202406232326
Status:
Tags: Paed
Pre-op paediatric assessment
Syndromes associated with difficult airways
| Syndrome | Abnormalities | Difficult mask ventilation/intubation |
|---|---|---|
| Apert syndrome | Midface hypoplasia | Mask ventilation |
| Beckwith Wiederman syndrome | Macroglossia | Both |
| Crouzon syndrome | Midface hypoplasia | Mask ventilation |
| Downs syndrome | Macroglossia, brachycephaly, atlanto-axial instability | Both |
| Goldenhar syndrome | Midface/hemifacial hypoplasia | Intubation |
| Mucopolysaccharidoses, e.g. Hurler's/Hunter's syndrome | Macroglossia, craniofacial abnormality, temporomandibular joint dysfunction adenoid and tonsil hypertrophy | Both |
| Klippel–Feil syndrome | Cervical fusion | Intubation |
| Pierre Robin syndrome | Micrognathia, retrognathia | Intubation |
| Treacher Collins syndrome | Micrognathia | Intubation |
Syndromes associated with congenital heart disease
| Syndrome | Associated cardiac anomalies |
|---|---|
| Down syndrome (trisomy 21) | Atrioventricular septal defects, atrial septal defects, ventricular septal defects, tetralogy of Fallot |
| Turner syndrome (45X) | Coarctation of the aorta, bicuspid aortic valve, aortic stenosis |
| DiGeorge syndrome (22q11.2 deletion) | Tetralogy of Fallot, aortic arch abnormalities, truncus arteriosus |
| Noonan syndrome (RAS-MPK) | Pulmonary stenosis, atrial septal defect, hypertrophic cardiomyopathy |
| Williams syndrome (7q11.23) | Aortic stenosis, peripheral pulmonary stenosis |
Premed
| Drug | Dose (oral unless stated otherwise) | Onset | Comments |
|---|---|---|---|
| Midazolam | 0.5 mg/kg | 20–30 minutes | Risk of paradoxical excitement |
| Lorazepam | 50–100 micrograms/kg | 30–60 minutes | Risk of prolonged sedation (up to 6 hours) |
| Clonidine | 2–4 micrograms/kg | 30–60 minutes | Unfavourable cardiovascular effects |
| Ketamine | 2–8 mg/kg | 30–40 minutes | Nurse in a quiet, calm environment. Emergent hallucinations may be unpleasant |
| Diamorphine | 0.1 mg/kg intranasal | 15–30 minutes | Mild, early stinging sensation |
Summary of the general recommendations for the perioperative management of patients with neuromuscular disorders
| Perioperative domain | Recommendation | Explanation |
|---|---|---|
| Surgical setting | 24-hour high care facility available | Poor respiratory reserve and high risk of complications. |
| Preoperative assessment | Multidisciplinary team involvement | Complexity of patient |
| Anaesthetic technique | Regional anaesthesia ± general anaesthesia | Minimize risk of complications, e.g respiratory depression |
| Preoperative fasting | - Schedule as first case - Gastric ultrasound |
Risk of hypoglycaemia Risk of gastric dysmotility |
| Body temperature management | Use preoperative warming techniques | Vulnerable to hypothermia |
| General anaesthesia | Short-acting agents | Minimize respiratory depression |
| Neuromuscular blockers | - Avoid suxamethonium | - Risk of hyperkalaemia |
| - Reduce dose of non-depolarizing muscle relaxant | - Increased sensitivity | |
| - Use sugammadex | - Sensitive to residual blockade | |
| - Use quantitative train-of-four monitoring | ||
| Volatile anaesthetics | Avoid prolonged use | RYR1 diseases – malignant hyperthermia (absolute contraindication) Muscular dystrophy – rhabdomyolysis (relative) |
| Propofol | Use total intravenous anaesthesia However caution in mitochondrial disease |
Risk of propofol infusion syndrome in mitochondrial disease |
| Postoperative | Monitor for 24 hours | Respiratory depression Rhabdomyolysis |
| Enhanced recovery pathway | Reduced complications | |
| Medical alert card | Patient safety |