Comparing narrative storytelling ability in individuals with autism and fetal alcohol spectrum disorders.

BACKGROUND: Narrative discourse, or storytelling, is used in daily conversation and requires higher-level language and social communication skills that are not always captured by standardised assessments of language. Many autistic individuals and individuals with fetal alcohol spectrum disorders (FASD) have difficulties with both social communication and language skills, and narrative discourse analysis offers an ecologically relevant approach to assessing those challenges. AIMS: This study investigated narrative discourse in individuals with autism and FASD, as well as an age- and sex-matched comparison group. METHODS AND PROCEDURES: Narratives from 45 adolescents and adults, 11 with autism, 11 with FASD and 23 age- and sex-matched comparison participants were elicited using a wordless storybook. They were then transcribed orthographically, formatted to the Systematic Analyses of Language Transcript (SALT) convention and scored based on the SALT Narrative Scoring Scheme (NSS), a standardised language analysis protocol. In addition to the NSS total score, which assesses the overall structure and cohesion of the narratives produced, local and global measures of language ability were also employed. The local language measures included the number of mental state and temporal relation terms produced, while the global language measures included mean length of utterance, total different words, total words, total utterances, rate of speech, the number of mazes (e.g., repetitions, 'um', 'uh' or self-corrections) per total word and the NSS total score. OUTCOMES AND RESULTS: Using the SALT Language Sample Analysis tool, our results revealed that on global language measures, group differences were found on rate of speech, number of mazes per total words and the description of conflict/resolution in the narratives produced. The autism group produced significantly more mazes per total word and scored higher on the NSS conflict/resolution category score compared to the FASD and comparison groups. Both the autism and FASD groups spoke at a lower rate than the comparison group. On local language measures of narrative production, all groups were comparable, on average. CONCLUSIONS AND IMPLICATIONS: While many aspects of narrative discourse in the autism and FASD groups were similar to each other and to the comparison group, we observed group differences on global measures of narrative production and significant individual variability within groups, suggesting that narrative abilities considered at an individual level may provide important clinical information for intervention planning. Future research should also consider additional variables that influence narrative discourse, such as motivation, distractibility or decision-making of individual participants. WHAT THIS PAPER ADDS: What is already known on the subject Narrative discourse, or storytelling, is used in daily conversational interactions and reveals higher-level language skills that may not be well captured by standardised assessments of language. Many autistic individuals and individuals with fetal alcohol spectrum disorders (FASD) show difficulty with pragmatic and expressive language skills. What this paper adds to existing knowledge We found that many aspects of the narratives produced by the adolescents/young adults in the autism and FASD groups were comparable to each other and to the neurotypical group. However, the groups differed on three global measures of narrative production: rate of speech, number of mazes per total words and the description of conflict/resolution in the narratives produced. Also, significant variability was observed within groups, suggesting that narrative abilities should be considered at an individual level as opposed to their clinical groups. What are the potential or actual clinical implications of this work? This study showed that narrative discourse is an appropriate task that can be added to routine clinical assessments of language abilities in autistic adolescents/young adults as well as those with FASD or typical development and has the potential to reveal higher-level, real-world language skills. An important clinical implication of this study is that narrative language abilities should be considered at an individual level and individual-tailored interventions based on ability level due to the variability observed across individuals.

Thresholds for the 28-joint disease activity score (DAS28) using C-reactive protein are lower compared to DAS28 using erythrocyte sedimentation rate in early rheumatoid arthritis.

OBJECTIVES: The 28-Joint Disease Activity Score (DAS28) using C-reactive protein (CRP) and DAS28 using erythrocyte sedimentation rate (DAS28-ESR) may not be interchangeable. We sought to compare and estimate optimal thresholds for the DA28-CRP for use in early rheumatoid arthritis (ERA). METHODS: Patients from the Canadian Early Arthritis Cohort with baseline and 12 months' data for both DAS28-ESR and DAS28-CRP were examined for correlations and differences between DAS28-CRP and DAS28-ESR across their range of values. Receiver operating characteristic analysis identified thresholds for DAS28-CRP that best corresponded to established thresholds for the DAS28-ESR using the total sample, then stratified by age and sex. Agreement between DAS28-CRP and DAS28-ESR thresholds was assessed with the kappa statistic. RESULTS: The sample included 995 patients with mean (SD) age of 53.7 (14.5) years, 5.8 (2.9) months of symptom duration and 74% were female. DAS28-CRP and DAS28-ESR scores were highly correlated (r= 0.92, p<0.0001), however DAS28-CRP values were consistently lower than DAS28-ESR values. Calculated thresholds for DAS28-CRP were lower with 2.5 for remission, 2.9 for low disease activity, and 4.6 for high disease activity but showed moderate agreement with the DAS28-ESR thresholds (kappa=0.70). CONCLUSIONS: In this large sample of ERA patients, newly estimated thresholds for DAS28-CRP were consistently lower than DAS28-ESR thresholds across the spectrum of disease activity. This may have important clinical implications if inflammatory markers are used interchangeably. Additional external validation of our findings is needed.

