Stereotactic neurosurgery as a symptomatic treatment for autism spectrum disorders: A systematic review.

Stereotactic neurosurgery has been employed in autism spectrum disorders (ASD). However, its safety and effectiveness remain unclear owing to limited sample size and other methodological limitations. We aimed to systematically investigate the safety and efficacy of stereotactic neurosurgery for ASD. Eleven studies with 36 patients were included. Stereotactic neurosurgery alleviated the obsessive-compulsive disorder and aggressive behavior symptoms in ASD, with a mean improvement of 42.74% and 59.59% in the Yale-Brown Obsessive Compulsive Scale and Overt Aggression Scale scores, respectively. Systematic studies are necessary to explore the role of deep brain stimulation for social and communication difficulties in ASD.

Surgery of autism: Is it possible?

Autism spectrum disorder (ASD) is a developmental disability of the brain that can be associated to severe conductual alterations, such as self or heteroaggression and obsessive and compulsive behavior. Many of these patients do not improve with any pharmacological or behavioral therapy and represent a major social problem. We describe the outcome of patients with ASD, treated with radiofrequency brain lesions combined with Gamma Knife radiosurgery for therapy-resistant aggressiveness, obsessive thoughts, and compulsions. The ASD adapted YBOCS, PCQ and EAE scales assessed the therapeutic effect on symptoms. All patients had a significant reduction of their symptoms (YBOCS:34 and 22 PCQ 42 and 35, EAE 11 and 5.5, respectively), although all needed more than one treatment to maintain this improvement. The treatments resulted very safe for the patients and their neurological status has not change. We conclude that in these patients after surgery, there is a marked improvement in behavior, quality of life and relationship with the environment, with no evidence of secondary damage. Changes in connectivity might mediate the clinical improvement, although it is necessary to confirm these results with further studies.

A balancing act: An interpretive description of healthcare providers' and families' perspective on the surgical experiences of children with autism spectrum disorder.

LAY ABSTRACT: Children with an autism spectrum disorder (autism) often have negative experiences within the surgical setting. We conducted individual interviews with 8 parents of children with autism who had recently undergone surgery, and 15 healthcare providers (HCPs) with experience caring for children with autism. We asked open-ended questions on the approaches used to support children with autism around the time of surgery, how effective they were, suggestions for improvement, and the barriers and facilitators to improvement. Three main themes emerged within an overarching metaphor of a balancing act. The first theme, finding your footing through an uncertain journey, described individual factors (e.g. anticipatory anxiety) that set the foundation for experiences. The second theme, relationships can help to keep everyone steady, highlighted how personal interactions (e.g. collaboration and empathy) influence the experience. Finally, the systems shape the experience theme captured how systemic factors (e.g. the hospital environment) affected the balancing act. These findings enriched our understanding of the surgical experiences of children with autism, families, and HCPs by demonstrating the importance of individual characteristics, relationships, and systemic factors. Future interventions should consider this complexity and intervene not just with children, but also their parents, healthcare providers, and in policy to improve experiences.

Cochlear Implantation Can Improve Auditory Skills, Language and Social Engagement of Children With Autism Spectrum Disorder.

OBJECTIVE: To review outcomes of cochlear implantation (CI) in children diagnosed with autism spectrum disorder (ASD). STUDY DESIGN: Retrospective case review and parent survey. SETTING: Tertiary care children's hospital. PATIENTS: Thirty children with ASD who underwent CI between 1991 and 2018. Mean age at CI = 3.5 years (0.8-11.8), mean age at diagnosis of ASD = 5.1 years (2.0-15.0) (22/30 diagnosed after CI), mean follow-up = 10.5 years (1.4-21.6). Parents of 7 children returned a survey. INTERVENTION: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Speech perception; expressive communication mode; educational placement; social engagement; consistency of CI use; parent survey of child behavior change. RESULTS: Thirty-three percent of all and 45% of the 22 consistent device users developed measurable open-set speech perception by an average of 4.5 years of device use. Educational placement at last follow-up included 13% mainstreamed without interpreter, 50% Special Education programs, 10% therapeutic residential or day programs, 23% total communication programs, and one home schooled. Spoken language alone was used by 31% and spoken plus sign by 14%, with the remainder using sign alone, augmentative communication devices or no mode of communication. By parent report, 86% showed improvement in social engagement compared to pre-CI. Survey results showed the behaviors most frequently ranked as most affected by CI were communication and attention, while awareness of environment had the lowest (most affected) mean ranking. CONCLUSIONS: Findings support a growing body of literature that cochlear implantation has the potential to improve auditory skills, language, and enhance social engagement in some deaf children with autism spectrum disorder.

