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

WANG Yanyan;XU Yanping;HE Xiao;HUANG Fanglin;Department of Otorhinolaryngology Head and Neck Surgery,the Second Hospital of Jilin University;Department of Otorhinolaryngology,Xiangya Hospital of Zhongnan University;  
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.
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