Anesthesia for the child with autism

INTRODUCTION — Children with autism spectrum disorder (ASD) require health services more commonly than children without these disorders, and may require anesthesia for surgical, dental, or diagnostic procedures. As the prevalence of autism is increasing, anesthesiologists are increasingly likely to care for children with ASD.

Children with ASD are often unable to cope with the unfamiliar perioperative environment, which can trigger behaviors that make the experience challenging for the patient, parents or caregivers, and clinicians. Advance planning, with the parent or caregiver actively involved in creation of an individualized perioperative plan, is important for a smooth perioperative course.

This topic will review perioperative and anesthetic management of children with ASD, and our multidisciplinary patient-centered approach. These principles may be applicable to children with other emotional or behavioral disorders.

Clinical features, epidemiology, and other aspects of management of ASD are discussed separately.

●(See "Autism spectrum disorder in children and adolescents: Clinical features".)

●(See "Autism spectrum disorder (ASD) in children and adolescents: Terminology, epidemiology, and pathogenesis".)

●(See "Autism spectrum disorder in children and adolescents: Overview of management".)

IMPLICATIONS FOR ANESTHESIA — By definition, autism spectrum disorder (ASD) is characterized by deficits in social communication and interaction; restrictive, repetitive patterns of behavior and interests that may include inflexible adherence to nonfunctional routines or rituals; and hyper- or hyposensitivity to sensory input (see "Autism spectrum disorder in children and adolescents: Clinical features"). Although there is a range of severity of ASD, most children with ASD are uncomfortable with or upset by unexpected or new situations. Challenging behaviors may be triggered by the unfamiliar perioperative environment and may make anesthesia related procedures (eg, application of a face mask for induction, intravenous [IV] catheter placement, application of monitors) difficult or impossible. Children with more severe ASD may exhibit verbal or physical aggression, antisocial or disruptive behavior, temper tantrums, screaming, panic attacks, and self-injurious behavior, and may have aberrant responses to sensory stimuli. A goal for perioperative care should be to avoid these difficulties, for patient comfort, and to avoid negative associations with the hospital environment.

Children with ASD may require sedation or general anesthesia in the hospital for procedures that would ordinarily be done in a clinic without systemic medication.

There is a paucity of literature to guide perioperative anesthetic management, mostly limited to case reports and series, and reports of institutional protocols [1].

MULTIDISCIPLINARY PREOPERATIVE PREPARATION — Careful multidisciplinary planning is required for a safe and stress-free perioperative period for the child with autism spectrum disorder (ASD) and for caregivers, even for minor procedures. Perioperative assessment and management may involve the patient's parents or caregivers, anesthesiologist, surgeon or proceduralist, primary care clinician, psychiatrist or behavioral specialist, and child life team, in addition to the perioperative nursing and administrative staff. The preoperative assessment process should produce a patient-specific flexible perioperative plan. Every health care provider involved in the patient's care should be familiar with the perioperative plan, and this should become a part of the patient's medical record.

Institutional protocol — Clinicians with extensive experience caring for children with ASD agree that existence of an institutional perioperative protocol improves the management of these children [2], and several such protocols have been published [3-8]. These protocols are necessarily institution-specific, but have generally similar goals, including:

●Advance identification of patients with ASD and notification of care providers,

●Involving the parents or caregivers in creation of a comprehensive individualized plan for all aspects of perioperative care, and

●Staff education

At our institution, a clinical nurse reviews the chart when a procedure is scheduled and refers the record of a patient with ASD to a child life specialist (CLS). The CLS contacts the parents or caregivers, creates a coping plan, and enters the plan into the patient's electronic medical record. An anesthesia clinician is contacted in advance if the child has challenging behavior that requires medical management.

Training of health care providers is mandatory for smooth perioperative transitions for patients with ASD. Lack of knowledge, training, and confidence in taking care of children with ASD may result in injury to both the health care provider and the patient and also may lead to maladaptive behavior postoperatively [3,6,9]. During implementation of our institutional protocol, training for care of patients with ASD and severe behaviors was provided by psychologists and a perioperative team of nurses, CLS, and anesthesiologists during multiple grand rounds presentations.

