal therapyØAnimal studies showing favorable resultsØPerformed in specialized centers - multi-D approachØNot compromise the reproductive futureØShould not increase maternal mortality
Harrison et al 2001
Level I evidence - RCTØTTTS (Laser ablation)
ØCDH (fetoscopic tracheal occlusion)
ØMMC (Open in-utero closure)
ØLUTO (vesico amniotic shunting)
Candidates for Fetal Surgery
• TTTS, TRAP Sequence• Thoracic: lung mass or
hydrothorax with hydrops • Teratoma: sacrococcygeal or
cervical teratoma with hydrops• Airway obstruction: Neck
masses or laryngeal atresia (CHAOS)
• EXIT procedure for predictable cardiorespiratory compromise
• Myelomeningocele• Amniotic band release
• Selective IUGR• Congenital diaphragmatic
hernia• Bladder outlet obstruction• Aortic or pulmonary outflow
obstruction• Gene/ stem cell therapy for
metabolic-cellular defects/ stem cell-enzyme defects
Established Benefit Probable Benefit
The hidden mortality
of monochorionic
twin pregnancies
Sebire et al 1997.
Dichorionic
Monochorionic
Level I evidence - RCT
ØTTTS (Laser ablation)
ØCDH (fetoscopic tracheal occlusion)
ØMMC (Open in-utero closure)
ØLUTO (vesico amniotic shunting)
Twin –Twin Transfusion Syndrome (TTTS)
Twin –Twin Transfusion Syndrome (TTTS)
AVRDRecipientTerritory
DonorTerritory
A-A
Twin-to-Twin Transfusion S. Laser vs. Amnioreduction
Laser Amnioreduction
Survival of one fetus 40% 26%
Survival of both fetuses 36% 26%
Survival of at least one fetus
76% 51%
GA at delivery 33.3 29.0
Alive w/o neurologic problems
52% 31%
Senat et al. N Eng J Med 2004; 351:136-44
60 - 70%
75 - 90%
Laser Photocoagulation
Laser Photocoagulation of Placental Anastomoses
“Solomonization” - connect the dots and decrease the chance of persistent anastamoses
Selective Solomon Technique
Lancet. 2014; 383: 2144-51
Lancet. 2014; 383: 2144-51
Am J Obstet Gynecol. 2014; 211: 285
Am J Obstet Gynecol. 2014; 211: 285
Anterior Placenta-Challenges
ØUse of curve scopes and lateral access if there is a “window” to place the fetoscope
ØIf no “window”: laparoscopic-assisted procedure
Laparoscopic-assisted laser surgery for TTTS
33
Preterm PROM
Twin Anemia Polycythemia Sequence(TAPS)
Level I evidence - RCT
ØTTTS (Laser ablation)
ØCDH (fetoscopic tracheal occlusion)
ØMMC (Open in-utero closure)
ØLUTO (vesico amniotic shunting)
Bowel
Liver
Lung ........
Congenital Diaphragmatic Hernia
Failure of closure of pleuroperitoneal folds during Weeks 4 – 10 post fertilization1:2200 – 1:5000
Left sided 85% and right sided 10-15%Bilateral is rare
50% isolated and 50% have other anomalies15% aneuploidy, 10% syndromic
Survival According to the Severity of CDH
Ruano et al 2012
Congenital Diaphragmatic Hernia
• 3 major issues:• lung hypoplasia• pulmonary hypertension• cardiac compression
Normal
Hypoplasia
CDH: Fetal MRI
CDH: 2 Predictors of Outcome
• Lung Volume• LHR: Lung-to-head ratio
• >1.2 = 79% survival (30/38)• 0.9-1.2 = 59% survival (13/24)• < 0.9 = 4% survival (1/24)
• MRI volumetric assessment
• Liver herniation:• No: 79% survival• Yes: 41% survival
Metkus AP, et al. J Pediatr Surg 31:148, 1996Walsh DS, et al. Am J Obstet Gynecol 18:1067, 2000
STLiver
BowelLUNG
Lung-Head Ratio
HeartLHR 0.67
(Long axis x Short axis)/HC
Fetoscopic Tracheal Occlusion (FETO)
Deprest, et al. Ultrasound Obstet Gyn 24:121, 2004
TRACHEAL OCCLUSIONFetoscopic endotracheal
balloon
Fetal ET Occlusion (FETO)
LHR 2.5
20 days Post - FETO
LHR 0.67
SEVERE CONGENITAL DIAPHRAGMATIC HERNIA
1.- FETOSCOPY. intra-tracheal ballon(PLUG).