A reliable surgical technique for the management of growing fractures of the frontal bone involving the orbit.

PURPOSE: Growing fractures involving the orbit occur infrequently. Due to the risk of neurological trauma and the ophthalmic sequelae of progressive intraorbital expansion of the fracture, surgical management is indicated. There is no accepted standard in the approach to the surgical management of these patients. The aim of this paper is to describe a reliable technique for the repair of growing fractures and discuss the long-term outcome. METHODS: We present two cases in which a technique using Norian CRS® (Craniofacial Repair System) fast set putty over a Medpor® scaffold was used to repair a growing fracture. RESULTS: This technique provides a secure repair with a good functional and cosmetic outcome. CONCLUSIONS: Growing fractures occur in the paediatric population so it is important to ensure that the repair does not restrict or adversely affect future bone growth. One case has been followed-up for over 10 years and has maintained normal growth.

Parental scaffolding in play: A comparison of fetal alcohol spectrum disorders and autism spectrum disorder.

BACKGROUND: Parental support of child play varies based on child needs; however, how parental play level differs from child play level remains an understudied area of research, especially in relation to specific developmental disabilities. AIMS: To preliminarily explore differences in child and parent play levels in age- and IQ-matched children with fetal alcohol spectrum disorders (FASD) and autism spectrum disorder (ASD). METHODS: and Procedures: Parent-child dyads were recorded during free-play sessions. Parent/child play levels were coded for highest level achieved during each minute of play. Mean play level and dPlay (difference in parent versus child play level) were calculated across play sessions for each dyad. OUTCOMES AND RESULTS: On average, parents of children with FASD demonstrated higher levels of play than other parents. Children with FASD demonstrated higher levels of play than their own parents. In contrast, the play level of parents of children with ASD did not differ from their child's. There were no between-group differences in dPlay. CONCLUSIONS AND IMPLICATIONS: This preliminary exploratory study suggests that parents of children with developmental disabilities may differentially 'match' their child's play level. Further research on developmental play levels during parent-child play is warranted.

[Formula: see text]Parental interaction style, child engagement, and emerging executive function in fetal alcohol spectrum disorders (FASD).

Children with fetal alcohol spectrum disorders (FASD) are known to experience cognitive and neurobehavioral difficulties, including in areas of executive function and social skills development. Interventions for these challenges have focused on a number of areas, including parent-based training. Despite the general consensus that specific parenting styles consistent with an "authoritative" - warm but firm - parenting approach may influence behavioral self-regulation, it is not known what specific parental interaction styles are associated with child engagement and emerging executive function in this population. The current study used an observation-based behavioral coding scheme during parent-child play interactions and associated parent report-based executive function measures in children with FASD. Here, we demonstrate that parental interaction styles with increased responsive/child-oriented behavior and parental affect are associated with higher levels of child play engagement, while parental interaction that has increased achievement-orientation is associated with higher levels of emerging executive function in children with FASD. These findings help inform future studies on behavioral targets in parent-based training programs and highlight the importance of considering certain parental interaction styles during parent-child play.

Relationship Between Task-Based and Parent Report-Based Measures of Attention and Executive Function in Children with Fetal Alcohol Spectrum Disorders (FASD).

A majority of children with fetal alcohol spectrum disorders (FASD) have demonstrated attention and executive function deficits as measured by both parent report measures and performance on tasks requiring sustained levels of attention. However, prior studies have consistently reported a lack of association between parental report-based and task-based performance measures. The current study investigated whether changes in performance over time within-task (i.e., first-half versus second-half) better correspond to parental reports of executive function and temperament in children with FASD. Greater differences in split-half performance during a continuous performance task were found to be associated with higher parent-reported levels of behavioral regulation and inhibitory control. These findings suggest that within-task performance differences may more accurately reflect individual differences in executive function and temperament as measured by parental report and help to further inform the way in which cognitive processes are measured in children with FASD.

