经瘘管一期肛门成形术治疗先天性无肛并直肠前庭瘘45例

直肠前庭瘘（RVF）是肛门直肠畸形（ARM）的最常见类型,矢状入路肛门成形术（ASARP）和后矢状入路肛门成形术（PSARP）是常用术式。本院采用经瘘管肛门成形术（TFARP）治疗45例,现报告如下。     

直肠内瘘修补术在小儿肛门直肠疾病中的应用

目的 探讨直肠内瘘修补术在小儿肛门直肠疾病中的应用及疗效。方法 对近十年间我院采用直肠内瘘修补术治疗196例各类小儿肛门直肠疾病进行回顾性分析。结果 196例患儿中后天性肛前瘘136例,无肛并直肠舟状窝瘘42例,无肛直肠尿道瘘4例,无肛直肠阴道瘘4例,复发性后天性肛前瘘3例,无肛术后损伤性直肠尿道或阴道瘘4例,先天性无肛舟状窝瘘术后复发3例,一次性手术总治愈率95．4％(187／196),术后瘘复发率4．6％(9／196)。结论 直肠内瘘修补术简便易行,损伤小,可用于治疗多种小儿肛门直肠疾病。     

瘘管切除直肠粘膜下拖覆盖瘘内口治疗小儿单纯性肛瘘

目的 探讨和开展一种新型Ⅰ期根治小儿单纯性肛瘘的手术方式。方法 采取Ⅰ期瘘管完整切除、直肠粘膜拖下覆盖已缝合修补的瘘内口的手术方法，对23例小儿单纯性肛瘘实施根治，并对其随诊获得的临床资料进行回顾性分析。结果 23例患儿中22例均获得临床良好愈合，随诊2～6年无复发迹象，排便功能良好；1例因术后严重腹泻而复发。结论 瘘管切除、直肠粘膜拖下覆盖瘘内口的术式，手术创伤小，手术操作简单易行、手术成功率相对高，是治疗小儿肛瘘的一种新术式，值得临床推广应用。     

脱肛注射治疗并发肛直肠会阴瘘24例报告

目的对儿童脱肛注射治疗后并发肛直肠会阴瘘的情况进行总结报告，探讨脱肛注射治疗的术前术后处理及并发症的预防措施。方法回顾性总结分析1987年6月-2004年8月收治的因脱肛注射治疗后并发肛直肠会阴瘘24例病例资料。结果并发肛瘘20例，直肠会阴瘘3例，行相应治疗后预后良好，并发直肠坏死1例，经治疗术后3月伤口仍未愈。结论严格掌握适应证，注意术中操作，可减少并发症发生，出现并发症采取对应治疗，一般能治愈。     

经直肠前手术治疗女童后天性直肠前庭瘘

目的 探讨经直肠前修补女童后天性直肠前庭瘘的手术措施.方法 本院1997年1月-2007年12月经直肠前修补后天性直肠前庭瘘患儿63例.手术年龄1～12岁.术前3 d温水坐浴,口服药物及清洁灌肠等准备.切开肛门前半周或1/3周游离直肠之瘘管,手术修补瘘管,术后随诊0.5～2.0 a.结果 痊愈60例,治愈率达95.2%.3例瘘管复发,其中2例经硼酸液坐浴控制感染后自愈,1例经2次手术治愈.结论 经直肠前修补女童后天性直肠前庭瘘手术简单、可靠,是一种切实可行的方法.     

经会阴手术治疗小儿直肠前庭瘘26例

目的探讨经会阴途径治疗小儿直肠前庭瘘的手术方法和疗效。方法对26例直肠前庭瘘患儿采取经会阴切除瘘管,重建会阴体的方式治疗。结果24例均一期愈合,1例术后3d发生伤口裂开,1例出现伤口感染,经保守治疗2周痊愈。24例术后随访2年,无复发及大便失禁等并发症。结论经会阴瘘管切除术具有手术视野好、手术彻底、复发率低等优点,是治疗直肠前庭瘘较为理想的术式。     

一期会阴肛门成形及瘘修补术治疗中位先天性无肛并直肠尿道球部瘘

目的 总结2005年1月至2010年2月采用一期会阴肛门成形并瘘修补术治疗中位先天性无肛并直肠尿道球部瘘的临床疗效.方法 应用一期会阴肛门成形并瘘修补术治疗中位先天性无肛并直肠尿道球部瘘患儿9例.结果 所有患儿术后恢复良好,痊愈出院,均获得随访,2例瘘复发,术后无尿道狭窄,肛门控制功能优良.结论 该术式简便易行,疗效满意,可选择应用于中位先天性无肛并直肠尿道球部瘘的病例.     

