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目的:探讨Crouzon综合征患儿的临床特点、家族史、治疗及预后。方法:回顾性分析6例Crouzon综合征患儿的临床资料。6例患儿睡眠打鼾伴张口呼吸,其中5例伴有憋气(可憋醒)。5例伴有听力下降,其中1例右耳反复流脓。入院后6例患者均在全身麻醉下行手术治疗,其中2例行扁桃体、腺样体切除及鼓膜切开术,1例行腺样体切除及鼓膜切开术,2例行单纯腺样体切除术,1例行右侧外耳道胆脂瘤清除术。结果:随访0.5~7.0年,6例患儿中5例术后睡眠时无憋气及憋醒,偶有轻微鼾声,可闭口呼吸;另1例行右侧外耳道胆脂瘤清除术的患儿术后睡眠打鼾无改善;术前伴有听力下降的5例患儿术后给予耳部对症治疗后听力提高;4例患儿尖颅畸形、眼球突出、下颌骨前突及上下齿反咬合情况无明显进展,2例整体颅面部畸形较前减轻。结论:掌握Crouzon综合征的临床特点,有助于该病的早期诊断,根据患儿的临床症状及体征制定个性化治疗方案,尽早干预,可有效改善患儿预后,提高生活质量。
Objective: To investigate the clinical features, family history, treatment and prognosis of children with Crouzon syndrome. Methods: The clinical data of 6 children with Crouzon syndrome were retrospectively analyzed. 6 cases of children with sleep snoring with mouth breathing, 5 cases with suffocation (can wake up). 5 cases accompanied by hearing loss, of which 1 case of right ear repeated pus. After admission, all the 6 patients underwent surgical treatment under general anesthesia. Among them, tonsillectomy, adenoidectomy and tympanotomy were performed in 2 cases, adenoidectomy and tympanotomy in 1 case, adenoidectomy in 2 cases Surgery, 1 case of right ear canal cholesteatoma removal. Results: During the follow-up of 0.5-7.0 years, 5 of 6 children had no suffocation and arousal during sleep, with occasional slight snoring, which could be closed breath. Another patient with pediatric cholesteatoma Postoperative sleep snoring no improvement; preoperative with hearing loss in 5 cases of children with postoperative hearing given ear symptomatic treatment to improve; 4 cases of children with cricoid deformity, prominence, mandibular protrusion and the upper and lower teeth anti-bite no Significant progress, 2 cases of craniofacial deformities than the previous reduction. Conclusion: To master the clinical features of Crouzon syndrome is helpful for the early diagnosis of the disease. According to the clinical symptoms and signs of children, we can formulate individualized treatment plan and make intervention as early as possible, which can effectively improve the prognosis of children and improve the quality of life.