Prevalence and patterns of sensory processing behaviors in a large clinical sample of children with prenatal alcohol exposure.

BACKGROUND: Atypical behavioral responses to sensation are reported in a large proportion of children affected by prenatal alcohol exposure (PAE). Systematic examination of symptoms across the fetal alcohol spectrum in a large clinical sample is needed to inform diagnosis and intervention. AIMS: To describe the prevalence and patterns of atypical sensory processing symptoms in a clinical sample of children with PAE. METHODS: Retrospective analysis of diagnostic clinical data from the University of Washington Fetal Alcohol Syndrome Diagnostic and Prevention Network (FASDPN). Participants were ages 3 through 11 years, had a diagnosis on the fetal alcohol spectrum, and Short Sensory Profile (SSP) assessment. The proportions of children categorized with definite differences on the SSP across selected clinical and demographic features were examined with chi-square analyses. OUTCOMES: The sample consisted of 325 children; 73.2 % had SSP total scores in the definite difference range. Atypical sensory processing symptoms were significantly more prevalent among children with higher reported levels of PAE. The prevalence of atypical symptoms was comparably high across age, levels of diagnostic severity, and other prenatal/postnatal risks. CONCLUSIONS: Results lend support for altered sensory processing as another domain of brain function affected by the teratogenic impact of PAE, guiding clinical work and research.

Endoscopic third ventriculostomy: predicting outcome with phase-contrast MR imaging.

PURPOSE: To determine whether phase-contrast magnetic resonance (MR) imaging measurements of preoperative cerebral blood and cerebrospinal fluid (CSF) hydrodynamics can be used as a biomarker of response to endoscopic third ventriculostomy (ETV). MATERIALS AND METHODS: Approval from the local research ethics committee and written informed consent were obtained for this prospective study. Thirteen patients (six female patients, seven male patients; median age, 43 years) with chronic obstructive hydrocephalus, 12 of whom went on to undergo ETV, were imaged with phase-contrast MR imaging at 1.5 T to determine rates of total cerebral blood flow (CBF) and ventriculostomy defect, foramen magnum (FM), and cerebral aqueduct CSF flow. Ten control subjects (10 men; median age, 37 years) were similarly imaged. Correlations between measured values were assessed by means of Pearson correlation coefficients. Measurements were compared between groups with a Mann-Whitney test, and measurements before and after surgical intervention were compared with a Wilcoxon test for paired samples. RESULTS: Rates of CBF (356 mL . min(-1) +/- 73 [standard deviation] vs 518 mL . min(-1) +/- 79, P < .001) and CSF flow in the FM (17.62 mL . min(-1) +/- 13.12 vs 36.35 mL . min(-1) +/- 8, P < .05) were significantly lower in patients than in control subjects. CONCLUSION: ETV induces changes in brain volume and CBF that can be predicted by using simple metrics. These pilot results support a formal trial of these techniques in a larger prospective study.

Outcome from surgery for vestibular schwannomas in children.

OBJECT: A review of sporadic and NF2-related vestibular schwannoma surgery in children (under 18 years of age) with a specific interest in resection rates, recurrence, facial nerve outcomes, hearing preservation, hearing rehabilitation and genetic analysis. METHODS: A retrospective analysis of prospectively collected data of 35 consecutively operated vestibular schwannomas in 29 paediatric patients that underwent 38 operations between 1992 and 2007. Pre- and post-operative radiology, facial nerve function, pure tone audiogram and speech discrimination tests were performed with a mean follow-up of 4.5 years. Tumour and blood mutations were analysed in 86% of patients. RESULTS: Total resection was achieved in all sporadic cases and 68% of NF2 cases. Near total resection led to tumour recurrence in 5 out of 10 cases. The facial nerve was anatomically preserved in 92%. Facial nerve function was excellent to good (Grades 1-3) in 88% with outcome related to tumour size. Hearing preservation was successful in 3 of 11 cases. CONCLUSIONS: Surgery with complete resection results in excellent tumour control, but it is more difficult to attain total resection in NF2 with a relatively high recurrence rate of persistently growing tumours. A better facial outcome is associated with smaller tumours, near-total resection and first time surgery. Hearing preservation is possible in a minority. Hearing rehabilitation can be successful by utilising cochlear implants and auditory brain stem implants (ABI) as appropriate. Overall there is a low complication rate and results are comparable with adult series.

