先天性食管狭窄15例诊疗体会

目的 总结先天性食管狭窄诊断和治疗体会,提出最佳的诊断、治疗方法.方法 回顾我院1990至2009收治的15例先天性食管狭窄临床资料,对临床表现(症状、体征、发病年龄等),诊断方法(UGI、胃镜等)进行分析.将患儿分为手术组与非手术组,比较两组治疗过程和预后情况(饮食状况、并发症等).结果 15例先天性食管狭窄患儿,男9例,女6例.发病年龄(10±5.5)个月(0～3岁9个月).主要临床表现为反复进食后呕吐.GI检查发现狭窄段位于食管下段者9例,位于中下段者6例.7例仅行胃镜辅助下食管扩张术,手术治疗8例,其中6例行狭窄段食管切除+端端吻合术,1例行狭窄段肌层切开+胃底折叠术,1例行狭窄段全层切开、纵切横缝+胃底折叠术.术后病理诊断2例为气管软骨异位型,1例为狭窄局部炎性假瘤形成,5例为肌层增厚型.术后4例出现吻合口狭窄,2例出现胃食管反流,1例伴发术侧膈膨升.结论 上消化道钡剂造影(UGI)是诊断首选方法,电子胃镜可作为诊断及辅助治疗的手段.治疗方法建议首选食管扩张术,对扩张2～3次以上无效者进行手术探查.下段狭窄者应行胃底折叠术避免术后并发胃食管反流.

Abstract：

Objective The aim of this study was to find an optimal procedure of diagnosis and treatment for congenital esophageal stenosis (CES). Methods From 1990 to 2009, 15 CES patients were treated in our hospital. Demographical, clinical, pathological and radiological data were collected retrospectively. Patients were further divided into surgery group and non-surgery group. Outcomes and complications were studied to identify the indication and timing for operation. Results The age of presentationof of these 15 patients (9 boys and 6 girls) were 10± 5. 5 months (1 day ～ 41months).Vomiting was the most significant and common symptomign. UGI were performed in all the 15 patients. 9 patients had stenosis in lower esophagus and 6 in mid-lower esophagus. In surgery group, 6patients underwent surgical resection; the remaining 2 patients underwent Heller myotomy esophagomyotomy and stricturoplasty, respectively. Tracheobronchial remnants were found in 2 patients and submucosal thickening were found in 5 patients. Inflammatory pseudotumor was found in the rest 1patient. The frequency of esophageal anastomotic stricture, gastroesophageal reflux and diaphragmatic erentration were 44.4 % (4/9), 22. 2 % (2/9) and 11.1% (1/9), repectively. Conclusions UGI is the first choice for the diagnosis of CES, while electronic gastroscope can play an alternative role. Esophageal dilatation could be the first therapy for CES, and operation is indicated when outcome dilatation fails.     

电子支气管镜在儿童上气道梗阻性疾病中的诊断价值

目的 评价电子支气管镜在儿童上气道梗阻性疾病中的诊断价值及安全性.方法 对91例上气道梗阻患儿行电子支气管镜检查,总结分析病因.结果 (1) 儿童上气道梗阻病因依次为先天性喉气管软化合并感染(45例)、声门下异物(13例)、喉气管占位性病变(9例)、声门区水肿(6例)、气管狭窄(5例)、喉咽腔病变(咽后脓肿3例、肿物2例)、声门区增生(3例)、声门下狭窄(2例)、声带麻痹(2例)、喉蹼(1例);(2) 不同年龄段病因不同:新生儿期依次为声带麻痹、喉气管软化;~6个月依次为先天性喉气管软化、会厌囊肿,其他少见病因如气管狭窄、咽后脓肿、喉蹼等疾病;~1岁依次为喉气管软化、气管狭窄、声门下异物等;~3岁依次为声门下异物、声门区水肿、喉气管软化等;>3岁儿童1例为喉乳头状瘤.(3) 疾病好发的年龄段不同:先天性喉气管软化好发于6个月以下婴儿,气管异物好发于1~3岁幼儿,先天性会厌囊肿多见于3个月以下婴儿.(4) 并发症:16例有一过性血氧饱和度下降,11例出现气管支气管痉挛,术后8例有一过性发热,6例有短期喉鸣加重.结论 电子支气管镜在儿童上气道梗阻性疾病的诊断中起重要作用,可迅速、直观地判断病变部位和性质,避免误诊,有助于指导治疗.

