Ⅴ型先天性食管闭锁一例

患儿,男,1个月25天.因“喝奶后呛咳1个月个余”入院.患儿出生后第1天喝奶后即出现呛咳,伴颜面青紫,呼吸费力,当地医院诊断为新生儿肺炎,行抗感染治疗后好转,但仍有呛奶.转上级医院后行上消化道碘水造影,显示食管气管瘘(图1).后转入我院在全身麻醉下行食管气管瘘修补术,术中取颈部近锁骨上窝处横切口,逐层切开各层组织,显露食管,贴气管后壁向上分离,可见瘘管斜行连接食管及气管,瘘管外径约0.5cm,术中诊断为V型先天性食管闭锁(图2).     

经胸、胸膜外Ⅰ期手术治疗食管闭锁

食管闭锁(esophageal atresia,ET)是以食管连续性中断为特征的先天性发育畸形,可伴有或不伴有气管食管瘘,ET最常见的类型是近段食管闭锁并远段气管食管瘘,占总数的86%[1].食管闭锁的发生率为2500～4500个活产儿中1个[2].其严重危及患儿生命,需要急诊手术矫治.自1943年Haight和Towsley报告了第1例Ⅰ期食管吻合成功的病例至今已有近70年.早期的手术死亡率高达30%～50%.由于手术技术、麻醉和新生儿监护水平的不断提高,死亡率越来越低.Spitz[3]报告体重>1500g,不存在严重心脏畸形的患儿如今的手术成活率达到98%.     

胸腔镜治疗先天性食管闭锁Ⅲ型74例北大核心CSCD

目的探讨胸腔镜技术治疗先天性食管闭锁Ⅲ型的临床疗效。方法回顾2015年1月~2020年10月74例胸腔镜治疗先天性食管闭锁Ⅲ型的临床资料,年龄(2.1±2.3)d,体重(2.66±0.50)kg。早产儿20例(27.0%),产前检查提示食管闭锁16例(21.6%),合并其他疾病31例(41.9%)。均行胸腔镜气管食管瘘结扎,73例食管端端吻合,1例胃造瘘。结果66例(89.2%)完成胸腔镜手术,8例中转开胸。手术时间(178.5±68.3)min,住院时间(19.7±11.7)d,住院费用(9.8±5.6)万元。术后4例放弃治疗死亡,70例(94.6%)治愈出院。术后吻合口漏9例(12.9%),乳糜胸1例(1.4%)。术后随访1~5(2.8±1.4)年,吻合口狭窄32例(45.7%),气管食管瘘复发3例(4.3%),严重胃食管反流2例(2.9%),严重气管软化1例(1.4%)。结论胸腔镜治疗先天性食管闭锁Ⅲ型安全有效。     

先天性食管闭锁镜下手术的发展

食管闭锁是腔镜手术涉及较迟的病种之一.自上世纪80年代末期腹腔镜探索性的涉足小儿外科病种后,至上世纪90年代初,开始见有大量腹腔镜应用于各类儿童消化道疾病的临床报告,延至1999年始见首例胸腔镜下新生儿食管闭锁纠治术的报告[1],此后在欧美国家也陆续见有小宗的临床报告[2-4],并且多数见于伴有远端食管气管瘘的Ⅲ型食管闭锁,而所有报告均给出结论,胸腔镜下修复食管闭锁和食管气管瘘是可行的.     

