单纯腹腔镜监视下经肛门直肠拖出次全结肠切除术

目的 总结单纯腹腔镜监视下经肛门直肠拖出次全结肠切除术治疗先天性巨结肠及其同源病的临床经验并评价其可行性和安全性.方法 2010年3月至12月,8例先天性长段型巨结肠及其同源病行腹腔镜Soave手术.作者改进这种技术.在单纯腹腔镜监视下通过肛门直肠肌鞘入路游离全部结肠.然后拖出体外完成次全结肠切除术.结果 全部操作均顺利完成,6例单一腹腔镜经脐部放置,2例经关闭剥离结肠造口后的小切口导入.手术时间155～240min,平均(178±23)min,术后1～2 d恢复肠蠕动.1例因小肠梗阻开腹探查.随访2～10个月大便次数由术后近期的每天5～15次降至2个月后的3～5次,临床效果良好,无大便失禁或便秘复发.结论 单纯腹腔镜监视下经肛门拖出次全结肠切除术安全、有效、可行,使手术创伤更小,经脐单孔腔镜手术可达到NOTES的美观效果.

Abstract：

Objective To summarize our experience and assess the feasibility and safety of transanal endorectal pull-through and laparoscopic subtotal colectomy for Hirschsprung's disease and allied disorder (HAD). Methods From March to December 2010,8 infants and children with long segment Hirschsprung's disease or/and allied disorder underwent Soave pull-through procedures under laparoscopic guidance. We modified this technique by mobilizing the whole colon via rectal muscular sleeve approach under laparoscopic vision, then transanal endorectal pull-through to complete the subtotal colectomy. Results All procedures were completed successfully. Single-port access laparoscope was introduced through the umbilicus in 6 cases and through a small incision after mobilized colostomy in 2 cases. The average operative time was 178 ± 23 min (range 155 to 240 min). The period of the full bowel action was 1-2 day. One infant developed postoperative intestinal obstruction that required open exploration. They were followed-up for 6 months (range 2-10 months). The bowel movement frequency was reduced from 5-15 per day postoperatively to 3-5 per day after 2 months. The clinical outcomes were excellent with no stool incontinence or constipation. Conclusions Transanal Soave pull-through subtotal colectomy with simple laparoscopic guidance is a safe, effective and feasible procedure, with minimal invasion,and good cosmetic result.     

先天性巨结肠Soave术后大便失禁的评价和治疗

目的 对先天性巨结肠Soave术后大便失禁进行评价和治疗.方法 先天性巨结肠Soave术后大便失禁患儿24例,男14例,女10例,年龄6～13岁,肛门功能临床评价为良19例,差5例.全部患儿行肛门直肠测压,并与18例肛门功能正常的Soave术后儿童比较.所有患儿在医院接受生物反馈训练2周后自行在家中行盆底肌收缩训练.结果 肛管静息压、收缩压、直肠初感觉分别为(18.9±6.2)mmHg、(179.9±17.8)mmHg、(45.4±9.4)ml,与18例对照组儿童相比[分别为(44.5±11.1)mmHg、(177.7±15.9)mmHg、(50.0±10.1)ml],静息压明显下降,收缩压、直肠感觉无明显变化.3例不配合治疗,21例1年后除5例未能坚持外,其余16例获得良好的肛门控制,肛管静息压、肛管收缩压、直肠初感觉分别为(35.4±8.7)mmHg、(195.3±15.0)mmHg、(45.9±8.4)ml.肛管静息压和收缩压均升高.结论 内括约肌损伤可能是先天性巨结肠Soave术后大便失禁的原因之一,对此盆底肌训练可取得满意的治疗效果.

Abstract：

Objective To evaluate the efficacy of pelvic floor muscle exercise for the treatment of fecal incontinence after Soave procedure for Hirschsprung's disease. Methods Twenty four patients who had Soave procedure for Hirschsprung's disease and presented fecal incontinence after surgery were recruited in this study. They were 14 males and 10 females, aged from 6 to 13 years old. Eighteen children who had normal defecation after Soave procedure were selected as controls. All the subjects had manometry to measure resting anal canal pressure, squeeze pressure, and rectal sensation.All the patients had two weeks biofeedback therapy during their stay in hospital. After being discharged, they were taught and prescribed to do pelvic floor muscle exercise at home. Results Of the children with fecal incontinence, the resting anal canal pressure, squeeze pressure and rectal sensation were ( 18. 9 ± 6. 2 ) mmHg, ( 179. 9 ± 17. 8) mmHg, (45. 4 ± 9. 4) ml, respectively. Compared with the controls, the resting anal canal pressure was lower [(18. 9±6. 2) mmHg vs (44. 5 ± 11.1) mmHg].But no difference of squeeze pressure and rectal sensation was found between the 2 groups [(179. 9 ±17. 8)mmHg vs (177. 7 ± 15. 9) mmHg, (45.4 ± 9. 4)ml vs (50. 0 ± 10. 1)ml]. Three patients dropped out of the therapy. Five patients stopped the exercise after the first year. The other 21 who kept exercise improved their defecation function. Their resting anal canal pressure, squeeze pressure and rectal sensation were (35. 4 ± 8. 7)mmHg, (195. 3 ± 15.0)mmHg and (45. 9 ± 8. 4)ml, respectively. Conclusions The dysfunction of internal anal sphincter might be the main cause for fecal incontinence after Soave procedure. Pelvic floor muscle training is helpful to improve the defecation function of the patients.     

