17.1 Trisomy 21 Flashcards
A 9-year-old child with Down’s syndrome
is scheduled for an adenotonsillectomy.
a) List airway/respiratory (30%),
features of the syndrome relevant to the anaesthetist.
Airway and respiratory:
> > Subglottic or tracheal stenosis: *
may require smaller tube size.
> > Atlantoaxial instability:
care with neck manipulation,
especially extension
(this is an issue for positioning during tonsillectomy
as well as during anaesthetic airway management).
> > Cervical spine ankylosis (Klippel-Feil):*
limits neck extension,
may make intubation difficult.
> > Obstructive sleep apnoea (OSA) caused
by craniofacial abnormalities
(macroglossia, narrow midface, oropharyngeal hypotonia, micrognathia, small mouth, short neck, adenotonsillar hypertrophy):
mask ventilation may be challenging.
> > Predisposition to
lower respiratory tract infection due
to hypotonia,
gastro-oesophageal reflux,
reduced immunity.
cardiovascular (10%) features of T21 relevant to the anaesthetist.
Cardiovascular:
» Congenital heart disease:
increased risk of atrioventricular canal defects,
atrioseptal and ventriculoseptal defects,
patent ductus arteriosus,
tetralogy of Fallot.
> > Pulmonary hypertension
with or without associated cardiac lesions.
> > Risk of conduction defects
following repair of congenital lesions.
> > Valve abnormalities in adulthood: mitral valve prolapse, aortic regurgitation.
and neurological (10%) features of T21 relevant to the anaesthetist.
Neurological:
> > Hypotonia: care with positioning.
> > Epilepsy.
> > Intellectual impairment:
consent, cooperation.
> > Postoperative agitation.
> > Reduced MAC requirement possibly
due to reduced central
catecholamine activity.
> > Increased sensitivity to
sedatives and anaesthetic agents.
b) What are the general principles involved in the preoperative (15%) management of this patient with Down’s syndrome?
Preoperative:
» Full patient assessment involving
parents or carers.
Explanations and consent
appropriate to patient’s understanding
and sensory function.
Arrange ward visit and opportunity
to meet staff to help manage anxiety.
> > Airway:
assess for difficulties associated with
Down’s syndrome, as
detailed previously.
Consider radiological assessment
of cervical spine if atlantoaxial
instability is likely.
> > Respiratory: assess for possibility of
OSA, especially considering that
the patient is requiring an
adenotonsillectomy.
Assess for symptoms and signs
of lower respiratory tract infection.
Consider antisialagogue
premedication with atropine.
> > Cardiovascular:
assess exercise capacity,
history of cardiac defects and
corrective surgery.
Discuss with the cardiology or
cardiothoracic team
caring for the patient if necessary.
Right heart strain on ECG may be
indicative of pulmonary hypertension –
investigate with echocardiogram.
Topical local anaesthetic to be
used in preparation for cannulation.
> > Neurological: consider radiological
assessment of cervical spine if
atlantoaxial instability is likely.
> > Endocrine:
consideration of
thyroid function testing
and blood glucose,
if diabetes is a possibility.
> > Gastrointestinal:
assess for presence of
gastro-oesophageal reflux.
Premedicate with proton-pump
inhibitor and plan for rapid sequence
induction if indicated.
What are the general principles involved in the
intraoperative (25%) management of this patient with Down’s syndrome?
Intraoperative:
» Ensure carer or parent accompanies
child to theatre.
Involve play specialist if required.
> > Airway:
intubate,
possibly rapid sequence.
Have range of tube sizes available,
smaller tube may be required if subglottic stenosis
(and children with Down’s often
smaller than other children of same age).
Consideration of need for
difficult intubation equipment.
Oropharyngeal airway may be required to facilitate mask ventilation.
> > Cardiovascular:
cannulation may be challenging due to tendency to raised BMI.
Prone to bradycardia after induction –
ensure atropine and glycopyrrolate
prepared according to child’s weight.
Avoid hypotension in order to preserve
spinal cord perfusion.
ECG monitoring due to
possibility of arrhythmia.
> > Neurological:
care with positioning
due to ligamentous laxity
and atlantoaxial instability –
care with all neck movement (even if
radiographically normal), especially with the hyperextension normally required for tonsillectomy.
Avoidance of long-acting opioids and
consideration of lower MAC.
> > Infection, immunology:
scrupulous attention to asepsis
due to increased
risk of infection.
What are the general principles involved in the
postoperative (10%) management of this patient with Down’s syndrome?
Postoperative:
» Risk of OSA.
Patient may need CPAP,
overnight oxygen saturations monitoring,
and close nursing observation.
> > Predisposed to postoperative agitation.
Prolonged recovery stay and
higher intensity nursing care on
a higher dependency ward may be indicated
> > Postoperative pain relief.
Consideration must be given to communication
abilities.
Parent or carer who knows child well may be required to assist in pain assessment.
Avoid long-acting opioids especially
if the patient has OSA.
> > Monitor for signs of postoperative infection, especially lower respiratory tract infection.
Alert parents/carers to signs to be aware of.
> > Monitor for signs of spinal cord
compromise and investigate promptly.
b) What are the potential
problems during induction of
anaesthesia and initial airway
management in this patient?
(6 marks)
Communication difficulties:
> > It may be more challenging to establish
good communication and rapport
with this child.
Parents, carer and/or play specialist
may be required to assist.
Airway management:
» Increased risk of gastro-oesophageal r
eflux may dictate plan for airway
management:
intubation, possibly rapid sequence intubation.
> > Increased likelihood of difficulty with mask ventilation due to craniofacial changes associated
with Down’s syndrome (as detailed above) and with
adenotonsillar hypertrophy.
May need oropharyngeal airway.
> > Possibility of difficulty intubating due to subglottic stenosis – smaller tube sizes should be available.
> > Neck extension may be limited by Klippel-Feil or by the presence of atlanto-axial instability – videolaryngoscopy or asleep fibreoptic intubation
may be indicated.