Listening Difficulties in Children With Fetal Alcohol Spectrum Disorders: More Than a Problem of Audibility.

Purpose Data from standardized caregiver questionnaires indicate that children with fetal alcohol spectrum disorders (FASDs) frequently exhibit atypical auditory behaviors, including reduced responsivity to spoken stimuli. Another body of evidence suggests that prenatal alcohol exposure may result in auditory dysfunction involving loss of audibility (i.e., hearing loss) and/or impaired processing of clearly audible, "suprathreshold" sounds necessary for sound-in-noise listening. Yet, the nexus between atypical auditory behavior and underlying auditory dysfunction in children with FASDs remains largely unexplored. Method To investigate atypical auditory behaviors in FASDs and explore their potential physiological bases, we examined clinical data from 325 children diagnosed with FASDs at the University of Washington using the FASD 4-Digit Diagnostic Code. Atypical behaviors reported on the "auditory filtering" domain of the Short Sensory Profile were assessed to document their prevalence across FASD diagnoses and explore their relationship to reported hearing loss and/or central nervous system measures of cognition, attention, and language function that may indicate suprathreshold processing deficits. Results Atypical auditory behavior was reported among 80% of children with FASDs, a prevalence that did not vary by FASD diagnostic severity or hearing status but was positively correlated with attention-deficit/hyperactivity disorder. In contrast, hearing loss was documented in the clinical records of 40% of children with fetal alcohol syndrome (FAS; a diagnosis on the fetal alcohol spectrum characterized by central nervous system dysfunction, facial dysmorphia, and growth deficiency), 16-fold more prevalent than for those with less severe FASDs (2.4%). Reported hearing loss was significantly associated with physical features characteristic of FAS. Conclusion Children with FAS but not other FASDs may be at a particular risk for hearing loss. However, listening difficulties in the absence of hearing loss-presumably related to suprathreshold processing deficits-are prevalent across the entire fetal alcohol spectrum. The nature and impact of both listening difficulties and hearing loss in FASDs warrant further investigation.

Comparison of the 4-Digit Code, Canadian 2015, Australian 2016 and Hoyme 2016 fetal alcohol spectrum disorder diagnostic guidelines.

BACKGROUND: As clinicians strive to achieve consensus worldwide on how best to diagnose fetal alcohol spectrum disorders (FASD), the most recent FASD diagnostic systems show convergence and divergence. Applying these systems to a single clinical population illustrates the contrasts between them, but validation studies are ultimately required to identify the best system. METHODS: The 4-Digit-Code, Hoyme 2016, Canadian 2015 and Australian 2016 FASD diagnostic systems were applied to 1,392 patient records evaluated for FASD at the University of Washington. The diagnostic criteria and tools, the prevalence and concordance of diagnostic outcomes, and validity measures were compared between the systems. RESULTS: The proportion diagnosed with fetal alcohol syndrome (FAS) and FASD varied significantly (4-Digit-Code 2.1%, ≤79%; Hoyme 6.4%, 44%, Australian 1.8%, 29%; Canadian 1.8%, 16%). Eighty-two percent were diagnosed FASD by at least one system; only 11% by all four systems. Key factors contributing to discordance include: requiring high alcohol exposure; excluding growth deficiency; relaxing the facial criteria; requiring brain criteria that prevent diagnosis of infants/toddlers; and excluding moderate dysfunction from the spectrum. Primate research confirms moderate dysfunction (1-2 domains ≤-2 standard deviations) is the most prevalent outcome caused by PAE (FAS 5%, severe dysfunction 31%, moderate dysfunction 59%). Only the 4-Digit-Code replicated this diagnostic pattern. CONCLUSION: The needs of individuals with FASD are best met when diagnostic systems provide accurate, validated diagnoses across the lifespan, the full spectrum of outcome, the full continuum of alcohol exposure; and utilize diagnostic nomenclature that accurately reflects the association between outcome and alcohol exposure.

A diagnostically promising technique for tallying nominal reference errors in the narratives of school-aged children with foetal alcohol spectrum disorders (FASD).