先天性巨结肠根治术后医源性直肠阴道瘘的再手术治疗CSCD

Objective To explore the causes of iatrogenic rectovaginal fistula after pull-through in Hirschsprung’s disease (HD) and summarize the experiences and efficacies of reoperation. Methods From December 2007 to December 2020 , retrospective review was conducted for clinical data of 6 HD children with iatrogenic rectovaginal fistula after pull-through. The surgical procedures for repairing rectovaginal fistula included transabdominal and transanal Soave , transperineal or transanal surgery. Results A total of six girls were included. Clinical manifestation was vaginal excretion. Injuries occurred in initial surgery (n =4) and during redo pull-through (n =2). The reasons for re-operation were postoperative abdominal hemorrhage and aganglionic segment residue. Rectovaginal fistula with anastomotic retraction and stenosis were successfully repaired by transabdominal and transanal Soave procedure in single time(n =4). The remaining two cases underwent simple local repair of rectovaginal fistula , including fistula closure after anal repair twice (n =1) and failed closure after five local (perineal/anal) repairs (n =1). Conclusion Rectovaginal fistula after pull-through in HD is a serious iatrogenic injury. It should be separated close to rectal submucosa/rectal wall to avoid vaginal injury. Transabdominal and transanal Soave procedure has a high success rate for repairing rectovaginal fistula and managing anastomotic retraction and stenosis. © 2022, Journal of Clinical Pediatric Surgery. All rights reserved.     

腹腔镜直肠代阴道肛门成形术治疗先天性肛门闭锁直肠前庭瘘并苗勒管发育不全综合征二例

肛门直肠畸形的发生率约为1/3500,其中先天性肛门闭锁前庭瘘是女性肛门直肠畸形中常见的类型。阴道及子宫不发育或发育不良即苗勒管发育不全综合征(Mayer-Rokitanski-Küster-Hauser,MRKH)通常表现为原发性无月经,在出生女婴中发病率约为1/4000~5000。Levitt等[1]报道在肛门直肠畸形前庭瘘的患儿中阴道畸形的发生率为0.6%。同时报道在子宫阴道发育异常的患儿中直肠前庭瘘的发生率为9.5%[2]。Wang等[3]总结病例发现在MRKH中直肠肛门闭锁前庭瘘的发病率为2.25%。我科近年收治了2例先天性肛门闭锁前庭瘘合并MRKH患儿,均一期行肛门成形,直肠前庭瘘代阴道成形术,现报告如下。     

先天性巨结肠根治术后医源性直肠阴道瘘的再手术治疗

目的分析先天性巨结肠根治术后医源性直肠阴道瘘的发生原因, 探讨再次手术治疗的方法及效果。方法回顾性分析2007年12月至2020年12月首都医科大学附属北京儿童医院普外科收治的6例先天性巨结肠根治术后医源性直肠阴道瘘患儿临床资料。再次手术治疗的方式包括经腹经肛Soave术、经会阴或经肛门修补手术。分析发生直肠阴道瘘的原因, 总结手术经验, 随访预后情况。结果 6例患儿临床表现均为自阴道内漏出粪便, 其中4例出现在初次手术后, 2例出现在再次手术后;行再次手术的原因分别为手术后腹腔出血、存在肠无神经节细胞段残留。4例合并吻合口回缩、狭窄, 采用经腹经肛Soave术治疗直肠阴道瘘及吻合口回缩、狭窄, 均经单次修复获成功;2例仅单纯局部修补直肠阴道瘘, 其中1例经肛门修补2次后瘘管闭合, 1例经会阴/经肛门局部修补5次后瘘管仍未闭合。结论先天性巨结肠根治术后直肠阴道瘘是一种较为严重的医源性损伤, 在进行先天性巨结肠拖出手术时应紧贴直肠黏膜下层或直肠壁分离, 以避免损伤阴道。经腹经肛Soave手术修复直肠阴道瘘成功率高, 可同时处理吻合口回缩、狭窄。     

Carcinoma of the rectum in a pre-teen girl

Inoperable carcinoma of the rectum was diagnosed in a 12-year-old girl during work-up for dysentery and fever. Clinical details, investigations, and the course of the disease are described. The literature on rectal carcinoma in childhood is reviewed. Offprint requests to: N. V. Doraiswamy at the above address     

小儿气管食管瘘手术治疗分析

目的：对小儿气管食管瘘病例的诊断、治疗和愈后进行回顾和总结。方法对本院近5年来收治的10例气管食管瘘患儿的手术治疗过程及疗效进行回顾性分析。10例均接受手术治疗11次,对气管瘘口进行修补,对食管明显狭窄者切除病变食管,行端端吻合或结肠代食管。结果10例均获治愈,术后无一例死亡,门诊随访时间3～15个月,仅2例出现食管狭窄需多次扩张,其余均正常。结论小儿气管食管瘘的常见原因为化学腐蚀性和医源性,常伴发呼吸道症状,病情较为严重,积极有效的手术治疗能改善患儿预后,提高存活率。     

