Exit Procedures

An Anaesthetic perspective

Dr Peter Gibson Senior Staff Anaesthetist Children’s Hospital at Westmead and Westmead Hospital

“We play as a team and we do it my way!”

Richie Benaud, The Twelfth man.

A Brief History of Exit

• Harrison MR, Anderson J, Rosen MA, et al. Fetal surgery in the primate, I: Anesthetic, surgical and tocolytic management to maximize fetal-neonatal survival. J Pediatr Surg 1982 17:115-23.

• Norris MC, Joseph J, Leighton BL. Anesthesia for perinatal surgery. Am J Perinatol1989;639-40. • Kelly MF, Greco R, Berenholz L, Wolfson P, Rizzo KA, Zwillenberg DA. Approach for oxygenation of

the newborn with airway obstruction due to a cervical mass. Ann Otol Rhinol Laryngol. 1990;99:179-182.

• Schulman SR, Jones BR, Slotnick N, Schwa MZ. Fetal Tracheal Intubation with Intact Utero-placental Circulation. Anesth Analg 1993;76197-9

• Stocks RMS, Egerman RS, Woodsonc GE Bower CM, Thompson JW , Wiet GJ. Airway Management of Neonates With Antenatally Detected Head and Neck Anomalies. Arch Otolaryngol Head Neck Surg.1997;123:641-645

• Michaliska GB, Beale JF, Graf JL et al. Operating on placental support: the ex-utero intrapartum treatment procedure. Journal of Pediatric Surgery 1997; 32: 227–231.

• The Ex utero intrapartum treatment procedure: Looking back at the Exit. Hirose S, Farmer DL, Lee H et al. Journal of Pediatric Surgery, Vol 39, No 3, 2004: pp 375-380

• Morris LM, Lim FY, Elluru RG et al. Severe micrognathia: indications for EXIT-to-Airway. Fetal Diagn Ther. 26(3):162-6, 2009

• Morris LM, Lim FY, Cromblehome TM, Ex Utero Intrapartum Treatment Procedure: A Peripartum Management Strategy in Particularly Challenging Cases . J Pediatr 2009;154:126-31

Indications for Exit procedure

• Masses of head and neck

• Craniofacial anomalies with mandibular hypoplasia – With other evidence of aerodigestive obstruction

such as polyhydramnios, tracheal compression

• Laryngeal/tracheal atresia, CHAOS • Lung masses, mediastinal masses, pulmonary

outflow obstruction, HPLH, Diaphragmatic hernia. Exit to ECMO

Amniotic fluid flux

Gilbert WM, Moore TR, Brace RA: Amniotic fluid volume dynamics. Fetal Med Rev 3:89, 1991.

Pre-procedural investigations/consultation

• Prenatal detection of foetal malformation • Further Imaging/?Amniocentesis • Multidisciplinary consultations • Neonatology/paediatrician, Geneticist, Surgeons,

ENT, Plastic and reconstructive, Anaesthetists. • Formulate plan with family

– Not Proceed with pregnancy – Proceed with pregnancy and schedule EXIT – Proceed with pregnancy and not have extraordinary

resuscitative efforts at birth

Case presentation

• Mrs TP, G5 P2, aged 49 years referred to Westmead feto-maternal unit at 28 weeks gestation with fetal micrognathia and polyhydramnios on US.

• Co-morbidities: Hypertension, Cutaneous SLE, Thyrotoxicosis, diet controlled gestational diabetes

• Medications: prednisolone, hydoxychloroquine, oxprenolol, carbimazole

Mandible / airway –larynx & trachea foetal MR T2 weighted

Assembling the team

• Obstetrician

• Neonatologist

• Anaesthetists

• Paediatric surgeons, ENT, General, Plastic

• Nursing staff

Obstetric management

• Plan delivery day!

• Amnio-reduction day prior

• Ultrasound to check fetal position and placental position.

• Lower segment hysterotomy ? Classical for breech or placenta praevia.

• Haemostatic clips during hysterotomy

• Delivery of head, shoulder, arm.

• Warm saline infusion to maintain uterine volume

Maternal Anaesthesia Goals

• Relax uterus, prevent placental separation

• Maintain utero-placental perfusion

• Anaesthetise/sedate/immobilise foetus

• Rapid return of uterine tone after delivery

• Minimise blood loss, transfusion requirements

Monitoring the foetus

• Doppler US

• Delivery of one arm and application of pulse oximeter

• IV access for fetus

• ?cardiac ultrasound

Anaesthetising the mother General Anaesthesia

• High dose volatile anaesthetic

• GTN

• Remifentanil

• Arterial monitoring

• Vasopressors

• Cell saver

Anaesthetising the mother Regional Anaesthesia

• CSE

• GTN

• Remifentanil Fink RJ, Allen TK, Habib AS. British Journal of Anaesthesia 106 (6): 851–5 (2011)

Volatile and Foetal cardiac dysfunction

• Supplementing desflurane with intravenous anesthesia reduces fetal cardiac dysfunction during open fetal surgery. Pediatric Anesthesia, 20: 748–756. doi: 10.1111/j.1460-9592.2010.03350.x

BOAT, A., MAHMOUD, M., MICHELFELDER, E. C., LIN, E., NGAMPRASERTWONG, P., SCHNELL, B., KURTH, C. D., CROMBLEHOLME, T. M. and SADHASIVAM, S. (2010)

Preparing the room

Photo of theatre crowded with multiple teams.