Abstract：

Objective To investigate the diagnostic value and safety of bronchovideoscope in the pediatric upper airway obstruction.Methods Bronchovideoscope was performed in 91 pediatric patients with upper airway obstruction.The etiology was analyzed and summarized.Results (1) Our study showed that the etiology of pediatric upper airway obstruction were as follow in turn:congenital laryngo-trachemalacia (45 cases),subglottic foreign body (13 cases),laryngotracheal occupying lesion (9 cases),vocal area edema (6 cases),tracheal stenosis (5 cases),laryngopharyngeal lesion (3 cases of retropharyngeal abscess,2 cases of tumor),vocal area hyperplasia (3 cases),subglottic stenosis (2 cases),glottic paralysis (2 cases),laryngeal web (1 case).(2) The common etiology of pediatric upper airway obstruction was different with age.Neonatal period:glottic paralysis,laryngomalacia in turn;1~6 month:laryngo-trachemalacia,tracheal stenosis,subglottic foreign body in turn;1~3 year:subglottic foreign body,vocal area edema,laryngo-trachemalacia in turn,>3 year:laryngeal papilloma.(3) Specific disease had predominant age:laryngo-trachemalacia predominated in infants less than 6 month;tracheal foreign body was most common in child aged 1~3 years old;epiglottic cyst was most common in infant less than 3 month.(4) Complication:during procedure,16 patients had transient decrease of saturation of blood oxygen and 11 patients had tracheobronchial spasm.After procedure,8 patients had transient fever and 6 patients had transient aggravation of laryngeal stridor.Conclusion Bronchovideoscope plays an important role in the diagnosis of pediatric upper airway obstruction.It can directly identify position and nature of disease,and then guide treatment.     

产前诊断的先天性胆管扩张症手术时机探讨

目的 对产前诊断的先天性胆管扩张症的手术时机进行探讨.方法 回顾我院2006年至2010年收治的产前诊断10例先天性胆管扩张症患儿,入院手术年龄27 d至2岁,9例接受腹腔镜胆总管囊肿切除术,肝管空肠Roux-Y吻合术,1例接受开腹手术.观察其临床表现、超声和实验室指标、术中情况、术后恢复情况等.结果 产前囊肿均随孕周逐渐增大.产后5例患儿曾经出现黄疸,4例曾经出现白便.4例超声提示囊肿增大明显,6例ALT升高.术中造影见全部发生胆总管远端梗阻,其中Ⅰ型3例,Ⅳ型7例,3例可见胰胆合流异常.5例患儿术中及术后病理可见肝硬化、肝脏损伤表现.全部10例患儿术后恢复好.结论 产前诊断的先天性胆管扩张症可能是由于胆总管远端梗阻,造成胆管扩张形成.产前诊断先天性胆管扩张症的患儿,应密切观察,发现黄疸白便表现、ALT和AST升高明显、超声提示囊肿短期增大明显应尽快手术治疗.如无上述表现,可定期复查,但也应尽早手术根治.

Abstract：

Objective To present the therapeutic strategy for prenatally diagnosed congenital biliary dilatation (CBD).Methods From 2006 to 2010,10 patients with prenatally diagnosed CBD were treated at this center.Their age ranged from 27 days to 2 years old.Among the 10 patients,9 underwent the laparoscopic total cyst excision with Roux-en-Y hepatojejunostomy,1 was performed open surgery.The clinical features,ultrasonography,laboratory tests and postoperative complications were retrospectively analyzed.Results Results The ultrasonograohy revealed the biliary cysts' size increased with the gestational ages.After birth,5 of the 10 patients had jaundice,4 had acholic stools,4 patients' cysts increased in size,and 6 had elevated transaminases.Intraoperative cholangiography was performed,and distal common bile duct stenosis was found on all patients.Three of them were type Ⅰ of CBD,7 of them were type Ⅳ,and the other 3 had pancreaticobiliary malunion.Liver cirrhosis was found in 5 of the 10 patients.All patients recovered from the surgery.Conclusions Distal common bile duct obstruction is the leading etiology of the prenatally diagnosed CBD.Surgical intervention is recommended for the patients with jaundice,acholic stool,elevated transaminase or cysts obviously increasing in size.     

