1. Tracheal Agenesis Rita Fakhoury CRNA Laura Wetzel CRNA

2. Objectives Discuss case report Define Tracheal Agenesis(TA) Embryology/Anatomy Incidence of TA Clinical Signs/Symptoms Neonatal Difficult Airway Algorithm Therapeutic Options

3. Case Report 2 do male Tetralogy of Fallot, pulmonary atresia with discontinuous pulmonary arteries supplied by bilateral patent ductuses arteriosus, ASD,VSD. Imperforate or anteriorly displaced anus Absent thymus, suspect DiGeorge Syndrome

4. CT ordered Patient was intubated in NICU Reported to be “difficult airway” On arrival, NICU team requested ETT change for large leak

5. NICU team reported ventilation difficulties and desaturations with slight adjustments in ETT positioning. Decision made to exchange ETT in NICU prior to transport to CT Difficult airway cart and neonatal exchange catheter called for

6. CMAC used to visualize placement of existing airway Vocal cords visualized without ETT ETT was presumably placed in esophagus Attempt to place ETT 3.0 through vocal cords

7. Resistance met after placement through glottic opening Checked for placement of ETT through vocal cords (capnometer) No change in color of capnometer No bilateral breath sounds

8. CRAP!

9. Decision to use FOB to visualize anatomy found beyond glottic opening Appeared to be blind pouch Patient remained stable Ventilated with existing esophageal tube

11. ENT called to bedside STAT “No, I don’t need a T&A, I need ENT to bedside STAT”!!!!!!

12. Decision made for emergent DLB in OR with possible tracheostomy Transported CAREFULLY with existing ETT Mouth and nose held shut to mitigate large leak

13. DLB confirmed blind pouch after glottic opening Decision to prep for tracheostomy After incision, NO TRACHEAL RINGS FOUND!!!!!!!

14. SAY WHAT???

15. Pediatric General Surgery consulted to OR Patient placed on ECMO Plan was possible esophageal conversion After long discussion with family decision was made to withdraw support

16. Epiglottis Fused Arytenoids Cricoid Plate RLN

18. Stomach Right bronchus Left bronchus Opened Esophagus

19. Hold on to your hats! Its time to review Tracheal Agenesis

20. What is TA? Extremely rare congenital anomaly characterized by a complete absence or interruption of the trachea Ergun, Suzan et al. “Tracheal Agenesis: A Rare but Fatal Congenital Anomaly” McGill Journal of Medicine (2011) 13.1:10.

21. Embryology y Holinger LD, Rodney PL and Christopher GG. Pediatric Laryngology and Bronchoesophagology. Philadelphia PA, Lippincott-Raven Publishers, 1997.

22. Phase III -Around 28 days GA -Stage 13 - Distance between respiratory diverticulum and carina lengthen - Vascular compromise at this phase leads to… - Esophageal Atresia - TEF - Tracheal Agenesis - Tracheal Stenosis Holinger LD, Rodney PL and Christopher GG. Pediatric Laryngology and Bronchoesophagology. Philadelphia PA, Lippincott-Raven Publishers, 1997

23. Phase IV - 48 days GA - Stages 17-18 - Laryngeal cecum descends caudally - Begins to recanalize at glottic level - Failure to re-canalize can cause… -Supraglottic webs -Glottic webs -Glottic atresia Holinger LD, Rodney PL and Christopher GG. Pediatric Laryngology and Bronchoesophagology. Philadelphia PA, Lippincott-Raven Publishers, 1997

24. Anybody feel like this yet?

25. Types of TA Floyd’s Classification of Tracheal Agenesis Type I Type II Type III Dijkman, et al. “Floyd’s Classification of Tracheal Agenesis.” Cases Journal (2009) 2:7212.

26. Incidence 1:50,000 Male to female incidence 2:1 52% associated with premature delivery ~50% associated with polyhydramnios 94% associated with other congenital abnomalities De Groot-van der Mooren, et al. “Tracheal Agenesis: Approach Towards this Severe Diagnosis. Case Report and Review of Literature.” European Journal of Pediatrics (2012) 171:425-431.

27. Prenatal-Diagnosis Polyhydramnios Can be confused with TEF when an esophogeal fistula is present When CHAOS syndrome suspected in absence of fistula, prenatal MRI should be obtained-(would need exit procedure or fetal intervention). Kalache, Karim D., et al. “Prenatal Diagnosis of Laryngeal Atresia in Two Cases of Congenital High Airway Obstruction Syndrome (CHAOS).” Prenatal Diagnosis (1997) 17.6: 577-581. De Groot-van der Mooren, et al. “Tracheal Agenesis: Approach Towards this Severe Diagnosis. Case Report and Review of Literature.” European Journal of Pediatrics (2012) 171:425-431.

28. Postnatal-Diagnosis By recognition of specific clinical signs in newborn Radiographic findings Postnatal CT scan if undiagnosed prenatally Bronchoscopy De Groot-van der Mooren, et al. “Tracheal Agenesis: Approach Towards this Severe Diagnosis. Case Report and Review of Literature.” European Journal of Pediatrics (2012) 171:425-431.

29. Clinical Signs Respiratory Distress Breathing movement without appropriate air entry No Audible cry Failed endotracheal intubations Usually associated with VACTERL De Groot-van der Mooren, et al. “Tracheal Agenesis: Approach Towards this Severe Diagnosis. Case Report and Review of Literature.” European Journal of Pediatrics (2012) 171:425-431.

30. This can’t be good!

31. Neonatal Algorithm

32. Therapeutic Options ECMO to stabalize Tissue engineering trachea Esophageal conversion

33. Double Cervical Esophagostomy and Esophageal Banding Usui, N. et al. “Three-Stage Reconstruction of the Airway and Alimentary Tract in a Case of Tracheal Agenesis” The Society of Thoracic Surgeons (2010) 89:2019-22.

34. First Recipient of Tracheal derived from stem cells

35. Discussion Several intubations Audible crying? Future treatments

36. Time to Wake Up For Questions!!!!!!!!!

37. References Ergun, S., et al. “Tracheal Agenesis: a Rare but Fatal Congenital Anomaly.” McGill Journal of Medicine (June 2011) 13.1:10. Holinger LD, et al. Pediatric Laryngology and Bronchoesophagology. Philadelphia PA, Lippincott-Raven Publishers, 1997. Dijkman, et al. “Floyd’s Classification of Tracheal Agenesis.” Cases Journal (2009) 2:7212. De Groot-van der Mooren, et al. “Tracheal Agenesis: Approach Towards this Severe Diagnosis. Case Report and Review of Literature.” European Journal of Pediatrics (2012) 171:425-431. Kalache, Karim D., et al. “Prenatal Diagnosis of Laryngeal Atresia in Two Cases of Congenital High Airway Obstruction Syndrome (CHAOS).” Prenatal Diagnosis (1997) 17.6: 577-581. Morrison, R. et al. airway algorithm…. Usui, N. et al. “Three-Stage Reconstruction of the Airway and Alimentary Tract in a Case of Tracheal Agenesis” The Society of Thoracic Surgeons (2010) 89:2019-22.