综述
先天性食管闭锁术后吻合口狭窄的治疗进展
中华小儿外科杂志, 2019,40(10) : 956-959. DOI: 10.3760/cma.j.issn.0253-3006.2019.10.020
摘要

食管闭锁(esophageal atresia,EA)作为新生儿期消化道重症疾病之一,是最常见的先天性食管畸形,发病率为1/4 500~1/2 500。随着围手术期护理和手术技术的改善,其总体生存率达到了91%~97%。手术方式有经典开胸手术和胸腔镜下手术,术后并发症主要有吻合口狭窄、吻合口瘘、胃食管反流、气管软化、气管食管瘘复发等。其中术后吻合口狭窄发病率最高,约为18%~60%,严重影响EA患儿的生活质量。本文就先天性食管闭锁术后吻合口狭窄的治疗进展做一综述。

引用本文: 张宾宾, 柳龚葆, 董岿然. 先天性食管闭锁术后吻合口狭窄的治疗进展 [J] . 中华小儿外科杂志, 2019, 40(10) : 956-959. DOI: 10.3760/cma.j.issn.0253-3006.2019.10.020.
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食管闭锁(esophageal atresia,EA)作为新生儿期消化道重症疾病之一,是最常见的先天性食管畸形,发病率为1/4 500~1/2 500[1,2,3]。随着围手术期护理和手术技术的改善,其总体生存率达到了91%~97%[4,5]。手术方式有经典开胸手术和胸腔镜下手术,术后并发症主要有吻合口狭窄、吻合口漏、胃食管反流、气管软化、气管食管瘘复发等[6]。其中术后吻合口狭窄发病率最高,约为18%~60%,严重影响EA患儿的生活质量[7,8,9,10,11,12,13,14,15]

目前,EA后食管狭窄的外科治疗方法分为3类:食管扩张治疗(球囊扩张和探条扩张)、手术治疗和支架治疗。

一、扩张治疗

食管扩张治疗既可以改善食管闭锁患儿术后食管狭窄引起的进食困难,满足生长发育对营养摄入的需求,同时又避免了再次开胸/腹手术带来的风险和再狭窄并发症,已成为食管闭锁术后吻合口狭窄治疗的首选方法[16,17]。但扩张治疗的指征、平均扩张次数、有效率和穿孔发生率均需进一步讨论。

1.扩张治疗指征

有机构在食管闭锁术后常规进行定期内镜下食管扩张,也有的是在食管闭锁术后有吞咽困难症状后再行内镜下食管扩张治疗[18,19]。Koivusalo等[16]在研究中对比2家医疗机构,A机构在出现吞咽困难症状后行扩张治疗,B机构术后3周常规行规律的扩张治疗(1~3周/次),直至狭窄段食管直径达到10 mm,结果提示A机构患儿平均扩张次数和平均住院日均明显低于B机构,而术中并发症发生率无明显差别,经过2年的随访2家机构患儿出现吞咽困难、呼吸道症状、食物梗阻的发生率也无明显差别,在一定程度上说明对有症状的食管狭窄行食管扩张的必要性。

2.扩张方法的比较

对食管狭窄术后吻合口狭窄行扩张治疗主要有2种方法:球囊扩张和探条扩张。球囊扩张时所受的力主要是放射状的力,而探条扩张的力是放射状的力加上从狭窄段近端至远端的纵向剪切力[20]

一项包含5项研究的系统评价针对球囊扩张(透视下/内镜下),其中139例食管闭锁术后吻合口狭窄患儿共经历401次扩张,成功率70%~100%,平均扩张次数2.88次/人,穿孔率为1.8%[21]。在一项大型研究中,对107例食管狭窄的患儿行沙式探条扩张术,成功率为93.7%(104/107),平均扩张次数为6次,穿孔率为0.9%(6/648),但其中包含有食管化学烧灼伤导致的食管狭窄[22]

在另外2项回顾性研究中,Thyoka等[21]报道在食管闭锁术后吻合口狭窄中球囊扩张平均扩张次数小于探条扩张次数,然而仅在行球囊扩张的患儿发生食管穿孔。Jayakrishnan等[23]报道透视下球囊扩张穿孔发生率较探条扩张的穿孔发生率低。

因此2种扩张方法的选择优劣缺乏有力证据,选择时主要根据术者的经验和喜好,除去工具本身,术者经验才是减少术中并发症的关键,欧洲小儿胃肠营养学会-北美小儿胃肠病专家指南仅建议使用导丝引导下行扩张治疗(球囊/探条)[20,24]

文献报道食管闭锁术后患儿发生吻合口狭窄年龄范围为1个月至16岁,接受扩张治疗的范围为2.8~6.4次,扩张有效率(治疗有效定义为患儿经过治疗后可正常进食)为73.6%~100%,穿孔发生率为0.3%~5.7%,术后随访时间为6~96个月[17,25,26,27,28,29,30,31,32]。另也有报道球囊扩张结合静脉注射甲基强的松龙可更有效治疗食管闭锁术后吻合口狭窄[33]

