Advanced | Co-existing Diseases or Disease States Part I

Question 1

A 30-year-old patient with Fibrodysplasia Ossificans Progressiva (FOP) requires a surgical procedure under general anesthesia. Which of the following would be the most appropriate approach for airway management in this patient?

A) Awake nasal fiberoptic intubation, as it avoids trauma to the airway and minimizes the risk of heterotopic ossification.

B) Orotracheal intubation without fiberoptic assistance, as it is the quickest and most effective method.

C) General anesthesia with muscle relaxants to facilitate intubation, as there is no significant risk of heterotopic ossification in the airway.

D) Intramuscular injection of a local anesthetic for airway management, since regional anesthesia is not an option.

Answer 1

A) Awake nasal fiberoptic intubation, as it avoids trauma to the airway and minimizes the risk of heterotopic ossification

Question 2

A 30 year old female is scheduled for removal of thymoma. She is a known case of Myasthenia Gravis for a year now and she’s on immuno-suppressant medications. Post-operatively, which of the predictors will require this patient for prolonged intubation?

A. Duration of disease less than or equal 24 months

B. History of a smoking

C. Pyridostigmine dose of > 750 mg/day

D. Vital capacity 3.5L or more

Answer 2

C. Pyridostigmine dose of > 750 mg/day

The predictors of a prolonged mech vent post-operatively are the following:

Duration of disease ≥ 72 months (≥ 6 years)**greatest value

History of a chronic respiratory disease (e.g. asthma and COPD)

Pyridostigmine dose of > 750 mg/day

Vital capacity < 2.9 liters

Question 3

Prader Willi patient will most likely have which of the following intraoperative issue?

A. Hypoglycaemia

B. Hypocalcaemia

C. Neuroleptic malignant syndrome

D. Malignant Hyperthermia

E. Hypothermia

Answer 3

A. Hypoglycemia

Hypoglycaemia

—

• Adrenal insufficiency occurs in up to 60%, which can lead to hypoglycemia

However in older age they may have diabetes which predisposes to hyperglycemia

Question 4

TRUE or FALSE

Patients with MG are very sensitive to succinylcholine.

Answer 4

FALSE

In fact, patients with MG are highly resistant to depolarizing muscular blocking agents.

Mg patients are resistant to SUX, sensitive to nondepolarizing blockers. In addition, patients being treated with cholinesterase inhibitors will also have a prolonged response to succinylcholine (prolonged phase I blockade) due to impaired plasma cholinesterase function.

Question 5

Plasma half-time of which of following is prolonged in patients with end stage cirrhotic liver disease?

A. Diazepam
B. Pancuronium
C. Fentanyl
D. All of the above

Answer 5

D. All of the above

Question 6

Malignant Hyperthermia is believed to involve a generalized disorder of membrane permeability to

A. Sodium
B. Potassium
C. Calcium
D. Mg
E. Phosphate

Answer 6

C. Calcium

Question 7

A 46-year-old man is scheduled for repair of an inguinal hernia. Six years ago, he had an episode of malignant hyperthermia during cholecystectomy. Which of the following is the most appropriate perioperative management?

A. Administration of a regional anesthetic

B. Administration of dantrolene orally for two days prior to surgery

C. Avoidance of all inhalational anesthetics except isoflurane

D. Avoidance of ester local anesthetics

E. Flushing the anesthesia machine with oxygen 10 L/min for a minimum of 12 hours

Answer 7

A. Administration of a regional anesthetic

Question 8

Drugs suitable for patients with MH include all of the following except:

A. Etomidate

B. Nitrous oxide

C. Calcium chloride

D. Ketamine

E. All of the above

Answer 8

E. All of the above

Question 9

Which of the following statements concerning myasthenia gravis is TRUE?