新生儿先天性Ⅲ型食管闭锁的护理

先天性食管闭锁是新生儿消化系统的一种严重的先天性畸形 ,在国内发病率为 1 /2 0 0 0～1 /40 0 0 [1] ,男性高于女性。在其病理解剖分类中以 型最多见 ,占 85%～ 90 % [2 ]。手术是治疗本病的唯一途径 ,而患儿的一般情况及合并病变如吸入性肺炎等是影响治疗成功的关键。我科 1 996年 1月至2 0 0 0年 6月收治新生儿先天性 型食管闭锁 1 2例 ,现将护理体会介绍如下。1　临床资料1 2例中 ,男 8例、女 4例 ,入院时年龄为 1 1 h至4d,手术时年龄 1 .5～ 1 6 d;体重 1 .9～ 3 .5kg。经食管碘油造影、胸腹 X线摄片而确诊。均有不同程度的口吐泡沫…     

Ⅲ型先天性食管闭锁一期食管吻合术17例疗效分析

先天性食管闭锁（CEA）是最常见的先天性食管畸形，其中以Ⅲ型最为多见。近年来随着新生儿监护水平的提高和肠外营养的应用，治愈率明显提高，但病死率及并发症发生率仍然较高。我们自1998年7月至2002年11月连续为17例Ⅲ型先天性食管闭锁患儿进行一期手术治疗均获成功（本组研究取得患儿家长知情同意），现报告如下。     

机器人辅助胸腔镜下Ⅰ型先天性食管闭锁的手术护理配合

总结1例新生儿机器人胸腔镜下Ⅰ型先天性食管闭锁手术的护理配合要点.术前做好访视和达芬奇机器人手术系统的布局摆放;术中,巡回护士做好手术体位的安置和调节,做好皮肤护理和预防低体温,建立和强化立体式无菌区域;器械护士关注手术配合要点和注意事项;术后强调体位和管道护理.     

新生儿Ⅰ期胃代食管术治疗长段型食管闭锁

目的 探讨Ⅰ期胃代食管术治疗长段型食管闭锁的疗效.方法 对2008年3月至2013年12月采用Ⅰ期胃代食管术治疗的11例长段型食管闭锁进行回顾性分析,其中男8例,女3例.入院后行食管造影确诊,其中Ⅰ型2例,Ⅲa型9例.结果 所有患儿均顺利完成手术.9例治愈出院,1例死亡,1例家长放弃治疗.术后6例有严重肺炎,近期吻合口瘘2例.随访6个月至5年,吻合口狭窄3例,均行食管扩张术治愈,轻度胃食管反流6例,均未行抗反流手术,采用少量多餐及体位喂养治疗后症状缓解.结论 新生儿期采用Ⅰ期胃代食管术治疗长段型食管闭锁临床可行,避免了分期手术,缩短了治疗周期,有助于提高治愈率.     

先天性食管闭锁Ⅲ型并骨骼畸形1例报告

患儿，男，5d。因吐沫5d，青紫2d入院。患儿系第一胎第一产，胎龄39^ 6周，顺产，出生体重3600g，生后无窒息。羊水过多、清。于生后即发现唾液多、不断从口腔溢出，吐白沫。生后6h喂奶，吃后即吐，呈非喷射性，并有呛咳。于生后第2天在当地医院住院治疗，诊为新生儿肺炎，予禁食补液等治疗，2d后出现颜面青紫逐渐加重而转我院。入院时     

先天性食管闭锁的外科治疗

目的 总结32例先天性食管闭锁手术治疗经验。方法 手术均采用食管上盲端前壁肌层U形翻转，即经胸膜外入路充分游离食管上端及瘘管，近气管侧缝扎切断瘘管。用剪刀将远端食管前壁纵行剪开5min，吻合前距吻合口上方10min处行食管上下两端浆肌层缝合3针，然后距吻合口上方15～20min处将食管前壁肌层切开，从切开处向吻合口方向从两侧纵行切开肌层至吻合口约5mm，轻轻分离前壁肌瓣，形成U形向下翻转，缝合在食管远端浆肌层上。结果 32例中17例采用此方法未发生吻合口瘘，1例吻合口狭窄。2例成功行一期胃代食管术。结论 食管上盲端前壁肌瓣U形翻转能有效预防吻合口瘘及狭窄。Ⅰ、Ⅱ型食管闭锁采用一期胃代食管术，可避免二次手术。     

Experience in treating congenital esophageal atresia in China

Purpose

The aim of the study was to evaluate our recent experience in treating esophageal atresia (EA) and the outcomes observed at a single center for pediatric surgery.

