Anaesthetic management in syndromic paediatric patients undergoing dental procedures: A case series

INTRODUCTION

A special child is defined as having physical, mental, sensory, cognitive, emotional, or behavioural disabilities that require special medical intervention, specialised services, or programmes.^[1]

Tooth decay is a common problem in this set of patients due to higher amounts of carbohydrates in the diet, sugar content in medications, and poor oral hygiene with lower salivary flow in the oral cavity.^[2] Patients on long-term antiepileptics have gingival hyperplasia, bleeding, petechiae and decreased platelet aggregation. Single-session dental treatment is appropriate and feasible.^[3] We present here a case series of paediatric patients who presented with congenital rubella, West syndrome, Down’s syndrome, mucopolysaccharidosis (MPS) and Pelizaeus-Merzbacher disease (PMD) posted for full mouth rehabilitation under general anaesthesia (GA) [Figure 1].

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Figure 1:

Syndromic patients. (a) Congenital rubella syndrome, (b) Cerebral palsy with West syndrome, (c) Down’s syndrome, (d) Mucopolysaccharidosis, (e) Pelizaeus-Merzbacher disease

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CASE SERIES

DISCUSSION

Multisystem involvement affecting syndromic children poses challenges to the anaesthesiologist. Apart from routine investigations, thyroid function, serum calcium and serum glucose may be required. Echocardiography is done to rule out congenital heart disease. Parents need to be counselled for the high risk involved in general anaesthesia. Placement of an intravenous cannula can be challenging due to poor nutrition, muscle wasting and spasticity of limbs. Premedication with opioids carries the risk of respiratory depression but is often required for separation from parents. These patients need anticholinergic premedication as they have excessive secretions due to overactive salivary glands. Dysfunction of cranial nerves reduces clearance of pharyngeal secretions which needs thorough suctioning at the time of induction and extubation. Difficult intubation cart should be ready in craniofacial malformed patients.^[4] Short trachea, tracheomalacia and bronchomalacia are some associated airway anomalies. Macroglossia, abnormal dentition and mandibular abnormalities cause difficult mask holding. Most dental procedures need nasal intubation, so adenoidal hypertrophy must be ruled out preoperatively. Pre-existing OSA and velopharyngeal dysfunction predispose to postoperative airway obstruction. Long-term anticonvulsants can alter liver function, hence anaesthetic drugs need careful titration. Fluid restriction may be needed in patients with cardiac abnormality. Padding of pressure points and careful positioning are required in osteoporotic and spastic children. Body temperature is maintained by warming blankets as malnourishment and hypothalamic dysfunction predispose to hypothermia and delayed emergence.

The syndromic child is not a good candidate for dental chair anaesthesia as the margin of safety for any type of sedation or inhalational technique is narrow due to limited resources, inadequate monitoring and lack of expertise in resuscitation.

West syndrome patients have spasticity of limbs, which is reduced after induction. Careful padding and positioning is done to prevent iatrogenic fractures.^[5]

PMD is a type of leukodystrophy affecting the nervous system. Due to pharyngeal muscle hypotonia, these patients need enteral feeding to prevent poor nutrition and aspiration.^[6] Due to prolonged neuromuscular blockade, a low dose of muscle relaxants and neuromuscular monitoring is advised under GA.

The significant features of Down’s syndrome are atlantoaxial instability (15%), subglottic stenosis, obstructive sleep apnoea, enlarged tongue and tonsils. Associated cardiac defects are atrial septal defect, tetralogy of Fallot, ventricular septal defect and pulmonary hypertension (PHT). These patients are prone to bradycardia, hypotension and arrhythmias if induced with a large dose of sevoflurane, so gradual dosing is advised.^[7]

The MPS patient in this case series was type IIIC (Sanfilippo), with a deficiency of acetyl coenzyme A. Progressive cellular and multisystemic damage occurs due to partially degraded glycosaminoglycans (GAG). This defect occurs due to a deficiency of specific lysosomal enzymes. As years pass, new GAG deposition may change the previous airway anatomy, affect cardiac and pulmonary functions, causing problems like OSA. Airway abnormalities predispose to obstruction and difficult intubation, with possible “cannot intubate/cannot ventilate” scenarios. It is safer to have the patient spontaneously breathing until successful intubation is done or (at least) confirmation of mask ventilation. He was extubated and shifted to the PICU with a nasal airway inserted for 24 hours, similar to the case managed by Walker R et al.^[8]

Maintaining the systemic vascular resistance (SVR) to PVR ratio is of utmost importance in congenital rubella patients posted for non-cardiac surgery. It is prudent to avoid hypercarbia, hypoxia, hypothermia and acidosis to maintain PVR. An increase in right to left shunt, reduced pulmonary perfusion and hypoxaemia may result due to a fall in SVR. Nitrous oxide is avoided due to evident PHT. It is beneficial to maintain a slightly lower heart rate and to augment preload in patients with pulmonary stenosis.^[9]

CONCLUSION

When preparing a child with a syndrome for GA, it is important to consider all systems that may be affected. Factors that may impact overall care and outcomes, like poor nutrition, repeated URTI, difficult intubation and chronic anticonvulsant drugs should be well managed for the safe conduction of syndromic patients. Multidisciplinary team involvement is mandatory for better care.

Careful planning includes anticipation and preparedness to deal with the airway and altered physiology with choice and dosing of anaesthetic agent and proper patient positioning. Postoperative care must be individualised and depending on the severity of clinical condition, high-level care may be required.