Raina K Patel, Valeria Mejia, Melanie Bakovic, Asli Pekcan, Marvee Turk, Alyssa Valenti, Mark M Urata, Jeffrey A Hammoudeh
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引用次数: 0
Abstract
Introduction: Stickler syndrome (SS) is a genetic collagenopathy characterized by visual impairment, hearing loss, joint pain, and cleft palate (CP). CP patients without SS typically undergo primary palatoplasty within the first year of life; in the SS population, however, medical comorbidities can result in palatoplasty occurring later than typically advised. In this study, we evaluate the impact of palatoplasty timing on long-term speech outcomes in SS patients.
Methods: A retrospective review was conducted of patients with SS who underwent palatoplasty at a tertiary children's hospital between 2006 and 2024. Data collection included patient demographics, medical and surgical history, and postoperative outcomes. Certified speech pathologists assessed speech using perceptual speech ratings. Spontaneous speech scales were used to assess articulation error severity relative to age-appropriate norms.
Results: A total of 26 patients were included, of whom 9 (35%) underwent palatoplasty within the first year of life and 17 (65%) underwent late palatoplasty after the first year of life. The overall median age at palatoplasty was 1.5 years. Palatoplasty was most commonly delayed by the need for mandibular distraction osteogenesis (n = 13), followed by respiratory distress and/or upper airway obstruction (n = 8). Late palatoplasty patients had an increased risk of obligatory errors (37.5% vs 0%, P = 0.010), compensatory errors (50% vs 11%, P = 0.044), and altered resonance (76.5% vs 44.4%, P = 0.024). Velopharyngeal insufficiency and nasal regurgitation did not significantly differ between groups.
Conclusions: Cleft surgeons should recognize that patients with SS may not adhere to the typical CP treatment paradigm due to additional medical comorbidities and may experience speech deficits as outlined above. Additional studies are required to optimize palatoplasty timing as it relates to speech outcomes in this population.
简介：Stickler综合征（SS）是一种以视力损害、听力损失、关节疼痛和腭裂（CP）为特征的遗传性胶原病。没有SS的CP患者通常在出生后一年内接受初级腭成形术；然而，在SS人群中，医学合并症可能导致腭裂成形术发生的时间比通常建议的要晚。在本研究中，我们评估腭成形术时机对SS患者长期言语预后的影响。方法：回顾性分析2006年至2024年在某三级儿童医院行腭裂成形术的SS患者。数据收集包括患者人口统计、病史和手术史以及术后结果。注册语言病理学家使用感知语音评分来评估语音。自发性言语量表用于评估相对于适龄标准的发音错误严重程度。结果：共纳入26例患者，其中9例（35%）在出生后一年内行腭成形术，17例（65%）在出生后一年内行晚期腭成形术。腭成形术的总中位年龄为1.5岁。腭成形术最常见的延迟原因是需要下颌牵张成骨（n = 13），其次是呼吸窘迫和/或上气道阻塞（n = 8）。晚期腭成形术患者出现强制性错误（37.5% vs 0%, P = 0.010）、代偿性错误（50% vs 11%, P = 0.044）和共振改变（76.5% vs 44.4%, P = 0.024）的风险增加。两组间腭咽功能不全和鼻反流无显著差异。结论：唇腭裂外科医生应该认识到，由于其他的医学合并症，唇腭裂患者可能不会坚持典型的唇裂治疗模式，并可能出现如上所述的语言缺陷。需要进一步的研究来优化腭成形术的时机，因为它关系到这一人群的语言预后。
期刊介绍：
The only independent journal devoted to general plastic and reconstructive surgery, Annals of Plastic Surgery serves as a forum for current scientific and clinical advances in the field and a sounding board for ideas and perspectives on its future. The journal publishes peer-reviewed original articles, brief communications, case reports, and notes in all areas of interest to the practicing plastic surgeon. There are also historical and current reviews, descriptions of surgical technique, and lively editorials and letters to the editor.
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