Prevention and treatment strategies for esophageal stenosis after thoracoscopy for type Ⅲ esophageal atresia北大核心CSCD

Objective To explore the roles of esophageal stenosis control strategies in children with esophageal atresia (EA). Methods Retrospective analysis was conducted for clinical data of 56 children with type Ⅲ EA undergoing thoracoscopy from January 2017 to December 2022.There were 30 boys and 26 girls.The clinical types were ⅢA (n=12) and ⅢB (n=11) in group A. Birth weight was (2573.26±112.77) gram and operative age (31.70±5.86) hours.Eight cases had concurrent congenital heart disease (CHD). And 33 patients received traditional postoperative observation (group B). The clinical types were ⅢA (n=18) and ⅢB (n=15) in group B. Birth weight was (2 598.79±94.88) gram and operative age (37.00±7.15) hour.Six cases had concurrent CHD.Preoperative profiles and postoperative recovery of two groups were compared along with differential efficacy between the prevention and treatment strategy after comparing EA and traditional management mode. Results No statistically significant inter-group differences existed in operative age (P=0.593), body weight (P=0.863) or proportion of CHD (P=0.438). One-stage surgery was performed for repairing tracheoesophageal fistula and esophageal reconstruction.Later they were cured and discharged with regular follow-ups.The incidence of postoperative anastomotic stenosis was 26.09%(6/23) in group A versus 60.61%(20/33) in group B. The inter-group difference was insignificant (P=0.125). There were 0 cases (0%) of anastomotic leakage in group A versus 3 cases in group B (9.09%)(P=0.001). Average dilation frequency was[(0.61±0.27) vs (2.00±0.41), P=0.014]and incidence of postoperative refractory stenosis[0(0%) vs 4(12.12%), P=0.001]. The differences were statistically significant. Conclusions Perioperative comprehensive management plays some roles in the prevention and treatment of esophageal stricture after EA thoracoscopy.The clinical outcomes are satisfactory. © 2023 Chinese Medical Journals Publishing House Co.Ltd.. All rights reserved.     

先天性肛门直肠畸形术后便秘合并巨结肠的临床特点及处理

目的 总结先天性肛门直肠畸形术后顽固性便秘合并巨结肠的临床特点和治疗效果.方法 回顾性分析2005年8月至2010年7月收治的顽固性便秘合并巨结肠23例临床资料,男10例,女13例.首次手术年龄3 d至6岁,术前诊断均为中低位肛门直肠畸形.23例患儿均以肛门成形术后便秘就诊.下消化道气钡造影显示下端结肠及直肠扩张,无移行段,排钡延迟.本次手术年龄1岁2个月至15岁8个月.均采用Soave巨结肠根治术.结果 术前见肛门外观大致正常13例,外观正常合并直肠尿道瘘1例,肛门开口位置前移6例,瘢痕回缩2例,肛门外口狭窄1例.术中进入盆腔后即为扩张肠管,系膜增生增厚,肠壁血管增生粗大.13例单纯经会阴手术,10例经腹会阴手术,其中1例同时回肠末端造瘘.9例巨结肠切除同时,再次行肛门成形术,1例巨结肠切除同时行前矢状入路直肠尿道瘘修补术.病理检查15例切除肠管远近段,可见神经节细胞者,8例肠管远端无神经节细胞者,23例均见肌层增生肥厚,肌纤维变性,肌层排列紊乱.二次术后随诊肛门功能李氏评分5～6分.结论 先天性肛门直肠畸形术后顽固性便秘往往并发巨结肠改变,影像显示为肠管局限性扩张、僵硬和动力紊乱.采取手术治疗,切除病变肠管,做必要的肛门解剖缺陷修复,术后效果良好.

Abstract：

Objective To summarize the experience of diagnosis and treatment of intractable constipation in children with repaired anorectal malformation.Methods Between August 2005 and July 2010,23 children with a history of anorectal malformation (ARM) repair were diagnosed with intractable constipation,and underwent surgical treatment at this center.The patients,including 10 boys and 13 girls,underwent primary surgeries to repair low or intermediate type of ARMs.Their ages at the primary surgery ranged from 3 days to 6 years old.Physical examinations found normal appearance of anus on 14 children including 1 with rectourethral fistula,anus antelocation on 6,cicatricial retraction of anus on 2,and anal stricture on 1.The positive barium enema revealed the dilated distal colon and rectum without transition zones,and delayed barium transit time.The patients' age at the second surgery ranged from 1 year and 2 months to 15 years and 8 months old.Results Dilated distal colon and rectum,with increased intestinal wall and mesentery thickness as well as proliferated blood vessels,was found during surgery on all patients.However,no transition zone was observed.To remove the dilated intestine,transanal Soave procedure was performed on 13 children,and the other 10 underwent abdomino-perineal Soave procedure including 1 had additional ileostomy.Besides the megacolon resection,9 patients also underwent anoplasty,and rectourethral fistula was also repaired on 1 patient.At the distal end of the removed intestine,pathological examination revealed ganglion cells in 15 patients,and no ganglion cells in 8 patients.Hypertrophy,degeneration and derangement of the intestinal smooth muscle cells were also observed.After reoperation,patients' anal function was graded as 5 or 6 according to Lizheng's anal function scoring criteria.Conclusions Intractable constipation after ARM repair is usually caused by acquired megacolon.It can be cured by megacolon resection.     

限制性后人字入路直肠肛门成形及外括约肌成形术治疗中低位无肛合并直肠会阴瘘

目的 针对中低位无肛合并直肠会阴瘘的患儿改良一种新的手术方法,使之较现行的方法如Pena、后切术更加简便、更符合生理以减少手术并发症.方法 切口采用限制性(即限制在3 cm以内)后人字切口,显露瘘管和直肠下端,在直肠下端的后方将肛门外括约肌复合体部分切开,将两侧的肌纤维环包于直肠下端.完成外括约肌成形术,在中线后切瘘管及直肠壁,于正常肛隐窝处完成肛门成形.结果 24例先天性无肛合并直肠会阴瘘(X线侧位片直肠盲端在P-C线或至Ⅰ线间)患儿进行了该手术,术后全部病例进行随诊:除1例5岁患儿术后便秘,全部患儿恢复自主排便,可控制,无失禁及溢粪.1例患儿术后肛门切口轻度感染经切开引流治愈.结论 该手术较Pe(n)a手术操作更加简便,局部创伤小,术后避免直肠回缩,减少了术后并发症,且术后不必扩肛;不同于传统的"后切术",术中进行肛门外括约肌成形,术后随诊疗效满意.

