Peds congenital abnormalities again DAN MILLER STYLE!! Flashcards
Tracheoesophageal Fistula
S+S
Excessive oral secretions
regurg of first feeding
Resp distress after several feedings
inability to pass OGT
Delayed S+S
recurring pnemonia
inability to pass an OGT
Associated abnormalities of
Tracheoesophageal Fistula
Vertebral abnormalities
Anus (impertorate Anus)
Tracheo-
Esophageal Fistula
Radial aplasia
Renal abnormalities
OR VATER
Most common Tracheoesophageal Fistula
85-90 % are type 3B espohageal atresia with TEF
Tracheoesophageal Fistula anesthesia implications
Often not emergent
Gastrostomy tube placed under MAC/Local
A line
Fiberoptic cart
Precordial Stethoscope
Only way to tell if you are in the correct place after ETT placement is if you
Right mainstem them then check with Fiber optics and slowly withdraw ETT until you are just distal to fistula
TEF positioning
Left Lateral with intermittent venting of the Gastrostomy tube to prevent aspiration
Post op TEF complications
Regurg
aspiration
anastomotic leak
tracheal compression
Malrotation and Midgut Volvulus
abnormal rotation of the GI systemduring developement may have a volvulus (ischemic Bowel) r/t obstruction of the mesentary
Malrotation and Midgut Volvulus
can be determied from vomit which would be
Bilious in nature aka bile
with abdominal distension / tenderness
metabolic acidosis
electrolyte abnormalities
hemodynamically unstable
Malrotation and Midgut Volvulus
if no volvulus its not an emergency
Malrotation and Midgut Volvulus
intra op
Narcotic / paralytic / VA anesthetic
Low incidence of extubation after surgery
Increase fluid shifts
Increased IAP
Poss. open abdomen
Malrotation & Midgut Volvulus
intra op
Aggressive fluid resusc.
IVF / Blood / Bicarbonate
Often times manipulation of abd. contents will facilitate edema formation
Increase IAP / Dec FRC & lung volumes.
Often times will leave abd. open with a Silastic silo in place
Congenital Diaphragmatic Hernia
Herniation of abdominal contents into the thoracic cavity
Through one of 3 foramen
Foramen of Morgagni (anterior)
Foramen of Bochdalek (posterolateral)
Left Foramen of Bochdalek 90%
Congenital Diaphragmatic Hernia
Resultant mediastinal shift to non-effected side
hypoplastic / aplastic lung tissue formation
Roughly 25% have congenital heart defects as well
Congenital Diaphragmatic Hernia
Congenital Diaphragmatic Hernia
S+S
Signs of respiratory distress
Tachypnea & cyanosis
Decreased transillumination on affected side
Bowel sounds in thorax
Scaphoid abdomen
Cardiovascular Anomalies
Right to Left Shunt / Obstruction of IVC
Critical pts may have to be placed on ECMO
Congenital Diaphragmatic Hernia
Pre op
Immediate stabilization is paramount
Secured airway / sedation / paralysis
Awake FOB preferred
Avoid mask ventilation prior to intubation
Unless in Acute Emergency
Read as CV Collapse - Gentle MV until airway secured
Prior to intubation, if possible, narcotics to facilitate reduction of catecholamine release
Reduction of PVR
Congenital Diaphragmatic Hernia
Pre op
Small TV with low pressure to reduce chance of pneumothorax / barotrauma
OGT / NGT placed ASAP to decompress fluid / gas
Reduce aspiration risk
Facilitate some ease of ventilation
Place on effected side to reduce pressures on heart and lung
Congenital Diaphragmatic Hernia
Intro op
Keep airway pressures below 30 cmH2O
No N2O
Frequent ABG’s
Preferrably from a preductal arterial line & umbilical line
Umbilical artery catheter is usually pre-existing
Facilitates degree of R to L shunt
Sudden reduction in lung compliance / BP / SpO2 => CONTRALATERAL pneumothroax
BAD!! One good lung now has a pneumo
Need a Chest Tube
Herculean efforts to expand ipsilateral lung immediately after surgery is contraindicated
Congenital Diaphragmatic Hernia
Intra op and post op
Intra-operative
Fluid management
Third spacing replaced via LR / NS
Mainenance - glucose containing
Albumin 5%
Post op
Pt. intubated to ICU while sedated
Omphalocele
Abdominal Wall Defect
Origin - Base of Umbilicus
Contained within hernia sac
Congenital Abn.
