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Obstructive sleep apnea (OSA) is a respiratory disorder that has a high prevalence in patients with craniofacial, neurocognitive, and neuromuscular disorders. Currently, the treatments for this population are diverse and depend on the individual conditions of the patient and the severity of the case. However, there are no multidisciplinary dental treatment guidelines. The aim of the present study was to determine the multidisciplinary dental treatment alternatives in patients with craniofacial, neurocognitive, and neuromuscular disorders with a diagnosis of OSA through evidence-based medicine. A systematic review of the literature has been performed by searching scientific articles in the PubMed, Cochrane, Ovid, ScienceDirect and Scopus databases, through controlled and uncontrolled language. Articles were classified according to the level of evidence and grades of recommendation through the Scottish Intercollegiate Guidelines Network. A total of 19,439 references were identified, of which 15 articles met the predetermined requirements to be included in the investigation. The articles included for this systematic review showed that mandibular distraction osteogenesis and adenotonsilectomy are the first-choice therapies for craniofacial and neurocognitive disorders. However, for neuromuscular disorders, the findings reported were not enough to provide information about surgical or nonsurgical alternatives. Despite the reported high frequency of OSA in those children with craniofacial, neurocognitive, and neuromuscular disorders, the evidence on the surgical and nonsurgical therapeutic success for OSA in these patients is scarce. It is necessary to perform future studies to investigate successful therapies for OSA in children. .
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http://dx.doi.org/10.3928/19382359-20231205-04 | DOI Listing |
Front Immunol
July 2025
Pediatria, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.
Introduction: Cardiofaciocutaneous syndrome (CFCS) is a rare syndromic disorder caused by germline mutations affecting the RAS/MAPK pathway. It is characterized by distinctive craniofacial dysmorphism, congenital heart defects, skin abnormalities, gastrointestinal dysfunction, neurocognitive impairment, and epilepsy. Emerging evidence suggests an association with hypogammaglobulinemia, but a comprehensive characterization of immunological abnormalities in CFCS is lacking.
View Article and Find Full Text PDFChilds Nerv Syst
July 2025
Division of Plastic Surgery, Department of Surgery, Yale School of Medicine, 330 Cedar Street, Boardman Building, New Haven, CT, 06510, USA.
Purpose: In this study, we explore the long-term cognitive capabilities and behaviors of patients in late adolescence and early adulthood who underwent corrective surgery for craniosynostosis as infants.
Methods: Patients 16 years of age or older who had undergone surgery as infants for any type of non-syndromic craniosynostosis were identified from the operating records of three craniofacial plastic surgeons from a single institution. Participants underwent standardized neurocognitive testing using the BEERY and WASI.
Orphanet J Rare Dis
May 2025
Hospital Pediátrico de Coímbra, Unidade Local de Saúde de Coímbra, Coimbra, Portugal.
Due to the craniofacial anatomy of people with achondroplasia, sleep-disordered breathing (SDB) occurs more frequently than in the average stature population. SDB, which comprises obstructive sleep apnoea (OSA), more rarely central sleep apnoea (CSA), and nocturnal alveolar hypoventilation (NH), may present at any age in patients with achondroplasia. Untreated SDB is associated with neurocognitive dysfunction, cardiovascular, and metabolic complications in children and adults.
View Article and Find Full Text PDFJ Neurosurg Pediatr
June 2025
1Faculty of Medicine, University of Basel.
Objective: Craniosynostosis is ideally treated within the 1st year of life to correct the physical deformity, achieve optimal aesthetic head shape, and facilitate normal neurocognitive development. While objective aesthetic assessments have been widely studied, limited reports on long-term parent-reported outcomes (PROs) exist. The authors herein report long-term follow-up after open midline strip craniectomy with barrel stave osteotomies (BSOs) and occipital release of nonsyndromic sagittal craniosynostosis (SC), including parental satisfaction; cosmetic, ophthalmological, and neurocognitive outcomes; and health-related quality of life (HRQOL).
View Article and Find Full Text PDFCraniosynostosis (CS) is the premature fusion of skull sutures, with all sutures except the metopic suture typically fusing in adulthood. Premature fusion constrains brain growth, leading to abnormal skull shape and potential neurocognitive or neurological issues, along with syndromic features in some cases. While CS is rare, its occurrence in siblings is exceptionally uncommon and holds significant academic importance.
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