Background Congenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES. Methods Medical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome. Results Over 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p?0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p?=?0.27). Conclusions CS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.
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机译:背景先天性食管狭窄(CES)是一种罕见的疾病,经常与食管闭锁(EA)相关。关于CES的表现,治疗和结局的小系列数据很少。方法回顾性分析法国食管畸形和先天性疾病网络中所有CES患者的病历,以诊断,治疗和预后。结果在18年的时间里,有61例患者(30名男孩)患有CES,其中29例(47%)患者也患有EA。确诊的平均年龄为24个月(1天至14岁),并且CES和EA患者比孤立CES的患者年轻(7个月与126个月,p <0.05)。 61例CES患者中有21例没有临床症状:在3例患者中,发现是偶然的;在29例伴发EA的患者中,有18例在外科手术或EA手术后被诊断出CES食管钡剂研究。在其他40例患者中,在诊断时,有50%出现吞咽困难,40%出现呕吐,50%患有食物感染和42%出现呼吸道症状。食管钡剂研究(56/61)和/或食管内镜检查(50/61)证实了CES的诊断。气管支气管残余(TBR)16例,纤维肌管狭窄(FMS)40例,膜狭窄(MS)5例。仅通过扩张术治疗了34例患者(56%)(随访时仍无症状)(13/34); 15例患者接受了扩张治疗,但由于失败而需要进行后续手术(随访中4/15仍无症状); 9例患者接受了主要的外科手术干预(4/9随访时无症状)。两名患者(3.4%)因食管穿孔而扩张。随访时,吞咽困难仍占36%(21/58),但是EA组和孤立CS组的发生率没有差异(10/29 vs. 7/32,p = 0.27)。结论与EA相关时,CS诊断可能会延迟。扩张术可以有效治疗FMS和MS患者,但是TBR患者通常需要手术修复。我们的结果清楚地表明,无论采用何种治疗方法,吞咽困难都会频繁发生,因此应长期随访CES患者。
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