Establishing an airway

• Micrognathia – Confirm very difficult/impossible intubation – Tracheostomy

• Head and neck masses – Laryngoscopy/video-laryngoscopy/rigid bronchoscopy – Tracheostomy – Tumour resection with tracheostomy – Use of ultrasound to identify the trachea

• Fibreoptic scope to confirm tube position • Over to neonatology

Series of 3 slides showing intubation and tracheostomy on placental support. Followed by 2 slides showing babies with micrognathia and large neck mass with tracheostomies in situ.

Neonatal resuscitation

• Full range of neonatal resuscitation equipment required

• Resuscitative sedative and resus drugs and fluids according to estimated babies weight

• Umbilical catheters

• Chest drains

3D CT

Slide of Mrs TP nursing baby with tracheostomy in situ.

Maternal outcome

• Increased blood loss

• Increased wound infection rate

• No mortality reported

• No increased hospital stay.

Noah MM, Norton ME, Sandberg P, et al: Short-term maternal

outcomes that are associated with the EXIT procedure, as compared with cesarean delivery. Am J Obstet Gynecol 186:773-777, 2002

Case Mass Micrognathia Other Hydramnios Airway

1 Teratoma ++ tracheostomy

2 Teratoma/Aicardi synd

++ fibreoptic intubation

3 Cystic hygroma resolved Not intubated

4 Epulis Easy intubation

5 Goitre Easy intubation

6 CCAM Not intubated

7 Treacher Collins ++ Difficult, rigid bronch,early tracheostomy

8 Cystic hygroma Easy intubation

9 Cystic hygroma Easy intubation

10 Cystic hygroma Easy mask ventilation, Mod difficult intubation

11 Teratoma ++ Tracheostomy

12 Severe/multiple anomalies

++ Tracheostomy

13 Severe with microstomia ++ Tracheostomy

Micrognathia and EXIT

• 3 cases in Westmead experience

– Treacher Collins

– Multiple Congenital anomalies, died in neonatal period after life sustaining measures withdrawn

– Isolated micrognathia, tracheostomy, tube feeding, alert and growing. Awaiting reconstructive surgery.

Fig. 1 Fetal MRI in the coronal (A) and sagittal (B) planes of a patient with fetal CHAOS (case no. 12). Note the atretic laryngeal segment (white arrows). This lesion measured 7 mm and was deemed a poor candidate for fetal tracheoplasty.

Payam Saadai , Eric B. Jelin , Amar Nijagal , Samuel C. Schecter , Shinjiro Hirose , Tippi C. MacKenzie , Larry R. Long-term outcomes after fetal therapy for congenital high airway obstructive syndrome. Journal of Pediatric Surgery Volume 47, Issue 6 2012 1095 - 1100 ..

http://dx.doi.org/10.1016/j.jpedsurg.2012.03.015

CHAOS and EXIT

CHAOS and EXIT

• Roybal JL, Liechty KW, Hedrick HL et al. Predicting the severity of congenital high airway obstruction syndrome. Journal of Pediatric Surgery (2010) 45, 1633–1639.

• Saadai P, Jelin EB, Nijagal A. Long-term outcomes after fetal therapy for congenital high airway obstructive syndrome. Journal of Pediatric Surgery (2012) 47, 1095–1100.

• “The overall prognosis for pregnancies complicated by CHAOS remains poor, however, and the long-term medical and surgical challenges for survivors remain numerous even after lifesaving fetal intervention” and “these are some of the sickest kids you are ever going to have to take care of, and their long-term outcome is still significantly challenged even after you are able to get them to survive”.

EXIT is only the beginning Emily’s story

• Photo of Emily, aged 6, child with Treacher - Collins syndrome and her mother

Acknowledgements

• Parents who gave permission for photography for educational purposes.

• Agnes Molnar previous Fellow in Paediatric Anaesthesia and Michael Cooper who collated records of patients.

• Jane Brown and Anaesthetists from Westmead who have looked after Maternal anaesthesia.

• Jane McDonald, Mark Lovell and other Paediatric anaesthetists • Neil Athyde Obstetrician from Westmead and Chris Dalton and

Cathy Birman ENT surgeons who have help work out the team way of doing things.

• Tony Peduto and Kristina Prelog radiologists • All the nursing staff in theatres at Westmead and our Anaesthetic

nurses from the Children’s hospital who have gone out of there way have the equipment and personnel we need to make things happen.