Down syndrome

Question 1

What proportion of pts with Down syndrome have tracheal stenosis? how does this impact Anaesthetic management?

Answer 1

5%, 25% require a smaller tube, 33% experience post-extubation stridor with a standard tube

Question 2

What proportion of DS pts have a congenital cardiac malformation? what are the common ones?

Answer 2

15-60%, usually AV (ASD, VSD, AVSD, PDA), some may have tetralogy of fallot, cleft anterior mitral leaflet (slits or holes, contributes to MR), pulmonary HTN is more common (28% incidence, due to sleep-disordered breathing & congenital heart defects)

Question 3

What are the 4 defects in tetralogy of fallot?

Answer 3

VSD, pulmonary stenosis, overriding aorta (arises from both ventricles) & RV hypertrophy

Question 4

What proportion with DS have epilepsy?

Answer 4

10%

Question 5

What’s a common endocrine condition associated with DS?

Answer 5

Hypothyroidism

Question 6

What are some findings on CBC that may be associated with AML?

Answer 6

May have isolated anaemia or thrombocytopenia, either leukocytosis or even leukopenia

Question 7

How high is the risk of AML in DS ?

Answer 7

20-fold higher

Question 8

What are some features of Down syndrome which may increase risk of a difficult airway?

Answer 8

Thicker neck (and higher risk OSA) and higher risk obesity & GORD
Risk atlanto-axial instability (may require C-spine precautions)
Macroglossia- more likely to obstruct with BMV, may be a challenge to laryngoscopy
May have tracheal stenosis (5%, 25% require smaller tube, 33% experience post-extubation stridor with standard tube)
Chronic LRTI common
Higher degree of vagal tone (more prone to bradycardia, particularly during laryngoscopy/insufflation/movement of peritoneum
More risk congenital heart defects