Trisomy 21
Cardiac Malform
Bladder Malform
Diaphragmatic Hernia
Gastroschisis
Abdominal Wall Defect
Origin - Lateral to Umbilicus
No hernia sac present (exp. content)
No congenital abnormalities usually seen
Omphalocele
Gastroschisis
Omphalocele & Gastroschisis
anesthetic implications
Often times will have planned C-Section to facilitate timely and acute management of neonate
At high risk neonate ICU / L&D Facility
Gastric decompression prior to induction
Induction can proceed as normal
Awake / asleep
With or without paralysis
Third spacing volume loss can be extensive
Must be aggressively replaced
5% albumin
LR or NS
Omphalocele & Gastroschisis
anesthetic implications
Rarely abdomen is completely closed with 1 surgery
If any of these parameters persist complete closure is held
Intragastric > 20 cmH2O - (EOP / Vent Effort)
PIP > 35 cm H2O
ETCO2 > 50 mmHg
Dacron graft placed (Silastic Silo)
Closed 2-3 days later
Obviously remains intubated until complete closure
Maintain fluid status / normothermia
Pyloric Stenosis
Hypertrophied muscular layer around the pylorus sphincter
Reduces ability of gastric contents to be emptied
Most commonly seen 2-6 weeks of age
Chronic vomiting (nonbilious)
Hyponatremia / hypokalemia / hypochloremia / alkalosis
Causes hypochloremic metabolic alkalosis
Pyloric Stenosis
Surgical correction is rarely if ever emergent
Fluid / electrolyte correction is paramount
With depletion of Ions
Kidneys excrete H+, even in the face of the present alkalosis
Na+ / H+ anti-port in kidneys….remember?? (Me neither)
Kidneys also exctrete bicarbonate in an effort to neutralize the system
If uncorrected it may lead to shock and renal failure
LR is metabolized to bicarbonate
Should be avoided
NS with K+ supplementation
Pyloric Stenosis
Anesthesia implications
Gastric Decompression
Supine / L Lateral / R Lateral
Not one, all three positions while sx is on
Facilitates complete emptying
Awake FOB or RSI
Short procedure, requiring muscular paralysis
Increased incidence of respiratory depression
Should be monitored postoperatively with apnea monitors
Infectious Croup vs. Acute Epiglottitis
Croup
Croup - Follow URTI (Viral)
3 mo - 3 yoa
Onset - gradual over Days
Subglottic
Laryngeotracheobronchitis
S/S
Low grade fever
Croupy / “Seal bark” cough
Inpiratory stridor
Rhinnorhea
Infectious Croup vs. Acute Epiglottitis
Acute Epiglottitis
Epiglottitis
Bacterial (H. Influenza)
2 - 6 yoa
Onset - sudden
Hours
Supraglottic
EMERGENCY!!
S/S
Low pitch insp. stridor
Drooling / Lethargic / Fever
Restless / Tachypnea /
Sitting upright / Pharyngitis
Croup Treatment
Treatment
Humidified Oxygen
Mist
Racemic Epi
Dexamethasone
IM / PO / Neb
ETT only when prolonged obstruct. persists and resp. muscles fail
Cool nights
Epiglottitis Treatment
Epiglottitis (ETT 0.5-1 size smaller)
Treatment
Emergent (Unconcious / Cyanotic / Bradycardic )
Induce / Intubate in ER
Urgent
To OR, inhal. Ind. in sitting position
NO DL until under GA (laryngeospasm)
Intub / Cultures / IV abx
Stable
Lat Neck Xray (thumb?)
IV abx / always on standby in case of progression of S/S
what is the most common human genetic malformation
Trisomy 21
Trisomy 21
Physical S/S
Decreased Muscle tone
Single crease in palm of hand
Small ears
Small mouth
Wide hands / short fingers
Excess skin of neck
Short neck
Irregular dentition
Protruding tongue
Other genetic abnormalities that amybe seen with trisomy 21
Cardiac (ASD / VSD)
Cataracts
Esophageal / duodenal atresia
Hip dysplasia
Weak Atlano-axial joint
OSA
Hypothyroidism
Trisomy 21 Airway
Possibly difficult
use 0.5 to 1 size smaller
Congenital Heart Defect catagories
Acyanotic and cyanotic
Acyanotic heart defects
ASD (atrial septal defect)
VSD (ventricular septal defect)
PDA( patent ductus arteriousis)
AS ???
PS ????