Surgery and Radiosurgery in Autism: A Retrospective Study in 10 Patients.

INTRODUCTION: A subgroup of patients with autism spectrum disorder (ASD) show self or heteroaggression, dyscontrol episodes, and others are of obsessive-compulsive disorder (OCD) profile; some of them are resistant to medical and behavioural treatment. We describe the long-term outcome in a group of these patients, treated with radiofrequency brain lesions or combined stereotactic surgery and Gamma Knife (GK) radiosurgery. METHODS: We reviewed the medical records of 10 ASD patients with pathological aggressiveness and OCD, who had undergone radiofrequency lesions and/or radiosurgery with GK in our institution. RESULTS: The 10 patients had a significant reduction of their symptoms (PCQ 39.9 and 33, OAS 11.8 and 5, CYBOCS-ASD 30.4 and 20), preoperatively and in the last follow-up, respectively; p < 0.005 (in all cases), although all but 2 needed more than 1 treatment to maintain this improvement. CONCLUSIONS: We observed a marked improvement in behaviour, quality of life, and relationship with the environment in all our 10 patients after the lesioning treatments, without long-lasting side effects.

Muenke syndrome: Medical and surgical comorbidities and long-term management.

Muenke syndrome (MIM #602849), the most common syndromic craniosynostosis, results from the recurrent pathogenic p.P250R variant in FGFR3. Affected patients exhibit wide phenotypic variability. Common features include coronal craniosynostosis, hearing loss, carpal and tarsal anomalies, and developmental/behavioral issues. Our study examined the phenotypic findings, medical management, and surgical outcomes in a cohort of 26 probands with Muenke syndrome identified at the Children's Hospital of Philadelphia. All probands had craniosynostosis; 69.7% had bicoronal synostosis only, or bicoronal and additional suture synostosis. Three male patients had autism spectrum disorder. Recurrent ear infections were the most common comorbidity, and myringotomy tube placement the most common extracranial surgical procedure. Most patients (76%) required only one fronto-orbital advancement. de novo mutations were confirmed in 33% of the families in which proband and both parents were genetically tested, while in the remaining 66% one of the parents was a mutation carrier. In affected parents, 40% had craniosynostosis, including 71% of mothers and 13% of fathers. We additionally analyzed the medical resource utilization of probands with Muenke syndrome. To our knowledge, these data represent the first comprehensive examination of long-term management in a large cohort of patients with Muenke syndrome. Our study adds valuable information regarding neuropsychiatric and medical comorbidities, and highlights findings in affected relatives.

Behavioral training and mirroring techniques to prepare elective anesthesia in severe autistic spectrum disorder patients: An illustrative case and review.

Children with autistic spectrum disorder are more likely to become distressed during induction of anesthesia. Inhalational induction is almost always the preferred route with acceptance of the face mask often presenting a considerable challenge. Tempering measures to facilitate gas induction such as forced premedication and physical restraint are no longer viable options except in extenuating circumstances. Recent research interest has focused on the need for advanced planning in collaboration with the caregiver to tailor an individualized perioperative plan. This plan may include both pharmacological and non-pharmacological interventions. Applied behavior analysis strategies have a well-documented efficacy in this unique population to systematically change an individual's usual behavior. These can be used, as a non-pharmacological strategy, to ensure a smooth perioperative course. We present a successful case of preoperative desensitization of a child with severe autistic spectrum disorder using a mirror demonstration technique associated with positive reinforcement to prepare him for general anesthesia. We discuss the potential application of applied behavior analysis strategies for anesthesia in this unique population. From a practical point of view, early communication with carers is required to establish who may benefit from this behavioral training. Planned individual preparation for general anesthesia must be provided by trained multidisciplinary staff.