新生儿食管闭锁的外科治疗

目的 总结新生儿食管闭锁的诊断和治疗经验.方法 回顾性分析我院2002年6月至2010年6月收治的新生儿食管闭锁61例,男34例,女27例.手术年龄18 h～7 d,平均(2.5±0.6)d,体重1 500～4 000 g,其中低体重儿(＜2 500g)16例.61例中按Gross病理解剖分类:Ⅰ型2例,Ⅲa型32例,Ⅲb型27例,Ⅲa型中有1例远端食管有局限狭窄,开口约0.2 cm;合并畸形:先天性心脏病15例,肠道畸形4例,泌尿系畸形3例.结果 61例中2例Ⅰ型食管闭锁先行近端食管引流、胃造瘘,2周后行结肠代食管手术,59例Ⅲ型均Ⅰ期食管气管瘘切断缝扎、食管端端吻合术,Ⅲa型中远端食管有1例局限性狭窄,行纵切横缝解除狭窄.术后并发单侧或双侧严重肺炎42例,硬肿症3例,近期吻合口狭窄24例,吻合口瘘3例.除1例术后2d合并肠穿孔死亡,3例放弃治疗(均为早期病例:1例为术后1周出现核黄疸,2例术后合并严重肺部感染不能脱离呼吸机),余57例均痊愈出院.术后随访3个月～8年,轻度胃食管反流3例,余均进食良好,生长发育正常.结论 尽早诊断、及时手术,积极预防和治疗并发症,新生儿食管闭锁可取得良好的效果.

Abstract：

Objective To summarize the diagnosis and treatment for congenital esophageal atresia (CEA) in neonates. Methods From June 2002 to June 2010, 61 neonates with congenital esophageal atresia underwent surgery at this center. Of these patients, 34 were boys and 27 were girls. Their age ranged from 18 hours to 7 days (mean, 2. 5 ±0. 6 days). Their weight ranged from 1500 grams to 4000 grams. Sixteen patients were very low-birth-weight infants (＜1500 g). According to the anomalies of CEA, 2 were diagnosed with type Ⅰ CEA, and the other 59 were type Ⅲ CEA (32 type Ⅲa and 27 type Ⅲb). The most common associated anomalies were cardiac anomalies (16, 26%), followed by intestinal anomalies (5, 8%) and renal anomalies (3, 5%). Results The 2 cases with type Ⅰ CEA underwent proximal drainage of esophagus and gastrostomy to stabilize their conditions. Two weeks later, they underwent the second stage surgery to replace esophagus with colon. The 59 patients with type Ⅲ CEA underwent fistulectomy and end-to-end esophagus anastomosis via thoracic approach. A stenosis in the medial-distal esophagus was found on 1 type Ⅲa CEA patients, and was repair with longitudinal incision and transverse suture. Postoperative complications included pneumonia on 42 patients (68. 8%), scleredema on 3 patients (4. 9%), mild anastomosis orifice stenosis on 24 patients (39. 3%), and anastomosis orifice fistula on 3 patients (4. 9%). One patient died of intestinal perforation 2 days after surgery. Three patients were given up including 1 developed kernicterus 1week after surgery, and the other 2 had serious pulmonary infection and couldn't be withdrawn from mechanical ventilation. The other 57 cases were discharged from hospital. The patients were followed up for 3 months to 8 years. Three patients had mild gastroesophageal reflux. The others ate and drank normally during follow-up. Conclusions Early diagnosis and carefully management of postoperative complications are important to improve clinical outcomes and prognosis of congenital esophageal atresia in neonates.     

Selective coronary angiography in follow - up and technical study of Kawasaki disease - induced giant coronary artery aneurysm北大核心CSCD