2.- Planned delivery or emergency (PPROM)E.X.I.T. strategy
3.- NEONATAL SURGERY (Defect Repair)
0 26 w 36 w
1 2 3
2nd FETOSCOPY. Balloon retrieval (UN-PLUG).
34 w
When to un-PLUG the lung?
TRACHEAL OCCLUSIONFetoscopic Endotracheal Balloon
Experience at TCH/BCM
• To evaluate the feasibility and initial outcomes of a comprehensive FETO program
• To investigate whether there is an independent additive benefit to FETO by having immediate ECMO availability and capacity
Methods and Materials• Prospective cohort: January 2012 – June 2015 • IRB and FDA approved protocol• FETO offered between 22-0/7 - 29-6/7 weeks:
– severe left-sided CDH (LHR < 1.0) and liver herniation – no chromosomal/structural anomalies/latex allergy – ability to relocate to live within 30 minutes of hospital
• Obstetrical and postnatal outcomes: – Feasibility and safety of FETO– Compared with similar cases at TCH without FETO
Subject Cohort• Evaluation with US and MRI at 24 +/- 3 wks
– US: LHR = 0.82+/ 0.09o/e LHR = 0.26+/- 0.04
– MRI: o/e TLV = 0.24 +/- 0.06 % liver herniation = 0.36 +/- 0.09
• FETO attempted in 11 patients at 28 +/- 1 wks– Successful in 10/11 (91%)
Demonstrable Surgical Feasibility• FETO balloon retrieval:
– Retrieved in 6/10 at 34 +/- 1 wks – Placement/removal interval = 5.9 +/- 1.5 wks
• Removal of tracheal balloon by:– Fetoscopy: balloon removal (n = 6), no balloon (n = 1)– Ultrasound-guided puncture of the balloon (n = 2)– EXIT procedure with balloon removal (n = 1)
• No abruption, chorioamnionitis or fetal demise
PPROM Occurrence• Spontaneous PPROM (< 35 weeks) in 3/11
(27%)– 31.7 weeks, 31.3 weeks, and 34.9 weeks
• Spontaneous PROM did not occur in any of the 7 patients who had 2 fetoscopy procedures:– 3 of these 7 patients (43%) had a vaginal delivery
Significantly Improved in utero Measures with FETO
Largely Late Preterm & Stable Delivery
• Interval from balloon removal/birth: 7 days [0-35]
• GA at birth (FETO, n=10) was 35.5 [32.6 - 40.0] wks
• 4/11 (36%) had vaginal delivery, and 7/11 (64%) CS
• No acidosis at delivery:– Median Apgar score at 5 minutes was 7 [4-9]
– Median UA pH was 7.30 [7.26 to 7.35]
• Postnatal surgical repair on day 2-4 of life– All had very large defects and all required a patch at repair
FETO Survival• Overall survival rate:
– To 6 months = 80% (8/10)– To 1 year = 67% (6/9)– To date = 70% (7/10)
• Survival to 6 months for our historical cohort of non-FETO patients = 47%
Improved Outcomes with FETO • 1/10 died from pulmonary hypertension after 4
months (pulmonary capillary hemangiomatosis)
• 3/10 required ECMO (30%) - 1/3 (33%) survived–70% of our historical cohort of non-FETO patients received ECMO
• 2/7 surviving FETO patients (29%) continue to require supplemental oxygen
ConclusionsFETO:
1.Feasible without adding significant complications
2.Significant increases in fetal lung volume
3.Improved postnatal outcomes:- Increased 6 month survival (47% to 80%)- Decreased need for ECMO (70% to 30%)
Level I evidence - RCTØTTTS (Laser ablation)
ØCDH (fetoscopic tracheal occlusion)
ØMMC (Open in-utero closure)
ØLUTO (vesico amniotic shunting)