Children with autism spectrum disorders and drug-resistant epilepsy can benefit from epilepsy surgery.

OBJECTIVE: The objective of this research was to evaluate a cohort of children with both autism spectrum disorder (ASD) and drug-resistant epilepsy (DRE) after epilepsy surgery to determine predictors of best outcome. METHODS: Retrospective chart review was done for 29 children ages 2 to 18 years with ASD and DRE who had neurosurgical intervention for seizure management over 15 years at one institution. All subjects had at least 1 year of follow-up. Data abstraction included demographic information, seizure diagnosis, treatment, investigations, surgical intervention, neuropsychological assessment, and outcome. Statistical analysis software (SAS) was used for statistical analysis. Engel classification was used to assess seizure outcome. RESULTS: Fifteen subjects had resective surgery. Fourteen had palliative surgery with vagal nerve stimulator (VNS) insertion (13) and corpus callosotomy (1). Of the 29 subjects, 35% had class I outcome (all in the resective group). When combining all subjects (resective and palliative), 66% of subjects benefited with class I-III outcomes. In the total cohort, age at time of surgery was significant, with class I outcome more frequently seen in the younger age group when compared with classes II-IV (p = 0.01). CONCLUSION: A subset of children with ASD can benefit from resective surgery, and for those who are not candidates, a VNS can offer significant improvements in seizure control.

A prospective study comparing perioperative anxiety and posthospital behavior in children with autism spectrum disorder vs typically developing children undergoing outpatient surgery.

BACKGROUND: Research describing the experience of youth with autism spectrum disorders in the perioperative setting is limited. This study compared youth with autism spectrum disorder to typically developing children in the perioperative setting and examined group differences in: child anxiety, parent anxiety, premedication patterns, induction compliance, and changes in behavior postprocedure. METHODS: Participants were 60 youth (32 with autism spectrum disorder, 28 typically developing) of ages 2-19 years undergoing outpatient surgery and their parents. Parents and research assistants rated children's anxiety at 3 time points (waiting room, preoperative holding, separation), and parents rated their own anxiety in the waiting room and at separation. The anesthesiologist rated induction compliance. Postprocedure behavior change was assessed via phone survey 1 and 7 days postprocedure. Analyses examined group differences in anxiety, medication patterns, and behavior. RESULTS: Children with autism spectrum disorder had higher research assistant reported anxiety than typically developing youth in the holding room only. There were no group differences in parent report of their own anxiety or their child's anxiety across time points. Compared to typically developing youth, children with autism spectrum disorder were more likely to receive a premedication (including nonstandard premedication), and had poorer induction compliance. Groups did not differ on posthospital behavior change 1 or 7 days postsurgery. CONCLUSION: Findings revealed ratings of anxiety in youth with and without autism spectrum disorder facing surgery varied by reporter and setting, highlighting the importance of using multiple reporters in research of youth with autism spectrum disorder in the perioperative period. Furthermore, while results showed group differences in premedication patterns and induction compliance, groups did not differ in level of negative behavior change after surgery. Future research can examine how individual differences in youth with autism impact anxiety in the perioperative setting and degree of behavior change postprocedure.

Epilepsy surgery in patients with autism.

