UBP 5.4 (Short Form): ENT – Bleeding Tonsil

Question 1

How would you evaluate this patient preoperatively?

(An 8-year-old, 46 kg, male presents for tonsillectomy. The mother reports that her son has a history of asthma, and that he has given prednisone 8-9 weeks ago because his asthma was “acting up”. His anesthetic history includes prolonged bleeding following a complicated tooth extraction 3 years ago.)

Answer 1

Considering the patient’s age, weight, the scheduled case, his history of asthma, and the prolonged bleeding he experienced following a tooth extraction,

• my preoperative evaluation would focus on his asthmatic condition and any additional history consistent with respiratory tract infection, airway obstruction, and/or a bleeding disorder.
  
  • Therefore, in evaluating his asthmatic condition, I would attempt to elicit information concerning
    
    • the age of onset,
    • triggering events,
    • allergies,
    • changes in symptomatology (cough, sputum, wheezing, etc.),
    • current medications,
    • anesthetic history, and
    • any asthma related hospital admissions.

Recognizing that active respiratory tract infections are common in this age group and would increase the risk of respiratory and bleeding complications,

• I would also try to identify any airway tract infection by
  
  • reviewing the patient’s recent history and examining him for cough, congestion, runny nose, sneezing, wheezing, rales, and malaise.

Given his weight and the orofacial abnormalities associated with adenotonsillar hyperplasia (high arching plate, retrognathic mandible, and dental abnormalities), I would –

• evaluate his airway (i.e. tonsillar hyperplasia may affect mask ventilation),
• ask the surgeon whether airway obstruction was the indication for surgery, and
• assess his risk for obstructive sleep apnea
  
  • (If airway obstruction were the indication for the tonsillectomy, the results of a sleep study would be helpful in identifying the patient’s at increased risk of rapid desaturation and difficult airway management).

Finally, considering his history of prolonged bleeding following tooth extraction, I would –

• order a coagulation profile and
• inquire about any additional history consistent with a bleeding disorder, such as a family history, easy bleeding and bruising, and prolonged bleeding following minor injuries.

Depending on my findings, I may consider:

1. an ECG or echocardiogram to identify the right ventricular hypertrophy and/or pulmonary hypertension that may result secondary to chronic airway obstruction;
2. a chest x-ray to identify cardiomegaly or a lower airway tract infection; and
3. PFTs to more accurately assess the severity of obstruction and his response to therapy.

Question 2

You order lab work, and get a normal PT and an elevated aPTT.

What do you think?

(An 8-year-old, 46 kg, male presents for tonsillectomy. The mother reports that her son has a history of asthma, and that he has given prednisone 8-9 weeks ago because his asthma was “acting up”. His anesthetic history includes prolonged bleeding following a complicated tooth extraction 3 years ago.)

Answer 2

The combination of a normal PT and an elevated aPTT is consistent with –

1. Von Willebrand’s disease,
2. Hemophilia A (Factor VIII deficiency),
3. Hemophilia B (Factor IX deficiency),
4. Hemophilia C (aka Rosenthal’s disease; Factor XI deficiency),
5. lupus anticoagulant, or
6. the administration of low-dose heparin.

Therefore, I would:

1. consult a hematologist;
2. verify that the patient was not receiving heparin; and
3. consider ordering lab to measure –
   
   1. vWF antigen,
   2. vWF activity,
   3. Factor VIII activity (the latter is decreased in both severe vWF deficiency and Hemophilia A),
   4. Factor IX activity (decreased in both severe vWF deficiency and Hemophilia B), and
   5. Factor XI activity.

I would be less concerned about lupus anticoagulant, since lupus anticoagulant antibodies are actually prothrombotic due to increased platelet adhesion and aggregation.

Question 3

What is the difference between Hemophilia A and Hemophilia B?

