Transcript Tracheal Agenesis - umichanesthesiaconference.com

Tracheal Agenesis
Rita Fakhoury CRNA
Laura Wetzel CRNA
Objectives
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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
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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
Fused Arytenoids
Epiglottis
RLN
RLN
Cricoid Plate
RLN
RLN
Opened Esophagus
Left bronchus
Right bronchus
Stomach
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
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- 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 TA
Floyd’s Classification of
Tracheal Agenesis
Type I
Type II
Dijkman, et al. “Floyd’s Classification of Tracheal Agenesis.” Cases Journal (2009) 2:7212
Type III
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Incidence
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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.