OBJECTIVEThe authors evaluated the long-term outcome of their treatment protocol for Muenke syndrome, which includes a single craniofacial procedure.METHODSThis was a prospective observational cohort study of Muenke syndrome patients who underwent surgery for craniosynostosis within the first year of life. Symptoms and determinants of intracranial hypertension were evaluated by longitudinal monitoring of the presence of papilledema (fundoscopy), obstructive sleep apnea (OSA; with polysomnography), cerebellar tonsillar herniation (MRI studies), ventricular size (MRI and CT studies), and skull growth (occipital frontal head circumference [OFC]). Other evaluated factors included hearing, speech, and ophthalmological outcomes.RESULTSThe study included 38 patients; 36 patients underwent fronto-supraorbital advancement. The median age at last follow-up was 13.2 years (range 1.3–24.4 years). Three patients had papilledema, which was related to ophthalmological disorders in 2 patients. Three patients had mild OSA. Three patients had a Chiari I malformation, and tonsillar descent < 5 mm was present in 6 patients. Tonsillar position was unrelated to papilledema, ventricular size, or restricted skull growth. Ten patients had ventriculomegaly, and the OFC growth curve deflected in 3 patients. Twenty-two patients had hearing loss. Refraction anomalies were diagnosed in 14/15 patients measured at ≥ 8 years of age.CONCLUSIONSPatients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.

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Objectives This study aimed to investigate the malocclusion complexity and orthodontic treatment need among children with and without autism spectrum disorder (ASD) referred for orthodontic treatment by quantifying the Discrepancy Index (DI) and Index of Orthodontic Treatment Need (IOTN). Materials and methods Dental records of 48 ASD and 49 non-ASD consecutive patients aged between 9 and 18 years (median age 13.0 years) referred for orthodontic treatment were reviewed and compared. The Discrepancy Index (DI) was quantified to determine the malocclusion complexity, and the Index of Orthodontic Treatment Need (IOTN), including the Dental Health Component (IOTN-DHC) and Aesthetic Component (IOTN-AC), was quantified to determine the orthodontic treatment need. Statistical analysis included descriptive analysis, Pearson chi-square tests, Fisher’s exact test, Mann–Whitney U tests, and several univariate and multivariate regression analyses. The statistical analysis used descriptive analysis, Pearson chi-square test, Fisher’s exact test, and multivariate logistic regression. Results The results show that both malocclusion complexity (DI, p = 0.0010) and orthodontic treatment need (IOTN-DHC, p = 0.0025; IOTN-AC p = 0.0009) were significantly higher in children with ASD. Furthermore, children with ASD had a higher prevalence of increased overjet (p = .0016) and overbite (p = .031). Conclusions Malocclusion complexity and orthodontic treatment need are statistically significantly higher among children with ASD than children without ASD, independent of age and sex. Clinical relevance Children with autism may benefit from visits to a dental specialist (orthodontist) to prevent, to some extent, developing malocclusions from an early age.

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