6例Crouzon综合征患儿的临床特点、家族史及预后分析  

Clinical analysis of the character,family history and prognosis of 6 cases with Crouzon syndrome

作　　者：王言言[1] 徐艳萍[1] 何晓[1] 黄芳林[2] 

机构地区：[1]吉林大学第二医院耳鼻咽喉头颈外科,长春130041 [2]中南大学湘雅医院耳鼻咽喉科

出　　处：《临床耳鼻咽喉头颈外科杂志》2017年第2期142-145,共4页Journal of Clinical Otorhinolaryngology Head And Neck Surgery

摘　　要：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 characteristics,family history,treatment and prognosis of patients with Crouzon syndrome.Method：We reviewed and analyzed the clinical data of 6patients with Crouzon syndrome.All patients suffered from snore during sleep,along with mouth opening,in which 5cases had apnea and could be awaken.In addition,five of six experienced hearing loss,including one case with right ear discharging repeatedly.During the treatment,all patients were operated under general anesthesia.Among six cases,two were performed by tonsillectomy,adenoidectomy,and myringotomy;one was treated by adenoidectomy and myringotomy;two were performed by adenoidectomy;one was treated by external auditory canal cholesteatoma exenteration.Result：Within half a year to seven years’ follow-up,5patients snore occasionally at night but without sleep apnea,while 1patient performed by external auditory canal cholesteatoma exenteration has no improvement in sleep snoring.Additionally,the hearing of 5patients have been greatly improved after operation and the cranial facial deformity of 2cases have been alleviated than before,while 4patients have no alleviation in oxycephaly,exophthalmos,mandibular prognathism and malocclusion.Conclusion：Understanding the clinical characteristics of Crouzon syndrome significantly contributes to early diagnosis of this disease.Individual-based treatment should be carried out according to the clinical symptoms and signs of patients.Early intervention benefit to the improvement of prognosis and the life quality of patients.

关 键 词：CROUZON综合征 颅面发育 眼球突出 

分 类 号：R816.98[医药卫生—放射医学]