Abstract：

Objective To study the clinical outcomes of anorectoplasty and external anal sphincterplasty via posterior approach with Y-shaped incision to repair intermediate and low anorectal malformation complicated with rectal perineal fistula in children. Methods Twenty four patients with intermediate and low anorectal malformation complicated with rectal perineal fistula were enrolled in this study. Via the posterior approach, a Y-shaped incision (＜3 cm) was made to expose the lower rectum and fistula. The external anal sphincter was identified and dissected at the posterior aspect of the lower rectum. The external anal sphincterplasty was performed by overlapping the ends of the muscle around the lower rectum. The rectal perineal fistula was resected and anorectoplasty was performed. Results All surgeries were successfully performed. Voluntary bowel movement and fecal continence was restored on 23 patients after surgery. One 5 years old patient still had constipation. Mild incision infection was found on 1 patient, and was cured after opening the incision and draining the pus. No fecal incontinence or anastomotic stricture was noted. Conclusions Compared with Pena surgery, anorectoplasty and external anal sphincterplasty via posterior approach with Y-shaped incision is less complicated and invasive to repair intermediate and low anorectal malformation complicated with rectal perineal fistula. By using this procedure, better sphincter shape and fecal function is achieved on patients.     

腹腔镜辅助肛门直肠成形术致后尿道憩室的预防措施探讨CSCD

Objective To explore the preventive measures of posterior urethral diverticulum (PUD) after laparoscopic assisted anorectoplasty (LAARP). Methods From August 2013 to December 2018, perioperative and postoperative clinical data were reviewed for 6 children with anorectal malformation (ARM) developing PUD after LAARP. PubMed database was searched for all published English literatures of PUD after ARM surgery from 2000 to 2021. Since international naming of PUD is not standardized, there are two major names, i. e. remnant of original fistula and PUD. Thus retrieval keywords were anorectal malformation, remnant of original fistula and PUD. Results The diagnostic age was 17 months to 5 years after LAARP. The clinical manifestations included mucuria, recurrent urinary tract infection and dysuria, etc. Computed tomography (CT) and retrograde urethrography revealed prerectal cyst. A total of 13 articles were retrieved, reporting 102 cases of PUD after ARM surgery. The age was 1 — 44 years and the diameter of PUD 3 —70 mm. The major clinical symptoms were frequent urination, dysuria, recurrent urinary tract infection, urinary calculi, hematuria and urinary incontinence, etc. Some non-symptomatic cases were only discovered accidentally during follow-ups. ARM procedures included LAARP (n = 36), posterior sagittal anorectopasty (PSARP,n = 15), abdominoperineal pull-through (n=15) and APP (n=50). Conclusion PUD is a rare complication after ARM surgery. And potential risks of PUD may be minimized by strengthening perioperative managements and improving surgical techniques. © 2022, Journal of Clinical Pediatric Surgery. All rights reserved.     

加速康复外科技术在肛门闭锁并直肠前庭瘘中的应用CSCD

Objective To explore the efficacy and safety of enhanced recovery after surgery (ERAS) in children with anal atresia and rectal vestibular fistula. Methods From January 2017 to October 2021, prospective review was conducted for 50 children with anal atresia and rectal vestibular fistula undergoing transanal analoplasty. They were randomized into two groups of ERAS and non-ERAS (n =25 each). ERAS group received enema once in the morning of operation day, oral 12.6% carbohydrate liquid 2 h pre-operation, intraoperative heat preservation, target-oriented rehydration ,no indwelling catheter post-operation, postoperative analgesia and other new measures; non-ERAS group had traditional perioperative treatment. Blood glucose at the beginning of anesthesia, blood glucose 24 h post-operation, white blood cell count ( WBC), C-reactive protein (CRP), time of initial exhaust and defecation post-operation, amount of intraoperative infusion, time of postoperative intravenous infusion ,time of postoperative hospitalization, expenditure of hospitalization incidence of complications and readmission rate at Day 30 post-discharge were compared between two groups. Results No significant inter-group differences existed in average age, weight, preoperative WBC, CRP, hemoglobin, albumin, prealbumin or blood glucose at admission. Blood glucose at the beginning of anesthesia in ERAS and non-ERAS groups was (4.92±0.50) and (4.53 ±0.42) mmol/L, blood glucose (5.03 ±0.66) and (5.96±41.18) mmol/L at 24 h post-operation. WBC at 24h post-operation (9.97 3.24) x 10°/L and (8.28 ±3.51) x 10°/L;CRP at 24 h post-operation (3.63±4.00) and (9.03±15.77) mg/L;initial postoperative exhaust and defecation time (12.1 ±6.4) and (14.0 ±9.3) h;intraoperative infusion volume (83.1 ±32.2) and (136. 1±68.4) mL;postoperative intravenous infusion time (4.68 ±1.25) and (6.6 ±1.68) days; postoperative hospital stay (7.12 ± 1.56) and (10.56 ±3.58) days; hospitalization expense (13 314. 34 ±2 856.86) and (16 088.69 ±3 282.34) CNY ;complications occurred (n =3,12%) and (n =6,24%) ;overall satisfaction was 88% and 60%. Both groups were cured and discharged and there was no re-admission within 30 days post-discharge. No inter-group differences existed in initial exhaust or defecation time, WBC and CRP at 24 h post-operation (P >0.05).In nonERASgroup, blood glucose declined at the beginning of anesthesia while postoperative blood glucose level rose with statistical difference (P <0. 05) ; overall satisfaction of ERAS group was higher than that of non-ERAS group with statistical difference (P lt; 0.05). Conclusion Conceptual application of ERAS is both safe and effective in children with anal atresia with rectovestibular fistula. It can effectively shorten the average time of hospital stay, curtain the length of postoperative hospitalization and lower hospitalization expenditure. A wider popularization is worthy. © 2022, Journal of Clinical Pediatric Surgery. All rights reserved.     

