6. Down’s Syndrome

Question 1

Intro

Answer 1

Down’s syndrome (DS) is one of the most common chromosomal abnormalities,

occurring in approximately 1 in 700 live births.

It is due to the presence of either a whole or
part of an extra 21st chromosome

and is hence termed trisomy 21.

In the majority of these cases
the cause is non-disjunction of the chromosomes (95%) but it can also be due to translocation.

Question 2

What are the anaesthetic considerations in a patient with

Down’s syndrome?

Answer 2

Patients with DS may have several
anatomical and physiological abnormalities
that can cause problems during anaesthesia.

Consequently, these cases should be
discussed with a consultant anaesthetist.

The anaesthetic considerations are best
described in terms of organ systems.

Question 3

> Nervous system:

Answer 3

• Learning difficulties may lead to an
anxious and uncooperative patient.

Try to establish a rapport with the patient, 
tailor your explanation of
the anaesthetic to their
 level of understanding and 
consider premedicating
with an anxiolytic or sedative agent.

Question 4

Respiratory

Answer 4

1 • Atlanto-axial and atlanto-occipital instability,

which is partly due to ligament laxity
and can lead to spinal cord compression.

Great care must be taken when
positioning the head during intubation.

Ask preoperatively about symptoms of
spinal cord compression
(numbness and tingling of the arms and fingers)
and consider requesting flexion extension
views of the C-spine.

2  Airway
• Micrognathia, 
small mouth, 
macroglossia, 
and short neck may all
contribute to difficulties during intubation 
and therefore these must be
anticipated. 

A difficult airway trolley with
appropriate emergency drugs
should be ready and available.

In reality, increased ligament laxity
usually means that intubation is not difficult.

3
• Excessive salivation
can obscure the view during laryngoscopy and
can be an aspiration risk.
Consider pre-medicating with an antisialogogue.

4
• Adeno-tonsillar hypertrophy and
oro-pharyngeal hypotonia may lead
to obstructive sleep apnoea.

Elicit features of this condition during
the pre-operative assessment
(snoring, daytime somnolence,
ECG evidence of right heart strain).

Upper airway obstruction can worsen
after anaesthesia and opioids so
consider extended recovery or HDU
environment in the post-operative period.

5
• Subglottic and tracheal stenosis –
consider using a smaller than
predicted endotracheal tube.

Question 5

> Cardiovascular system:

Answer 5

• Congenital heart disease occurs in up to
50% of patients with DS
and common abnormalities include
ASD, VSD, PDA and TOF.

Apart from TOF, all of these defects cause
a left-to-right shunt,
which can ultimately lead to pulmonary hypertension.

Full review of the medical notes with a thorough history and examination is essential.

If congenital heart disease is suspected,
patients must be investigated and optimised
by a cardiologist prior to surgery.

Consider the need for prophylactic antibiotics at induction to prevent endocarditis.

• Difficult venipuncture –
this can be due to obesity and/or learning
difficulties.

Consider pre-medicating patients with
topical local anaesthetic agents
applied to potential venipuncture sites.

Question 6

> Gastrointestinal system:

Answer 6

• Gastro-oesophageal reflux, 
duodenal atresia and 
gastric paresis occur
with increased frequency and all 
increase the risk of aspiration during
induction of anaesthesia. 

Therefore, consider early NGT placement,
pre-medicating with prokinetic agents and
antacids and/or a rapid
sequence induction technique.

Question 7

> General:

Answer 7

• Obesity
• Increased incidence of hypothyroidism
• Impaired immunity
• Increased incidence of leukaemia
• Increased incidence of hepatitis B