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Principles II > Peds congenital abnormalities again DAN MILLER STYLE!! > Flashcards

Peds congenital abnormalities again DAN MILLER STYLE!! Flashcards

1
Q

Tracheoesophageal Fistula

S+S

A

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

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2
Q

Associated abnormalities of

Tracheoesophageal Fistula

A

Vertebral abnormalities

Anus (impertorate Anus)

Tracheo-

Esophageal Fistula

Radial aplasia

Renal abnormalities

OR VATER

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3
Q

Most common Tracheoesophageal Fistula

A

85-90 % are type 3B espohageal atresia with TEF

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4
Q

Tracheoesophageal Fistula anesthesia implications

A

Often not emergent

Gastrostomy tube placed under MAC/Local

A line

Fiberoptic cart

Precordial Stethoscope

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5
Q

Only way to tell if you are in the correct place after ETT placement is if you

A

Right mainstem them then check with Fiber optics and slowly withdraw ETT until you are just distal to fistula

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6
Q

TEF positioning

A

Left Lateral with intermittent venting of the Gastrostomy tube to prevent aspiration

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7
Q

Post op TEF complications

A

Regurg

aspiration

anastomotic leak

tracheal compression

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8
Q

Malrotation and Midgut Volvulus

A

abnormal rotation of the GI systemduring developement may have a volvulus (ischemic Bowel) r/t obstruction of the mesentary

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9
Q

Malrotation and Midgut Volvulus

can be determied from vomit which would be

A

Bilious in nature aka bile

with abdominal distension / tenderness

metabolic acidosis

electrolyte abnormalities

hemodynamically unstable

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10
Q

Malrotation and Midgut Volvulus

A

if no volvulus its not an emergency

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11
Q

Malrotation and Midgut Volvulus

intra op

A

Narcotic / paralytic / VA anesthetic
Low incidence of extubation after surgery
Increase fluid shifts
Increased IAP
Poss. open abdomen

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12
Q

Malrotation & Midgut Volvulus

intra op

A

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

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13
Q

Congenital Diaphragmatic Hernia

A

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%

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14
Q

Congenital Diaphragmatic Hernia

A

Resultant mediastinal shift to non-effected side
hypoplastic / aplastic lung tissue formation
Roughly 25% have congenital heart defects as well

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15
Q

Congenital Diaphragmatic Hernia

A
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16
Q

Congenital Diaphragmatic Hernia

S+S

A

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

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17
Q

Congenital Diaphragmatic Hernia

Pre op

A

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

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18
Q

Congenital Diaphragmatic Hernia

Pre op

A

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

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19
Q

Congenital Diaphragmatic Hernia

Intro op

A

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

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20
Q

Congenital Diaphragmatic Hernia

Intra op and post op

A

Intra-operative
Fluid management
Third spacing replaced via LR / NS
Mainenance - glucose containing
Albumin 5%
Post op
Pt. intubated to ICU while sedated

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21
Q

Omphalocele

A

Abdominal Wall Defect
Origin - Base of Umbilicus
Contained within hernia sac
Congenital Abn.
Trisomy 21
Cardiac Malform
Bladder Malform
Diaphragmatic Hernia

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22
Q

Gastroschisis

A

Abdominal Wall Defect
Origin - Lateral to Umbilicus
No hernia sac present (exp. content)
No congenital abnormalities usually seen

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23
Q

Omphalocele

A
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24
Q

Gastroschisis

A
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25
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
26
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
27
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
28
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
29
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
30
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
31
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
32
Croup Treatment
Treatment Humidified Oxygen Mist Racemic Epi Dexamethasone IM / PO / Neb ETT only when prolonged obstruct. persists and resp. muscles fail Cool nights
33
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
34
what is the most common human genetic malformation
Trisomy 21
35
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
36
Other genetic abnormalities that amybe seen with trisomy 21
Cardiac (ASD / VSD) Cataracts Esophageal / duodenal atresia Hip dysplasia Weak Atlano-axial joint OSA Hypothyroidism
37
Trisomy 21 Airway
Possibly difficult use 0.5 to 1 size smaller
38
Congenital Heart Defect catagories
Acyanotic and cyanotic
39
Acyanotic heart defects
ASD (atrial septal defect) VSD (ventricular septal defect) PDA( patent ductus arteriousis) AS ??? PS ???? Coarctation of the aorta
40
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)
41
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
42
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
43
VSD S+S
Holosystolic Murmur Large Defect- RVH - PHTN - Cyanosis - CHF
44
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
45
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
46
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
47
PDA medical TX
Nonselective COX inhibitor Reduces prostaglandin production Allows for anatomical closure Indomethacin SE - Dec Renal BF / Cerebral BF / mesenteric BF
48
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
49
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
50
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 /
51
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
52
Coarctation of the Aorta Blood supply to the spinal cord
2 posterior arteries (lateral) Dorsal 1 anterior artery (median) Ventral
53
Coartation Complications
Prolonged intubation Paralysis HTN Bleeding Infection Chylous effusion (chylothorax) Recurrent laryngeal nerve injury
54
Tetralogy of Fallot Major defects
4 major defects Large VSD Right Ventricular Outflow Tract Obstruction Overriding Aorta Right Ventricular Hypertrophy
55
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
56
TOF
57
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
58
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
59
Tetralogy of Fallot Cerebral vascular accidents why
Related to high hgb concentration Often times severe arterial hypoxemia
60
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
61
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
62
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
63
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)
64
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
65
Total Anomalous Pulmonary Venous S+S
Acute CHF Severe PHTN Shock Pulmonary Edema
66
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
67
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
68
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
69
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
70
Transposition of the Great Vessels S+S
Cyanosis CHF RVH - systemic circ
71
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
72
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
73
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
74
Hypoplastic LV S+S
Cyanosis CHF Systemic Hypoperfusion
75
Hypoplastic LV causes systemic circulation to be from a
PDA
76
Hypoplastic LV PDA closure will cause
Ischemia / acidosis / death
77
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
78
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
79
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
80
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.
81
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

Principles II (25 decks)

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