Plastic and reconstructive surgery · 2026
Patient-Reported Speech Outcomes in Patients with Beckwith-Wiedemann Syndrome.
СтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Hu Allison C, George Andrew M, Viswanathan Aravind, Casalnova Abigail, Somma Makaela, Han Nicholas A, Tolley Philip D, Liao Eric C, Kalish Jennifer M, Taylor Jesse A
Аннотация
BACKGROUND: Macroglossia is a hallmark feature of Beckwith-Wiedemann Syndrome (BWS) and may affect a child's appearance, speech, and feeding. Tongue reduction surgery (TRS) is often recommended to address functional concerns, but its effects on patient-reported speech outcomes remain unclear. This study evaluates self-reported speech outcomes in individuals with BWS.
METHODS: Patients with BWS (aged 4-18 years) were prospectively administered the FACE-Q Craniofacial measures (speech distress, breathing, eating and drinking, facial function, speech function). Surveys were Rasch transformed score (0-100, 100=best outcome). Clinical characteristics, BWS Index of Macroglossia (BIG), and Intelligibility in Context Scale (ICS) scores were reviewed.
RESULTS: Thirty-six patients (50% male; mean age 8.6±3.8 years at survey) were included. Most patients had an IC2-LOM genetic diagnosis (n=24, 66.6%). Average BIG score was 1.3±1.1 (range 0-3). Speech therapy was received by 28 patients (77.8%) and 16 (44.4%) underwent TRS at mean age 2.3±2.0 years. FACE-Q scores averaged 84.7±15.0 for speech distress, 84.9±15.0 for breathing, 80.9±15.9 for eating and drinking, 92.0±12.1 for facial function, and 75.8±19.5 for speech function. There was moderate correlation between ICS score and speech function (r=0.697, p=0.001). Patients who received speech therapy at school and clinically had lower speech function (p=0.014). No significant differences were found between surgical and non-surgical patients or by BIG scores (p>0.1).
CONCLUSIONS: While overall reported outcomes were favorable, speech function scores were lower, suggesting persistent speech challenges in this population. These findings underscore the multifactorial nature of speech development in BWS and the importance of individualized, longitudinal management to optimize outcomes.
METHODS: Patients with BWS (aged 4-18 years) were prospectively administered the FACE-Q Craniofacial measures (speech distress, breathing, eating and drinking, facial function, speech function). Surveys were Rasch transformed score (0-100, 100=best outcome). Clinical characteristics, BWS Index of Macroglossia (BIG), and Intelligibility in Context Scale (ICS) scores were reviewed.
RESULTS: Thirty-six patients (50% male; mean age 8.6±3.8 years at survey) were included. Most patients had an IC2-LOM genetic diagnosis (n=24, 66.6%). Average BIG score was 1.3±1.1 (range 0-3). Speech therapy was received by 28 patients (77.8%) and 16 (44.4%) underwent TRS at mean age 2.3±2.0 years. FACE-Q scores averaged 84.7±15.0 for speech distress, 84.9±15.0 for breathing, 80.9±15.9 for eating and drinking, 92.0±12.1 for facial function, and 75.8±19.5 for speech function. There was moderate correlation between ICS score and speech function (r=0.697, p=0.001). Patients who received speech therapy at school and clinically had lower speech function (p=0.014). No significant differences were found between surgical and non-surgical patients or by BIG scores (p>0.1).
CONCLUSIONS: While overall reported outcomes were favorable, speech function scores were lower, suggesting persistent speech challenges in this population. These findings underscore the multifactorial nature of speech development in BWS and the importance of individualized, longitudinal management to optimize outcomes.