Chiari II Malformation
Incidence
• 3.4 per 10,000 live births in US• Folic acid supplementation• Improved prenatal screening
• 1,400 to 1,500 infants born with MMC per year in the US
MMC: Fetal Surgery
Two-Hit Hypothesis
• 2 Hit Hypothesis: The final neurologic deficit results from• A combination of failure of neural tube formation• Injury from prolonged exposure of the neural
elements to the intrauterine environment
Methods
• Randomized control trial • Recruitment done at 3 MFM surgery centers• All other centers in USA agreed to not perform the surgery for
the duration of the trial• Prenatal repair:
• Standardized technique and perioperative management• Participants stayed near by until CD at 37 weeks
• Postnatal repair• Delivered by CD at 37 weeks• Postnatal repair done by the same surgical team
Exclusion Criteria:• Fetal anomaly• Severe kyphosis• Risk of PTB• Placental abruption• BMI≥ 35kg/m2
• Contraindication to surgery (ie previous classical hysterotomy)
Inclusion Criteria:• singleton pregnancy• MMC with upper boundary between T1 and S1• Evidence of hindbrain herniation• GA 19-25.9 weeks at randomization• Normal karyotype• US residency• At least 18 years old
Methods
• All children were evaluated at 12 and 30 months with physical and neurological exams
• Primary outcomes:• 12 months:
• composite of fetal or neonatal death• Need for a cerebrospinal fluid shunt
• 30 months: • composite score of the Mental Development Index of
the Bayley Scales of Infant Development II and the child’s motor function (adjusted for lesion level)
Methods
• Secondary outcomes:• Maternal/fetal/neonatal
• Pregnancy complications• Surgical complications• Neonatal morbidity and mortality
• Infant• Radiographic appearance of components of the Chiari II malformation• Time to first shunt placement• Locomotion• Psychomotor Development Index of the Bayley Scales• Scores on the Peabody Developmental Motor Scales• Degree of functional impairment• Degree of disability (measured by Functional Independence Measure for
Children)
MOMs Trial ResultsPrimary Outcome: Death or hydrocephalus at 12 months
68% 40%
98% 82%
Prenatal-Surgery Group
Postnatal-Surgery Group
Met criteria for primary outcome
Actually had a shunt placed
Decreased the risk of hydrocephalus by 30-50%
2/2003-12/2010
Maternal and pregnancy complications were more common with prenatal surgery
1/3 of subjects had a dehiscence or very thin hysterotomy site at time of delivery
Conclusions
• Despite having more severe lesions and a nearly 13% incidence of preterm delivery before 30 weeks, the prenatal surgery group had significantly better outcomes than the postnatal surgery group
• Benefits must be balanced against the risks of prematurity and maternal/ fetal morbidity
78
Case Report Fetoscopic Repair of MeningomyeloceleMichael A. Belfort, MD, PhD, William E. Whitehead, MD, Alireza A. Shamshirsaz, MD, Rodrigo Ruano, MD, PhD, Darrell L. Cass, MD, and Oluyinka O. Olutoya, MD
Fetoscopic NTD Repair
Fetoscopic NTD Repair
ENDO
(N = 18 )
OPEN
(N = 31)p
Maternal age (years) 29 � 5 28 � 6 0.55
Race or ethnic groups, no.