Objective To investigate the characteristics of long - term changes in giant coronary artery aneurysm (GCAA) caused by Kawasaki disease (KD) and the technical approach of using selective coronary angiography (SCAG) in children. Methods A retrospective analysis was made in 52 patients with KD and GCAA in acute phase, including 38 males and 14 females with an average age of (4. 1 ±2.5) years old ranging from 1 to 14 years old,from January 2008 to December 2018 at the Department of Cardiology, Guangzhou Women and Children's Medical Center. The selective coronary angiography (SCAG) was performed in the patients who were followed up for (10.6 ±3.6) years (1-19 years). The changes in coronary artery lesions were analyzed and the technical approach of SCAG was discussed in children. Results Among 52 patients investigated by SCAG, the location of coronary artery lesion was found the left - anterior descending branch in 21 cases(40.4%),20 cases(38. 5%) in the right coronary artery,8 cases (15.4%) in the left main trunk and 3 cases(5.7%) in circumflex. The left coronary artery in 2 cases and the right coronary artery in 4 cases were completely occluded,and collateral vessel formation was found in 12 cases. There were 21 cases of right coronary artery stenosis, including 7 cases of the right coronary artery occlusion and bridge - like neovascularization, and 4 cases of the right coronary artery woven lesions (thrombotic occlusion and clustered neovascularization). There were 27 cases of the left coronary artery stenosis with different degrees, including 5 cases of the left coronary artery occlusion and bridge - like neovascularization, and 2 cases of the left coronary artery woven lesions (thrombotic occlusion and clustered neovascularization). All of 52 patients with GCAA were followed up for 1 to 19 years. GCAA still existed in 40 cases. Regression to small coronary artery aneurysm was found in 8 cases,and mild - dilation regression was observed only in 4 patients. The resting electrocardiogram showed pathological Q wave and the left ventricular enlargement in 6 cases,and normal in the remaining 46 patients. The children had no subjective symptoms and their activities were not restricted.compared with SCAG,echocardiography detected 52 cases with proximal morphological changes in the coronary artery, but distal changes were found only in 3 cases. And 12 cases with coronary artery occlusion and neovascularization failed to be prompted by echocardiography. Conclusions GCAA induced by KD can persist for a long time, and has characteristics of coronary occlusion, stenosis, and collateral vessel formation. The conventional electrocardiogram and echocardiography are insensitive to the coronary artery morphology and dysfunction in KD sequelae. The SCAG is of great value in tracking this disease. For using SCAG in children,as long as the appropriate equipment and projection angle are selected, and the operation skills are mastered, satisfactory images and high success rate can be obtained. © 2019 Chinese Medical Journals Publishing House Co.Ltd. All rights reserved.     

完全性大动脉转位患儿外科术后早期并发症和近期随访

Objective To present the short-term outcomes of the patients with transposition of the great arteries after repair. Methods Between 2008 and 2009,71 patients with transposition of the great arteries (TGA) underwent corrective surgeries at this center. The 71 TGA patients were divided into TGA/IVS group whose ventricle septum was intact, and TGA/VSD group who complicated with ventricle septum defect. Their clinical data and postoperative short-term outcomes were analyzed and compared. Results The postoperative complications included 7 (9. 9% ) patients presented with low cardiac output syndrome, 12 ( 16. 9% ) with pulmonary stenosis, 21 ( 29. 6% ) with arrhythmia, 6 (8. 5%) with diaphragmatic paralysis, 6 (8. 5%) with airway abnormalities, and 13 ( 18. 3% ) with bacteria in sputum culture. Three patients (4. 2%) died after surgery. Fifty four out of the 68 patients were followed up for an average period of 12. 0 ± 7. 4 months. Four (7. 4%) patients died during follow-up. Three patients (5. 6%) underwent interventional therapies for pulmonary stenosis. The survival rate of 57 patients was 87. 7% at the end of the first postoperative year. No differences of the postoperative complications and survival rate were noted between the TGA/IVS and TGA/VSD group.Conclusions The postoperative short-term outcomes of the patients with transposition of the great arteries are good.     

先天性纠正型大动脉错位不同手术方法结果的比较

目的 比较先天性纠正型大动脉错位(ccTGA)的各种手术治疗结果方法,为临床治疗ccTGA提出更合理的手术方式.方法 1999年6月至2009年12月外科手术治疗95例ccTGA,包括传统纠治手术25例,功能性单心室纠治手术26例,双调转术(Double Switch)手术26例,姑息手术15例,另有3例接受其他手术.结果 术后死亡7例,其中传统手术组死亡2例,Double Switch手术组死亡4例,其他手术组死亡1例,单心室手术组全部存活.结论 纠正型大动脉错位的手术方法的选择取决于解剖条件和生理参数.在各类手术结果的比较中,功能性单心室纠治手术的死亡率低、并发症少、再手术率低,可适当放宽功能性单心室纠治手术指征,可能得到更好的治疗效果.但长期的手术疗效还有待于进一步随访.

Abstract：

Objective To study the clinical outcomes of various corrective surgeries for congenitally corrected transposition of the great arteries (ccTGA) in children. Methods Between June 1999 and December 2009, 95 patients with ccTGA who underwent corrective surgeries at this center were enrolled in this study. Among the 95 patients, 25 patients were performed conventional surgical repairs, 26 had functionally univentricular surgical repair, 26 had double switch operation, 15 had palliative surgeries, and 3 underwent other surgical treatments. Results Seven deaths after surgery were recorded, including 2 patients who had conventional surgical repairs, 4 with double switch operation and 1 with other surgical treatments. No death of the patients who underwent functionally univentricular surgical repair was noted. Conclusions The surgical strategy to correct ccTGA should be made according to patient's individual anatomic anomalies and pathophysiology. Short term outcome study revealed that functionally univentricular surgical repair is safe and effective.     

