第一鳃弓综合征并发皮样囊肿1例报告及文献复习

First branchial arch syndrome complicated with dermoid cyst:A case report and literature review

目的:分析第一鳃弓综合征并发皮样囊肿患者的临床资料,阐明其可能的发生机制,提高临床医生对该病的认识。方法:收集1例第一鳃弓综合征并发皮样囊肿患者的病例资料,并结合文献复习,探讨其临床特征、影像学表现、诊断和手术方法。结果:患者,女性,9岁,因右面部无明显诱因肿胀1周,自行抗炎治疗无效,肿胀缓慢加重入院。专科检查见面部左右不对称,右侧面部肿胀明显,表面可见窦道,挤压可见黄白色液体。两侧腮腺咬肌区可见附耳,右侧外耳道形态异常。患者父亲左侧外耳道畸形,耳屏形态异常,右侧耳屏前有一圆形赘生物。CT和MRI检查可见患者颌骨发育不全,右侧下颌升支后份及髁状突部分骨质缺如,右侧腮腺体积较对侧减小。彩超检查显示右面颊部皮下1.6 cm×1.1 cm以囊性为主囊实性回声。术后病理诊断为皮样囊肿。结论:第一鳃弓综合征伴有无明显诱因肿胀,抗炎治疗无效时可考虑第一鳃弓综合征并发皮样囊肿。该病有家族遗传倾向,手术为首选治疗方法。

Objective:To analyze the clinical materials of the patient with the first branchial arch syndrome complicated with dermoid cyst and clarify its pathogenesis,and to improve the understanding of the clinicians on this disease.Methods:The clinical materials of one patient with the first branchial arch syndrome complicated with dermoid cyst were collected.Combined with the relevant literatures,the clinical features,radiological appearance,and diagnosis and operation methods were discussed.Results:A 9-year-old girl was hospitalized because the right facial area had swollen with no obvious incentives for a week,the anti-inflammatory treatment was ineffective,and the swelling was slowly aggravated.The special examination showed that the face was asymmetrical,the right face was swollen,and the sinus was visible and yellow-white liquid could be squeezed out.The preauricular appendages from both sides of the parotideomasseteric region could be seen,and the right external ear canal was abnormal.The left external auditory canal and tragus of patient’s father was deformity and one round preauricular excrescence could be seen.The CT and MRI results showed maxillary and mandibular bone hypoplasia of the patient,sclerotin absent of the posterior portion of the right mandible ramus and smaller right parotid gland than the contralateral one.The color ultrasound results showed a subcutaneous 1.6 cm×1.1 cm echo on the right cheek containing cystic and solid components in which that cystic component was main.The postoperative pathological diagnosis was dermoid cyst.Conclusion:The first branchial arch syndrome exists unclear swelling;when anti-inflammatory treatment is ineffective,the first branchial arch syndrome complicated with dermoid cyst could be firstly considered.This disease has a familial hereditary tendency and the operation is the primary treatment method.