(%)
White 9/18 (50) 23/31 (74) 0.16Black 1/18 (6) 3/31 (10) 0.61Hispanic 8/18 (44) 5/31 (16) 0.07Other 0/18 (0) 0/31 (0) -
Nulliparity (%) 4/18 (31) 13/31 (42) 0.28
BMI at screening 27 � 4 28 � 5 0.47
Anterior placenta (%) 8/18 (44) 10/31 (32) 0.59
EGA at surgery (weeks) 24.7 � 2.0 24.4 � 1.3 0.53
Prior uterine surgery (%) 4/18 (22) 3/31 (10) 0.43
EFW < 10 % 1/18 (6) 4/31 (13) 0.74
Cervix (mm) 38 � 6.0 39 � 7.0 0.61
ENDO (N = 18)
OPEN(N = 31 )
p
GA at PROM (weeks) 33.5 � 2.0 29.7 � 4.4 0.10
PPROM (%) 5/17 (29) 9/29 (31) 0.91
PPROM < 30 weeks (%) 0/17 (0) 5/29 (17) 0.19
PPROM 30-34 6/7 wks (%) 4/17 (24) 2/29 (7) 0.24
PPROM ≥ 35 weeks (%) 1/17 (6) 2/29 (7) 0.89
No Difference in Preterm PROM
ENDO
(N = 18)
OPEN
(N = 31 )p
GA at delivery (weeks) 35.4 � 3.4 34.1 � 4.0 0.27
Delivery < 30 weeks (%) 1/17 (6) 6/29 (21) 0.36
Delivery ≥ 37 weeks (%) 8/17 (47) 9/29 (31) 0.44
Vaginal Delivery (%) 7/17 (41) 0/29 (0) <0.01
Repair to delivery (wks) 10.7 � 3.6 9.9 � 4.2 0.52
PROM-delivery (days) 1.8 � 1.7 5.4�4.5 0.11
Higher Proportion of Vaginal Deliveries
ENDO(N = 18)
OPEN (N = 31 )
P value
Placental abruption (%) 1/18 (6) 1/29 (3) 0.73Membrane separation (%) 6/18 (33) 2/29 (7) 0.05
Oligohydramnios (%) 3/18 (19) 7/29 (25) 0.81Pulmonary edema (%) 2/18 (11) 1/29 (4) 0.67Chorioamnionitis (%) 0/18 (0) 2/29 (7) 0.69 Well healed scar (%) 10/ (100) 23/29 (79) 0.29Partial dehiscence (%) 0/10 (0) 5/29 (17) 0.39Any adhesions (%) 3/10 (30) 18/29 (62) 0.17
Adhesions to omentum (%) 3/10 (30) 12/29 (41) 0.79
Blood transfusion (%) 0/18 (0) 1/31 (3) 0.45Maternal LOS 5 [3-8] 6 [2-23] 0.81
No Differences in Obstetrical Complications
ENDO(N = 18 )
OPEN(N = 31 ) P value
Birth weight
Mean (g) 2444 � 694 2360 � 853 0.73
<10% (%) 1/17 (6) 1/29 (4)* 0.7
Fetal demise (%) 0/17 (0) 0/29 (0)* -APGAR at 5 min < 7 (%) 1/17 (6) 3/29 (10)* 0.60NICU ventilation (%) 1/17 (6) 4/29 (14)* 0.73Early sepsis, (%) 0/17 (0) 4/29 (14)* 0.29
Retinopathy of prematurity (%) 0/17 (0) 3/29 (11)* 0.45
NICU LOS (days) 9.5 [2-38] 9.5 [2-76] -Perinatal death (%) 0/18 (0) 3/29 (10) 0.43RDS (%) 2/17 (12) 9/29 (31) 0.26
Similar Perinatal Outcomes
Level I evidence - RCT
ØTTTS (Laser ablation)
ØCDH (fetoscopic tracheal occlusion)
ØMMC (Open in-utero closure)
ØLUTO (vesico amniotic shunting)
Fetal Lower Urinary Tract Obstruction (LUTO)-Bladder Shunts
Lancet 2013; 382: 1496–506
PLUTO trial
Lancet 2013; 382: 1496–506
Lancet 2013; 382: 1496–506
PLUTO trial
Complications of vesico-amniotic shunting
Lancet 2013; 382: 1496–506
Atrial Stent Placement
Atrial Stent Placement
Atrial Stent Placement
Fetal Procedures offered at TCH
• Laser ablation for TTTS and SIUGR• Bipolar coagulation for Acardiac Twin • FETO for congenital diaphragmatic hernia• Intracardiac balloon valvuloplasty/shunt
placement• Amniotic band release• Open fetal neural tube repair/Fetoscopic closure• Open fetal chest mass resection• EXIT for airway and SCT
Thanks for your Attention