Anaesthesia for child with Down syndrome

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INTRODUCTION Commonest chromosomal abnormality John Langdon Down, described the syndrome in 1866 Down’s syndrome is also referred to as Trisomy 21 due to the presence of an extra copy of chromosome number 21 Incidence of 1.5 per 1000 live births Maternal age is a risk factor for down’s syndrome A 20 year-old mother has a risk of 1 in 2000 A 35 year-old mother has risk of 1 in 400. The risk increases to 1 in 40 at 45 years of age

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Multi-system problems in Down’s syndrome


GENERAL APPEARANCE Small for age, generalised hypotonia, lax joints Flat occiput, short neck, small low set ears, flattened nasal bridge with midface hypoplasia and protuding large tongue Prominent epicanthic folds (fold of skin of the upper eyelid covering the inner corner of the eye) and upward slanting eyes (Mongoloid slant) Brushfield spots (light coloured spots near the periphery of the iris) Single transverse palmar crease of the hand (Simian crease) Larger than normal space between the big and second toes

Cardiovascular system:

Cardiovascular system Atrioventricular septal defects (AVSD) (40%) Ventricular septal defects (VSD) (27%) Patent ductus arteriosus (PDA) (12%) Atrial septal defect (10%) Tetralogy of Fallot (TOF) (8%) Pulmonary vascular disease

Respiratory system:

Respiratory system Recurrent respiratory tract infections Sub-glottic stenosis Enlarged tongue Enlarged tonsils and adenoids Obstructive sleep apnoea

Central nervous system:

Central nervous system Developmental delay and moderate to severe mental retardation Microcephaly Epilepsy (5-10%)

Skeletal system:

Skeletal system Atlanto-axial instability: This is seen in about 15% of patients. The instability may be due to abnormality in the C1 vertebra (atlas) or C2 vertebra (axis), with laxity of the transverse ligament. Spinal cord compression is seen in 2% of children. There may also be laxity of other joints (finger, thumb, elbow or knee). Generalised poor muscle tone

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Endocrine system Down’s syndrome is associated with hypothyroidism in about 50% of patients Haematology Acute lymphoblastic leukaemia, acute myeloid leukaemia (1.5%) Polycythaemia

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GIT Duodenal atresia is 300 times more common than in the general population GERD Hirschsprung disease Immunology Impaired cellular immunity - risk of infectious disease (especially pneumonia) is 12 times more common than in the general population Others Obesity Difficult peripheral venous access Dental problems

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PREOPERATIVE PREPARATION History and examination The Anaesthetist should try to explain (to the child) what is going to happen in a language appropriate to the child The presence of the parent or carer will be greatly reassuring for the child Play therapists may also have a role to ensure smooth induction of anaesthesia.

Cardiovascular system:

Cardiovascular system Structural heart disease in children with down’s syndrome is common Symptoms suggestive of congenital heart disease include Failure to thrive Breathlessness Fatigue on exertion Unexplained ‘funny turns’ Important signs include Central cyanosis Finger clubbing Respiratory distress Signs of cardiomegaly with displaced cardiac apex Hepatomegaly The presence of a heart murmur with associated ‘thrill’ (palpable murmur).

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The characteristics of pathological murmurs are as follows: All murmurs associated with cardiac signs or symptoms All pansystolic and diastolic murmurs Late systolic murmurs Loud murmurs, those associated with a thrill, or continuous murmurs A detailed cardiovascular examination, ECG, and ideally a cardiology opinion and echocardiography should be obtained in all children with down’s syndrome before proceeding with surgery

Respiratory system:

Respiratory system A thorough examination of the respiratory system and airway is necessary to rule out a difficult airway or intubation. History of snoring during sleep associated with daytime lethargy and somnolence, behavioral changes, poor concentration and inattention at school may suggest the presence of severe obstructive sleep apnoea (OSA). Children with severe OSA are not suitable for day case surgery.