低出生体重食管闭锁患儿预后因素的研究

Objective To investigate the prognostic factors of survival rate after repair of esophageal atresia (EA) and/or tracheoesophageal fistula (TEF). Methods The clinical data of the 101 patients underwent TEF/EA repair between January 1999 and December 2008 at this center were retrospectively reviewed. The patients were divided into low-birth-weight group (29 patients, birth-weight＜2 500 grams) and normal-birth-weight group (72 patients, birth-weight＞2. 5 kg). The possible prognostic factors were retrospectively analyzed including patients status, the incidence of preoperative and intraoperative critical events, postoperative complications, postoperative mechanical ventilation time,stress hyperglycemia and survival rate. Logistic regression analysis was employed to predict the prognostic factors of the survival rate after repair of EA and/or TEF. Results Of the 29 low-birth-weight EA patients, the survival rate was significantly lower than that of the normal birth weight patients (75.9% vs 92. 3% ,P = 0. 027) ,and the survival rate positively correlated with the birth weight (r=0. 946,P= 0. 015). The incidence of preoperative and intraoperative critical events and postoperative complications in low-birth-weight EA patients were significantly higher than those of the normal birth weight patients (67. 7% vs 32. 9% ,P = 0. 002; 70% vs 26%, P = 0. 001 ). The morbidity of anastomotic stricture and leak was higher, and postoperative mechanical ventilation time was longer compared with the normal birth weight patients (P = 0. 001 ). The survival rate of the patients had postoperative stress hyperglycemia was lower than that of patients without stress hyperglycemia (93. 6% vs 81.0%,P = 0. 024) ,and the survival rate negatively correlated with the severity of postoperative stress hyperglycemia (r = - 0. 931, P = 0. 022). Conclusions Low-birth-weight and postoperative stress hyperglycemia are the prognostic factors of survival rate after repair of EA and/or TEF.     

Integrated management during the perinatal period for total anomalous pulmonary venous connection北大核心CSCD

Objective To evaluate the clinical effectiveness of integrated management during the perinatal period for fetuses diagnosed with total anomalous pulmonary venous connection (TAPVC) by prenatal echocardiography. Methods Clinical data of 64 cases of TAPVC fetuses diagnosed by prenatal echocardiography and managed with integrated perinatal care in Qingdao Women and Children's Hospital from January 2017 to December 2021 were retrospectively analyzed. Integrated perinatal care included multidisciplinary collaboration among obstetrics, fetal medicine, ultrasound, pediatric cardiology, pediatric anesthesia, and neonatology. Results Among the 64 TAPVC fetuses, there were 29 cases of supracardiac type, 27 cases of intracardiac type, 2 cases of infracardiac type, and 6 cases of mixed type. Chromosomal analysis was performed in 42 cases, and no obvious abnormalities were found. Among the 64 TAPVC fetuses, 37 were induced labor, and 27 were followed up until term birth. Among the 27 TAPVC cases, 2 cases accepted palliative care, 2 cases were referred to another hospital for treatment and lost to follow-up, while the remaining 23 cases underwent primary repair surgery. One case died within 6 months after the operation due to low cardiac output syndrome, while the other 22 cases were followed up for (2.1±0.3) years with good outcomes (2 cases underwent a second surgery within 1 year after the first operation due to anastomotic stenosis or pulmonary vein stenosis). Conclusions TAPVC fetuses can achieve good outcomes with integrated management during the perinatal period. © 2023 Xiangya Hospital of CSU. All rights reserved.     

SOX10在先天性巨结肠相关性小肠结肠炎发病中的作用

目的 探讨SOX10、潘氏细胞发育及分泌防御素-5与先天性巨结肠相关性小肠结肠炎的关系.方法 收集50例先天性巨结肠病变肠管,根据术前是否发生小肠结肠炎分为HAFC组(n=14)和HD组(n=36),并以20例正常结肠标本作对照组.采用免疫组织化学方法观察结肠中防御素-5蛋白质表达、潘氏细胞发育情况以及SOX10蛋白质表达情况.采用实时荧光定量PCR技术检测防御素-5 mRNA及Sox 10 mRNA表达情况.结果 防御素-5在正常肠管中不表达,HAEC组和HD组在肠腺隐窝基底处呈不同程度阳性表达.但前者阳性区域平均光密度值明显增高(0.33±0.039比0.10±0.031,P＜0.05),HAEC组防御素-5mRNA亦呈显著增高趋势(2.72±0.80比0.78±0.21,P＜0.05).对结肠组织同层切片进行潘氏细胞特异性产物溶菌酶免疫组化染色发现,对照组肠管中除1例存在弱阳性外其他均无阳性表达.HD组和HAEC组结肠中同样在隐窝基底处存在溶菌酶阳性细胞,可鉴别为化生的潘氏细胞,但HAEC组在发生率(78.6%)和细胞个数(2.97±0.80)明显高于HD组(27.8%,0.43±0.85)(P＜0.05).SOX10免疫产物主要在结肠神经节细胞膜及胞浆中表达,对照组、HAEC组、HD组阳性区域的平均光密度值递减(0.75±0.041,0.61±0.048,0.35±0.025),差异具有统计学意义(P＜0.05),同时RT-PCR检测显示Sox10 mRNA在各组中的表达与蛋白质水平呈平行结果.结论 SOX10可能通过影响潘氏细胞发育及分泌防御素-5在先天性巨结肠相关性小肠结肠炎的发生发展中起一定作用.