Early preanesthesia evaluation — Preanesthesia preparation should begin as soon as the surgical or diagnostic procedure is scheduled. Goals for preanesthesia evaluation should be to facilitate a smooth admission process, minimize the need for evaluation on the day of the procedure, and reduce delays.

Active involvement of parents or caregivers — The parents or caregivers are best able to provide advice for behavioral management, and their input should be actively solicited. Parents or direct caregivers should be contacted in advance of the day of the procedure to gather information on severity of ASD, past health-related and anesthesia experiences, effects of premedication used in the past, preferred method of administration of medication, cognitive level, methods of communication, motivators, stressors or anxiety triggers, sensory challenges, coping strategies, comfort objects and preferences for food and drink. The parent/caregiver questionnaires we use are shown in tables (table 1 and table 2).

Parents or caregivers should be given specific information about the plans for the day of the procedure, including preoperative fasting, expected wait times, plan for premedication, and expected sequence of preoperative and postoperative events. If appropriate, parents or caregivers are encouraged to discuss and reinforce the perioperative process with the patient. The child's behavioral specialist can help with role playing activities, visual symbols [10], play therapy, or rehearsal of the expected sequence of events [11]. As an example, in one case report, a successful individualized program of desensitization to anxiety-provoking perioperative stimuli was created by a severely autistic boy's psychologist [12]. The program involved mirroring activities using a large doll, with tokens and rewards for completing desired actions (eg, allowing stethoscope placement, placement of anesthesia mask), using twice weekly sessions over the course of six weeks. This type of protocol is time consuming and labor intensive, and may not be possible at many institutions. However, as a simple alternative, a parent or caregiver can be asked to role play (eg, placement of an anesthesia mask over the child's face, placement of a blood pressure cuff, wearing surgical gown and mask) in the weeks leading up to the day of anesthesia. We've found such role playing to be helpful for some patients in our practice.

In the authors' experience based on parent/caregiver satisfaction surveys that we have used since 2017, most respondents have indicated that creating a preanesthesia coping plan was helpful for both the child and the parent or caregiver, and that the coping plan would be useful for health care in the future and at other health care settings. The survey we use and the responses we’ve received are shown in a table (table 3).

Preanesthesia medical evaluation — All children require an anesthesia-directed medical history and physical examination, including an airway evaluation. These issues are discussed separately. (See "General anesthesia in neonates and children: Agents and techniques", section on 'Preoperative evaluation'.)

Children with ASD may be in robust physical health or have significant comorbidities, including seizures. They may be taking medications that have implications for anesthesia, including antipsychotics, antidepressants, mood-stabilizing medications, sedatives, and anxiolytics. Perioperative management of these medications is discussed separately. (See "Perioperative medication management", section on 'Psychotropic agents'.)

DAY OF SURGERY PREOPERATIVE PROTOCOL — Our protocol for the day of surgery is described here and is generally similar to other published protocols.

On the day of surgery, we aim to minimize stimulation (eg, bright lights, loud noises, unfamiliar personnel), avoid unnecessary delays, and provide comforting objects, as follows:

●The child life specialist (CLS) who has built a relationship with the family or caregivers is involved throughout the perioperative process. The CLS may provide comfort items (eg, weighted blankets, noise-canceling headphones, toys, electronic devices) in addition to those the parents or caregivers bring. The CLS works closely with the nurse assigned to the patient and makes modifications to the existing plan as needed.

●On arrival to the hospital, the patient is brought into a private, quiet preoperative holding room capable of dim lighting. Any necessary registration is completed in this room. The perioperative management plan is posted outside the room to alert involved staff.

●The number of health care providers involved is specifically kept to a minimum. Whenever possible, the same staff nurse cares for the patient in the preoperative area and for recovery from anesthesia.

●The CLS may accompany the patient to the operating room (OR) or procedure room if hospital policy does not allow parental or caregiver presence in the operating room. At our institution, the CLS accompanies the patient into the OR along with the anesthesia clinician. The parents or caregivers typically come with the child as far as the door to the OR, though in select cases they may enter the OR as well.