(A) Neostigmine is inappropriate for antagonism of neuromuscular blockade

(B) The number of acetylcholine receptors is decreased

(C) Plasma cholinesterase concentration is decreased

(D) The risk for malignant hyperthermia is increased

(E) Succinylcholine is contraindicated

Answer 9

(B) The number of acetylcholine receptors is decreased

Question 10

Which of the following is a known effect of propofol?

(A) Decreased amplitude of somatosensory evoked potentials

(B) Induction of malignant hyperthermia

(C) Inhibition of cytochrome P450

(D) Initiation of porphyria

(E) Suppression of adrenocortical function

Answer 10

(A) Decreased amplitude of somatosensory evoked potentials

*INCREASED Latency

*DECREASED Amplitude

Question 11

A 27-month-old, 14-kg infant with intestinal obstruction is anesthetized with nitrous oxide at 1 L/min, oxygen at 0.4 L/min, and halothane at 0.8% using a Bain circuit. Ventilation is controlled at a rate of 30/min. The child’s temperature is 39°C, and the PaCO2 is 65 mmHg. Which of the following is most likely?

(A) Malignant hyperthermia

(B) Low gas inflow rate

(C) Low respiratory rate

(D) Pulmonary aspiration of gastric contents

(E) Inaccurate flowmeters

Answer 11

(B) Low gas inflow rate

Question 12

Most common ECG among Myotonic Dystrophy Type - 1?

Answer 12

Prolonged PR interval and QRS duration

Question 13

This is a progressive multisystem disorder characterized by the DELAYED relaxation of skeletal muscle after voluntary contraction.

A. Myotonic Dystrophy

B. Hypokalemic Periodic Paralysis

C. GBS

D. ALS

Answer 13

MYOTONIC DYSTROPHY

2 types

DM-1 is the more COMMON and SEVERE form, with a wide phenotype ranging from
asymptomatic to life threatening.

Question 14

FEATURES of Myotonic Dystrophy Type 1:

Answer 14

• A trinucleotide expansion (CTG) on chromosome 19q
• Effects on virtually any organ system, but most seriously the musculoskeletal, cardiovascular, gastrointestinal, respiratory, central nervous, and endocrine systems
• dystrophic process, begins DISTALLY and
  progressing proximally
• The Myotonic grip often precedes weakness

Question 15

A patient with Myotonic Dystrophy is at risk of aspiration due to:

A. Gastric atony

B. Delayed gastric emptying

C. Diaphragmatic Hernia

D. Pyloric anomaly

Answer 15

Gastric ATONY and pharyngeal muscle weakness

Question 16

True of False

(Myotonic Dystrophy) DM-2 commonly complain of myalgia and fatigue, and weakness is usually MILD.

Answer 16

True

• DM-2 is caused by a quadnucleotide expansion (CCTG) on chromosome 3q.

Overall, the clinical course of DM-2 is milder than DM-1.

Most commonly, patients present with mild proximal muscle weakness in the third decade of life or later.

Question 17

TRUE or FALSE

Annual ECG are recommended among Myotonic patients?

Answer 17

TRUE

Question 18

High yield tip | Anesthesia Technique:

Is succinylcholine indicated for Myotonic Dystrophy?

Answer 18

NO. Sux should be AVOIDED

Succinylcholine should be avoided, as patients have an exaggerated contracture response to succinylcholine that can potentially make ventilation and tracheal intubation difficult.

If NMB is needed, short acting NMB is preferred, and dosage of NMB should be adjusted in proportion to the degree of muscle wasting.

Question 19

Is Neuromuscular monitoring reliable intraoperatively in Myotonic Dystrophy?

Answer 19

YES however myotonic response to stimulation can mimic sustained tetany, hence the degree of block could be UNDERESTIMATED.

• Neostigmine and Sugammadex is SAFE

Question 20

TRUE or FALSE

Shorter-acting opiates and sedatives are preferred to reduce the need for postoperative ventilation.