Abstract：

Objective To study the expression of SOX10 and Human α-defensins-5(HD-5 )in Hirschsprung's disease associated enterocolitis(HAEC) and expore the possible relationship between SOX10 and HAEC.Methods Fifty pathological colons of Hirschsprung's disease (HD) were divided into HAEC group (n = 14) and HD group(n = 36) according to the presence of preoperative enterocolitis,Twenty normal colons as control group.The protein and mRNA expression of HD-5 and SOX 10 were measured by immunohistochemical staining and real-time quantitative PCR Results Normal colons did not express HD-5 but positive expression of HD-5 was detected at the base of the crypts of Lieberkuhn in HAEC and HD groups in different degree.The mean optical density of HD-5 immunohistochemical staining (0.33 ± 0.039 vs 0.10 ± 0.031 )and HD-5 mRNA expression (2.72 ± 0.80 vs 0.78 ± 0.21 ) in HAEC group was apparently higher than those in HD group(P＜0.05).The expression of lysozyme,a specific product by Paneth Cell,on sequential sections was negative in control group except one sample.In HAEC group and HD group,positive expression of lysozyme can be seen in the crypts of Lieberkuhn,where the cells can be identified as Metaplastic Paneth cell,but the incidence (78.6% vs 27.8%) and the number of cells 2.97 ± 0.80 vs 0.43 ± 0.85) in HAEC group were obviously higher than those in HD group(2.97 ± 0.80 vs 0.43 ± 0.85) (P＜0.05).SOX10 was mainly located in the plasmalemma and cytoplasm of ganglion cell and its mean optical density in control group (0.75 ±0.041 ),HAEC group (0.61 ± 0.048)and HD group(0.35 ± 0.025) were decrement,the difference between three groups were statistically significant (P＜0.05).Meanwhile,Sox10mRNA detected by real-time quantitative PCR indicated the parallel result.Conclusions SOX10 may be an important factor in the pathogenesis and development of HAEC.     

改良Soave结合结肠切端缩口缝合术治疗先天性巨结肠疗效评价

目的 评价改良Soave结合结肠切端缩口缝合术治疗先天性巨结肠的术后疗效.方法 回顾性分析2005年7月至2009年8月在我院行改良Soave结合缩口缝合术的32例巨结肠患儿术后恢复情况,设为观察组,选取32例行单纯改良Soave根治术的患儿为对照组.观察术中扩张段肠管断端病理切片、术后复查肛诊、肛门镜、钡灌肠及直肠肛管测压并对二组患儿排便功能进行评分.结果 观察组中26例扩张段断端神经节细胞数量正常,无明显变性,6例神经节细胞数量减少,存在变性,对照组中前者28例,后者4例.肛诊及肛门镜示术后半年以上缩口缝合结肠黏膜皱褶基本消失.钡灌肠示结肠均无明显扩张,结肠形态恢复良好.观察组和对照组术后1年排便功能评分及直肠肛管测压中的肛管高压区长度、肛管静息压、直肠感觉阈值,分别为6.6±1.3、(2.0±0.7)cm、(53.1±3.9)mmHg、30～55 ml;6.8±1.2、(1.9±0.6)cm、(52.6±4.7)mmHg、35～60 ml,均无统计学差异.术后并发症观察组有吻合口狭窄1例,二组轻度污粪各1例.结论 改良Soave结合结肠切端缩口缝合巨结肠根治术后结肠及排便功能恢复满意,与单纯改良Soave术式疗效无差异.扩张但功能良好的近端结肠切端管径大于肛门口径时,可行结肠切端缩口缝合术,该术式可成为改良Soave手术较好的辅助手段.

Abstract：

Objective To evaluate the efficacy of modified Soave procedure combined with colon plication for the treatment of Hirschsprung's disease (HD) in children.Methods Between July 2005 and August 2009,32 HD patients of the plication group underwent modified Soave procedure combined with colon plication at this center.Another 32 patients who underwent modified Soave procedure were enrolled as the controls.The pathological examinations,follow-up,barium enema,anorectal manometry,and anorectal function were retrospectively reviewed and analyzed to evaluate the surgical efficacy.Results Of the 32 patients of the plication group,26 had normal ganglion cells at both ends of the dilated intestine,and decreased ganglia number and ganglia degeneration were found on the other 6 patients.However,among the 32 control patients,decreased ganglia number and ganglia degeneration was only found on 4 patients.Rectoscopy suggested that mucosa rugae caused by colon plication could eventually disappear half a year after the surgery.On patients' barium enema radiography,no dilated intestines were observed.No difference of the defecation function scores between the plication group and control group at 1 year after operation was found (defecation function score,6.6 ± 1.3 vs 6.8 ±1.2).Moreover,there was no difference of high pressure zone length (2.0 ± 0.7 cm vs 1.9 ± 0.6 cm),anal canal resting pressure (53.1 ± 3.9 mmHg vs 52 ± 4.7 mmHg) and sensation threshold (30～55 ml vs 35～60 ml) between the 2 groups 1 year after operation.Postoperative complications included anastomotic stenosis was noted on 1 patients of the placation group,1 of the plication group and 1 control patient had mild soiling.Conclusions Modified Soave procedure combined with colon plication improves the defecation function of the HD patients.     

