Current characteristics and management of congenital esophageal stenosis: 40 consecutive cases from a multicenter study in the Kyushu area of Japan
Current characteristics and management of congenital esophageal stenosis: 40 consecutive cases from a multicenter study in the Kyushu area of Japan
复制标题
DOI：
10.1007/s00383-017-4133-0
复制
发表时间：
2017-10-01
影响因子：
1.8
通讯作者：
Ieiri, Satoshi
中科院分区：
文献类型：
--
作者：
Suzuhigashi, Masaya;Kaji, Tatsuru;Ieiri, Satoshi
先天性食管狭窄（CES）较为罕见，现有的临床资料有限。我们探讨了CES目前的诊断、治疗及预后情况。利用九州地区小儿外科中心的病历进行了一项问卷调查。在10多年间，有40例患者（24例为男性）患有CES。合并畸形的发生率为52.5%（21/40），食管闭锁的发生率为20.0%（8/40）。诊断时的平均年龄为12.0个月（范围为1天 - 8.8岁）。7例（17.5%）患者在新生儿期被诊断出。10例（25.0%）因气管支气管残留导致CES，27例（67.5%）因纤维肌性狭窄（FMS）导致，1例（2.5%）因膜性狭窄 + FMS导致。36例（90.0%）患者接受了球囊扩张治疗（平均3次；范围为1 - 20次）。7例（17.5%）患者在扩张时发生穿孔，且均被诊断为FMS。18例（45.0%）患者接受了根治性手术（3例为初次手术，15例为扩张后继发手术）。我们的研究明确了CES的特征和预后，包括新生儿期的诊断情况。在九州地区，每33,000例新生儿中就有1例发生CES。即使是FMS的扩张病例也应格外注意。CES在经过充分且反复的治疗后，若症状持续存在，则需要长期随访。
Congenital esophageal stenosis (CES) is rare, and the available clinical data are limited. We explored the current diagnosis, treatment and outcomes of CES.A questionnaire survey was performed using medical records at pediatric surgical centers in the Kyushu area.Over 10 years, 40 patients (24 males) had CES. The incidence of associated anomalies was 52.5% (21/40), and that of esophageal atresia was 20.0% (8/40). The mean age at the diagnosis was 12.0 months (range, 1 day-8.8 years). Seven (17.5%) patients were diagnosed in the neonatal period. Ten (25.0%) developed CES due to tracheobronchial remnants, 27 (67.5%) due to fibromuscular stenosis (FMS) and 1 (2.5%) due to membranous stenosis + FMS. Thirty-six (90.0%) were treated by balloon dilatation (mean, 3 times; range, 1-20). Perforation at dilatation occurred in 7 (17.5%) patients, and all were diagnosed with FMS. Eighteen (45.0%) patients underwent radical operation (3 primary, 15 secondary to dilatation).Our study clarified the characteristics and outcomes of CES, including neonatal diagnoses. CES occurred in 1 in every 33,000 births in the Kyushu area. Careful attention should be paid, even in cases of dilatation for FMS. CES requires long-term follow-up for symptom persistence after adequate and repeated treatment.