复杂畸形伴肌部多发室间隔缺损的直视镶嵌封堵

目的 介绍复杂畸形伴肌部多发室间隔缺损心内直视镶嵌封堵的经验.方法 全组10例,男7例,女3例.复杂心内外畸形包括右心室双出口Taussig-Bing畸形2例、右心室双出口主动脉瓣下室缺2例、完全性大动脉错位伴室间隔缺损肺动脉高压2例、法乐四联症1例、室间隔缺损伴重度二尖瓣反流2例、室间隔缺损伴主动脉重度缩窄1例.其他伴发畸形有:二尖瓣反流、单支右冠状动脉畸形、房间隔缺损、冠状窦隔缺损、主动脉弓发育不良、动脉导管未闭等.全组均解剖纠治镶嵌心内直视封堵肌部多发室间隔缺损,平均手术年龄(8.8±5.4)个月,平均手术体重(6.3±2.0)kg.结果 手术无死亡.无心内直视镶嵌封堵相关并发症.3例术后并发肺动脉高压危象;1例术后3 d延迟关胸;1例拔肺动脉测压管出血,心包填塞,床边开胸止血.随访无死亡,2例封堵器下缘残留少量分流.结论 心内直视封堵肌部多发室间隔缺损,可避免传统手术纠治需心室切口、心室内肌束切开,对心功能影响大的缺点;可缩短体外循环时间,同时镶嵌复杂畸形Ⅰ期解剖纠治,使手术简化、安全、尤其适用于婴幼儿,可获得较佳中长期疗效.

Abstract：

Objective To present our experience using hybrid approaches to repair muscular ventricular septal defects (mVSDs) associated with additional congenital heart diseases in children.Methods From April 2006 to September 2010, 10 patients with mVSDs associated with additional congenital heart diseases underwent corrective surgery at this center. Their mean age was 8. 8 month old, and their mean body weight was 6. 3 kg. The additional congenital heart defects included 2 patients with D-transposition of great arteries, 2 with double outlet right ventricle-Taussig-Bing anomaly, 1 with Tetralogy of Fallot, 2 with double outlet right ventricle, 1 with severe coarctation of aorta,and 2 with severe mitral valve regurgitation. Results All patients underwent the single stage hybrid approach repair under direct vision and all recovered from surgery. After surgery, 3 patients developed pulmonary hypertension crisis among whom 1 had delayed chest closure and 1 underwent emergence surgery to relief cardiac tapenade. The patients were followed up for 3 months to 3 years No late deaths were noted. Complete closure of muscular ventricular septal defects was achieved on 5 patients.Clinically insignificant residual shunt was observed on 2 patients. Conclusions It is safe and effective using hybrid approaches to repair muscular ventricular septal defects (mVSDs) associated with additional congenital heart diseases in children.     

婴幼儿先天性气管狭窄伴复杂先心病的Ⅰ期纠治

目的：探讨婴幼儿先天性气管狭窄伴复杂先心病I期纠治的治疗效果。方法：2例手术年龄分别1.5岁和3岁的先天性气管狭窄伴法洛四联症患儿在体外循环下行气管狭窄纠治和四联症根治术。1例气管狭窄长约1.2cm，切除气管狭窄段，然后端端吻合；另1例气管狭窄长约6cm，切除3cm剪开形成两块补片，分别扩大剪开的气管前壁。总扩大气管狭窄段达6cm。结果：术后恢复良好。随访6个月-1年，呼吸平稳，CT示气管通畅，心脏超声检查无残余分流和梗阻。结论：纠治先天性气管狭窄半先心病，以I期手术治疗为好，不但术中在体外循环下纠治气管狭窄较安全，而且同时纠治先心病有利于手术后患儿康复。     

腓骨瓣加内外固定结合治疗先天性胫骨假关节

目的 评价利用改良手术方式,治疗先天性胫骨假关节的临床效果.方法 对先天性胫骨假关节10例患儿,采用彻底切除病灶,带监测皮岛的游离或局部转移腓骨瓣移植,环式固定架外固定加髓内针内固定相结合的综合方法治疗.结果 经1～3.5年(平均2.1年)随访,10例患儿中9例胫骨假关节完全愈合,不需支具保护即能负重行走,且随访期间未出现再骨折现象,但均遗留肢体短缩,1例假关节复发.结论 最大限度的避免一切影响骨质愈合因素:彻底切除病变组织,改善局部血循环,牢固的外固定及中心固定,改良术式能提高先天性胫骨假关节的疗效.

