Tracheal Agenesis Rita Fakhoury CRNA Laura Wetzel CRNA

Tracheal Agenesis

Rita Fakhoury CRNALaura Wetzel CRNA

Objectives

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

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

• CT ordered • Patient was intubated in NICU• Reported to be “difficult airway”• On arrival, NICU team requested ETT

change for large leak

• 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

• 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

• 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

CRAP!

• Decision to use FOB to visualize anatomy found beyond glottic opening

• Appeared to be blind pouch• Patient remained stable • Ventilated with existing esophageal tube

ENT called to bedside STAT

“No, I don’t need a T&A, I need ENT to bedside

STAT”!!!!!!

• Decision made for emergent DLB in OR with possible tracheostomy

• Transported CAREFULLY with existing ETT

• Mouth and nose held shut to mitigate large leak

• DLB confirmed blind pouch after glottic opening

• Decision to prep for tracheostomy• After incision, NO TRACHEAL RINGS

FOUND!!!!!!!

SAY WHAT???

• 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

Epiglottis

Fused Arytenoids

Cricoid Plate

RLN

RLN

RLN

RLN

Stomach

Right bronchus

Left bronchus

Opened Esophagus

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

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.

Embryology

y

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

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

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

Anybody feel like this yet?

Types of TAFloyd’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.

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.

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.

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.

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.

This can’t be good!

Neonatal Algorithm

Therapeutic Options

• ECMO to stabalize• Tissue engineering trachea• Esophageal conversion

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.

First Recipient of Tracheal derived from stem cells

Discussion

• Several intubations• Audible crying?• Future treatments

Time to Wake Up For Questions!!!!!!!!!

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.