Intra uterine fetal surgery

Intra Uterine Fetal Surgery Dr.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
AICOG 2011 [email_address]
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 AICOG 2011 [email_address]

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

It is contraindicated in conditions that are incompatible with life
Severe affliction
Other associated life threatening abnormalities
Chromosomal & Genetic conditions

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

Types of Fetal Surgery
Open Surgery
FETENDO (Fetal Endoscopic Surgery)
FIGS (Fetal Image Guided Surgery)
EXIT (Ex-Utero Intrapartum Treatment Procedure)

FIGS (Fetal Image Guided Surgery)
Ultrasound image guided procedure
Needle or a Trocar-Canula -Shunt introduced

Least invasive
Least risk of amniotic fluid leak
Least risk of PT labour

Examples
Diagnostic
Chorion Villus Sampling
Amniocentesis
Cordocentesis
Fetal skin Biopsy

Therapeutic
RFA (Radio Frequency Ablation) of anomalous Twins
Cord cauterization in Twins
Vesical / Pleural Shunts
Balloon Dilatation of Aortic Stenosis

FETENDO (Fetal Endoscopic Surgery)
Fetoscopic access to the Fetus
Real time visualisation of the Fetus

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

Less invasive
Less risk of amniotic fluid leak
Less risk of PT labour

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

Open Surgery
Mother is anaesthetised
Uterus is opened similar to LSCS
Special stapling device to prevent bleeding & amniotic fluid leak

Intra operative sonography to locate the placenta and to determine the surface anatomy of the fetus
Fetal part is exteriorized
Fetal Surgery

Examples
CCAM (Congenital Cystic Adenomatoid Malformation of Lung)- Lobectomy
SCT (Sacro-coccygeal Teratoma)- Resection
MMC (Meningo Myelocoele)- Repair

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 O 2 to the lungs after separation of placenta

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

Bronchoscopy of the fetal airway
Endotracheal intubation attempted
If unsuccessful then tracheostomy is done
O 2 delivery to lungs confirmed

Cord is cut
Baby is delivered

Examples
CHAOS (Congenital High Airway Obstruction Syndrome)
Removal of balloon after CDH
Pulmonary Sequestration
CCAM (Congenital Cystic Adenomatoid Malformation)

Challenges before the field of fetal surgery…..
Ethical dilemma
Maternal & Fetal anaesthesia
Risks both to mother and fetus
Post surgical tocolysis

Ethical Dilemma
Not all procedures are performed regularly
The results are not guaranteed
Risks to mother and fetus

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 PERFORMED on refusal of mother?

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

Maternal Risks
Tocolytic therapy can cause pulmonary edema
Subsequent delivery by LSCS
Intra op blood loss

Amniotic fluid leak
Wound infection
Intra uterine infection

“ Maternal Mirror Syndrome” in cases of fetal Hydrops
Chorioamnionic membrane separation

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

Fetal Risks
Prematurity
Intra Uterine Infection
Fetal vascular embolic events
Intestinal atresia
Renal agenesis

Premature closure of Ductus Arteriosus
CNS injuries due to maternal hypoxia or fetal circulatory disturbance

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

Fetal circulating volume is low, hence little intra-operative bleeding can cause hypovolemia
Maternal anesthesia depress myocardium, circulation

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

Maternal anesthesia reduces placental blood flow, this reduces the amount of O 2 delivered to the fetus
Normal Fetal oxygen saturation is 60-70% and the aim is to maintain it above 40%

Intra-operative fetal distress is manifested by bradycardia, decreased fetal oxygen saturation and reduced stroke output
During prolonged surgery, fetus may be transfused O neg blood

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

Fetal Monitoring during surgery
In case of open surgery
Fetus monitored by echocardiography and miniature pulse oxymeter

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

Epidural analgesia for 24-48 hours is recommended

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

Assessment of the fetus
Detailed USG to r/o other malformations
3D and 4D examination
Detailed examination of affected organ system

Detailed Fetal Echocardiography
Amniocentesis
Localisation of placenta
Fetal MRI

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

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

Incision to be taken close to the area of interest
Uterine Stapler to seal amnion and reduce blood loss

Dr.Michael Harrison
University of California, San Francisco
Father of open fetal surgery

Uterine Stapler AICOG 2011 [email_address]

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

The fetus is monitored with
Fetal Echocardiography
Pulse Oxymetry
PO 2 from Cord Blood
Fetal Hb from Cord Blood

Infusion of 50 ml aliquots of O neg Blood
Infusion of warmed Ringer Lactate to replace amniotic fluid
Fetal Surgery is performed

At the time of closure, IV MagSulph 6g over 20 minutes
3G/hr infusion post operative

Maternal Anaesthesia
Regional Anaesthesia -Lumbar Epidural
Deep GA -(Sodium Pentothal + Scoline) + (Isoflurane + Fentanyl+O 2 + Vecuronium)
GA with N 2 O - (Sodium Pentothal + Scoline) + (Isoflurane + N 2 O + Vecuronium)

Fetal Pain….
“ Pain” by definitive is a subjective phenomenon
Hence it is not possible to assess “Fetal Pain” directly

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

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

Fetal pain has been said to contribute to exaggerated pain response in 8 week old infants
It is also said to stimulate preterm labour

Future possibilities
Deliver stem cells or DNA to treat sickle cell anemia or other genetic conditions

Inherited Genetic Diseases Treatable with Stem Cells AICOG 2011 [email_address]

Inherited Genetic Diseases Treatable with Stem Cells
Haemoglobinopathies
Immunodeficiency diseases
Mucopolysaccharidoses
Mucoliposes
Diamond Blackfan Syndrome
Fanconi anemia

Prevention of graft v/s host disease
Prevents further damage to the fetus
Intra-amniotic or Intra-umbilical vein

The key in fetal surgery is not when to operate, but to know when NOT to operate!!! AICOG 2011 [email_address]

Sacrococcygeal Teratoma (SCT)
Open Surgery for excision of the Teratoma
The tumours are benign
But can caused Fetal Hydrops due to vascular shunts

Cystic SCTs AICOG 2011 [email_address]

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

Solid SCT AICOG 2011 [email_address]

Solid SCTs have vascular shunts
High risk of Hydrops and fetal death
Hence Intra Uterine Surgery is indicated

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

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

These fetuses need intra partum intervention for postpartum surgery to succeed
FETENDO with temporary tracheal occlusion
EXIT procedure to remove the balloon before birth

Why open intra partum surgery fails????
Reduction of the liver into abdomen kinks the Ductus Venosus

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

Thoraco amniotic shunt
EXIT procedure for securing airway
Open Fetal Surgical Resection

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

Fetal Surgery is a roller coaster for the fetus…. AICOG 2011 [email_address]

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

Thank you AICOG 2011 [email_address]

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Intra uterine fetal surgery

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