双侧第四鳃裂畸形1例并文献复习

Bilateral fourth branchial fissure:a case report and literature review

目的探讨第四鳃裂畸形的诊断与治疗方法。方法对1例颈部双侧第四鳃裂畸形的患者临床资料进行总结与文献回顾分析。结果患者,17岁,男性,颈部无痛性肿块10年,专科检查:颈前偏右皮下可触及一肿块,大小约4.0 cm×3.0 cm,边界清楚,形态规则,质软,触有波动感,无明显压痛。颈前偏左可见一大小约0.5 cm×0.5 cm的瘘口,瘘口可见黄色清亮液体流出,周围皮肤局部红肿,皮肤表面温度升高;计算机断层扫描检查示:右侧锁骨上区、颈前区见类圆形囊性低密度影,大小约4.4 cm×3.4 cm,其内片絮状等密度影,边缘见结节状钙化,周围脂肪间隙模糊,增强扫描囊壁轻度强化,内容物未见明显强化;其左侧可见直径约1.4 cm类圆形结节影,增强扫描环形强化,周围皮肤增厚,皮下脂肪间隙模糊;双侧颈部见多发小淋巴结显示,较大者短径约0.8 cm;甲状腺大小、形态未见明显异常,其内未见明显异常密度影。入院诊断为右颈部第四鳃裂囊肿,左颈部第四鳃裂瘘管。在全麻静脉复合麻醉下行右侧鳃裂囊肿切除术+左侧鳃裂瘘管切除术。术后病理检查提示为右鳃裂囊肿,左鳃裂瘘管。伤口I期愈合,术后随访6个月无复发。文献回顾复习结果表明,第四鳃裂畸形是鳃器的先天性发育异常,发生率仅占所有鳃裂畸形的1%,常发生于左侧。解剖学位置常位于颈根部、锁骨上区,表现为与甲状腺相毗邻的囊肿或窦道。通过其解剖学位置、影像学检查或喉镜检查结合术后病理结果明确诊断,需要与颈部肿块如甲状舌管囊肿、淋巴结转移瘤等相鉴别。主要治疗手段为外科手术和内窥镜烧灼内瘘口,预后一般较好,有复发风险,但很少发生癌变。结论第四鳃裂畸形十分罕见,应早期识别,避免过多无效的手术引流,减少切除时的潜在并发症并彻底切除病变防止复发。

Objective To explore the diagnosis and treatment of fourth branchial cleft deformity.Methods The clinical data of a patient with bilateral fourth branchial cleft deformity in the neck were summarized,and the literature was reviewed.Results The patient was a 17-year-old male who had a painless lump in his neck for 10 years.During specialized examination,a lump approximately 4.0 cm×3.0 cm in size could be palpated subcutaneously on the right side of the neck,with clear boundaries,a regular shape,a soft texture,and a wave-like sensation without obvious tenderness.A fistula with a size of approximately 0.5 cm×0.5 cm could be observed on the left side of the neck,and yellow clear liquid could be seen flowing out of the fistula.The surrounding skin was locally red and swollen,and the surface temperature of the skin was elevated.Computed tomography examination demonstrated a circular cystic low-density shadow approximately 4.4 cm×3.4 cm in size in the right supraclavicular and anterior cervical regions.A flocculent isodense image could be observed in the middle;moreover,nodular calcification could be observed at the edge,and the surrounding fat spaces were blurred.The enhanced scan showed mild enhancement of the cyst wall but no obvious enhancement of the contents.On the left side,a circular nodular shadow with a diameter of approximately 1.4 cm could be seen,with enhanced scanning and circular enhancement.The surrounding skin was thickened,and the subcutaneous fat gap was blurred.Multiple small lymph nodes could be observed on both sides of the neck,with the larger nodes having a short diameter of approximately 0.8 cm.The size and morphology of the thyroid gland were not significantly abnormal,and there was no obvious abnormal density shadow inside of the gland.Upon admission,the diagnosis was a fourth gill fissure cyst in the right neck and a fourth gill fissure fistula in the left neck.Under general anesthesia and intravenous anesthesia,right branchial cleft cyst resection and left branchial cleft fistula resection were performed.Postoperative pathological examination demonstrated a left branchial cleft fistula and a right branchial cleft cyst.The wound healed by first intention,and there was no recurrence after 6 months of follow-up.According to the literature,fourth branchial cleft deformity is a congenital developmental abnormality of the branchial apparatus,the incidence of which accounts for only 1%of all branchial cleft deformities;moreover,it often occurs on the left side.The anatomical position is often located in the cervical root and supraclavicular region,thus demonstrating cysts or sinuses adjacent to the thyroid gland.The diagnosis should be confirmed by anatomical location,imaging examination or laryngoscopy combined with postoperative pathological results and should be differentiated from cervical masses such as thyroglossal duct cysts and lymph node metastasis.The main treatment methods include surgical procedures and endoscopic cauterization of the internal fistula.The prognosis is generally good,and there is a risk of recurrence;however,cancer rarely occurs.Conclusion Deformity of the fourth branchial fissure is very rare;thus,it should be identified early to avoid excessive and ineffective surgical drainage,reduce potential complications during resection and completely remove the lesion to prevent recurrence.