PREMEDICATION — We premedicate most patients with autism spectrum disorder (ASD), though practice regarding premedication varies [3-6]. We do not premedicate patients whose caregivers report that maladaptive behavior is worsened as a result of attempts at administering medication. Instead, we create an individualized plan that includes calming techniques, in collaboration with parents or caregivers, well in advance of the day of the procedure. In one institution, after implementation of a protocol that involved creation of an individualized perioperative management plan based on the severity of ASD and parental/caregiver input, 60 percent of children with ASD who had anesthesia for diagnostic or surgical procedures did not receive any form of premedication [6]. The decision to premedicate was made by the anesthesiologist on the day of the procedure. Cooperation with induction of anesthesia was approximately 90 percent overall, with greater cooperation in patients with less severe ASD. There was no difference in cooperation between children who received premedication and those who did not. In contrast, in another institution, 50 percent of patients with ASD who were assessed as not requiring premedication or who refused were uncooperative at the time of induction of anesthesia [4].

Parents or caregivers may be able to provide valuable information regarding the effects of specific sedatives in the past and the need for sedation prior to hospital or clinic visits.

Premedication may have to begin at home in patients with significant, challenging behaviors and usually involves administration of lorazepam [13]. The patient's usual medications for behavior modification should be administered on the morning of the procedure.

Route of administration — Any route of administration of medication may be stressful for a child with ASD. Oral premedication is preferred whenever possible, to avoid the trauma of an injection. The medication should be added to a small volume of the clear liquid preferred by the patient, to mask any bitter taste. It should be administered in a quiet room, with adequate time for full effect before the procedure. The parent or caregiver may be the best person to administer the medication.

If the patient refuses oral premedication, intramuscular injection may be the best option, and provides a relatively rapid and reliable onset. A quick intramuscular injection during a brief period of restraint, with distraction and the parent or caregiver present, may be preferable to escalating disruptive behavior, anxiety, or fear.

Intranasal administration of medication may be considered, but may not be possible in children with challenging behaviors.

For children who will accept intravenous (IV) catheter placement, IV premedication is reliable and titratable. Topical local anesthetic may be helpful for IV placement, but only if the patient allows, as most patients with ASD resist being touched and have altered sensitivity to some textures on the skin. (See "Autism spectrum disorder in children and adolescents: Clinical features", section on 'Atypical responses to sensory stimuli'.)

Choice of medication — Parents or caregivers of children with ASD can often report which medications have been either effective or problematic for the child in the past. Options for premedication for pediatric patients in general, including doses and routes of administration, are discussed separately. Considerations for patients with ASD are discussed here. (See "General anesthesia in neonates and children: Agents and techniques", section on 'Prevention and treatment of preoperative anxiety'.)

●Midazolam – We usually administer midazolam 0.5 mg/kg orally for premedication in children, including children with ASD. Midazolam may cause paradoxical dysphoria in some children with ASD [14].

●Ketamine – If the parents or caregivers report dysphoria with midazolam, oral ketamine (3 to 8 mg/kg orally) alone or in combination with midazolam may be well tolerated [15]. If necessary, intramuscular ketamine (4 to 5 mg/kg) provides effective sedation within five minutes of administration, and is effective for approximately 45 minutes [16].

●Alpha-2 agonists – Oral clonidine (alpha-2 adrenergic agonist) 2 to 4 mcg/kg [17] and dexmedetomidine (selective alpha-2 adrenergic agonist) oral or nasal route 1 to 4 mcg/kg maybe used in situations of inadequate sedation [18]. However, when used alone, alpha-2 adrenergic agonists do not have amnesic properties.

In one report, oral dexmedetomidine (mean dose: 2.6 ± 0.83 mcg/kg orally) prior to procedural sedation was effective for allowing IV catheter placement without difficulty in seven of eight patients with neurobehavioral disorders, and with mild resistance in the other [18]. In another review, 18 children with autism, neurobehavioral disorders, or seizure disorders were premedicated with oral dexmedetomidine, mean dose 3.6 mcg/kg, prior to sedation for electroencephalography [19]. Topical local anesthetic cream was applied after the patient took the medication. An IV catheter was placed without difficulty in 15 patients, and required mild restraint in 3.

