Friedmacher Florian, Kroneis Birgit, Huber-Zeyringer Andrea, Schober Peter, Till Holger, Sauer Hugo, Höllwarth Michael E
Department of Pediatric and Adolescent Surgery, Medical University of Graz, Auenbruggerplatz 34, 8036, Graz, Austria.
J Gastrointest Surg. 2017 Jun;21(6):927-935. doi: 10.1007/s11605-017-3423-0. Epub 2017 Apr 19.
Esophageal atresia (EA) and tracheoesophageal fistula (TEF) represent major therapeutic challenges, frequently associated with serious morbidities following surgical repair. The aim of this longitudinal study was to assess temporal changes in morbidity and mortality of patients with EA/TEF treated in a tertiary-level center, focusing on postoperative complications and their impact on long-term gastroesophageal function.
One hundred nine consecutive patients with EA/TEF born between 1975 and 2011 were followed for a median of 9.6 years (range, 3-27 years). Comparative statistics were used to evaluate temporal changes between an early (1975-1989) and late (1990-2011) study period.
Gross types of EA were A (n = 6), B (n = 5), C (n = 89), D (n = 7), and E (n = 2). Seventy (64.2%) patients had coexisting anomalies, 13 (11.9%) of whom died before EA correction was completed. In the remaining 96 infants, surgical repair was primary (n = 66) or delayed (n = 25) anastomosis, closure of TEF in EA type E (n = 2), and esophageal replacement with colon interposition (n=2) or gastric transposition (n=1). Long-gap EA was diagnosed in 23 (24.0%) cases. Postoperative mortality was 4/96 (4.2%). Overall survival increased significantly between the two study periods (42/55 vs. 50/54; P = 0.03). Sixty-nine (71.9%) patients presented postoperatively with anastomotic strictures requiring a median of 3 (range, 1-15) dilatations. Revisional surgery was required for anastomotic leakage (n = 5), recurrent TEF with (n = 1) or without (n=9) anastomotic stricture, undetected proximal TEF (n = 4), and refractory anastomotic strictures with (n = 1) or without (n = 2) fistula. Normal dietary intake was achieved in 89 (96.7%) patients, while 3 (3.3%) remained dependent on gastrostomy feedings. Manometry showed esophageal dysmotility in 78 (84.8%) infants at 1 year of age, increasing to 100% at 10-year follow-up. Fifty-six (60.9%) patients suffered from dysphagia with need for endoscopic foreign body removal in 12 (13.0%) cases. Anti-reflux medication was required in 43 (46.7%) children and 30 (32.6%) underwent fundoplication. The rate of gastroesophageal reflux increased significantly between the two study periods (29/42 vs. 44/50; P = 0.04). Twenty-two (23.9%) cases of endoscopic esophagitis and one Barrett's esophagus were identified.
Postoperative complications after EA/TEF repair are common and should be expertly managed to reduce the risk of long-term morbidity. Regular multidisciplinary surveillance with transitional care into adulthood is recommended in all patients with EA/TEF.
食管闭锁（EA）和气管食管瘘（TEF）是主要的治疗挑战，手术修复后常伴有严重的并发症。这项纵向研究的目的是评估在三级中心接受治疗的EA/TEF患者的发病率和死亡率的时间变化，重点关注术后并发症及其对长期胃食管功能的影响。
对1975年至2011年间出生的109例连续性EA/TEF患者进行了中位时间为9.6年（范围3 - 27年）的随访。采用比较统计学方法评估早期（1975 - 1989年）和晚期（1990 - 2011年）研究期间的时间变化。
EA的大体类型为A（n = 6）、B（n = 5）、C（n = 89）、D（n = 7）和E（n = 2）。70例（64.2%）患者存在合并畸形，其中13例（11.9%）在EA矫正完成前死亡。在其余96例婴儿中，手术修复为一期吻合（n = 66）或延迟吻合（n = 25）、E型EA的TEF闭合（n = 2）以及结肠代食管（n = 2）或胃转位（n = 1）。23例（24.0%）诊断为长段EA。术后死亡率为4/96（4.2%）。两个研究期间的总体生存率显著提高（42/55对50/54；P = 0.03）。69例（71.9%）患者术后出现吻合口狭窄，平均需要3次（范围1 - 15次）扩张。因吻合口漏（n = 5）、复发性TEF伴（n = 1）或不伴（n = 9）吻合口狭窄、未发现的近端TEF（n = 4）以及难治性吻合口狭窄伴（n = 1）或不伴（n = 2）瘘需要进行再次手术。89例（96.7%）患者实现了正常饮食摄入，而3例（3.3%）仍依赖胃造口喂养。测压显示78例（84.8%）婴儿在1岁时存在食管动力障碍，在10年随访时增至100%。56例（60.9%）患者有吞咽困难，其中12例（13.0%）需要内镜下取出异物。43例（46.7%）儿童需要抗反流药物治疗，30例（32.6%）接受了胃底折叠术。两个研究期间胃食管反流率显著增加（29/42对44/50；P = 0.04）。发现22例（23.9%）内镜下食管炎和1例巴雷特食管。
EA/TEF修复术后并发症常见，应进行专业管理以降低长期发病风险。建议对所有EA/TEF患者进行定期的多学科监测，并提供过渡性护理直至成年。