Answer 20

TRUE

Question 21

Disorder NOT typically associated with Malignant Hyperthermia:

A. Central core disease

B. Duchenne muscular dystrophy

C. King-Denborough Syndrome

D. Evans Myopathy

Answer 21

B. Duchenne muscular dystrophy

The effect of INHALATION anesthesia on patients with DMD is now thought to be a form of anesthesia-induced rhabdomyolysis

Question 22

One of the following is evidently one of the earliest SIGNS of malignant hyperthermia:

A. Fever

B. Hematuria

C. Elevated ETCO2

D. Metabolic acidosis

Answer 22

C. Elevated ETCO2

Question 23

TRUE of Malignant Hyperthermia:

A. Hypercarbia that is resistant to increases in minute ventilation.

B. After confirming adequate ventilation, hypercarbia resistant to increases in minute ventilation should prompt a suspicion of MH

C. The initial dose of dantrolene is 2.5mg/kg. Repeat as frequently as needed until there is a decrease in EtCO2 and muscle rigidity

D. All are TRUE

Answer 23

ALL OF THESE ARE TRUE statement

Hypercarbia that is resistant to increases in minute ventilation. After confirming adequate ventilation, hypercarbia resistant to increases in minute ventilation should prompt a suspicion of MH.

The initial dose of dantrolene is 2.5mg/kg. Repeat as frequently as needed until there is a decrease in EtCO2 and muscle rigidity

Question 24

Differential diagnosis of MH includes the following EXCEPT:

A. Serotonin Syndrome

B. Fat Embolism Syndrome

C. Thyroid storm

D. Central anticholinergic syndrome

E. Hypokalemic Periodic Paralysis

Answer 24

E. Hypokalemic Periodic Paralysis

Question 25

Which of the following findings is NOT consistent with a diagnosis of malignant hyperthermia? a. PaCO2 150 mm Hg b. MVO2 50 mm Hg c. pH 6.9 d. Onset of symptoms an hour after end of operation

Answer 25

b. MVO2 50 mm Hg MH reflects a hypermetabolic state. Clinical signs include tachycardia, tachypnea, arterial hypoxemia, hypercarbia, metabolic acidosis, hyperkalemia, hypotension, muscle rigidity, trismus after succinylcholine administration, and increased temperature. * Mixed venous oxygen tension would be very low (normal MVO2 is 30-35, so an elevated MVO2 of 50 would not be consistent with MH).

Question 26

What type of channelopathy is the main culprit in Hyperkalemic periodic paralysis: A. Na+ channel myopathy B. K+ channel myopathy C. Ca+ channel myopathy D. Cl- channel myopathy

Answer 26

A. Na+ channel myopathy It is an autosomal dominant channelo-pathy caused by a mutation of the sodium channel. The muscles responsible for respiration are usually spared The use of potassium-wasting drugs such as thiazide diuretics or carbonic anhydrase inhibitors, along with maintaining a diet of carbohydrate-rich meals, can help prevent episodes.

Question 27

What type of channelopathy is the main culprit in Hypokalemic periodic paralysis: A. Na+ channel myopathy B. K+ channel myopathy C. Ca+ channel myopathy D. Cl- channel myopathy

Answer 27

C. Ca+ channel myopathy Hypokalemic periodic paralysis (hypoPP) is also autosomal dominant and is the result of mutations in both calcium ion (most common) and sodium ion channels. Patients begin having episodes of weakness, usually in their teenage years; these episodes last hours to days and are the result of a low serum potassium concentration. Proximal muscles are most often affected, while the diaphragm and muscles supplied by the cranial nerves are spared.