(An 8-year-old, 46 kg, male presents for tonsillectomy. The mother reports that her son has a history of asthma, and that he has given prednisone 8-9 weeks ago because his asthma was “acting up”. His anesthetic history includes prolonged bleeding following a complicated tooth extraction 3 years ago.)

Answer 3

Hemophilia A (85% of hemophiliacs) and Hemophilia B (14% of hemophiliacs) are both sex-linked recessive disorders that are clinically indistinguishable.

While Hemophilia A leads to deficient or defective Factor VIII, Hemophilia B results in deficient or defective Factor IX.

Both disorders are associated with a prolonged aPTT (the aPTT may be normal in mild disease) and a normal PT, but may be distinguished from each other by determining the patient’s Factor VIII and Factor IX activity levels.

Question 4

The lab reports that his factor VIII activity level is 5%.

Would desmopressin be helpful?

(An 8-year-old, 46 kg, male presents for tonsillectomy. The mother reports that her son has a history of asthma, and that he has given prednisone 8-9 weeks ago because his asthma was “acting up”. His anesthetic history includes prolonged bleeding following a complicated tooth extraction 3 years ago.)

Answer 4

Desmopressin (DDAVP) may be helpful in preparing a patient with mild hemophilia A (Factor VIII levels > 5%) for surgery because – it rapidly increases the amount of circulating factor VIII and vWF by inducing the release of these factors from endothelial cells.

The subsequent increase in vWF, a carrier molecule for factor VIII, may provide further benefit by decreasing the clearance of Factor VIII from the circulation.

However, it should be recognized that DDAVP is subject to tachyphylaxis, is most effective in patients with Factor VIII levels > 5%, and exhibits a variable patient response that can be significant.

Question 5

You explain the anesthetic plan to the patient and his mother.

When you are done, the child tells you that he does not want to have his tonsils taken out.

The mother tells him, “You are having this surgery”.

What would you do?
(An 8-year-old, 46 kg, male presents for tonsillectomy. The mother reports that her son has a history of asthma, and that he has given prednisone 8-9 weeks ago because his asthma was “acting up”. His anesthetic history includes prolonged bleeding following a complicated tooth extraction 3 years ago.)

Answer 5

Children should be involved in making decisions related to their healthcare to the extent that they are able (capacity).

Furthermore, in the case of an elective procedure that can be delayed with very little risk, the refusal of assent may be ethically binding.

Therefore, I would attempt to identify and allay his concerns.

If I believed that anxiety and fear were the primary reasons for his refusal, I would suggest removing him from the preoperative area, allowing him some time to compose himself, and offering him a preoperative anxiolytic.

If he continued to refuse the procedure, despite these interventions, I would initiate a discussion with the patient, his mother, and the surgeon about the risks and benefits of delaying the procedure until he was of sufficient age and maturity to make his own health care decisions, independent of his parents.

Question 6

How would you prepare this patient for surgery?

(An 8-year-old, 46 kg, male presents for tonsillectomy. The mother reports that her son has a history of asthma, and that he has given prednisone 8-9 weeks ago because his asthma was “acting up”. His anesthetic history includes prolonged bleeding following a complicated tooth extraction 3 years ago.)

Answer 6

In preparing this patient for surgery, I would provide:

1. a short-acting B2-agonist (i.e. albuterol), to optimize his asthmatic condition and minimize the risk of bronchoconstriction during intubation;
2. virally inactivated factor VIII concentrate to increase his factor VIII levels to 100%, to prevent excessive bleeding (while factor VIII levels of 30% should prevent surgical bleeding, most authors recommend correcting levels to 50-100% of normal, depending on the bleeding risk);
3. perioperative steroid supplementation, to avoid adrenal insufficiency in this patient who received prednisone several weeks ago for his asthma; and
4. atropine to reduce secretions and augment upper airway function (there is some evidence that the muscarinic effects of atropine on the upper airway may decrease the risk of post-adenotonsillectomy respiratory complications).