Of Rings, Slings and many other things Diego Gonzalez M.D. Gregory Gordon M.D. Metrohealth Medical Center November 26, 2002

Pre Op 7 month old 7 month old 8kg 8kg PMHx & PSHx: PMHx & PSHx: Former Premie intubated x 6 weeks in CCF NICU Former Premie intubated x 6 weeks in CCF NICU History of previous “asthma episode” admitted to hospital 2-3 weeks prior History of previous “asthma episode” admitted to hospital 2-3 weeks prior Double aortic arch repair Double aortic arch repair Presenting for evaluation of stridor Presenting for evaluation of stridor

Procedure Mask induction Start IV and Change to TIVA Confirm Satisfactory bag/mask control Short acting muscle relaxant Reconfirm good bag mask ventilatory control Ventilation via bronchoscope inserted by surgery

Physical Exam Airway unable to assess Airway unable to assess Pt was noticed to have biphasic stridor Pt was noticed to have biphasic stridor Lungs with adventitious breath sounds Lungs with adventitious breath sounds Regular rate and rhythm Regular rate and rhythm Neurologically was awake and alert, acting like a 7 month old. Neurologically was awake and alert, acting like a 7 month old.

Infant Airway Differences from adult Larger tongue Larynx is higher in the neck Epiglottis is short and stubby and angled over the laryngeal inlet Vocal cords are angled Infant airway is funnel shaped narrowest portion

Causes of Stridor Expiratory stridor: (also prolonged expiration) Expiratory stridor: (also prolonged expiration) Bronchiolitis Bronchiolitis Asthma Asthma Intrathoracic foreign body Intrathoracic foreign body Inspiratory stridor: Inspiratory stridor: Epiglotitis Epiglotitis LTB LTB Laryngeal foreign body Laryngeal foreign body

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

Vascular rings Frequency: Frequency: < 1% of congenital heart defects < 1% of congenital heart defects Equal in both sexes Equal in both sexes 2 most common are (85-90% of cases) 2 most common are (85-90% of cases) Double aortic arch Double aortic arch Right aortic arch with left ligamentum aretriosum Right aortic arch with left ligamentum aretriosum Other anomalies make like left pulmonary artery sling make 10%, incomplete ie sling Other anomalies make like left pulmonary artery sling make 10%, incomplete ie sling

Etiology of rings and slings Non-regression or incomplete regression of any of the 6 embryonic branchial arches Normally what happens: 1 and 2 arches irrigate the face 3 arch forms carotids Dorsal aorta in 3 and 4 involutes 4 form aortic arch 5 arch involutes 6 becomes the proximal right pulmonary artery 7 distal right subclavian and left subclavian

Intra Operative Course Easy mask induction Easy mask induction Easy to ventilate Easy to ventilate Difficult time getting IV in (probably from previous surgeries) Difficult time getting IV in (probably from previous surgeries) Once IV started changed to TIVA pt paralyzed with Mivacron Once IV started changed to TIVA pt paralyzed with Mivacron Bag mask ventilatory control confirmed Bag mask ventilatory control confirmed Surgeon unable to introduce bronchoscope Surgeon unable to introduce bronchoscope

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

Intra op 3 Unable to pass Shiley, Number 3ETT tube passed and could not ventilate adequately. Unable to pass Shiley, Number 3ETT tube passed and could not ventilate adequately. PALS started Atropine and Epi given for bradycardia. PALS started Atropine and Epi given for bradycardia. Now WHAT???? Now WHAT????

Intra OP 4 Shiley replaced with Shiley 3# cuffed Ventilation possible via right lung Tube pulled back able to ventilate both lungs Shiley # 3 replaced with Shiley #4 adequate ventilation of both lungs

Follow 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 7 Pt consequently followed days 2,3,4,5 and 7

Round 2 7 days later pt taken for rigid bronchoscope, tracheoscopy possible flex bronchoscopy 7 days later pt taken for rigid bronchoscope, tracheoscopy possible flex bronchoscopy Able to pass small rigid suction tube Able 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 severe stenosis at the carina level. Flex scope through trachea which showed severe stenosis at the carina level. Pt transferred to CCF for further evaluation possible surgery. Pt transferred to CCF for further evaluation possible surgery.

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 Stenosis Congenital malformation of the cricoid cartilage Congenital malformation of the cricoid cartilage Acquired- Pathophysiology Acquired- Pathophysiology Mucosal edema Mucosal edema Hyperemia Hyperemia Pressure necrosis of mucosa Pressure necrosis of mucosa Fibrosis Fibrosis

Risk factors for SGS ETT ETT Duration of intubation Duration of intubation Repeated intubation Repeated intubation GERD GERD Factors that affect healing Factors that affect healing Systemic illness Systemic illness Malnutrion Malnutrion Anemia Anemia Hypoxia Hypoxia

SGS Incidence 1-2% of graduated NICU patients Incidence 1-2% of graduated NICU patients Morbi-mortality Morbi-mortality Difficulty breathing Difficulty breathing Exercise intolerance Exercise intolerance Death Death

Clinically Inspiratory stridor Inspiratory stridor Supraglottic lesion Supraglottic lesion Expiratory stridor Expiratory stridor Tracheal, bronchial, pulmonary lesion Tracheal, bronchial, pulmonary lesion In SGS that is moderate to severe they may have biphasic stridor In SGS that is moderate to severe they may have biphasic stridor

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

Staging Grade 1- Obstruction of 0-50 % of the lumen Grade 1- Obstruction of 0-50 % of the lumen Grade 2- Obstruction of 51-70% of the lumen Grade 2- Obstruction of 51-70% of the lumen Grade 3- Obstruction of 71-99% of the lumen Grade 3- Obstruction of 71-99% of the lumen Grade 4- Obstruction of 100% (no visible lumen) Grade 4- Obstruction of 100% (no visible lumen)

Treatment No medical treatment No medical treatment Surgery indicated with SGS + symptoms present Surgery indicated with SGS + symptoms present

Residents hard at work!!