目的:评价解剖肌解剖修复对22q11.2缺失综合征粘膜下腭裂(SMCP)患者咽喉功能不全(VPI)的疗效。
方法:对一名外科医生超过10年的管理进行回顾性盲法随机分析。
方法:本研究在英国一家儿科专科医院进行。
方法:SMCP和22q11.2缺失综合征的儿童。
方法:所有参与者都接受了根治性肌肉剥离术。
方法:术前和术后测量包括解剖缺损的严重程度,数字化的语音样本和横向图像,随机化,然后使用经过验证的技术进行外部和盲目分析。采用Stata软件进行统计分析。
结果:57例22q11.2缺失综合征患儿被纳入本分析。术中,大多数病例被确定为SMCPI级异常.术后,鼻塞的显著改善,静息腭长度,最大闭合时的腭长度,观察到最大闭合时的腭偏移和间隙大小。到10年,59%的患者进行了二次手术。
结论:肌肉剥离修复可改善鼻塞,22q11.2缺失综合征患者的腭闭合功能和闭合间隙。尽管超过50%的患者可能需要进一步手术,肌肉解剖修复应该是第一步,因为它在年轻的时候就很实用,当侵入性调查是不可能的时候,其发病率较低,语音和语言的好处或改变计划,以减少阻塞的二次手术,当它导致减少咽喉间隙和改善上颚活动时,即使没有实现足够的咽喉闭合。
OBJECTIVE: To evaluate the benefit of anatomical muscle dissection repair for velopharyngeal insufficiency (VPI) in patients with submucous cleft palate (SMCP) with 22q11.2 deletion syndrome.
METHODS: Retrospective blinded randomised analysis of a surgeon\'s management over 10 years.
METHODS: The study was performed at a specialised Paediatric hospital in the United Kingdom.
METHODS: Children with SMCP and 22q11.2 deletion syndrome.
METHODS: All participants underwent radical muscle dissection veloplasty.
METHODS: Pre- and post- operative measurements included severity of anatomical defect, speech samples and lateral images which were digitised, randomised then externally and blindly analysed using validated techniques. Stata software was used to perform statistical analysis.
RESULTS: 57 children with 22q11.2 deletion syndrome were included in this analysis. Intra-operatively, the majority of cases were identified as SMCP Grade I anomalies. Post-operatively, a statistically significant improvement in hypernasality, resting palate length, palate length at maximum closure, palate excursion and gap size at maximum closure was observed. Secondary surgery was performed for 59% of patients by ten years.
CONCLUSIONS: Muscle dissection repair improves hypernasality, palate closure function and the closure gap in patients with 22q11.2 deletion syndrome. Although over 50% of patients may require further surgery, muscle dissection repair should be a first step due to its utility at a younger age, when invasive investigations are impossible, its lower morbidity, speech and language benefits or altering the plans for less obstructive secondary surgery when it lead to reduced velo-pharyngeal gap and improved palate mobility even when adequate velo-pharyngeal closure was not achieved.