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开口于皮肤的窦道型先天性第四鳃裂畸形十例分析

Fourth branchial cleft deformity with skin orifice: a series of 10 cases

摘要目的 探讨一种开口于胸锁关节浅面皮肤的先天性窦道的胚胎来源及诊治.方法 回顾分析2010年1月-2015年6月广东省人民医院耳鼻咽喉头颈外科收治的10例开口于胸锁关节浅面皮肤的先天性窦道患儿的临床资料.结果 10例患儿,其中男4例、女6例;确诊年龄为11~96个月(平均为33.4个月);均位于左侧;所有病例均表现为生后左侧胸锁关节周围、胸锁乳突肌下端前内侧皮肤瘘口,感染后出现流脓、肿痛.细菌学培养,5例金黄色葡萄球菌,1例为克雷白杆菌.术前下咽造影5例次,双侧梨状窝底部未见瘘管;B超3例次,提示胸锁关节邻近椭圆形囊实性病灶,内部无回声,后方回声增强.MRI 6例次提示胸锁关节邻近等T1长T2信号影,增强扫描呈不均匀强化,病灶与胸锁乳突肌下端关系密切.10例患儿均于全麻下行“支撑喉镜检查+先天性鳃裂窦道切除术”.术后病理病灶呈管腔结构,内附复层鳞状上皮或纤毛柱状上皮,窦道平均长度11 mm (5~22 mm).术后无出血、感染等并发症,随访6~70个月(中位时间33个月)未见复发.结论 开口于胸锁关节浅面颈胸交汇处皮肤的鳃源性窦道较为罕见,可能为开口于皮肤的窦道型先天性第四鳃裂畸形,临床需与其他下颈部先天性疾病鉴别,炎症静止期彻底切除是根治的关键.

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abstractsObjective To report rare cases of congenital neck cutaneous sinus with an orifice near the sternoclavicular joint and to investigate their origins and managements.Methods A total of ten patients with congenital neck cutaneous sinus having an orifice near the sternoclavicular joint treated in the Guangdong General Hospital from January 2010 to June 2015 were retrospectively analyzed.Results There four boys and six girls,aging from 11 months to 96 months with an average of 33.4 months,and they had a common feature showing a congenital cutaneous sinus with an orifice near sternoclavicular joint.Discharge of pus from the orifice or abscess formation was commonly seen soon after infection.With bacteriological study,staphylococcus aureus was positive in five cases and klebsiella pneumonia in a case.Another orifice of fistula/sinus was not depicted in pyriform with barium swallow X-ray in five cases Ultrasound studies of three cases demonstrated anechoic (i.e.,nearly black) and solid-cystic lesion near sternoclavicular joint with posterior acoustic enhancement.Magnetic resonance imaging (MRI) showed isointensity of the lesion on T1 and T2 weighted images with heterogeneous enhancement and a close relationship with sternoclavicular joint.All patients underwent laryngoscopic examination,which showed no orifice of sinus in pyriform at same side.Surgical resection of fistula/sinus was performed in all cases.The lengths of the fistula varied from 5 mm to 22 mm with an average of 11 mm.Postoperative pathological examination showed all specimens were accordance with fistula.No complications were noticed.Recurrence was not observed in the cases by following-up of 6 months to 70 months (median:33 months).Conclusion Congenital neck cutaneous sinus with orifice near the sternoclavicular joint maybe a special clinical phenotype of the fourth branchial cleft sinus with skin orifice in cervicothoracic junction.Differential diagnoses between low cervical diseases are required.The curative treatment is a complete excision during inflammatory quiescent period.

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DOI 10.3760/cma.j.issn.1673-0860.2016.10.013
发布时间 2016-11-15
基金项目
“十二五”国家科技支撑计划“中国罕见疾病防治研究与示范”项目(2013BAI07B00)National Science and Technology Infrastructure Program during the 12th Five-Year Plan Period
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