先天性巨结肠患儿切除肠管肠黏膜ENS干细胞的初步鉴定

目的 我们已经证实p75NTR阳性细胞在HD(Hirschsprung's disease)患儿切除肠管的移形段和扩张段肌间丛及黏膜下丛存在,本研究拟对肠黏膜层进行研究.另外,为了明确Metzger等在肠黏膜活检标本中分离体外培养出的肠黏膜ENS干细胞是胚胎残留还是其他肠黏膜干细胞在诱导分化过程中转化而来,本研究选用了不同时期肠神经干细胞标记物(早期:SOX10和Nestin中晚期:TUJ1;晚期:GFAP),对病理已证实的婴儿组(年龄≤1岁)和幼儿组(年龄＞1岁)HD患儿的切除肠管标本连续切片后进行研究.同时对黏膜层、黏膜下丛及肌间丛的SOX10、Nestin、TUJ1及GFAP强阳性表达率进行综合分析,明确GNCSCs在三层间的存在差异及其与年龄间的关系,以指导临床取材.方法 收集临床诊断及病理确诊的HD患儿手术切除标本15例,分为婴儿组(8例)及幼儿组(7例),长段型(2例)及常见型(13例).沿肠管纵轴各取材一处并将其分为3组:第一组为狭窄段肠管,第二组为移行段肠管,第三组为扩张段肠管.对照组选用切除扩张段肠管的近端肠管,阴性对照应用TBS缓冲液作为一抗.应用S-P法分析四个指标在各标本、各部位的表达情况.结果 SOX10、Nestin和TUJ1在移行段和扩张段黏膜层均有强阳性表达,且在黏膜固有层呈散在分布,但主要位于近黏膜肌层,GFAP在移行段和扩张段黏膜层未见阳性表达.肌间丛SOX10、Nestin、TUJ1和GFAP免疫阳性细胞的强阳性表达率高于黏膜下丛,黏膜下丛强阳性表达率高于黏膜层.婴儿组患儿在扩张段肌间丛SOX10和Nestin强阳性表达率较幼儿组高.婴儿组扩张段黏膜层SOX10和Nestin强阳性表达率较幼儿组高.结论 在HD患儿有神经节段肠管肠黏膜可能存在ENS干细胞或前体细胞,且在肠黏膜固有层呈散在分布,但主要定位于近黏膜肌层.SOX10、Nestin、TUJ1和GFAP在HD患儿肌间丛表达水平较黏膜下丛高,而黏膜下丛表达水平较黏膜层高.婴儿组扩张段黏膜层SOX10和Nestin表达水平可能较幼儿组高,随年龄增长可能逐渐降低.初步判定存在于肠黏膜的ENS干细胞或前体细胞可能来源于肠神经嵴细胞.

Abstract：

Objective To identify the enteric nervous system (ENS) stem cells in intestinal mucosa of the patients with Hirschsprung's disease (HD). Methods Fifteen patients were pathologically diagnosed with Hirschsprung's disease. They underwent surgery to remove the aganglionic intestines.The removed intestines were collected for this study. Of the 15 patients, 8 were infants (age≤1 year)and 7 were toddlers (age＞ 1year). Two were long-segment HD, and 13 were short-segment HD.Three pieces of tissues were collected from the stenotic segment, transitional zone and dilated segment of the removed intestines. The intestines proximate to the dilated segment were selected as controls.Immunohistochemical staining of stem cell markers including SOX10, Nestin, TuJ1 and GFAP was performed to indentify the ENS stem cells in intestinal mucosa. The TBS buffer was used as the negative control of the primary antibody. The S-P method was applied to analyze the expressions of the 4stem cell markers through the intestine. Results The expressions of SOX10, Nestin, and TUJ1 in the mucosa of the transitional zone and dilated segment were strong. And scattered SOX10, Nestin, and TUJ1 positive cells were observed in the lamina propria. But most of the SOX10, Nestin, and TUJ1 positive cells located in mucosa closed to the muscularis mucosa. The expression of GFAP in the mucosa of the transitional zone and dilated segment was negative. The expressions of SOX10, Nestin,TUJ1 and GFAP in myenteric plexus were stronger than those of submucosal plexus (SOX10 expression, infant 50% vs 31.3%, toddler 35. 7% vs 21.4%; Nestin expression, infant 43. 8% vs 37. 5%,toddler 28. 6% vs 14. 2%; TUJ1 expression, infant 37. 5% vs 18. 5%; toddler 28. 6% vs 28. 6%;GFAP expression 25.0 % vs 18. 5 % ; toddler 57. 1% vs 35. 7 % ; P ＜ 0. 05 ). The expressions of SOX10, Nestin and TUJ1 were stronger in submucosal plexus than those in mucosa (SOX10 expression, infant 31.3% vs 25. 0%, toddler 21.4% vs 7. 1% ; Nestin expression, infant 25. 0% vs 18. 8%,toddler 14. 3% vs 14. 3%; TUJ1 expression, infant 18. 5% vs 12. 5 %, toddler 28. 6% vs 7. 1%; P＜0. 05). The expressions of SOX10 and Nestin in myenteric plexus of the dilated segment in infants were stronger than thoset in toddler (SOX10, 75. 0% vs 42. 8% ; Nestin, 62. 5% vs 42. 8%, P＜0. 05). The expressions SOX10 and Nestin in mucosa of the the dilated segment in infants were stronger than those in toddler (SOX10, 37. 5% vs 14. 3%; Nestin, 25. 0% vs 14. 3%, P＜0. 05). Conclusions The ENS stem cells may exist in the mucosa of the ganglionic intestines of HD patients, whichare scattered and mostly located in the mucosa near muscularis mucosa. In HD patients, the expressions of SOX10, Nestin, TUJ1 and GFAP in myenteric plexus are higher than those in submucosal plexus. However, the expressions of SOX10, Nestin, TUJ1 and GFAP in the submucosal plexus are higher than those in the mucous layer. The expressions of SOX10 and Nestin in mucosa of the dilated segment in infants are higher than those in toddlers, and it decreases with age. These observations suggest the ENS stem cells or precursor cells in the gut mucosa may be derived from the gut neural crest cells.     

Surgical options for esophageal stenosis in children北大核心CSCD

Objective To explore the clinical efficacy of progressive individualized options for esophageal stenosis (ES) in children. Methods From January 2017 to December 2022, retrospective review was conducted for 106 ES children with regular follow-ups. There were 59 boys and 47 girls with an age range of (3.00 ± 0. 75) year and a body weight of (9.00 ± 1. 25) kg. The causes were anastomotic (n: 58), corrosive (n: 47) and congenital (n: 1). Sequential treatments of direct balloon dilation under gastroscope, submucosal injection of drugs into esophagus, esophageal stenting and surgical procedures were applied. After interventions, degree of ES was observed by esophagography and gastroscopy along with dysphagia grade for evaluating clinical efficacy. Differences were compared by I or yr test. Results All of them were discharged uneventfully. After balloon dilatation, the outcomes were excellent under direct gastroscopic view alone (n: 55). Among 35 children with balloon dilation plus esophageal submucosal drug injection, the outcomes were excellent (n: 25) and esophageal stenting (n: 10). Among 25 children with esophageal stenting, the outcomes were excellent (n:21) and additional surgery (n: 4). Surgical procedures were performed with excellent outcomes (n:5). Conclusions The clinical outcomes progressive individualized options of direct visualization balloon dilation under gastroscope, balloon dilation plus submucosal injection of drugs into esophagus, esophageal stenting and surgical procedure are precise, convenient and efficacious for ES in children. © 2023 Chinese Medical Journals Publishing House Co.Ltd. All Rights Reserved.     

