Co-existing diseases

Question 1

Considerations with achondroplasia

Answer 1

atlantoaxial instability

Foramen magnum stenosis
OSA
inc lordosis/kyphoscoliosis - difficult neuraxial
Restrictive lung disease

Question 2

Beckwith-Wiedemann Syndrome

Answer 2

Omphalocele
organomegaly
macrosomia
large fontanelles
macroglossia
polycythemia
hypoglycemia

Question 3

Williams Syndrome

Answer 3

Elfin-like
Supravalvular aortic stenosis - if unknown can lead to death on induction of anesthesia
HyperCa

Question 4

Hyperkalemic Periodic Paralysis Triggers

Answer 4

Acidosis
Rest after exercise
Stress
Sux
Temp extremes
Hypoglycemia

Mutation in Na channel

Question 5

How do they following change in elderly people:

1. Beta receptor response
2. Catecholamine levels and response
3. Sensitivity to drugs
4. Lung volumes
5. Cardiac output
6. Neuraxis

Answer 5

1. Dec Beta receptor response –> less inc in chronotropy, ionotropy = less ability to inc CO
2. Dec baroreceptor activity, dec ACH release with vagal stim + INC NE levels
3. Dec lean mass + more fat (prolongs duration) + dec TBW (small Vd) = more sensitive. Dec clearance of remi.
4. TLC same
   - CC inc = small airway collapse
   - CC inc faster than FRC = inc A-a
   - VC dec
   - RV dec
   - Chest wall compliance dec
   - Lung compliance inc
5. Fibrosis –> diastolic dysfunction, SA node dysfunction = MORE reliant on atrial kick
6. • Dura more permeable = more sensitive
   • Dec myelinated fibers
   • Smaller epidural space - age = #1 factor for local spread

Question 6

Pre-op testing needed for myotonic dystrophy

Answer 6

EKG, ECHO
Cardiology consult with any conduction block - can progress rapidly and need transcutaneous pacing, not responsive to atropine

Avoid Sux, Neo, hypothermia…NMBDs not able to relive contracture

Tx = phenytoin or quinine

Question 7

1 inherited bleeding disorder

Answer 7

vWD

Carrier for factor VIII

Tx = DDAVP, Factor VIII or vWF concentrate, Cryo

Question 8

Causes of acquired vWD

Answer 8

Severe AS
Non-pulsatile VAD
ASD/VSD
Hypothryoidism 
SLE
Malignancies

Valproic acid
Cipro
Tetracyclines
Griseofulvin

Question 9

Signs of Multiple Myeloma

Answer 9

Anemia
HyperCa
Bone Fractures
Acute/chronic renal failure
Frequent pneumonia, pyelonephritis
Neuropathies

Question 10

Pyloric stenosis labs and optimization

Answer 10

hypoK, hypoCl metabolic alkalosis
Inc serum bicarb
Elevated urine SG
Dec urine Cl

Tx = NaCl bolus and D5 1/2NS infusion to rususictate before surgery

Inc CSF pH = apnea risk

Question 11

Abnormalities in DiGeorge Syndrome

Answer 11

CATCH-22
Cardiac (interrupted arch, truncus, TOF)
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia

Question 12

Induction and management of TEF

Answer 12

Inhalation
Spontaneous ventilation, avoid PPV
ETT cuff deep, past fistula

Question 13

Associations with Pierre-Robin

Answer 13

Stickler syndrome
Velocardiofacial
Treacher-Collins

Question 14

Things associated with POCD

Answer 14

Age >60
Lower educational level
Previous TIA/CVA
POCD at time of dishcarge

Question 15

Prolonged in hemophilia B

Answer 15

aPTT

Question 16

Deficiency in hereditary angioedema

Answer 16

C1 esterase inhibitor deficiency

• will have low C4 levels
• accumulation of bradykinin

Question 17

Spirometry with GBS

Answer 17

Restrictive pattern

-Dec TLC

Question 18

Paramytonia congenita

Answer 18

ion channel disease
avoid hypo or hyperthermia
avoid SUX
avoid ND-NMBDs if possible but are safe to use

Question 19

Hunter and Hurler syndromes

Answer 19

Accumulation of dermatans and glycoaminoglycans

• Large tongue, airway soft tissue = difficult airway
• obstructive lung disease
• valve abnormalities and diffuse coronary narrowing

Question 20

Spirometry with ALS

Answer 20

Restrictive pattern
-Dec TLC

= SAME as GBS

Question 21

Associations with choanal atresia

Answer 21

CHARGE

• Coloboma of the eye
• Heart defects
• Atresia of choana
• Retardation in growth or development
• GU abnormalities
• Ear abnormalities and deafness

Question 22

Associations with ankylosing spondylitis

Answer 22

Difficult intubation
A-A subluxation
TMJ
Inc risk epidural hematoma, difficult placement, narrower and potential excessive spread
Aortic insufficiency

Question 23

Test to diagnose acromegaly

Answer 23

IGF1

• remember inc lung volumes

Question 24

Causes of sickling

Answer 24

Six H’s cause SICKling

• hypothermia
• hyperthermia
• hypotension
• hypoxemia
• hypovolemia
• H+ ions (acidosis)

Question 25

Abnormalities with Goldenhar Syndrome

Answer 25

Craniofacial microsomia
Epibulbar dermoid
Rib or vertebral abnormalities

Facial asymmetry —> OSA, difficult mask, difficult intubation

Question 26

Fanconi Syndrome

Answer 26

Proximal tubule defect in kidney
Growth restriction, rickets

Non-anion gap metabolic acidosis, polyuria, dehydration, hypoK

Question 27

1 complication during anesthesia in Down syndrome

Answer 27

Bradycardia

Question 28

Things to avoid in AIP

Answer 28

Benzos
Barbiturates
Nifedipine
etOH
Glucocorticoids
Hypothermia

Question 29

Precipitants of hemolysis in G6PD deficiency

Answer 29

Methylene blue
Sulfa
ASA
Quinidine
Fava
Acidosis
Nitrofurantoin
Isoniazid

Question 30

Signs and Symptoms with autonomic dysreflexia

Answer 30

Vasoconstriction BELOW lesion

Vasodilation above lesion, HA, ICH, Stroke, Seizures,

Tx = vasodilators