TRANSFER TO THE OPERATING ROOM — We usually ask a parent or caregiver to apply the pulse oximeter tape to the patient's finger for monitoring after premedication in the preoperative holding area, and disconnect the cable from the monitor just prior to moving the patient into the operating room (OR).

A parent, caregiver, or child life specialist (CLS) should accompany the patient to the OR. The patient's preferred comfort items should be brought into the OR.

We aim to reduce stimulation prior to induction of anesthesia, with dim lighting and minimal noise, and restrict personnel to only those involved in the patient’s care. Additional help should be available as necessary to provide physical restraint during induction.

MANAGEMENT OF ANESTHESIA

Choice of anesthetic technique — For many procedures, and for many patients with autism spectrum disorder (ASD), general anesthesia will be necessary. Regional anesthesia alone is rarely used without general anesthesia in children. The decision to use sedation with monitored anesthesia care, particularly for diagnostic procedures, should be individualized based on patient factors and clinician preference [20]. (See "Anesthesia for magnetic resonance imaging and computed tomography procedures", section on 'Anesthetic management of pediatric patients'.)

General anesthesia

Physiologic monitors — We apply standard anesthesia monitors prior to induction of anesthesia only if the patient accepts them readily.

Operating room environment — The operating room (OR) atmosphere should be minimally stimulating prior to and during induction of anesthesia. The OR should be quiet, with lighting dimmed, and with only the necessary personnel present.

Choice of anesthetic agents in patients with autism — The pathogenesis of ASD is incompletely understood and likely complex, involving genetic, neurobiologic, and possibly environmental factors. (See "Autism spectrum disorder (ASD) in children and adolescents: Terminology, epidemiology, and pathogenesis", section on 'Pathogenesis'.)

Some families believe that biologic or metabolic conditions contribute to the pathogenesis of ASD, whether or not there is scientific evidence to support this hypothesis, and that drugs or environmental toxins may worsen symptoms of ASD (see "Autism spectrum disorder in children and adolescents: Complementary and alternative therapies", section on 'Rationale for use in ASD'). Thus, some families are concerned that administration of anesthetic agents, particularly nitrous oxide, could exacerbate symptoms of ASD [21]. The risks of nitrous oxide in patients with ASD are theoretical, based on effects on methionine synthase [22]. There are no clinical studies of the effects of anesthetic agents in patients with ASD, and we select anesthetic agents the way we would for children without ASD. We avoid nitrous oxide for induction and maintenance of anesthesia if the parents or caregivers express concern over its use. In our experience, most of them accept brief use of nitrous oxide to facilitate inhalation induction.

The neurotoxic effects of anesthetic agents in young children is an area of active research, without compelling evidence that any particular anesthetic agent should be avoided. (See "Neurotoxic effects of anesthetics on the developing brain".)

Induction of anesthesia — Inhalation induction, with administration of anesthetic gas by face mask, is the most common induction technique in young children, and is used primarily to avoid the trauma of intravenous (IV) catheter placement while the child is awake. The choice of inhalation versus IV induction of anesthesia and techniques for induction are discussed separately. (See "General anesthesia in neonates and children: Agents and techniques", section on 'Choice of induction technique'.)

Maintenance of anesthesia — Maintenance of anesthesia is similar in patients with ASD and other children, and is discussed separately. (See "General anesthesia in neonates and children: Agents and techniques", section on 'Maintenance of anesthesia'.)

For children with ASD, a goal for anesthetic management is to achieve a rapid, smooth emergence, to allow early removal of the IV catheter and rapid discharge to home. Important concerns include the following:

●Prophylaxis for postoperative nausea and vomiting (see "Postoperative nausea and vomiting")

●Plan for optimal multimodal opioid-sparing postoperative pain control, with regional anesthesia techniques and nonopioid analgesics as appropriate (see "Pharmacologic management of acute perioperative pain in infants and children")

●Adequate IV hydration

●The IV catheter should be protected with bandages to prevent it from being pulled out by the patient upon awakening

Emergence — Similar to children without ASD, the goal should be to achieve a smooth emergence from anesthesia. We often administer dexmedetomidine 0.3 mcg/kg IV near the end of surgery to smooth emergence and reduce the incidence of emergence delirium, though it may increase sedation and delay discharge from the post-anesthesia care unit (PACU). (See "Emergence delirium and agitation in children", section on 'Prevention'.)

