Abstract：
OBJECTIVE: Apert syndrome (AS) is a rare congenital craniofacial disorder that requires a multidisciplinary approach to treatment and multiple surgeries. Given that cleft palate (CP) is presented in some of these cases, this poses an additional risk of aggravating obstructed airways after closure. The timing and outcome of CP repair in these patients remains disputed and requires additional attention.
METHODS: This retrospective analysis included patients diagnosed with CP and AS, born between 1950 and 2020, and treated at our institution. Data were collected from medical records and evaluated using descriptive statistics.
METHODS: Data analyses were conducted at Sahlgrenska University Hospital in Gothenburg, Sweden.
METHODS: A registry of 83 patients with AS resulted in a cohort of 26 patients also presenting with CP.
METHODS: Postoperative complications, requirement for intensive care, and reoperations following CP repair.
RESULTS: CP incidence among all registered patients was 31%. Patients undergoing CP repair at low age (mean: 22.5 months) tended to experience more frequent postoperative complications and requirements for intensive care. Among the evaluated cohort with medical records describing CP repair (n = 14), 29% experienced postoperative complications, all of which involved aggravation of obstructed airways.
CONCLUSIONS: This study highlights the importance of airway assessment before and after CP repair in AS. The findings suggest that surgical outcomes might benefit from postponing CP repair, avoiding combined surgeries, and operating in two stages when indicated. However, additional and larger studies are required.
METHODS: This retrospective analysis included patients diagnosed with CP and AS, born between 1950 and 2020, and treated at our institution. Data were collected from medical records and evaluated using descriptive statistics.
METHODS: Data analyses were conducted at Sahlgrenska University Hospital in Gothenburg, Sweden.
METHODS: A registry of 83 patients with AS resulted in a cohort of 26 patients also presenting with CP.
METHODS: Postoperative complications, requirement for intensive care, and reoperations following CP repair.
RESULTS: CP incidence among all registered patients was 31%. Patients undergoing CP repair at low age (mean: 22.5 months) tended to experience more frequent postoperative complications and requirements for intensive care. Among the evaluated cohort with medical records describing CP repair (n = 14), 29% experienced postoperative complications, all of which involved aggravation of obstructed airways.
CONCLUSIONS: This study highlights the importance of airway assessment before and after CP repair in AS. The findings suggest that surgical outcomes might benefit from postponing CP repair, avoiding combined surgeries, and operating in two stages when indicated. However, additional and larger studies are required.
摘要：
目的：Apert综合征（AS）是一种罕见的先天性颅面疾病，需要多学科的治疗方法和多种手术。鉴于其中一些病例存在腭裂（CP），这给关闭后阻塞的气道带来了额外的风险。这些患者CP修复的时机和结果仍然存在争议，需要额外的关注。
方法：这项回顾性分析包括诊断为CP和AS的患者，出生于1950年至2020年，在我们的机构接受治疗。从医疗记录中收集数据，并使用描述性统计进行评估。
方法：数据分析在哥德堡的Sahlgrenska大学医院进行，瑞典。
方法：一项83例AS患者的注册研究结果发现，有26例患者也出现CP。
方法：术后并发症，重症监护的要求，和CP修复后的重新操作。
结果：所有登记患者的CP发生率为31%。在低年龄（平均22.5个月）接受CP修复的患者往往会经历更频繁的术后并发症和重症监护要求。在描述CP修复的医疗记录的评估队列中（n=14），29%经历了术后并发症,所有这些都涉及气道阻塞的恶化。
结论：本研究强调了AS患者CP修复前后气道评估的重要性。研究结果表明，手术结果可能受益于推迟CP修复，避免联合手术，并在指示时分两个阶段运行。然而,需要更多和更大的研究。
方法：这项回顾性分析包括诊断为CP和AS的患者，出生于1950年至2020年，在我们的机构接受治疗。从医疗记录中收集数据，并使用描述性统计进行评估。
方法：数据分析在哥德堡的Sahlgrenska大学医院进行，瑞典。
方法：一项83例AS患者的注册研究结果发现，有26例患者也出现CP。
方法：术后并发症，重症监护的要求，和CP修复后的重新操作。
结果：所有登记患者的CP发生率为31%。在低年龄（平均22.5个月）接受CP修复的患者往往会经历更频繁的术后并发症和重症监护要求。在描述CP修复的医疗记录的评估队列中（n=14），29%经历了术后并发症,所有这些都涉及气道阻塞的恶化。
结论：本研究强调了AS患者CP修复前后气道评估的重要性。研究结果表明，手术结果可能受益于推迟CP修复，避免联合手术，并在指示时分两个阶段运行。然而,需要更多和更大的研究。
