Intra uterine fetal surgery

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Intra Uterine Fetal SurgeryDr.Sameer Dikshit

Dr.Sameer Dikshit MD,DGO,FCPS,FICOG

Member, Genetic & Fetal Medicine Committee

Past Secretary, Palghar Ob Gy Society

Trained at King’s College, London under Prof. Nicolaides

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Hon Sonologist Wadia Hospital, Mumbai

Fetal Medicine Consultant BSES MG Global Hospital, Mumbai

Boisar Fetal Medicine Centre

Irla Nursing Home, Mumbai

Sanket Sonography, Mumbai

The allure of

Fetal Surgery is

the possibility of

interrupting the

in utero

progression of an

otherwise

treatable

condition

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Fetal Surgery is…….. Indicated in conditions which

interfere with the normal development of the fetus

Which when corrected will allow normal development of the fetus

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It is contraindicated in conditions that are incompatible with life Severe affliction Other associated life threatening

abnormalities Chromosomal & Genetic conditions

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Father of Fetal Surgery Sir A.W.Liley in 1965 Intra Uterine Transfusion for Hydrops

due to Rh incompatibility Dr.Michael Harrison in 1982 First open fetal surgery for obstructive

uropathy

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Types of Fetal Surgery Open Surgery

FETENDO (Fetal Endoscopic Surgery)

FIGS (Fetal Image Guided Surgery)

EXIT (Ex-Utero Intrapartum Treatment Procedure)

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FIGS (Fetal Image Guided Surgery)

Ultrasound image guided procedure

Needle or a Trocar-Canula -Shunt introduced

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Least invasive

Least risk of amniotic fluid leak

Least risk of PT labour

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Examples Diagnostic

Chorion Villus Sampling

Amniocentesis

Cordocentesis

Fetal skin Biopsy

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Therapeutic RFA (Radio Frequency Ablation) of

anomalous Twins

Cord cauterization in Twins

Vesical / Pleural Shunts

Balloon Dilatation of Aortic Stenosis

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FETENDO (Fetal Endoscopic Surgery)

Fetoscopic access to the Fetus

Real time visualisation of the Fetus

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The fetal visualisation is a combination of endoscopic and sonographic on two different screens

It is called FETENDO because the movements are like the children’s video game NINTENDO

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Less invasive

Less risk of amniotic fluid leak

Less risk of PT labour

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Examples CDH (Congenital Diaphragmatic Hernia)-

Balloon Occlusion of trachea

TTTS (Twin to Twin Transfusion Syndrome)- Laser coagulation of vessels

Cord ligation in cases of acardiac Twins

Amniotic bands division

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Open Surgery Mother is anaesthetised

Uterus is opened similar to LSCS

Special stapling device to prevent bleeding & amniotic fluid leak

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Intra operative sonography to locate the placenta and to determine the surface anatomy of the fetus

Fetal part is exteriorized

Fetal Surgery

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Examples CCAM (Congenital Cystic Adenomatoid

Malformation of Lung)- Lobectomy

SCT (Sacro-coccygeal Teratoma)- Resection

MMC (Meningo Myelocoele)- Repair

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EXIT (Ex-utero Intrapartum treatment procedure)

It is the intervention that occurs at the time of delivery

It is primarily used in cases where baby’s airway requires surgical intervention

Provide the baby with patent airway that can provide O2 to the lungs after separation of placenta

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It starts as a routine LSCS but under GA

Head of the baby is delivered, but the placenta is in situ

The baby gets oxygen from placenta via umbilical cord

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Bronchoscopy of the fetal airway

Endotracheal intubation attempted

If unsuccessful then tracheostomy is done

O2 delivery to lungs confirmed

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Cord is cut

Baby is delivered

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Examples CHAOS (Congenital High Airway

Obstruction Syndrome)

Removal of balloon after CDH

Pulmonary Sequestration

CCAM (Congenital Cystic Adenomatoid Malformation)

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Challenges before the field of fetal surgery….. Ethical dilemma

Maternal & Fetal anaesthesia

Risks both to mother and fetus

Post surgical tocolysis

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Ethical Dilemma Not all procedures are performed

regularly

The results are not guaranteed

Risks to mother and fetus

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Should a procedure which is not guaranteed to produce results BE PERFORMED on the insistence of mother?

Should a procedure which is guaranteed to produce results NOT BE NOT BE PERFORMED PERFORMED on refusal of mother?

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Research in Fetal Surgery is ethically controversial as it poses a risk to both the fetus and the mother

Surgical Animal models do not always replicate in human beings

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Maternal Risks Tocolytic therapy can cause pulmonary

edema

Subsequent delivery by LSCS

Intra op blood loss

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Amniotic fluid leak

Wound infection

Intra uterine infection

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“Maternal Mirror Syndrome” in cases of fetal Hydrops

Chorioamnionic membrane separation

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Deep anaesthesia is required to provide with adequate uterine relaxation for fetal manipulation and to prevent PT labour

This depth can cause fetal and maternal myocardial depression also can affect placental perfusion

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Fetal Risks Prematurity

Intra Uterine Infection

Fetal vascular embolic events Intestinal atresia Renal agenesis

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Premature closure of Ductus Arteriosus

CNS injuries due to maternal hypoxia or fetal circulatory disturbance

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Fetal response to maternal anaesthesia Fetal organs system is immature

Fetal Cardiac Output is sensitive to heart rate changes

Fetus has high vagal tone and hence responds to stress with precipitous bradycardia

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Fetal circulating volume is low, hence little intra-operative bleeding can cause hypovolemia

Maternal anesthesia depress myocardium, circulation

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Fetus tends to lose heat much easily from the exposed skin resulting in hypothermia

Immature coagulation system predispose the fetus to bleeding and difficulty in hemostasis