BACKGROUND: Foetal Alcohol Spectrum Disorders (FASD) include the range of disabilities that occur in children exposed to alcohol during pregnancy, with Foetal Alcohol Syndrome (FAS) on the severe end of the spectrum. Clinical research has documented a range of cognitive, social, and communication deficits in FASD and it indicates the need for diagnostic tools that can identify children with diminished communicative capacities resulting from prenatal alcohol exposure. Previous research indicates that analysis of nominal reference errors within narrative discourse may provide such a tool. AIMS: To demonstrate the potential diagnostic utility of a new tool for tallying nominal reference errors in the oral narratives of school-aged children with FASD by presenting quantitative measurement data that address interrater agreement and predictive accuracy. METHODS & PROCEDURES: Retrospective analysis was conducted on spontaneously produced oral narratives from 32 school-aged children (8;5-11;7) with a range of socio-economic and ethnic profiles. Sixteen of the children had been previously diagnosis with an FASD, including five with full or partial FAS (pFAS). The remaining 16 children were considered typically developing (TD). A range of methods for calculating the rate of nominal reference errors (rNRE) were used to predict which narratives were produced by children from each group. Accuracy (sensitivity and specificity) for two predictions (FASD versus TD, and FAS/pFAS versus all others) was quantified using receiver-operating characteristic curve analyses. Pairwise statistical comparisons were made between methods to determine which had the most diagnostic potential. OUTCOMES & RESULTS: The proposed system for calculating the rNRE was highly accurate at predicting which narratives were produced by children with FASD (versus TD, 88% overall accuracy), and which were produced by children with FAS/pFAS (versus all others, 97% overall accuracy), and outperformed all other methods tested. Agreement on coding decisions between independent judges was high (kappa = 0.90). CONCLUSIONS: The strong predictive accuracy demonstrated in this study provides empirical evidence that the system proposed in this feasibility study has sufficient sensitivity and diagnostic utility to warrant further development for use with children suspected of prenatal alcohol exposure. It also points to the potential for the tool to be used with other clinical populations that, even in the absence of a confirmed alcohol exposure, share many of the communication challenges of this complex clinical population.

Twin study confirms virtually identical prenatal alcohol exposures can lead to markedly different fetal alcohol spectrum disorder outcomes-fetal genetics influences fetal vulnerability.

BACKGROUND: Risk of fetal alcohol spectrum disorder (FASD) is not based solely on the timing and level of prenatal alcohol exposure (PAE). The effects of teratogens can be modified by genetic differences in fetal susceptibility and resistance. This is best illustrated in twins. OBJECTIVE: To compare the prevalence and magnitude of pairwise discordance in FASD diagnoses across monozygotic twins, dizygotic twins, full-siblings, and half-siblings sharing a common birth mother. METHODS: Data from the Fetal Alcohol Syndrome Diagnostic & Prevention Network clinical database was used. Sibling pairs were matched on age and PAE, raised together, and diagnosed by the same University of Washington interdisciplinary team using the FASD 4-Digit Code. This design sought to assess and isolate the role of genetics on fetal vulnerability/resistance to the teratogenic effects of PAE by eliminating or minimizing pairwise discordance in PAE and other prenatal/postnatal risk factors. RESULTS: As genetic relatedness between siblings decreased from 100% to 50% to 50% to 25% across the four groups (9 monozygotic, 39 dizygotic, 27 full-sibling and 9 half-sibling pairs, respectively), the prevalence of pairwise discordance in FASD diagnoses increased from 0% to 44% to 59% to 78%. Despite virtually identical PAE, 4 pairs of dizygotic twins had FASD diagnoses at opposite ends of the fetal alcohol spectrum-Partial Fetal Alcohol Syndrome versus Neurobehavioral Disorder/Alcohol-Exposed. CONCLUSION: Despite virtually identical PAE, fetuses can experience vastly different FASD outcomes. Thus, to protect all fetuses, especially the most genetically vulnerable, the only safe amount to drink is none at all.

Responses to conflict and cooperation in adolescents with anxiety and mood disorders.