Clinical and radiological characteristics of congenital spinal deformity associated with anorectal malformationCSCD

Objective To explore the clinical subtypes and radiological characteristics for children with anorectal malformation (ARM) associated with congenital spine deformity. Methods A retrospective review was conducted for 72 ARM children patients who had received treatment between January 2008 and December 2019. There were 38 boys and 34 girls. Based upon the range of vertebral anomalies, they were assigned into Group | (n =30,41.7% ) :ARM associated with simple thoracic/lumbar vertebral anomalies;Group [I (n = 35 ,48.6% ) :those with simple sacral agenesis and Group I (n =7,9.7% ) : those with both sacral agenesis and thoracic/lumbar vertebral anomalies. Demographic profiles, ARM type, type/location of vertebral anomalies, sacral agenesis ,rib anomalies and concomitant defects of other systems were recorded. SPSS 18.0 was used for statistical analysis. Since all measurement data in this study did not obey normal distribution, M( Q1, Q3) was used to describe the measurement data. Numerical comparison among three groups was conducted by KruskalWallis rank sum test for comparison of multiple groups, and Nemenyi test was used for pound-wise comparison. Fisher’s exact probability method was used to compare the sex ratio, types of anorectal malformations , combined with other systemic malformations, distribution of vertebral malformations, rib malformations, and sacral malformations among the three groups. P < 0. 05 showed statistical significance. Results The average evaluation age of Group JI was 4.00(3.00,5.00) months and it was greater than Group I /Il (P =0.009). No differences existed in gender or ARM type among three groups. Spinal deformity predominated in main thoracic region (24/30) and proximal thoracic region (17/30) in Group I whereas lumbar region (6/7) and thoracolumbar region (4/7) were affected in Group Il (P =0.002). No significant differences in type/level of vertebral anomaly or percentage of multiple anomalies existed between Groups I and JI. Severe sacral agenesis was more common in Group [[ than Group II (P =0.020). The prevalence of associated rib anomalies was higher in Group J than Group I/II (P =0.002). And Group [ had higher incidence of cardiac defects (P =0.031) and a lower incidence of intraspinal anomalies (P =0.001) than Group I/II. Conclusion ARM patients associated with spine deformity may be divided into three clinical subtypes. Clinical and radiological characteristics vary among three subtypes and carry important implications for disease evaluations and treatments. © 2022, Journal of Clinical Pediatric Surgery. All rights reserved.     

腹腔镜辅助高位肛门直肠畸形成形术后中期随访研究

Objective To evaluate postoperative anorectal functions of patients with high-type anoreetal malformations who underwent laparoscopically assisted anorectal pull-through (LAARP). Methods From Jan 2002 to Dec 2005, LAARP was performed on 10 patients with high-type anorectal malformations. All the patients were followed up and their clinical data were compared with patients who underwent posterior sagittal anorectoplasty (PSARP) (n = 12) performed before 2002. All pa-tients underwent colostomy at newborn period. Anorectal functions were evaluated with Kelly's clinical scoring and anoreetal manometry. At follow-up, the age of patients ranged from 3 to 5 years and from 5 to 6 years in the LAARP group and in the PSARP group respectively. Results The age at evaluation in the LAARP group was significantly younger than that in the PSARP group (47. 2 ± 8. 3 months vs 74. 5 ± 9. 3 months, P<0. 05). Kelly's clinical score was 3. 7 ± 0. 9 vs 3. 4± 0. 8 for the LAARP group and PSARP group respectively. The results of resting anal pressure and high pressure zone length showed that no significant difference was noted between the two groups, while the positive rate of rec-toanal inhibitory reflex in the LAARP group was significantly higher than that in the PSARP group (80 % vs 41%, P<0. 05). Conclusions Midterm follow-up study revealed that satisfactory fecal con-tinence can be achieved in patients with high-type anorectal malformations treated with LAARP, which may be an effective alternative. However, long-term follow-up is needed to evaluate the anorec-tal functions of patients treated with LAARP or PSARP     

先天性巨结肠同源病:临床诊断与手术指征的确立

Objective To determine the diagnostic criteria and surgical indications of allied diseases of Hirschsprung's disease (HAD) in children. Methods During the period from Jun. 2007 to Dec. 2009,65 consecutive patients were performed "radical surgery for megacolone" by this surgery team. Based on the clinical symptoms,anorectal manometry, AChE staining of rectal mucosa and barium enema,the preoperative suspected diagnosis of Hirschsprung's disease (HD) or HAD was made.Surgical indications were determined once the suspected HD diagnose was made. On the patients withsuspected HAD, strictly conservative treatments should be carried out for at least 3 months. If conservative treatments were ineffective or the symptoms were recurrent, surgical indication of HAD patients could be determined. Results The preoperative diagnosis was 38 patients with HD and 27 with HAD.However, the final pathologic diagnosis was 36 with HD and 29 with HAD. The misjudged preoperative diagnosis was made in 10. 5% (4/38) HD patients and 7. 4% (2/27) HAD patients. The HAD patients included 22 (75. 9%) hypoganglionosis (HG) cases and 7 (21.1 %) intestinal neuronal dysplasia (IND) cases. Among the 27 HAD patients, 27 (93. 1%) had anorectal inhibitory reflex, and varied and delayed reflection waveforms. AChE staining was positive in 12 (41.1%) patients. The results of anorectal manometry and AChE staining of the HAD patients were significantly different from those of the HD patients (P＜0. 01 ). Barium enema of the HAD patients appeared severe expanded rectum which occupied more than 90% space of the pelvic cavity. Redundant sigmoid colon was also found in more than 90% patients. All HAD patients had 24-hour barium retention. Subtotal colectomies were performed on 9 (31. 0%) HAD patients,and 8 (22. 2%) HD patients. Conclusions Anorectal manometry, AChE staining and barium enema are still important in determining the diagnosis and surgical indications for HAD. The efficacy of 3 months or longer conservative treatment is crucial to determine the surgical indications for HAD.     

