Congenital chromosomal aberration characterized by the presence of an extra chromosome 21 (trisomy 21 nondisjunction) or with one chromosome 21 containing the genetic content of two copies of chromosome 21 (trisomy 21 by translocation). Average prevalence: 1/800 (parallel increases with the age of the mother: 1/1500 to 20 years 1/100 to 40 years). The translocation trisomy 21 is always inherited from a parent.
Anesthetic implications :
- Developmental delay of varying degrees.
- Immunosuppression (high risk of leukemia).
- Small.
- Sometimes associated with duodenal atresia, Hirschsprungs disease.
- Congenital heart-disease in 50% of cases ( atrioventricular canal).
- Short neck,
- subglottic stenosis.
Mandibular hypoplasia with narrow pharynx and macroglossia:
- Obstructive apneas.
- Hypotonia.
- Risk of instability of C1 to C 2 and hypoplasia of the posterior arch of C 1 : dynamic shots of the cervical spine and neurosurgical opinion if neurological signs or symptoms (stiff neck, change in behavior).
Attention to the positioning of the head and neck during intubation. Avoid significant rotation of the head.
Significant risk of bradycardia and hypotension in cases of inhalational induction with sevoflurane, even in the absence of heart defects
Risk of airway obstruction on induction and awakening (hypotonia, obstructive sleep-apnea)
Translated from: sites.uclouvain.be/anesth...
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Macroglossia may make laryngoscopy difficult, as will minimising neck extension in consideration of possible atlanto-occiptal instability.
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Atlanto-axial instability is present in a significant number of patients with Down's Syndrome putting them at risk of atlanto-occipital subluxation/dislocation.
dx.doi.org/10.1007/BF0300...
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