This study examined patterns of behavioral and emotional responses to conflict and cooperation in adolescents with anxiety/mood disorders and healthy peers. We compared performance on and emotional responses to the Prisoner's Dilemma (PD) game, an economic exchange task involving conflict and cooperation, between adolescents with anxiety/depressive disorders (A/D) (N=21) and healthy comparisons (n = 29). Participants were deceived to believe their co-player (a pre-programmed computer algorithm) was another study participant. A/D adolescents differed significantly from comparisons in patterns of play and emotional response to the game. Specifically, A/D participants responded more cooperatively to cooperative overtures from their co-players; A/D girls also reported more anger toward co-players than did comparison girls. Our findings indicate that A/D adolescents, particularly females, respond distinctively to stressful social interchanges. These findings offer a first step toward elucidating the mechanisms underlying social impairment in youth with internalizing disorders.

Exploring the utility of narrative analysis in diagnostic decision making: picture-bound reference, elaboration, and fetal alcohol spectrum disorders.

PURPOSE: To evaluate classification accuracy and clinical feasibility of a narrative analysis tool for identifying children with a fetal alcohol spectrum disorder (FASD). METHOD: Picture-elicited narratives generated by 16 age-matched pairs of school-aged children (FASD vs. typical development [TD]) were coded for semantic elaboration and reference strategy by judges who were unaware of age, gender, and group membership of the participants. Receiver operating characteristic (ROC) curves were used to examine the classification accuracy of the resulting set of narrative measures for making 2 classifications: (a) for the 16 children diagnosed with FASD, low performance (n = 7) versus average performance (n = 9) on a standardized expressive language task and (b) FASD (n = 16) versus TD (n = 16). RESULTS: Combining the rates of semantic elaboration and pragmatically inappropriate reference perfectly matched a classification based on performance on the standardized language task. More importantly, the rate of ambiguous nominal reference was highly accurate in classifying children with an FASD regardless of their performance on the standardized language task (area under the ROC curve = .863, confidence interval = .736-.991). CONCLUSION: Results support further study of the diagnostic utility of narrative analysis using discourse level measures of elaboration and children's strategic use of reference.

Accentuate the Negative: Grammatical Errors During Narrative Production as a Clinical Marker of Central Nervous System Abnormality in School-Aged Children With Fetal Alcohol Spectrum Disorders.

Purpose: The purpose of this study was to examine (a) whether increased grammatical error rates during a standardized narrative task are a more clinically useful marker of central nervous system abnormality in Fetal Alcohol Spectrum Disorders (FASD) than common measures of productivity or grammatical complexity and (b) whether combining the rate of grammatical errors with the rate of cohesive referencing errors can improve utility of a standardized narrative assessment task for FASD diagnosis. Method: The method used was retrospective analysis of narrative and clinical data from 138 children (aged 7-12 years; 69 with FASD, 69 typically developing). Narrative analysis was conducted blind to diagnosis. Measures of grammatical error, productivity and complexity, and cohesion were used independently and in combination to predict whether a story was told by a child with an FASD diagnosis. Results: Elevated grammatical error rates were more common in children with FASD, and this difference facilitated a more accurate prediction of FASD status than measures of productivity and grammatical complexity and, when combined with an accounting of cohesive referencing errors, significantly improved sensitivity to FASD over standard practice. Conclusion: Grammatical error rates during a narrative are a viable behavioral marker of the kinds of central nervous system abnormality associated with prenatal alcohol exposure, having significant potential to contribute to the FASD diagnostic process.

Comparison of the FASD 4-Digit Code and Hoyme et al. 2016 FASD diagnostic guidelines.

BACKGROUND: As clinicians strive to achieve consensus worldwide on how best to diagnose fetal alcohol spectrum disorders (FASD), the most recent FASD diagnosstic systems exhibit convergence and divergence. Applying these systems to a single clinical population illustrates contrasts between them, but validation studies are ultimately required to identify the best system. Currently, only the 4-Digit Code has published comprehensive validation studies. METHODS: The 4-Digit Code and Hoyme 2016 FASD systems were applied to the records of 1,392 patients evaluated for FASD at the University of Washington to: 1) Compare the diagnostic criteria and tools used by each system, 2) Compare the prevalence and concordance of diagnostic outcomes and assess measures of validity. RESULTS: Only 38% of patients received concordant diagnoses. The Hoyme criteria rendered half as many diagnoses under the umbrella of FASD (n=558) as the 4-Digit Code (n=1,092) and diagnosed a much higher proportion (53%) as fetal alcohol syndrome/partial fetal alcohol syndrome (FAS/PFAS) than the 4-Digit Code (7%). Key Hoyme factors contributing to discordance included relaxation of facial criteria (40% had the Hoyme FAS face, including patients with confirmed absence of alcohol exposure); setting alcohol exposure thresholds prevented 1/3 with confirmed exposure from receiving FAS/FASD diagnoses; and setting minimum age limits for Alcohol-Related Neurodevelopmental Disorder prevented 79% of alcohol-exposed infants with neurodevelopmental impairment a FASD diagnosis. The Hoyme Lip/Philtrum Guides differ substantively from the 4-Digit Lip-Philtrum Guides and thus are not valid for use with the 4-Digit Code. CONCLUSIONS: All FASD diagnostic systems need to publish comprehensive validation studies to identify which is the most accurate, reproducible, and medically valid.

