Of Rings, Slings and Of Rings, Slings and many other thingsmany other things

Diego Gonzalez M.D.Diego Gonzalez M.D.Gregory Gordon M.D.Gregory Gordon M.D.

Metrohealth Medical CenterMetrohealth Medical CenterNovember 26, 2002November 26, 2002

Pre OpPre Op

7 month old7 month old 8kg8kg PMHx & PSHx: PMHx & PSHx:

Former Premie intubated x 6 weeks in CCF NICUFormer Premie intubated x 6 weeks in CCF NICU History of previous “asthma episode” admitted to History of previous “asthma episode” admitted to

hospital 2-3 weeks priorhospital 2-3 weeks prior Double aortic arch repairDouble aortic arch repair

Presenting for evaluation of stridorPresenting for evaluation of stridor

ProcedureProcedure

Mask inductionMask induction Start IV and Change to TIVAStart IV and Change to TIVA Confirm Satisfactory bag/mask controlConfirm Satisfactory bag/mask control Short acting muscle relaxantShort acting muscle relaxant Reconfirm good bag mask ventilatory controlReconfirm good bag mask ventilatory control Ventilation via bronchoscope inserted by Ventilation via bronchoscope inserted by

surgerysurgery

Physical ExamPhysical Exam

Airway unable to assessAirway unable to assess Pt was noticed to have biphasic stridorPt was noticed to have biphasic stridor Lungs with adventitious breath soundsLungs with adventitious breath sounds Regular rate and rhythmRegular rate and rhythm Neurologically was awake and alert, acting Neurologically was awake and alert, acting

like a 7 month old.like a 7 month old.

Infant AirwayInfant Airway

Differences from adultDifferences from adult Larger tongueLarger tongue Larynx is higher in the Larynx is higher in the

neckneck Epiglottis is short and Epiglottis is short and

stubby and angled over stubby and angled over the laryngeal inletthe laryngeal inlet

Vocal cords are angledVocal cords are angled Infant airway is funnel Infant airway is funnel

shaped narrowest portionshaped narrowest portion

Causes of StridorCauses of Stridor

Expiratory stridor: (also prolonged expiration)Expiratory stridor: (also prolonged expiration) BronchiolitisBronchiolitis AsthmaAsthma Intrathoracic foreign bodyIntrathoracic foreign body

Inspiratory stridor:Inspiratory stridor: EpiglotitisEpiglotitis LTBLTB Laryngeal foreign bodyLaryngeal foreign body

Vascular ringsVascular rings

Definition: when any Definition: when any anomalous anomalous configuration or vessel configuration or vessel surrounds trachea or surrounds trachea or esophagus forming a esophagus forming a ring around them, they ring around them, they can be complete or can be complete or incomplete incomplete

Vascular ringsVascular rings

Frequency:Frequency: < 1% of congenital heart defects< 1% of congenital heart defects Equal in both sexesEqual in both sexes

2 most common are (85-90% of cases)2 most common are (85-90% of cases) Double aortic archDouble aortic arch Right aortic arch with left ligamentum aretriosumRight aortic arch with left ligamentum aretriosum

Other anomalies make like left pulmonary Other anomalies make like left pulmonary artery sling make 10%, incomplete ie slingartery sling make 10%, incomplete ie sling

Etiology of rings and slingsEtiology of rings and slings

Non-regression or incomplete regression of any of the Non-regression or incomplete regression of any of the 6 embryonic branchial arches6 embryonic branchial arches

Normally what happens:Normally what happens: 1 and 2 arches irrigate the face1 and 2 arches irrigate the face 3 arch forms carotids3 arch forms carotids Dorsal aorta in 3 and 4 involutesDorsal aorta in 3 and 4 involutes 4 form aortic arch4 form aortic arch 5 arch involutes5 arch involutes 6 becomes the proximal right pulmonary artery6 becomes the proximal right pulmonary artery 7 distal right subclavian and left subclavian7 distal right subclavian and left subclavian

Intra Operative CourseIntra Operative Course

Easy mask inductionEasy mask induction Easy to ventilateEasy to ventilate Difficult time getting IV in (probably from Difficult time getting IV in (probably from

previous surgeries)previous surgeries) Once IV started changed to TIVA pt paralyzed Once IV started changed to TIVA pt paralyzed

with Mivacronwith Mivacron Bag mask ventilatory control confirmedBag mask ventilatory control confirmed Surgeon unable to introduce bronchoscopeSurgeon unable to introduce bronchoscope

Intra Op 2Intra Op 2 Pt desaturated into low 90’sPt desaturated into low 90’s Try to reventilate by mask.Try to reventilate by mask. Unable to maskUnable to mask Sats droppingSats dropping Repositioned and remaskedRepositioned and remasked Sats droppingSats dropping Some air entry to stomach and lungsSome air entry to stomach and lungs Sats dropping, cyanosis markedly increasedSats dropping, cyanosis markedly increased DL glottis visualized unable to inserted styletted #3 OETTDL glottis visualized unable to inserted styletted #3 OETT Unable to intubate, sats in the low 40sUnable to intubate, sats in the low 40s Slash trach performed by surgeonSlash trach performed by surgeon

Intra op 3Intra op 3

Unable to pass Shiley, Number 3ETT tube Unable to pass Shiley, Number 3ETT tube passed and could not ventilate adequately.passed and could not ventilate adequately.