Coarctation of the aorta
ASD S+S
May be undetectable for years
When shunting worsens
Increase in RAP -> RVP
Problematic when Pulm circ amount 1.5 times greater than systemic circ amount
DOE
Irreversible PHTN (Severe defect)
RCHF (Severe defect)
ASD anesthesia implicaitons
Minimize the L to R shunt
Facilitate the maintenance or slight lowering of SVR
Reduces shunt
VA has this effect
Increase in PVR
PPV has this effect
Most defects are repaired in the cath lab
Need antibx prophylaxis
VSD
Ventricular Septal Defects
Most common CHD in infants and children
Most spontaneously close by 2 YOA
Holosystolic murmur left sternal border
If large enough defect
Equalization of ventricular pressures
Initial L to R shunt
PVR increases
Eventually R to L shunt with PHTN / RVH
Cyanosis will then ensue
VSD S+S
Holosystolic Murmur
Large Defect- RVH
- PHTN
- Cyanosis
- CHF
VSD anesthetic implications
As with ASD, maintain
Low / normal SVR
Normal / high PVR
Will require ventriculostomy (CPB)
Arterial Line
Possible surgically placed left atrial line
TEE
Careful fluid administration
Pt. already in heart failure to have ped surgical correction
IE pts on digoxin / lasix / etc
PDA
Distal to L subclavian takeoff of aorta to pulmonary artery
Should functionally close 24 to 48 hours after birth in a full term newborn
Premature infants often times will not have a closure
PDA S+S
Often times asymptomatic until adolescence
Continuous systolic / diastolic murmur
LVH / RVH on ECG
If large and untreated can lead to
Irreversible PHTN
CHF
PDA medical TX
Nonselective COX inhibitor
Reduces prostaglandin production
Allows for anatomical closure
Indomethacin
SE - Dec Renal BF / Cerebral BF / mesenteric BF
PDA surgical TX
PDA ligation (NICU)
Small thoracotomy
Must be ready for potential blood loss
VA (ideal) / PPV
Usually Narc / NMB
Post op HTN
SNP infusion for acute control
Coarctation of the Aorta
Narrowing of the aortic lumen
Diaphragm-like ridge that protrudes into aortic lumen
Most frequently post ductal
IE after the L subclavian artery
Ligamentum arteriosum
Although can be pre ductal
In newborns / infants with severe Coarct.
Many will have a PDA to facilitate passage of blood out of the heart
COarctation of the Aorta S+S
Most are asymptomatic
SBP higher in BUE vs BLE
DBP similar in both
Widened pulse pressure
Weakened / absent femoral pulses
When symptomatic
HA / Dizziness / Epistaxis / Palpiations / Claudication
Peds - shock /
Coarctation of the Aorta
Anesthesia considerations
3 main issues
Perfusion of lower body during cross clamping
Resultant systemic HTN r/t cross clamping
Neurologic deficits r/t prolonged hypotension / cross clamping
Arterial lines Right Radial / Femoral
Femoral art line should be > 50 mmHg in a healthy pt. w/o CAD….w/ CAD must be higher
If there is an inability to facilitate such a pressure
Partial CPB must be instituted
Coarctation of the Aorta
Blood supply to the spinal cord
2 posterior arteries (lateral)
Dorsal
1 anterior artery (median)
Ventral
Coartation Complications
Prolonged intubation
Paralysis
HTN
Bleeding
Infection
Chylous effusion (chylothorax)
Recurrent laryngeal nerve injury
Tetralogy of Fallot
Major defects
4 major defects
Large VSD
Right Ventricular Outflow Tract Obstruction
Overriding Aorta
Right Ventricular Hypertrophy
Tetralogy of Fallot has what kind of shunt
R-L
or
L-R
Right to Left shunt r/t RVOT obstruction
RVOT is a fixed lesion
SVR & PVR very important in determining the severity of the shunt
TOF
Tetralogy of Fallot
S+S
Cyanosis develops from birth to 1st year of life
ECG RAD & RVH
PaO2 < 50mmHg even on 1.0 FiO2
Compensatory erythropoiesis r/t severity of shunt (hypoxemia)
Squatting to increase SVR and reduce shunt
“Tet spells
Tetralogy of Fallot
Hypercyanotic Attacks
Caused by decreased SVR or infundibulum muscle spasm
Usually exercise or crying induced
Severe cyanosis
Can lead to
Tachypnea
Loss of consciousness
Seizures
CVA
Death
Tetralogy of Fallot
Cerebral vascular accidents why
Related to high hgb concentration
Often times severe arterial hypoxemia
TOF
Anesthetic considerations
Intimate knowledge of your anesthetic plan and medications
Must be able to treat and recognize cyanosis quickly and efficiently
Reduce or eliminate any reductions in SVR
Reduce or eliminate any excitatory phases for the heart
TOF
Anesthetic considerations
No beta agonists
Reduced conc of VA