二、手术治疗

对于行扩张治疗和支架治疗无效的术后吻合口狭窄,才建议行手术治疗,手术方法有吻合口狭窄切开和吻合口狭窄切除再吻合,以及代食管术[20,34,35]。难治性和复发性食管狭窄治疗困难,但最后需要手术治疗的患儿比例也明显很低,约为3%~7%,没有大型数据对这些患儿进行长期随访[36,37]

1.狭窄段切除食管重建术

狭窄段食管切除再重建是治疗难治性食管狭窄的最常见的手术方式[37,38]。虽然经纵隔开胸会使手术复杂化,但直视下手术会增加成功率[39]。尽管如此,食管二次端端吻合仍然可能需要术后扩张或二次手术修复[36]

2.代食管术

代食管术治疗难治性食管狭窄很少被报道[36]。因舍弃原来的食管并且行代食管术很少应用,需要很有经验的医生讨论决定,并且可能发生近期和远期术后并发症,对于经历过多次手术而无效的患儿,代食管术的效果非常明显[39]。代食管的选择可以是胃、空肠、结肠等[40,41]。结肠代食管术是应用时间最长的代食管术,并发症主要包括口臭、反流、结肠段扩张、吻合口狭窄等;胃代食管术术式较简单,可应用于紧急情况或新生儿时期,并发症主要包括胃储留、胆汁反流、肺功能减退、生长受限等;空肠代食管术因为肠系膜短而极少应用,但术后效果佳[42]

3.狭窄段食管切开术

内镜下电刀切开术(endoscopic electrocautery incisional therapy,EIT)已经应用在难治性食管狭窄的治疗中[43]。EIT的原理是在直视下通过针式电刀破坏狭窄段的纤维环,多个放射状切口平行于食管长径,以达到满意的食管直径,有的也可在切开后后给予扩张治疗,切开的有效深度为深至固有肌层,术中需密切观察术口是否有出血或食管穿孔。对于狭窄段长度>1.5 cm,需在辐形切开后放置可覆膜支架,这样可更有效的治疗食管吻合口狭窄[20,35]。标准的针式电刀仅可切割刀片旁边的组织,尽可能的避免了食管穿孔,有的EIT技术还结合氩气等离子凝固设备[44]。Muto等[44]对此技术进行改良,即在进行放射状的食管切开后,切除切口间的纤维组织,并达到满意的效果。Hordijk等[45]在一项前瞻性研究中指出EIT技术在对未经其他疗法的食管狭窄患儿中,其有效率几乎等同于沙式探条扩张,并且安全性高、术中穿孔率低。Muto等[44]指出EIT在对难治性食管狭窄患儿(已经历>3次的扩张治疗)中有效率高于扩张治疗,而长段的食管狭窄(>1 cm)则复发率较高,并且需要反复治疗。但EIT技术在儿童食管狭窄的报道很少,仅在一项对7例食管狭窄患儿的回顾性研究中报道其中4例仅行狭窄段切开,3例在狭窄段切开后置入支架,均达到良好的效果,术中也未出现并发症[35]。虽然儿童食管狭窄关于切开治疗的报道很少,但近期证据表明EIT可作为治疗食管狭窄,尤其是短段型食管狭窄的一项选择。

三、支架治疗

食管支架已经在成人患者中广泛应用,主要用于治疗自发性食管穿孔、食管气道瘘、吻合口漏、食管狭窄等,全覆盖的自膨胀金属支架在折叠时放置,可减少增生组织的过度增长导致的移除困难和放置支架带来的食管穿孔而被广泛应用于临床[34]。因支架尺寸的限制,儿童食管定制支架应用并不广泛。

传统的探条或球囊扩张虽然疗效确切,需要反复到医院治疗,并且经历多次麻醉,有时候难以被患儿或家属接受,而食管支架的应用克服了这些缺点,在应用支架期间患儿也可正常进食,食管支架可以在没有破坏的情况下清洁后重复利用,扩张治疗失败后使用可移除支架要比反复的扩张更能提高患儿的生活质量,近年来有多篇食管闭锁后吻合口狭窄应用支架治疗的报道[34,35]。随着材料科学的进步,可降解支架也逐渐应用在食管狭窄治疗中,其优点为无需移除支架[46]。另外支架也可用于治疗食管扩张穿孔的患儿,Manfredi等[47]报道应用全覆膜金属/塑料支架对24例扩张穿孔的患儿行放置支架治疗,治愈率达79%。Lange等[34]报道应用全覆膜金属支架治疗食管吻合口狭窄后扩张穿孔的患儿,4例患儿经治疗后痊愈。

四、总结展望

综上所述,目前针对EA术后吻合口狭窄的治疗主要包括扩张治疗、手术治疗、支架治疗。扩张治疗已经作为EA术后吻合口狭窄的首选治疗方法,疗效确切,球囊扩张和探条扩张均建议在导丝引导下进行。扩张无效的患儿可行手术治疗,最常用的是狭窄段切除食管重建术,EIT及代食管术报道较少。支架治疗疗效确切,并且可减少反复的扩张,正逐渐应用在EA术后吻合口狭窄治疗中。

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