33例先天性食管狭窄的诊治分析

Diagnosis and treatment of congenital esophageal stenosis

目的总结先天性食管狭窄的诊治经验,探讨先天性食管狭窄的早期诊治方法,降低误诊率。方法回顾性分析1990—2012年我们收治的33例先天性食管狭窄患儿临床资料,对其临床表现、诊断、治疗方法及病理结果进行分析。结果 33例患儿中,男性16例,女性17例,仅17例(51%)首次就诊得以确诊,首次就诊误诊率达49%,平均首次发病年龄为8.6个月,确诊平均年龄为33.8个月。以进食后呕吐、生长发育迟缓、吞咽固体食物困难、贫血为主要表现。患儿术前均行钡餐检查。26例(79%)予手术治疗,疗效好,术后并发症少,随访1个月至10年,远期预后良好。术后病理活检21例,诊断为气管软骨食管异位症13例,纤维肌层肥厚8例。结论对于进食后反复呕吐及吞咽困难的患儿均应想到先天性食管狭窄的可能。食管钡餐检查是最简单有效的诊断方法。食管扩张术治疗先天性食道狭窄存在争议,手术是治疗先天性食管狭窄可靠、有效、安全的方法 。

Objetive Congenital esophageal stenosis （CES）is a rare malformation.And esophageal dilations and surgical resection are routine treatments.To explore the clinical diagnosis and treatment of CES soas to reduce the rate of its misdiagnosis and improve its early treatment. Methods A retrospective study wasconducted for 33 CES cases undergoing esophageal dilatation or operation from 1990 to 2012.Their clinicalmanifestations,diagnosis,treatment and pathological findings were reviewed. Results Only 17 cases（51%）were diagnosed correctly preoperative.And the rate of misdiagnosis was 49% for initial visitors and even higherfor tracheobrochial remnants.（72%）Symptoms of dysphagia and regurgitation developed at a mean age of8.6,definitive treatment was offered at a mean age of 33.8 with a time lag of almost 2 years from the onset ofsymptoms.Clinical manifestations included postprandial vomiting,regurgitation,failure of thriving,dysphagiaand anemia.The underlying diagnosis was usually confirmed by medical history,esophagogram and histopathological examination of resected segment.Esophagography,as a primary diagnostic tool,was performed in allpatients.After a mean followup period of 3 years （1 month to 10 years ）,26 patients（79%）required operations.The postoperative complications included anastomotic stenosis,anastomotic,gastroesophageal reflux aftera mean follow -up period of 3 years （range,1 month to 10 years）.There was no case of mortality.Operationwas the treatment of choice and carried little morbidity and mortality.CES could be classified based on the histological type of stenosis.There were tracheobronchial remnants （TBR,n =13）and fibromuscular hypertrophyor web （FMH,n =8）.Conclusions CES should be suspected in patients with a typical history of recurrentvomiting and dysphagia and characteristic esophagographic findings.If dilation is ineffective,operation is anoptimal treatment with a low morbidity and mortality.