Emergence from anesthesia in children is discussed separately. (See "General anesthesia in neonates and children: Agents and techniques", section on 'Emergence and extubation'.)

The association between preoperative mental status and the development of emergence delirium is unclear, and is discussed separately. (See "Emergence delirium and agitation in children", section on 'Preoperative behavior'.)

Monitored anesthesia care — For sedation with monitored anesthesia care (MAC), premedication must be adequate to allow IV catheter placement. (See 'Premedication' above.)

Similar to children without ASD, it makes sense to use short-acting anesthetic agents to facilitate a rapid recovery and early hospital discharge. Children with ASD may require higher doses of sedatives during MAC compared with children with other neurologic disorders [23-25].

POSTOPERATIVE CARE — The basic principles of ensuring a minimally stimulating environment and multidisciplinary awareness of the patient's care plan should be carried into the postoperative period. When possible, the patient should recover in a private post-anesthesia care unit (PACU) space with the parent or caregiver, or child life specialist (CLS) present. A detailed handover report should be given by the anesthesia team to the PACU staff. The intravenous (IV) catheter should be removed as early as it is considered safe to do so.

Postoperative pain may be difficult to assess in some patients with autism spectrum disorder (ASD), especially in those with communication deficits, and may contribute to postoperative behavioral problems [26]. Assessment and management of pain in children with communication problems is discussed separately. (See "Pain in children: Approach to pain assessment and overview of management principles", section on 'Nonverbal children with neurologic impairment'.)

The patient should be discharged from the PACU as quickly as possible to home or to the inpatient floor.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Pediatric anesthesia".)

SUMMARY AND RECOMMENDATIONS

●Preoperative planning

•The unfamiliar perioperative environment can be upsetting for children with autism spectrum disorder (ASD), and may trigger challenging behaviors. (See 'Implications for anesthesia' above.)

•Multidisciplinary preparation, including the parents, caregivers and other care providers, should result in a patient-specific, individualized plan for perioperative care (table 1 and table 2). (See 'Multidisciplinary preoperative preparation' above.)

•Ideally, an institutional protocol for management of children with ASD should be in place. Basic principles of management on the day of anesthesia include:

•Parental or caregiver presence as much as possible throughout the perioperative period

•Avoidance of delays

•Minimized stimulation, with dim lighting, quiet, minimized number of personnel

•Accommodation of patient preferences, including preferred comfort items and methods for taking medication (see 'Day of surgery preoperative protocol' above)

●Premedication – Oral premedication is preferred over other routes of administration whenever possible. We administer premedication, usually midazolam 0.5 mg/kg orally, for most patients with ASD. Other options for premedication include oral, or if necessary intramuscular, ketamine, or oral/intranasal clonidine or dexmedetomidine. (See 'Premedication' above.)

●Operating room environment

A child life specialist (CLS) and, if institutional policy permits, a parent or caregiver as well, should accompany the child into the operating room (OR), along with the child's preferred objects. (See 'Transfer to the operating room' above.)

The OR environment should be minimally stimulating prior to and during induction of anesthesia, and kept quiet, with dim lighting and only necessary personnel present. (See 'Operating room environment' above.)

●Anesthetic management

•There is no evidence that any anesthetic agent exacerbates symptoms of ASD. We select anesthetic agents as we would for children without ASD, and avoid particular agents if parents or caregivers express concern. (See 'Choice of anesthetic agents in patients with autism' above.)

•Induction and maintenance of anesthesia are conducted as they would be for children without ASD. (See 'Induction of anesthesia' above and 'Maintenance of anesthesia' above.)

•Goals for anesthesia include a rapid, smooth emergence, early removal of the intravenous (IV) catheter, and early hospital discharge, with prophylaxis for postoperative nausea and vomiting, and a plan for multimodal, opioid-sparing analgesia. (See 'Maintenance of anesthesia' above.)

●Postoperative care – The basic principles of ensuring a minimally stimulating environment and multidisciplinary awareness of the patient's care plan should be carried into the postoperative period. (See 'Postoperative care' above.)