Abstract：

Objective To evaluate the long-term results of upgrade method in the treatment of congenital pseudarthrosis of tibia Methods Ten patients with congenital pseudarthrosis of tibia were admitted in this institute. All of them underwent our new operation including resection of the tibia pseudarthrosis, ablation of part of tibia, transplantation of free fibula with flap, and vascular bundle implantation. The long-term results were recorded and analyzed. Results Results was obtained in all 10 cases after 6 months. Nine cases healed completely and could walk with no support. There was no refracture during 1 to 3. 5 years follow-up (mean 2. 1 years). All patients had limb shortening. And only one case recurred. Conclusions The new surgical method, resection of impaired tissue and improvement of blood supply,could get satisfactory results in children with congenital pseudarthrosis of tibia.     

The role of bronchoscopy in slide tracheoplasty in children北大核心CSCD

目的 总结支气管镜在儿童Slide气管成形术中的作用。方法 回顾性分析2017—2020年湖南省人民医院收治的4例气管狭窄患儿的诊疗经过,总结支气管镜在Slide气管成形术术前评估、术中定位与测量及术后创面评估和治疗中的作用。结果 Slide气管成形术术前支气管镜评估显示,4例患儿中3例存在完全气管环,2例合并肺动脉吊带,2例存在多处狭窄。3例在该院完成Slide气管成形术,术中支气管镜下判断狭窄段中点及测量狭窄段长度,协助离断气管狭窄段,术后灌洗明确病原;1例外院Slide气管成形术术后9个月出现瘢痕牵拉,经支气管镜下介入治疗好转。2例术后第4天支气管镜下发现气管黏膜改变,调整治疗方案;2例术后1个月出现肉芽增生,经支气管镜下冷冻治疗好转。1例因吻合口坏死放弃治疗死亡,存活的3例随访6个月以上,预后可,但均存在气管支气管软化。结论 支气管镜可用于气管狭窄患儿Slide气管成形术的管理,有助于术后康复及随访。     

Tracheal surgery in children.

BACKGROUND: The aim of this study is to show that five distinct types of tracheal anomalies should be differentiated with respect to therapy and prognosis. METHODS: The records of 12 infants and children seen over a period of 20 years for different tracheal anomalies such as laryngotracheal stenosis (n = 3), long or short-segment stenosis of the upper (n = 2), middle (n = 6), and lower (n = 1) trachea were reviewed. In addition to these 12 patients with congenital stenosis, 3 other patients needed tracheal resections because of oncologic or traumatic disease: in 2, the trachea was infiltrated by a papillary carcinoma of the thyroid gland and in one, the upper part of the trachea was injured by an oral explosion accident. 25 patients presenting during the same period for other tracheal pathologies including esophagotracheal cleft syndrome (n = 7), tracheomalacia (n = 4), total tracheal agenesis (n = 3), or for placement of a tracheostomy (n = 11) due to other diseases were excluded from this study. RESULTS: There was 1 early death after repair of a laryngotracheal stenosis by cricoid-split and cricoid-splint due to both cerebral hemorrhage and cardiac insufficiency secondary to Fallot's tetralogy. Another child died four weeks after slide tracheoplasty as a result of hypoxic cerebral lesions induced by severe central catheter-related septicemia. One child with therapy-resistant obstructing granulation tissue which developed after a slide tracheoplasty required a tracheostomy. The patient with the tracheal injury died after another accident one year after discharge. All other patients (n = 11) are doing well. CONCLUSIONS: With respect to therapy of congenital and post-intubation tracheal stenosis, four types should be distinguished. Each of these types requires an adequate surgical procedure. The most important postoperative problem in tracheal surgery is the development of granulation tissue. However, the pathogenesis of granulation is still unknown.     