Materials and Methods

The records of infants with EA from 2006 to 2009 were reviewed. Birth weight, associated anomalies, details of management, complications, and outcomes were examined.

Results

Forty-eight consecutive infants with EA were identified from 2006 to 2009, of which 33 (69%) were boys. Mean birth weight was 2668 g (range, 1700-3800 g). Common associated malformations (35%) were cardiac anomalies, imperforate anus, limb anomalies, and chromosomal anomalies. Forty-seven were Gross type C, and one was Gross type A. Forty-five infants underwent ligation of the tracheoesophageal fistula and end-to-side primary anastomosis, and one received a colonic interposition. Six patients died (12.5% mortality). Three died before or during operation because of severe pneumonia and complex cardiac anomalies, and 3 died during recovery (within 1 month after repair) because of aspiration and severe pneumonia (early postoperative mortality was 6.67%). Complications included pneumonia, anastomotic leakage (16%, all recovered after conservative treatment), wound sepsis (11%), recurrent tracheoesophageal fistula (9%) (3/4 recovered after conservative treatment), anastomotic stricture (10%), and gastroesophageal reflux in about 2 of 3 patients. Preoperative computed tomographic imaging and 3-dimensional graphic reconstruction used in 15 patients were useful.

Conclusions

Most patients with EA have excellent short- to midterm surgical outcomes. The main factors for mortality are complex cardiac anomalies, aspiration, and pneumonia. Computed tomographic imaging and 3-dimensional graphic reconstruction can provide surgeons with excellent preoperative reference about the anatomy of the defect. Most anastomotic related complications resolve with conservative treatment. Patients of low-risk prognosis group with type A and long gap EA can be managed with a primary colonic interposition with good results. The main midterm complications are gastroesophageal reflux and stricture.     

Thoracic skeletal anomalies following surgical treatment of esophageal atresia. Lessons from a national cohort

Introduction

Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity.

室间隔完整的肺动脉闭锁外科治疗

目的探讨适当的手术方式,评价手术矫治室间隔完整的肺动脉闭锁的临床疗效。方法1992年1月至2004年8月手术治疗17例室间隔完整的肺动脉闭锁病儿,男9例,女8例;年龄15d~12岁,平均(25.5±7.9)个月;体重3.5~28.0kg,平均(7.8±5.4)kg。其中根治手术10例,三尖瓣Z值-2.3~1.2,平均(-0.78±0.34),肺动脉发育指数149.53~297.89mm2/m2,平均(206.35±82.15)mm2/m2,其中2例病儿因严重低氧血症术后次日加行体肺分流手术;生理矫治手术6例,Z值-6.1~0.2,平均(-2.7±0.92),肺动脉发育指数39.88~218.29mm2/m2,平均(131.85±72.93)mm2/m2,包括双向格林手术2例,体肺动脉分流手术1例,右室流出道重建合并体肺动脉分流术3例;一个半心室矫治术1例,首先行双向格林手术,2年后行房间隔缺损修补术,动脉导管结扎术和右室流出道重建术。结果围术期死亡3例(16.67%),2例为先行根治术次日加行体肺分流手术者,1例为右室流出道重建合并体肺动脉分流术者。术后并发症低心排综合征3例,低氧血症3例,乳糜胸1例,右心衰3例,余者均顺利康复。结论合并室间隔完整的肺动脉闭锁是一种少见的先天性心脏病,依照右心室大小、三尖瓣环大小、肺动脉发育情况及冠状动脉解剖等采用适当的手术方式可取得满意的治疗效果。     

Dynamic esophageal lengthening for long gap esophageal atresia: experience with two cases

This report describes two infants with long gap esophageal atresia managed by dynamic esophageal lengthening. Preliminary traction on the atretic ends of the esophagus permitted subsequent primary anastomosis under significant tension. One patient has done well, while the other developed an anastomotic stricture requiring resection. The esophagus was preserved in both infants.     

