Literature DB >> 30376501

Improved Quality of Life for Children With Beckwith-Wiedemann Syndrome Following Tongue Reduction Surgery.

Takeshi Harada1, Tadashi Yamanishi, Takayuki Kurimoto, Juntaro Nishio.   

Abstract

Macroglossia is observed in the majority of patients with Beckwith-Wiedemann syndrome (BWS), a congenital condition with characteristic anomalies. In addition, sleep disordered breathing (SDB) associated with macroglossia has occasionally been noted in BWS patients, for which tongue reduction surgery is generally performed. However, macroglossia is related to various health problems, such as eating and articulation disorders, attention deficit disorder, enuresis, and thorax deformation, as well as aesthetic issues. Therefore, in addition to treating airway obstruction, tongue reduction surgery can also exert favorable effects on the overall quality of life (QOL) of patients. However, surgical indications for tongue reduction have yet to be established and reports on QOL assessments after surgery are rare. The OSA-18 questionnaire is used for screening SDB and is also useful for assessing the effect of tongue reduction surgery on QOL. We report a patient whose QOL was improved by tongue reduction surgery. On the basis of our patient's results, we recommend tongue reduction surgery in early childhood not only to treat SDB but also to improve QOL.

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Year:  2019        PMID: 30376501     DOI: 10.1097/SCS.0000000000004823

Source DB:  PubMed          Journal:  J Craniofac Surg        ISSN: 1049-2275            Impact factor:   1.046


  3 in total

1.  Maxillo-Facial Morphology in Beckwith-Wiedemann Syndrome: A Preliminary Study on (epi)Genotype-Phenotype Association in Caucasians.

Authors:  Patrizia Defabianis; Alessandro Mussa; Rossella Ninivaggi; Diana Carli; Federica Romano
Journal:  Int J Environ Res Public Health       Date:  2022-02-20       Impact factor: 3.390

2.  The Utility of Early Tongue Reduction Surgery for Macroglossia in Beckwith-Wiedemann Syndrome.

Authors:  Jennifer L Cohen; Christopher M Cielo; Jonida Kupa; Kelly A Duffy; Evan R Hathaway; Jennifer M Kalish; Jesse A Taylor
Journal:  Plast Reconstr Surg       Date:  2020-04       Impact factor: 4.730

3.  Oral Health-Related Quality of Life among Children and Adolescents with Beckwith-Wiedemann Syndrome in Northern Italy.

Authors:  Patrizia Defabianis; Rossella Ninivaggi; Federica Romano
Journal:  J Clin Med       Date:  2022-09-26       Impact factor: 4.964

  3 in total

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