Kartagener综合征诊疗及漏诊讨论

Missed diagnosis and treatment discussion of Kartagener syndrome

目的:探讨Kartagener综合征的临床特点、诊断标准及鼻窦炎鼻息肉的治疗方案。方法:回顾性分析河北医科大学第四医院耳鼻咽喉头颈外科门诊,首发症状为鼻窦炎,依次行心尖区听诊、X线正位胸片及胸部CT平扫检查,确诊为完全性Kartagener综合征的5例患者,行门诊干预治疗及1例入院鼻内镜鼻窦手术患者的病例临床诊疗资料,对门诊诊断、围手术期综合治疗疗效进行分析和研究。结果:本组5例患者的胸、腹腔脏器完全镜面内脏转位,胸部水平位CT示右位心、支气管扩张伴感染,同时表现不同程度的"印戒征"、"轨道征"及"蜂窝征"。鼻窦冠状位及平扫CT均见双侧上颌窦及筛窦炎症。5例患者均出现不同程度双侧分泌性中耳炎伴传导性聋。4例门诊予抗炎、黏液稀释及改善黏膜纤毛活性药对症治疗,1例患者因鼻部症状明显要求手术治疗,故收入院行局部麻醉下鼻内镜双侧上颌窦自然口扩大下鼻道开窗、筛窦轮廓化术,同时对症治疗。多年定期随诊,5例鼻部病变均减轻,支气管扩张伴感染复发次数减少及程度减轻,其中1例手术患者鼻部病变减轻明显,基本上皮化,无术后并发症发生。结论:完全性Kartagener综合征虽属罕见疾病,但提高对该病的认识结合心脏听诊、影像学检查可以减少漏诊,早期对症治疗及内镜鼻窦手术,可有效减轻鼻窦炎症状,降低支气管扩张肺部感染机会,有利于该病的转归。

Objective：To investigate the clinical feature and diagnostic criteria of Kartagener syndrome,and related treatment of sinusitis and nasal polyps. Method：The clinical data of 5 patients which were diagnosed as complete Kartagener syndromen in The forth hosipital of Hebei medical university were analyzed retrospectively. The first symptom of these patients was sinusitis. All of them accepted cardiac apical auscultation, X ray of orthopho- ria chest film and chest CT plain scan examination before diagnosis. All of them were treated intervention therapy in outpatient department,and 1 case accepted endoscopic sinus surgery in the hospital. The analysis of the data was to investigate the diagnosis in outpatient department and the perioperative therapy effect. Result： All of the 5patients＇ clinical feature were total mirror situs inversus viscerum,Dexcrocardia and brochiectasis associated with infection,＂ signet ring sign＂, ＂tram line sign＂, ＂honeycomb sign＂ at different degree on chest CT, bilateral maxillary and ethmoidal sinus inflammation on sinus CT. All the 5 patients appeared bilateral secretor otitis media associated with conductive deafness at different degree. 4 cases accepted anti-inflammation,mucus diluted and medicine treatment to improve cilia activity, i case accepted medicine and endoscopic sinus operation for obvious nasal symptoms. After following up for years, all symptoms of the 5 patients relieved, so did recurrent bronchiectasis infection. 1 case with operation had obviously relieved symptom and no postoperative complication. Conclusion.. Although complete Kartagener syndrome is infrequent disease, heart auscultation diagnosis and image examination are effective method to reduce missed diagnosis. Symptomatic treatment and suitable endoscopic sinus surgery method can reduce sinus symptom and the infection possibility of bronchiectasis effectively,which is beneficial for KS outcome.