ABBREVIATIONS CZS Congenital Zika syndrome JBI Joanna Briggs Institute ZIKV Zika virus AIM To describe the standardized neurodevelopmental outcomes after the first year of life in children with congenital Zika syndrome (CZS) and those exposed to Zika virus (ZIKV) during fetal life, but without microcephaly at birth. METHOD This scoping review included observational studies about the standardized neurodevelopmental outcome in children with CZS or exposed to ZIKV, but without microcephaly, assessed after 12 months of age. The databases searched were MEDLINE/ Pubmed, LILACS, Scielo, Scopus, PsycINFO, CINAHL, and Embase. Risk of bias was assessed with the Joanna Briggs Institute Critical Appraisal Checklists. RESULTS Seventeen papers were included: 12 focused on children with CZS, four on children born without microcephaly, and one described both. Only one of the studies about CZS reported a child with microcephaly and typical development; the remainder described a severe pattern of global developmental delay and cerebral palsy. The prevalence of epilepsy was 74.6%. In the reports about children born without microcephaly, 6.9% to 8.7% had some domain with a score below À2 SD, and three children developed autism spectrum disorder. INTERPRETATION CZS is associated with severe global developmental delay and cerebral palsy after 1 year of age. In children born without microcephaly, although most have typical development, some may be at risk for impairments.

Purpose To characterize swallowing in children with congenital Zika virus syndrome in comparison to typical children. Methods This cross-sectional study enrolled 45 children diagnosed with congenital Zika virus syndrome and 45 others with typical development. Swallowing was evaluated through clinical feeding evaluations Protocolo de Avaliação Clínica da Disfagia Pediátrica and using acoustic swallowing parameters (Doppler sonar). Results The mean age of children with congenital Zika virus syndrome was 26.69 ± 4.46 months and the mean head circumference was 29.20 ± 1.98 cm. Moderate/severe oropharyngeal dysphagia was found in 32(71.1%) of the children with congenital Zika virus syndrome. Significant differences were found between the groups on clinical evaluation: Children with congenital Zika virus syndrome presented insufficient lip closure 42(93.3%) and altered tonus of the tongue 35(77.8%) and cheeks 34(75.6%). In the children in the comparison group, only 6(13.3%) presented insufficient lip closure and 1(2.2%) had inadequate tongue posture. Changes during swallowing with liquid and spoonable food were not observed in the comparison group. When liquid/food was offered, affected children presented difficulties in sipping movements 14(77.8%) and lip/spoon contact 35(75%). The presence of residual food in the oral cavity after swallowing 38(86.4%) and clinical signs indicative of laryngotracheal penetration/aspiration, such as coughing, gagging and/or labored breathing, were also notable. No differences were found between the groups with regard to the acoustic parameters evaluated instrumentally. Conclusion Children with congenital Zika virus syndrome present alterations in the oral phase of swallowing, as well as clinical signs indicative of pharyngeal phase impairment.

Objective:The main aim of this study was to determine the impact of botulinum toxin A (BTX-A) on severity and frequency of drooling in children with Cerebral Palsy (CP) secondary to Congenital Zika Syndrome (CZS). Methods: This is a prospective longitudinal observational study including 23 children who received bilateral injections of BTX in the parotid and submandibular glands. The Thomas-Stonell & Greenberg Drooling Severity and Frequency Scale was applied by a multidisciplinary team including Speech, Language and Hearing professionals. The Global Impression of Improvement (GII) Scale was also applied to assess parents' subjective perceptions of therapeutic response. Swallowing was assessed using Doppler ultrasonography. Univariate logistic regression was used to analyse differences between responders and non-responders. Results: Participant age varied from 27 to 38 months (mean 31.78, SD = 2.61) all presented with Gross Motor Function Classification System (GMFCS) V. Drooling Severity and Frequency Scale scores ranged from 7 to 9 points (median = 9) prior to BTX administration and from 4 to 6 (median = 6) after. Pre-and post-treatment reduction in drooling severity occurred (Z = −3.746; p < 0.001). No cases of drooling worsening were reported. Only two subjects presented adverse effects attributed to BTX administration. Correlation was only confirmed with GII. Discussion: This article presents the safe and positive impact of BTX-A administration guided by anatomical references described in the literature, even on children with microcephaly. Further studies are needed to facilitate the use of Doppler ultrasonography as a tool to characterize changes in sensory processing and motor response following intraoral input in children with CP.

Schuurs-Hoeijmakers syndrome, an autosomal dominant disorder associated with mutations in the PACS1 gene, was initially identified in two unrelated children of European descent from a cohort of individuals with intellectual disabilities. This gene alteration significantly reduced cranial cartilaginous structures, inducing craniofacial alterations predominantly in a dominant-negative fashion. In this paper, we report a novel variant of PACS1 associated with Schuurs-Hoeijmakers syndrome: a boy aged two years and nine months of indigenous descent presenting with motor stereotypies, atypical sensory searches, language delay, and low socio-interactional reciprocity. Whole exome sequencing confirmed the presence of a heterozygous missense mutation c.943C>T p. (Arg315Trp) in the PACS1 gene. The phenotypic profile identified was similar to the other cases of Schuurs-Hoeijmakers syndrome described in the literature. This report highlights the importance of considering the possibility of PACS1 gene alterations and a diagnosis of Schuurs-Hoeijmakers syndrome in patients presenting craniofacial alterations associated with autistic features, psychomotor and language development delay.

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