腹腔镜手术治疗先天性无肛并直肠尿道瘘15例

目的探讨腹腔镜辅助下瘘修补＋直肠拖出肛门成形术治疗先天性无肛并直肠尿道瘘的可行性。方法在腹腔镜监视下,电灼游离直肠至瘘管颈部,缝扎后离断直肠尿道瘘管,电刺激仪引导下定位盆底肌中心,将直肠从盆底肌中心拖出形成肛门。结果15例均在腹腔镜辅助下完成肛门成形术,腹腔镜操作时间（108±16）min,出血量〈5mL,无需要输血者。术后（7—10）d拔除尿管,术后住院时间6～15d,平均（11．6±0．4）d。术后随访3—22个月,根据kelly评分及直肠肛管测压,肛门功能优9例,良6例。结论腹腔镜辅助肛门成形术,能准确辨别盆底肌群中心,避免损伤肌肉,创伤小,对于直肠膀胱颈部瘘及尿道前列腺部瘘而言,处理瘘管方便,但对尿道球部瘘显露较困难。     

Isolated tracheoesophageal fistula in a 10-year-old girl

Isolated tracheoesophageal fistula (H-TOF) is a rare type of tracheoesophageal anomaly and is in most cases diagnosed in the neonatal period because of choking and cyanosis during feeding. Diagnosis may be delayed even until adulthood because of nonspecific and sometimes intermittent symptoms, and because false-negative results of all diagnostic tools are not uncommon. We report a 10-year-old child with H-TOF, whose symptoms had nearly disappeared completely between the ages of 4 and 10 years. Diagnosis was only possible after the recurrence of the symptoms at the time of an episode of bronchitis, profound interrogation of the child’s medical history, and questioning of the results of a former diagnostic work-up. In this article, we discuss the potential pitfalls in both clinical diagnosis and diagnostic work-up.     

Surgical management of hepatic arterioportal fistula in a neonate

Congenital arterioportal fistulae in the liver are rare malformations which can lead to portal hypertension. We report a hepatic arterioportal fistula in a neonate who presented with intestinal hypoperfusion. Computerised tomography angiography showed a fistulous communication between the left hepatic artery and portal vein with hypoperfusion of small and large bowel. A formal left hepatectomy was done followed by clinical improvement and reduction in portal venous pressures. The case and the literature pertaining to it are discussed.     

Surgical ligation of patent ductus arteriosus on the intensive care unit in small premature infants

This paper summarizes the experience with ligation of persistent ductus arteriosus in prematures performed in the intensive care unit during the years 1986 to 1988. Records of 35 operated prematures on artificial ventilation were analyzed retrospectively. Management and organization of the operation are described. For comparison, records were analysed of 47 prematures, also artificially ventilated, who during the years 1978 to 1986 had to be transported to an operating theatre over a distance of 3 or 10 km for the ligation.     

Surgical therapy of a coronary artery fistula draining into the left atrium

Coronary artery fistulas (CAFs) are rare vascular anomalies. We describe a case of CAF draining into the left atrium, combined with an atrial septum defect (ASD) and azygos continuity in a 3-year-old girl. Surgical therapy included closure of the ASD combined with closure of the CAF through a left atrial approach. Even though CAF can be treated by interventional means, surgical correction may be required in the presence of associated cardiac abnormalities.     

Is ligation of azygos vein necessary in primary repair of tracheoesophageal fistula with esophageal atresia?

INTRODUCTION: Congenital tracheoesophageal fistula with esophageal atresia (TEF with EA) is not an uncommon disease of newborns. Classical approach for primary repair of TEF with EA is right thoracotomy with extrapleural approach, ligation of the azygos vein, identification and ligation of tracheoesophageal fistula, identification of upper esophageal pouch and end-to-end anastomosis. This study was conducted to evaluate if the ligation of the azygos vein is a must during primary repair of TEF with EA. METHOD: We studied 50 randomly selected cases in the last two years (Jan 2003 - Jan 2005). In 25 babies (group A), primary repair was done with preservation of the azygous vein while 25 babies (group B) were operated with a classical approach with ligation and division of the azygos vein. The postoperative result was comparative in both cases. RESULT: Postoperative pneumonitis was higher in the babies operated with the classical approach (56 % in group B and 12 % in group A) and was found to be statistically significant. In postoperative complication, anastomotic leak was slightly higher in group B but was not statistically significant. Mortality rate was similar in both groups. Babies in group A were hemodynamically more stable in comparison to group B. CONCLUSION: Preservation of the azygos vein maintains the normal venous drainage of mediastinum and hence decreases the postoperative chest congestion and pneumonitis in the postoperative period in cases of congenital esophageal atresia with tracheoesophageal fistula, so it should be preserved whenever possible.     

Conservative treatment of massive hemothorax in a girl with neuroblastoma

We report the case of a 1‐year‐old girl with stage 4 neuroblastoma who developed massive hemothorax due to tumor invasion before treatment. She presented with tachypnea, worsening anemia, and oxygen desaturation. Hemothorax was diagnosed based on chest radiography, ultrasonography, and diagnostic thoracic puncture results. High neuron‐specific enolase, vanillylmandelic acid, and homovanillic acid as well as computed tomography strongly supported a diagnosis of neuroblastoma. Chemotherapy along with intermittent puncture drainage, oxygen, and blood transfusion reduced the accumulated blood, and hemothorax disappeared within 1 week. Thus, it is possible to avoid invasive treatment for massive hemothorax by initiating chemotherapy for chemosensitive solid tumors, including neuroblastoma.