先天性心脏病伴气管狭窄或受压的诊治探讨

目的 探讨先天性心脏病伴气管狭窄或受压的诊断和治疗措施,以提高对气管狭窄的认识.方法 收集本院先心病伴气管狭窄或受压患儿41例,平均年龄14.6个月,体重7.8 kg.主要病种:室间隔缺损/肺动脉高压;法乐四联症肺动脉闭锁、肺动脉瓣缺如;主动脉缩窄伴室间隔缺损;右室双出口;肺动脉吊带等.9例患儿伴双主动脉弓、迷走锁骨下动脉.经胸部CT、气管造影和纤维支气管镜确诊:气管弥漫性狭窄9例,局限性狭窄16例,支气管狭窄7例,多发性狭窄伴支气管桥4例,气管软化5例.38例行体外循环心脏修补术,6例同时行气管狭窄纠治术(端端吻合术3例;自身气管片移植扩大2例;补片扩大1例).5例行气管切开术,用套管支撑狭窄段.结果 住院、近期死亡8例(19.50%).随访22例,平均(2.9±2.4)年.气管端端吻合及自身气管补片扩大者,气管通畅;补片修复者,肉芽组织增生再狭窄.带套管支撑者存活4例,分别于术后3个月、1年、2年、8年拔除气管套管.狭窄未处理者,狭窄段内径与气管整体生长成比例狭窄.结论 先天性心脏病伴气管狭窄诊断需及时,重度气管狭窄与心脏手术Ⅰ期矫治,以滑片吻合术,自身气管片移植术为好.轻、中度狭窄则等待其成长是最好策略.     

21例合并气管狭窄肺动脉吊带患儿的治疗

目的：回顾性分析合并气管狭窄的肺动脉吊带患儿的治疗和预后,探讨单纯左肺动脉移植,避免气管成形术治疗策略的可行性。方法2009年4月至2015年11月,共有21例合并气管狭窄肺动脉吊带患儿在本单位接受手术治疗。其中6例患儿采用左肺动脉移植加气管干预手术策略。另外15例患儿采用左肺动脉移植、避免气管成型手术,术后采用早期拔管,无创CPAP过渡治疗策略。对所有患儿病例资料进行收集分析。结果21例合并气管狭窄患儿,男12例,女9例,年龄1个月~10岁,体重2.9~25.0 kg,术前均有中度至重度呼吸道症状,其中5例为重度,需要机械通气辅助呼吸。左肺动脉移植加气管干预手术患儿6例,存活出院1例,存活率16.7％;其中3例接受Slide气管成形术,2例气管吻合口肉芽组织增生,呼吸衰竭死亡;另外3例接受气管支架植入术,无存活。左肺动脉移植手术患儿共15例,采用经胸骨正中切口行左肺动脉移植术,存活出院13例,存活率86.7％;另外2例撤离呼吸机失败,体外膜肺辅助下行Slide气管成形术,术后气管吻合口愈合不良死亡。结论中、重度气管狭窄的肺动脉吊带患儿,采取左肺肺动脉移植,避免气管成形手术,术后早期拔除气管插管改无创CPAP过渡的治疗策略是可行的,预后也比较好,可以成为此类患儿的理想治疗策略。     

先天性心脏病围手术期气管狭窄的综合治疗

目的 通过分析先天性心脏病围手术期气管狭窄患儿的治疗及转归,探讨不同原因导致气管狭窄的治疗方法.方法 回顾性分析2012年1月至2015年10月收治的60例气管狭窄合并先天性心脏病患儿的临床资料.其中,男42例,女18例;年龄1～144个月,平均4个月;体重(6.65±3.40) kg.先天性心脏病合并先天性气管狭窄39例,继发性气管狭窄21例.结果 先天性气管狭窄合并先天性心脏病39例,实施先天性心脏病矫治+气管狭窄旷置28例,先天性心脏病矫治+气管成形术5例,先天性心脏病矫治+气管支架置入1例;术后远期死亡共计4例,放弃手术治疗死亡4例,放弃治疗门诊随诊1例.继发性气管狭窄21例,实施心脏畸形矫治后气管软化狭窄减轻,不需手术治疗.结论 先天性心脏病合并先天性气管狭窄的主要致病原因在于其完全性软骨环导致气管内径增速缓慢,气道梗阻严重,即使实施心脏手术也不能完全改善其气管狭窄症状,必要时需同时实施气道成形术.继发性气管狭窄的主要致病原因在于气管临近大血管的压迫,缓解血管环畸形后气管狭窄即刻缓解,疗效满意,无需实施气管成形术.     