Atlanto-axial instability is seen in 15% of children with Down’s syndrome.:

Atlanto-axial instability is seen in 15% of children with Down’s syndrome . History of neck pain, limited neck mobility or head tilt, change in gait pattern, clumsiness, abnormal neurological reflexes, abnormal sensation or presence of bladder and bowel dysfunction suggest the possibility of neck problems. Atlanto-axial instability is diagnosed by identifying an increase in the distance between the posterior border of the arch of the atlas and the anterior border of the odontoid peg on lateral flexion and extension radiographs of the cervical spine (atlantodental interval, ADI)

In children over eight years old, the ADI should be 3 mm or less while in younger children the ADI should be 4 mm or less (some consider up to 5 mm as normal):

In children over eight years old, the ADI should be 3 mm or less while in younger children the ADI should be 4 mm or less (some consider up to 5 mm as normal) Figures 1 & 2. Line diagram of the atlas (C1) and axis (C2) vertebrae showing increased atlantodental interval (shaded area) in extension. The odontoid peg (dens) of C2 projects from the superior surface of the body of C2 and may move to cause spinal cord compression


ANAESTHESIA PLAN Discussion with the parents/carers regarding the mode of induction of anaesthesia and any associated procedures such as use of analgesic suppositories Topical local anaesthetic (EMLA/ametop) should be applied to facilitate intravenous cannulation If the child is anxious and unco-operative, sedation with oral midazolam (0.5 mg/kg – maximum dose 20mg) or oral temazepam (0.5-1 mg/kg – maximum 20mg) will be helpful. Occasionally, intra-muscular ketamine may be required. Premedication should be used cautiously in children with severe osa.

The following problems should be anticipated and appropriate measures should be taken::

The following problems should be anticipated and appropriate measures should be taken: Possibility of difficult airway and laryngoscopy due to enlarged tongue, tonsils and adenoids. The incidence of airway obstruction and difficult intubation have been reported to be 1.83% and 0.54% respectively. Successful use of a laryngeal mask airway (LMA) has been reported in a child with Down’s syndrome with atlanto-axial dislocation. A small tracheal tube may be needed due to sub-glottic stenosis. Care of the neck during laryngoscopy and intubation is necessary. Avoid forceful flexion and extension of the neck in these patients due to the possibility of atlanto-axial instability. The head should ideally be placed in neutral position during surgery. Anticipate difficult venous access in younger children; gaseous induction with halothane or sevoflurane will be helpful. If gastro-oesophageal reflux is present, premedication with an H2 antagonist such as ranitidine may be beneficial


INTRAOPERATIVE MANAGEMENT Monitoring should include ECG, pulse oximetry, end tidal CO2 monitor, blood pressure and temperature monitor. Children with Down’s syndrome are prone to infection and all invasive lines should be inserted under full aseptic precautions. Abnormal response to anaesthetic drugs has not been noted but sensitivity to atropine has been reported Complications under general anaesthesia may include bradycardia (3.66%), bronchospasm (0.43%) and hypotension. Postoperative neurological sequelae due to atlanto-axial subluxation are rare. Consideration should be given regarding antibiotic prophylaxis for infective endocarditis in children with structural heart lesions.


POSTOPERATIVE MANAGEMENT Patients should be observed closely in the recovery room until full recovery from anaesthesia. The presence of parents/carers can help to avoid disturbances in the postoperative period. Hypotonia may affect the ability to maintain airway. This can be managed by simple airway manoeuvres (head tilt, chin lift or jaw thrust), or appropriate positioning of the child in the lateral position to maintain patency of the airway. Use of airway adjuncts (oropharyngeal or nasopharyngeal airway) may be helpful

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The incidence of Post intubation croup is estimated to be about 1.83% (8). This can be treated by administering humidified oxygen, nebulised adrenaline (400 mcg/kg – max. dose 5 mg i.e; 5 ml of 1:1000 adrenaline) and intravenous dexamethasone (initial dose 250 mcg/kg followed by 100 mcg/kg six hourly for 3 doses). If nebulised adrenaline is used, the ECG should be monitored and it should be stopped if the heart rate exceeds 200/min or if any arrhythmias are seen. Chronic chest infections are common as is postoperative atelectasis and respiratory tract infection. Infections are treated preoperatively and postoperative chest physiotherapy may be prescribed to minimize chest complications Adequate analgesia should be prescribed to keep the child comfortable. Regional techniques may avoid the respiratory depressant effects of opioids and improve compliance with chest physiotherapy. Some patients are able to use PCA.

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