OBJECTIVE The purpose of this study was to report outcomes of epilepsy surgery in 56 consecutive patients with autism spectrum disorder. METHODS Medical records of 56 consecutive patients with autism who underwent epilepsy surgery were reviewed with regard to clinical characteristics, surgical management, postoperative seizure control, and behavioral changes. RESULTS Of the 56 patients with autism, 39 were male, 45 were severely autistic, 27 had a history of clinically significant levels of aggression and other disruptive behaviors, and 30 were considered nonverbal at baseline. Etiology of the epilepsy was known in 32 cases, and included structural lesions, medical history, and developmental and genetic factors. Twenty-nine patients underwent resective treatments (in 8 cases combined with palliative procedures), 24 patients had only palliative treatments, and 3 patients had only subdural electroencephalography. Eighteen of the 56 patients had more than one operation. The mean age at surgery was 11 ± 6.5 years (range 1.5-35 years). At a mean follow-up of 47 ± 30 months (range 2-117 months), seizure outcomes included 20 Engel Class I, 12 Engel Class II, 18 Engel Class III, and 3 Engel Class IV cases. The age and follow-up times are stated as the mean ± SD. Three patients were able to discontinue all antiepileptic drugs (AEDs). Aggression and other aberrant behaviors observed in the clinical setting improved in 24 patients. According to caregivers, most patients also experienced some degree of improvement in daily social and cognitive function. Three patients had no functional or behavioral changes associated with seizure reduction, and 2 patients experienced worsening of seizures and behavioral symptoms. CONCLUSIONS Epilepsy surgery in patients with autism is feasible, with no indication that the comorbidity of autism should preclude a good outcome. Resective and palliative treatments brought seizure freedom or seizure reduction to the majority of patients, although one-third of the patients in this study required more than one procedure to achieve worthwhile improvement in the long term, and few patients were able to discontinue all AEDs. The number of palliative procedures performed, the need for multiple interventions, and continued use of AEDs highlight the complex etiology of epilepsy in patients with autism spectrum disorder. These considerations underscore the need for continued analysis, review, and reporting of surgical outcomes in patients with autism, which may aid in better identification and management of surgical candidates. The reduction in aberrant behaviors observed in this series suggests that some behaviors previously attributed to autism may be associated with intractable epilepsy, and further highlights the need for systematic evaluation of the relationship between the symptoms of autism and refractory seizures.

Perioperative considerations in children with autism spectrum disorder.

PURPOSE OF REVIEW: Children with autism often present a challenge to the anesthesiologist. This review summarizes the current experiences and recommendations for the perioperative management of this unique group of patients. RECENT FINDINGS: Autism is the fastest growing neurodevelopmental disorder in the world. Increased recognition and public awareness of the disease is driven largely by the advances in research. A large body of evidence exists that identifies the role of genetic, environmental, biological, and developmental factors in the origin of autism. The anesthesia literature consists mostly of case reports. Recent publications are reporting management strategies and evaluation of this patient population's perioperative experiences. SUMMARY: Patients with autism spectrum disorder are a heterogeneous group and often need general anesthesia for different procedures and studies. Familiarity with each patient's behavioral specifics and efforts to alleviate stress is of paramount importance for a smooth perioperative course with minimal adverse events.

Autism spectrum disorder (ASD) and its perioperative management.

Autism spectrum disorder (ASD) is now diagnosed in more than 1 in 100 children, so it is not surprising that anesthetists are increasingly providing care for children with this diagnosis. The diagnostic classification for ASD has recently changed and our understanding of the causes and management of ASD are also changing rapidly. This review provides a timely update to increase understanding and awareness of the problems that children with ASD experience, and to minimize perioperative problems. Current literature on premedication and the increasing use of alpha-2 agonists such as clonidine and dexmedetomidine as well as the use of old favorites midazolam and ketamine is reviewed. Some simple strategies that will improve care and decrease anxiety, like social stories, the use of tablet computers, other comfort items or games for distraction, and using favorite drinks to disguise the bitter taste of medications, are described. Remember, the parents are their child's expert and will know what agitates and settles them. Talking to them prior to the day of the procedure is ideal. The importance of staff training and having a clinical practice guideline available at every institution cannot be overstated.

Electroencephalography for children with autistic spectrum disorder: a sedation protocol.

OBJECTIVES: To report the effectiveness and efficiency of a predetermined sedation protocol for providing sedation for electroencephalograph (EEG) studies in children with autism. METHODS: Sleep EEG has been advocated for the majority of children with autism spectrum disorder. In most cases, sedation is required to allow adequate studies. Most sedation drugs have negative effects on the EEG pattern. The sedation protocol we adopted included chloral hydrate, dexmedetomidine, and ketamine and was evaluated prospectively for 2 years. RESULTS: One hundred and eighty-three children with autistic spectrum disorder were sedated with the described drug protocol that was efficient, provided adequate EEG readings, and was not associated with serious adverse events. CONCLUSIONS: Our protocol kept costs to a minimum but provided appropriate escalation in care when required.