Question 28

Which of the following DOES not present as periodic paralysis? A. Hyperkalemic periodic paralysis B. Andersen–Tawil syndrome C. Myotonia congenita D. Thyrotoxic periodic paralysis

Answer 28

C. Myotonia congenita

Question 29

What type of channelopathy is the main culprit in Andersen–Tawil syndrome? A. Na+ channel myopathy B. K+ channel myopathy C. Ca+ channel myopathy D. Cl- channel myopathy

Answer 29

B. K+ channel myopathy Anderen–Tawil syndrome (ATS) is caused by a mtation in the potassium ion channel of skeletal muscle. This results in a periodic flaccid paralysis similar in presentation to hypokalemic periodic paralysis. Unlike other channelopathies, Andersen-Tawil Syndrome affects the smooth muscle such as the cardiac muscles. May present with arrhythmia. Andersen-Tawil syndrome (ATS) is characterized by a triad of: episodic flaccid muscle weakness (i.e., periodic paralysis); ventricular arrhythmias and prolonged QT interval; and anomalies including low-set ears, widely spaced eyes, small mandible, fifth-digit clinodactyly, syndactyly, short stature, and scoliosis. Affected individuals present in the first or second decade with either cardiac symptoms (palpitations and/or syncope) or weakness that occurs spontaneously following prolonged rest or following rest after exertion.

Question 30

Atlantoaxial subluxation is diagnosed when the distance between the: A. anterior arch of the atlas and the odontoid process is >3mm B. posterior arch of the atlas and the odontoid process >5mm C. posterior arch of the axis and the odontoid process is >3mm D. anterior arch of the axis and the odontoid process is >5mm

Answer 30

A. anterior arch of the atlas and the odontoid process is >3mm Atlantoaxial subluxation is diagnosed when the distance between the anterior arch of the atlas and the odontoid process is > 3 mm

Question 31

A known epileptic 18 year old is scheduled for electrocorticography to map out the foci-source of her seizure. Which of the following drugs SHOULD be AVOIDED? A. Midazolam B. Propofol C. Alfentanil D. Rocuronium

Answer 31

A. Midazolam

Question 32

A patient undergoing eCOG(electrocorticography) is at higher risk of developing "awareness". To prevent this, which of the following drugs can be given? A. Scopolamine B. 1.5 MAC isoflurane C. Atracurium D. Midazolam

Answer 32

A. Scopolamine

Question 33

A patient with long-standing epilepsy is scheduled for an elective surgery. Pre-operatively, should the maintenance anti-epileptics be DISCONTINUED?

Answer 33

NO. Unless there is a need for foci-mapping, all anti-epileptics are continued on the day of surgery.

Question 34

Which of the following medications needs dose up-titration peri-operatively if a patient is on anti-epileptic medications? A. Dexmedetomidine B. Fentanyl C. Rocuronium D. All of the above E. A and B only

Answer 34

D. All of the above

Question 35

All of the following conditions are associated with acromegaly EXCEPT A. T2 DM B. Hypertension C. Cardiomyopathy D. Obstructive Sleep Apnea E. COPD

Answer 35

E. COPD Associated conditions with Acromegaly: * OSA (70%), larger airways with calcinosis of larynx (may actually need smaller tube), macroglossia, macrognathia, extubation issues common * HTN (40%), Heart disease, cardiomyopathy, valvular disease, heart block * Carpal tunnel, raised ICP and headache from 3rd ventricle obsruction * T2DM Other Arthritis, large Bowel polyps/tumours, erectile dysfunction / dysmenorrhoea

Question 36

The illustration depicts flow-volume loops for the same person at two different times in his life. Which of the following is indicated by the dotted loop? (A) Bronchial tumor (B) Chronic asthmatic bronchitis (C) Morbid obesity (D) Paralysis of a vocal cord (E) Subglottic stenosis

Answer 36

(B) Chronic asthmatic bronchitis

Question 37

A 6-year-old child is transported to the recovery room after a tonsillectomy. The patient was anesthetized with isoflurane, fentanyl, and N2 O. Twenty minutes before emergence and tracheal extubation, droperidol was administered. The anesthesiologist is called to the recovery room because the patient is “making strange eye movements.” The patient’s eyes are rolled back into his head, and his neck is twisted and rigid. The most appropriate drug for treatment of these symptoms is A. Dantrolene B. Diazepam C. Glycopyrrolate D. Diphenhydramine

Answer 37

D. Diphenhydramine