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Maternal anesthesia reduces placental blood flow, this reduces the amount of O2 delivered to the fetus

Normal Fetal oxygen saturation is 60-70% and the aim is to maintain it above 40%

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Intra-operative fetal distress is manifested by bradycardia, decreased fetal oxygen saturation and reduced stroke output

During prolonged surgery, fetus may be transfused Oneg blood

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Top up fetal anaesthesia may be needed to augment the maternal anaesthesia

When fetus is hydropic, it is very sensitive to fluctuating maternal hemodynamics

Maternal hyperventilation is avoided as maternal hypocapnia causes fetal placental vasoconstriction and fetal hypoxia

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Fetal Monitoring during surgery In case of open surgery

Fetus monitored by echocardiography and miniature pulse oxymeter

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Post op care High risk of Pre term labour

Mag Sulph is the tocolytic of choice and maintained for 2-3 days

Maternal analgesia is important because maternal pain can cause PT labour and Fetal distress

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Epidural analgesia for 24-48 hours is recommended

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Protocol for open Fetal Surgery Assessment of the mother for fitness for

anaesthesia

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Assessment of the fetus Detailed USG to r/o other malformations

3D and 4D examination

Detailed examination of affected organ system

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Detailed Fetal Echocardiography

Amniocentesis

Localisation of placenta

Fetal MRI

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Maternal blood cross matched

Mother given GA with intubation as the uterus has to be relaxed to allow manipulation of the uterus

Indomethacin rectal suppository

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O neg blood for fetus kept ready

Abdomen opened as in LSCS

Intra operative USG to localise placenta and to assess the surface anatomy of the fetus

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Incision to be taken close to the area of interest

Uterine Stapler to seal amnion and reduce blood loss

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Dr.Michael Harrison University of

California, San Francisco

Father of open fetal surgery

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Uterine Stapler

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Fetus is given Inj Atropine 0.02 mg/kg Inj Epinephrine 1 μg/kg Inj Vecuronium 0.2 mg/kg Inj Fentanyl 1-2 μg/kg

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The fetus is monitored with Fetal

Echocardiography Pulse Oxymetry PO2 from Cord

Blood Fetal Hb from Cord

Blood

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Infusion of 50 ml aliquots of O neg Blood

Infusion of warmed Ringer Lactate to replace amniotic fluid

Fetal Surgery is performed

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At the time of closure, IV MagSulph 6g over 20 minutes

3G/hr infusion post operative

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Maternal Anaesthesia Regional Anaesthesia-Lumbar

Epidural Deep GA-(Sodium Pentothal + Scoline)

+ (Isoflurane + Fentanyl+O2 + Vecuronium)

GA with N2O- (Sodium Pentothal + Scoline) + (Isoflurane + N2O + Vecuronium)

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Fetal Pain…. “Pain” by definitive is a subjective

phenomenon

Hence it is not possible to assess “Fetal Pain” directly

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It is assessed indirectly by the ability of the fetus to mount a stress response to a noxious stimulus

Increased fetal cortisol, beta-endorphins and “central sparing” hemodynamic changes

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Fetal administration of a narcotic inhibits cortisol and beta-endorphin release but does not inhibit “central sparing” hemodynamic changes

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Fetal pain has been said to contribute to exaggerated pain response in 8 week old infants

It is also said to stimulate preterm labour

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Future possibilities Deliver stem cells or DNA to treat sickle

cell anemia or other genetic conditions

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Inherited Genetic Diseases Treatable with Stem Cells

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Inherited Genetic Diseases Treatable with Stem Cells Haemoglobinopathies

Immunodeficiency diseases

Mucopolysaccharidoses

Mucoliposes

Diamond Blackfan Syndrome

Fanconi anemia

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Prevention of graft v/s host disease

Prevents further damage to the fetus

Intra-amniotic or Intra-umbilical vein

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The key in fetal surgery is not when to operate, but to know when NOT to operate!!!

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Sacrococcygeal Teratoma (SCT) Open Surgery for excision of the

Teratoma

The tumours are benign

But can caused Fetal Hydrops due to vascular shunts

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Cystic SCTs

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Cystic SCTs do not have vascular shunts

Hence the fetus does not land up with Hydrops

Hence, there is NO INDICATION for Intra Uterine Surgery in these cases

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Solid SCT

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Solid SCTs have vascular shunts

High risk of Hydrops and fetal death

Hence Intra Uterine Surgery is indicated

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Congenital Diaphragmatic Hernia (CDH) The key to the successful management

is to have a fetus with competent lungs after birth

If the lungs are collapsed, then post natal surgery fails

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The status of the lungs can be predicted by- Presence of liver in the thorax (presence

of liver more severe disease)

LHR (Lung to Head ratio) less than 1.0

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These fetuses need intra partum intervention for postpartum surgery to succeed

FETENDO with temporary tracheal occlusion

EXIT procedure to remove the balloon before birth

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Why open intra partum surgery fails????

Reduction of the liver into abdomen kinks the Ductus Venosus

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Congenital Cystic Adenomatoid Malformation of the lungs (CCAM) Most fetuses do well in utero

Indications for intra uterine surgery are:- Progressive increase in the size Mediastinal shift Hydrops Polyhydramnios

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Thoraco amniotic shunt

EXIT procedure for securing airway

Open Fetal Surgical Resection

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CCAM Prenatal Steroid Trial University of California, San Francisco

Cases with large CCAM who would otherwise need intra uterine surgery

2 doses of Betamethasone 12 mg IM, 24 hours apart

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Fetal Surgery is a roller coaster for the fetus….

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Fetal Surgery is a roller coaster ride for the fetusIt is our endeavor to ensure that fetus comes through it smiling and unharmed….

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Thank you

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