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.     

High-mobility group box-1 and receptor for advanced glycation end products in preterm infants with brain injury

Background:High-mobility group box-1 (HMGB1) protein acts as an important pro-inflammatory mediator,which is capable of activating inflammation and tissue repair.HMGB1 can bind to its receptor such as advanced glycation end products (RAGE).RAGE,in turn,can promote the production of pro-inflammatory cytokines.Soluble RAGE (sRAGE) is a truncated form of the receptor comprising the extracellular domain of RAGE and can inhibit RAGE-activation.The objective of this study was to investigate whether HMGB1 and RAGE are involved in the development of brain injury in preterm infants.Methods:In total,108 infants ≤34 weeks gestation at birth were divided into 3 groups according to cranial altrasound scan:mild brain damage (n=33),severe brain damage (n=8) and no brain damage (n=67).All the placentas were submitted for pathologic evaluation.Histological chorioamnionitis (HCA) was defined as neutrophil infiltration of amniotic membranes,umbilical cord or chorionic plate.Expressions of HMGB1 and RAGE proteins were assessed by immunohistochemical analysis.The concentration of HMGB1 and sRAGE in umbilical cord blood were measured by enzyme-linked immunosorbent assay.Results:The frequency of HCA was 30.12％.HCA was associated with elevated concentrations of HMGB1 and decreased sRAGE in umbilical cord blood.The severe brain injury group demonstrated higher cord blood HMGB1 concentrations (P＜0.001) and lower sRAGE concentrations (P＜0.001) than both other groups.Brain injury in the premature infants was linked to intense staining for HMGB1/RAGE,particularly in inflammatory cells.Conclusions:Changes of cord blood HMGB1 and sRAGE of premature infants had direct relationship with the degree of inflammation and severity of brain damage.Monitoring sRAGE and HMGB1 levels may be helpful to predict intrauterine infection and brain injury in premature infants.     

腹腔镜下十二指肠吻合术治疗新生儿十二指肠梗阻

Objective To evaluate the feasibility and indication of laparoscopie duodenoduoden-ostorny for neonates with congenital duodenal obstruction- Methods From May 2004 to Feburary 2008,6 newborns with duodenal obstruction underwent exploratory laparoscopy. With a lower-pressure pneumoperitoneum of 5～8 mmHg and a suspending suture for right liver elevator, the procedure was performed using 3 cannulas of 3.3 mm to 5.5 mm diameter. Under the laparoscopic vision, the cause of duodenal obstruction was diagnosed and a sutured anastomosis was performed after the duodenum mo-bilized. Results Findings at laparoscopy included duodenal diaphragm in 3 cases,annular pancreas in 2 cases, and preduodenal portal vein in 1 case. Three cases with duodenal diaphragmatic stenosis were en-countered a partial excision of the diaphragm after vertical incision of the anterior part of duodenum followed laparoscopically by a transverse suture. A diamond-shaped side-to-side duodenoduodenal anas-tomosis was successfully carried out in 2 cases of annular pancreas through a laparoseopic approach, but a duodenojejunostomy was converted to mini-laparotomy during the laparoscopic course of a predu-odenal portal vein. The average operative time was 102 16.5 min (85～135 min). Visualization was ex-cellent, and there were no intraoperative complications. Feedings were started on postoperative day 3 to 5. All cases were on full feedings after 8 to 10 days. Follow-up upper gastrointestinal tests showed no evidence of stricture or obstruction. Conclusions The duodenoduodenostomy with laparoseopy can be performed in neonates securely and appropriated for a full-term newborn with tolerance CO2 pneumo-peritoneum. It provides an excellent and micro-invasive way to evaluate and treat congenital duodenal obstruction.     

腹腔镜下十二指肠吻合术治疗新生儿十二指肠梗阻

Objective To evaluate the feasibility and indication of laparoscopie duodenoduoden-ostorny for neonates with congenital duodenal obstruction- Methods From May 2004 to Feburary 2008,6 newborns with duodenal obstruction underwent exploratory laparoscopy. With a lower-pressure pneumoperitoneum of 5～8 mmHg and a suspending suture for right liver elevator, the procedure was performed using 3 cannulas of 3.3 mm to 5.5 mm diameter. Under the laparoscopic vision, the cause of duodenal obstruction was diagnosed and a sutured anastomosis was performed after the duodenum mo-bilized. Results Findings at laparoscopy included duodenal diaphragm in 3 cases,annular pancreas in 2 cases, and preduodenal portal vein in 1 case. Three cases with duodenal diaphragmatic stenosis were en-countered a partial excision of the diaphragm after vertical incision of the anterior part of duodenum followed laparoscopically by a transverse suture. A diamond-shaped side-to-side duodenoduodenal anas-tomosis was successfully carried out in 2 cases of annular pancreas through a laparoseopic approach, but a duodenojejunostomy was converted to mini-laparotomy during the laparoscopic course of a predu-odenal portal vein. The average operative time was 102 16.5 min (85～135 min). Visualization was ex-cellent, and there were no intraoperative complications. Feedings were started on postoperative day 3 to 5. All cases were on full feedings after 8 to 10 days. Follow-up upper gastrointestinal tests showed no evidence of stricture or obstruction. Conclusions The duodenoduodenostomy with laparoseopy can be performed in neonates securely and appropriated for a full-term newborn with tolerance CO2 pneumo-peritoneum. It provides an excellent and micro-invasive way to evaluate and treat congenital duodenal obstruction.