法洛四联症合并肺动脉闭锁的外科治疗

目的探讨法洛四联症合并肺动脉闭锁(TOF-PA)的外科治疗，重点讨论不同类型的手术时机和方法及适应证。方法1984年6月至2003年12月对24例TOF—PA病儿进行矫治手术，其中男10例，女14例；年龄6个月～9岁；Ⅰ型14例，Ⅱ型8例，Ⅲ型和Ⅳ型各1例。全组均采用中度低温体外循环下进行一期矫治手术，Ⅰ型采用跨瓣环的带单瓣的人工血管补片加宽，Ⅱ型采用右心室到肺动脉带瓣管道，Ⅲ型和Ⅳ型TOF-PA采用胸骨正中切口一期单源化和心内修复术。结果术后早期死亡4例，其中Ⅰ型、Ⅱ型各2例，死因为严重低心排血量综合征2例、灌注肺和多脏器功能衰竭各1例。Ⅰ型和Ⅱ型长期随访16例，随访1个月～15．5年，NYHA心功能Ⅰ或Ⅱ级14例，Ⅲ和Ⅳ级各1例；Ⅲ型和Ⅳ型分别随访1个月和6个月，效果满意。结论TOF-PA一经诊断应尽快手术，条件较好的早期行矫治手术可得到满意的效果。     

产前超声联合MRI诊断胎儿先天性食管闭锁

目的探讨产前超声联合MRI对胎儿先天性食管闭锁(CEA)的诊断价值。方法回顾性分析31胎经引产后尸体解剖或出生后手术及影像学检查证实的CEA胎儿(CEA组)的产前超声及MRI资料,并与31胎产后正常胎儿(正常对照组)进行对照。以病理结果为金标准,分析产前超声联合MRI诊断胎儿CEA的阳性率。比较2组间产前超声指标胎儿双顶径(BPD)、头围(HC)、腹围(AC)、股骨长(FL)、羊水深度(AFD)、羊水指数(AFI)、胎盘厚度、脐动脉峰谷比(S/D)、胎儿吞咽指数及估算胎儿体质量(EFW)的差异。结果产前超声联合MRI诊断胎儿CEA的阳性率为67.74%(21/31)。CEA组胎儿HC、AC、吞咽指数、EFW均低于正常对照组(P均0.05),S/D、AFI均高于对照组(P均0.05);2组间BPD、FL、AFD、胎盘厚度差异均无统计学意义(P均0.05)。结论产前超声无法直接诊断胎儿CEA,当发现胃泡持续72h不显示(间隔72h复查)或羊水过多时,结合MRI对产前诊断胎儿CEA具有一定价值。产前超声指标中,胎儿HC、AC、AFI、S/D、吞咽指数、EFW可能对CEA具有一定提示作用。     

Delayed primary anastomosis in poor-risk patients with esophageal atresia associated with tracheoesophageal fistula

Twelve patients with esophageal atresia with tracheoesophageal fistula (TEF) who were treated with delayed primary anastomosis were reviewed. All patients except two were classified as Waterston's group C, for whom multiple staged procedures usually can be considered. Gastrostomy for decompression was performed as the initial operation shortly after admission and adequate nutritional support was provided by a transpyloric or jejunostomy tube. Extrapleural division of the TEF and esophagoesophagostomy were delayed until these operations were considered safe to perform. Delayed primary anastomosis was done at varying ages from the 11th day of life to the 150th day. Eleven patients survived. It is concluded that the therapeutic program consisting of gastrostomy, enteral feeding and delayed division of TEF and esophagoesophagostomy is useful in treating poor-risk patients and should replace multiple staged procedures.