If inhal induc necessary, use caution
Use of ketamine
Avoid dehydration
Maintenance - N2O & Ketamine
Drawback - Marginal increase in PVR, cannot admin 1.0 FiO2
Muscular paralysis - pancuronium is preferred
TOF
Anesthetic considerations
CPB
Art / CVP line
Blood in room
Routine H/H & Glucose checks
Often times need inotropic support off CPB
To ICU intubated and sedated
Total Anomalous Pulmonary Venous
Condition in which the 4 pulmonary veins anastomose and empty into the RA / venous circulation, usually via the innominate vein (most common)
Total Anomalous Pulmonary Venous
Diagnosed via angiocardiography
50% of pts. have acute CHF by 1 MOA
90% of pts. have acute CHF by 1 YOA
Most viable pts. have an ASD or PFO to allow oxygenated blood into the systemic circulation more easily
Total Anomalous Pulmonary Venous
S+S
Acute CHF
Severe PHTN
Shock
Pulmonary Edema
TAPVR
Surgical correction
Surgical Correction is paramount and imminent
Dissection of the 4 Pulm Vein anastomosis sites from current location
Reanastomose to the LA
On CPB
Closure of ASD &/or PFO
TAPVR
Anesthetic considerations
Mechanically ventilate and administer PEEP as soon as possible
Reduce amount of fluid administered
Pt is in acute CHF, and related to vascular anomoly, administering large quantities of fluid will further exacerbate the CHF
Rarely can patients tolerate a VA
Narcotic / NMB / Benzo is anesthetic of choice in severely ill
TAPVR
Anesthetic considerations
Arterial line should be in place
ETT should be in place
CVL should be in place
Poss placing PA or LA per surgical team
CPB will be used
Transport to ICU intubated / sedated
Possibility of NO use
Transposition of Great Vessels
what it is???
Aorta is connected to the RV
Deoxygenated blood is now via the systemic circulatory system
Pulmonary trunk is connected to the LV
Oxygenated blood is now via the pulmonary circulatory system
Two completely separate systems
Survival only if you have a circulatory communication
ASD / VSD / PDA
Transposition of the Great Vessels
S+S
Cyanosis
CHF
RVH - systemic circ
Transposition of the Great Vessels
Surgical Correction
Prostaglandin infusion
Facilitates the fetal circulation, allows for mixing of the systems
Arterial Switch
Resection of the ascending aorta and pulmonary artery
Transposing them onto the appropriate anatomical structure
Coronary arteries must be transposed as well
Transposition of the Great vessels
Anesthetic Considerations
Ketamine / sedation / oxygen
Facilitates a maintained / increased SVR
Oxygen only facilitates a decreased PVR
Avoid VA for cardio depressant effects
Avoid histamine releasing meds
If NMB needed, pancuronium
Sympathomimetic
Hypoplastic LV??
Left Ventricular Hypoplasia
Aortic Valve Atresia
Ascending Aortic hypoplasia
Mitral Valve Hypoplasia
Complete mixing of blood
Both venous and arterial in RV only
Only way to oxygenate the systemic circulation is through
PFO
PDA
Hypoplastic LV
S+S
Cyanosis
CHF
Systemic Hypoperfusion
Hypoplastic LV causes systemic circulation to be from a
PDA
Hypoplastic LV PDA closure will cause
Ischemia / acidosis / death
What needs to stay increased in a hypoplastic LV
Pulmonary vascular resistance
Promotes forward flow into systemic circulation
Pulmonary steal
Dec. PVR will shunt blood to Pulm circ system
Reduces or eliminates BF to Systemic
Hypoplastic LV
Treatment
Medical
Increasing PVR / decreasing SVR / Maintaining fetal circulation (PGE1 gtt)
Low FiO2
Inotrope(s) infusions
Treatment for acidosis
Most pts with this condition will not live past 1-2 weeks if not surgically corrected
Hypoplastic LV Surgical TX
Norwood procedure
PDA ligation / PA trunk is oversewn to Ao & Ao arch. Graft placed. Direct shunt from PA to Ao
Glenn procedure
Bidirectional shunt to facilitate reduced volume load on single ventricle. To systemic and pulmonary now
Fontan procedure
Separation of Systemic and Pulmonary venous return to the heart
Hypoplastic LV
Anesthetic Considerations
Should have ETT / Art / CVP from NICU
Fentanyl / Panc induction
No VA r/t increased risk of Vfib
Coronaries underperfused with VA r/t retrograde flow
Keep FiO2 low (maintain PVR) PaO2 < 100mmHg
Hypoventilate
Air only
Add CO2
Clamp Pulm art.
Hypoplastic LV
Anesthetic Considerations
CPB
Will circ arrest during procedure
Reconstruct of Ao
CPB reinstituted for graft placement
Most likely need inotropes after CPB
Transported to ICU intubated and sedated