Nonsynostotic Posterior Brachycephaly with Hindbrain Herniation.

BACKGROUND: Positional plagiocephaly is the most common cause of cranial asymmetry. The underlying cause of Chiari-1 malformation has many possible theories, and anecdotally some pediatric neurosurgeons have had experience of severe cases of positional brachycephaly with Chiari-1. However, to date, there have been no published cases linking nonsynostotic plagiocephaly with Chiari-1 malformation. CASE DESCRIPTION: An 18-month-old boy presented with a head injury. On examination he had a Glasgow Coma Score of 15 with no focal neurologic deficits, but he was noted to have marked posterior brachycephaly. A computed tomography scan showed a slim left-sided hemispheric acute subdural hematoma with no mass effect, which was treated conservatively. Of note, all of his cranial vault sutures were open, and a diagnosis of incidental positional plagiocephaly was made. Subsequent magnetic resonance imaging as part of a work-up to exclude nonaccidental injury showed a small posterior fossa with a steep tentorium and herniation of the cerebellar tonsils to the level of the body of the second cervical vertebra. CONCLUSIONS: Chronic hindbrain herniation is well reported in cases of craniosynostosis, but to our knowledge this is the first published case associated with nonsynostotic deformational plagiocephaly. We hypothesize that severe posterior plagiocephaly can cause disproportion of the posterior fossa: hindbrain volume ratio and acquired chronic cerebellar herniation. Nevertheless, positional plagiocephaly and Chiari-1 are common entities, and it is possible that the dual diagnoses were coincidental in this case. This report serves to raise awareness of a putative causal relationship between positional plagiocephaly, reduced posterior fossa volume, and hindbrain herniation.

Multiple bur hole surgery for the treatment of moyamoya disease in children.

OBJECT: The authors' aim in this study was to review their experience in the use of indirect revascularization alone in a series of 14 children with moyamoya disease, in which numerous bur holes and arachnoid openings were made over each affected hemisphere. METHODS: Revascularization through multiple bur holes and arachnoid openings was performed in 14 children (mean age at diagnosis 6.5 years [range 3-15 years]) who suffered from progressive moyamoya disease. The authors performed surgery in a total of 24 hemispheres during 18 procedures. Ten children underwent bilateral multiple bur hole procedures, three underwent a unilateral procedure in the more severely affected hemisphere, and one child had previously undergone an encephaloduroarteriomyosynangiosis on the contralateral side. Ten to 24 bur holes were made in the frontotemporoparietooccipital area of each hemisphere, depending on the site and extent of the disease. Early postoperative perfusion magnetic resonance imaging studies, performed in the five most recent cases, showed restoration of cortical perfusion as early as 3 months, which was confirmed on subsequent angiography studies (performed between 8 and 12 months postoperatively) that showed excellent revascularization of the ischemic brain by external carotid artery collateral vessels. None of the children sustained further ischemic attacks postoperatively. Motor improvement was noted in those who had presented with paresis. A single seizure episode occurred in two patients at 2 weeks and 5 months after surgery; both children had presented with epilepsy. There were no postoperative deaths, and only one complication (an infected lumbar shunt in the patient who required cerebrospinal fluid [CSF] drainage). Five of the 18 procedures were complicated by subcutaneous CSF collections, which resolved with tapping and compressive head dressings; a transient lumbar drain was necessary in one case. CONCLUSIONS: The results obtained in this series suggest that in children with moyamoya disease this simple technique is both effective and safe. Furthermore, it is effective as a sole treatment without supplementary revascularization procedures.