PALS started Atropine and Epi given for PALS started Atropine and Epi given for bradycardia. bradycardia.

Now WHAT????Now WHAT????

Intra OP 4Intra OP 4

Shiley replaced with Shiley 3# cuffedShiley replaced with Shiley 3# cuffed Ventilation possible via right lungVentilation possible via right lung Tube pulled back able to ventilate both lungsTube pulled back able to ventilate both lungs Shiley # 3 replaced with Shiley #4 adequate Shiley # 3 replaced with Shiley #4 adequate

ventilation of both lungsventilation of both lungs

Follow UpFollow Up

Followed up pt that same day.Followed up pt that same day. H/D stable purposeful neurologically.H/D stable purposeful neurologically. Pt consequently followed days 2,3,4,5 and 7Pt consequently followed days 2,3,4,5 and 7

Round 2Round 2

7 days later pt taken for rigid bronchoscope, 7 days later pt taken for rigid bronchoscope, tracheoscopy possible flex bronchoscopytracheoscopy possible flex bronchoscopy

Able to pass small rigid suction tubeAble to pass small rigid suction tube Pt noticed to have severe sub-glottic stenosis.Pt noticed to have severe sub-glottic stenosis. Able to pass OETT 2.5 styleted with snug fit. Able to pass OETT 2.5 styleted with snug fit. Flex scope through trachea which showed Flex scope through trachea which showed

severe stenosis at the carina level.severe stenosis at the carina level. Pt transferred to CCF for further evaluation Pt transferred to CCF for further evaluation

possible surgery.possible surgery.

Round 3?Round 3?

Pt transferred back to Metro Pt transferred back to Metro Plan?Plan? Stent?Stent? Outgrow stenosis?Outgrow stenosis? Surgical repair?Surgical repair?

Sub Glottic StenosisSub Glottic Stenosis

Congenital malformation of the cricoid Congenital malformation of the cricoid cartilagecartilage

Acquired- PathophysiologyAcquired- Pathophysiology Mucosal edemaMucosal edema HyperemiaHyperemia Pressure necrosis of mucosaPressure necrosis of mucosa FibrosisFibrosis

Risk factors for SGSRisk factors for SGS

ETTETT Duration of intubationDuration of intubation Repeated intubationRepeated intubation GERDGERD Factors that affect healingFactors that affect healing

Systemic illnessSystemic illness MalnutrionMalnutrion AnemiaAnemia HypoxiaHypoxia

SGSSGS

Incidence 1-2% of graduated NICU patientsIncidence 1-2% of graduated NICU patients Morbi-mortalityMorbi-mortality

Difficulty breathingDifficulty breathing Exercise intoleranceExercise intolerance DeathDeath

ClinicallyClinically

Inspiratory stridorInspiratory stridor Supraglottic lesionSupraglottic lesion

Expiratory stridorExpiratory stridor Tracheal, bronchial, pulmonary lesionTracheal, bronchial, pulmonary lesion

In SGS that is moderate to severe they may In SGS that is moderate to severe they may have biphasic stridorhave biphasic stridor

StagingStaging

Percentage is evaluated Percentage is evaluated by using ETT of by using ETT of different sizes the different sizes the largest ETT that can be largest ETT that can be place with 20cm place with 20cm pressure is evaluated pressure is evaluated against a scale against a scale developed by Myers and developed by Myers and CottonCotton

StagingStaging

Grade 1- Obstruction of Grade 1- Obstruction of 0-50 % of the lumen0-50 % of the lumen

Grade 2- Obstruction of Grade 2- Obstruction of 51-70% of the lumen51-70% of the lumen

Grade 3- Obstruction of Grade 3- Obstruction of 71-99% of the lumen71-99% of the lumen

Grade 4- Obstruction of Grade 4- Obstruction of 100% (no visible 100% (no visible lumen)lumen)

TreatmentTreatment

No medical treatmentNo medical treatment Surgery indicated with SGS + symptoms Surgery indicated with SGS + symptoms

presentpresent

Residents hard at work!!Residents hard at work!!