Development of acquired tracheal stenosis in premature infants due to prolonged endotracheal ventilation: etiological considerations and surgical management

The aim of this study was to determine the etiology and appropriate surgical treatment for acquired tracheal stenosis that developed in patients who had undergone prolonged endotracheal mechanical ventilation as premature neonates. During the period 2000–2004, four patients aged 1–16 years were referred for tracheal stenosis characterized by stridor, choking, and recurrent pulmonary infection. All patients had undergone endotracheal mechanical ventilation for 2–5 months for respiratory distress related to prematurity (gestational age 25–29 weeks, birth weight 648–1,222 g). During this period, methicillin-resistant Staphylococcus aureus was predominantly cultured from the trachea. All patients exhibited a stenotic lesion encompassing 30–37% of the entire tracheal length on spiral CT. On palpation and inspection of the trachea during surgery, however, the stenotic segment appeared to encompass over 50% of the entire trachea. The carina was intact. Three patients underwent slide tracheoplasty with a tracheal resection and the other underwent resection and end-to-end anastomosis. Of the three patients treated by slide tracheoplasty, two are currently free of respiratory symptoms. However, one patient in this group required secondary resection of the remaining stenotic lesion with end-to-end anastomosis. This patient is currently asymptomatic. The remaining patient who underwent a resection and end-to-end anastomosis is doing well. The resected specimen showed fibrosis and degeneration of tracheal cartilage. A combination of prematurity, prolonged endotracheal mechanical ventilation and tracheal infection seem to be responsible for tracheal inflammation and stenosis. When considering surgical procedures for acquired tracheal stenosis, resection and end-to-end anastomosis are desirable. However, slide tracheoplasty with a partial tracheal resection is indicated for the treatment of stenosis involving a long tracheal segment.     

Surgical intervention strategies for congenital tracheal stenosis associated with a tracheal bronchus based on the location of stenosis

Purpose

The aim of this study was to determine the appropriate surgical intervention strategies for congenital tracheal stenosis (CTS) associated with a tracheal bronchus based on the location of stenosis.

Methods

The medical records of 13 pediatric patients with CTS associated with a tracheal bronchus at a single institution between January 2006 and December 2015 were retrospectively reviewed.

Results

Type 1: tracheal stenosis above the right upper lobe bronchus (RULB) (n = 1). One patient underwent slide tracheoplasty and was successfully extubated. Type 2: tracheal stenosis below the RULB (n = 7). Tracheal end-to-end anastomosis was performed before 2014, and one patient failed to extubate. Posterior–anterior slide tracheoplasty was performed since 2014, and all three patients were successfully extubated. Type 3: tracheal stenosis above the RULB to the carina (n = 5). One patient underwent posterior–anterior slide tracheoplasty and was successfully extubated. Two patients with left–right slide tracheoplasty and another two patients with tracheal end-to-end anastomosis for the stenosis below the RULB could not be extubated.

Conclusion

Tracheal end-to-end anastomosis or slide tracheoplasty can be selected for tracheal stenosis above the RULB according to the length of stenosis. Posterior–anterior slide tracheoplasty appears feasible for tracheal stenosis below the RULB or above the RULB to the carina.

     

Is Tracheoplasty Necessary for All Patients With Pulmonary Artery Sling and Tracheal Stenosis?

Pulmonary artery sling frequently accompanies various degrees of tracheal stenosis. The authors reviewed their surgical outcomes for pulmonary artery sling from two institutions. From 1997 to September 2011, 16 patients with pulmonary artery sling underwent surgical treatment. Their median age was 4.6 months and their body weight 6.2 kg at the time of the operation. Of the 16 patients, 12 showed preoperative respiratory symptoms such as recurrent airway infection or dyspnea, with three of these patients receiving ventilator therapy preoperatively. Most of the patients showed various degrees of tracheal luminal stenosis and stenotic length, except for three patients who did not show significant airway stenosis. Left pulmonary artery reimplantation was performed for all the patients, but an accompanying tracheoplasty was performed for only two patients (one patch augmentation and one sliding tracheoplasty). After surgery, early extubation and intensive lung care were performed. Two operative mortalities occurred: one due to cardiac tamponade associated with postoperative bleeding and one due to aggravation of preexisting multiorgan failure. Two late mortalities occurred: one due to airway obstruction by repetitive granulation tissue growing at the tracheoplasty site and one due to pneumonia. The 12 living patients, including 11 patients who did not undergo tracheal surgery, showed no clinically significant airway problems at their last follow-up visit. The surgical outcomes for pulmonary artery sling without tracheoplasty were acceptable. The authors think tracheoplasty may be avoided by intensive postoperative airway management for a significant portion of the patients with pulmonary artery sling and tracheal stenosis.     

The efficacy of slide tracheoplasty in a growing rabbit model

Purpose  

Slide tracheoplasty for congenital tracheal stenosis was reported by Tsang. This procedure provides good results, but there are few studies about the tracheal growth of post-surgical condition. Recently, not only traditional posterior to anterior slide plasty but also right side to left side slide plasty was reported. We evaluated the tracheal growth after slide tracheoplasty in growing Japanese white rabbit model.