先天性心脏病术后漏斗胸行Nuss手术28例

目的 探讨Nuss手术治疗先天性心脏病(CHD)术后漏斗胸的可行性和疗效.方法 回顾分析Nuss手术治疗CHD术后漏斗胸28例的病例资料.观察其手术时间、住院时间、手术效果、手术前后心功能指标及术后并发症.结果 CHD术后漏斗胸28例患儿行Nuss手术均顺利完成.手术时间55～75 min,平均(61.1±7.6)min;住院时间7～13 d,平均(9.2±1.5)d.术中出现心包积血、心脏压塞1例及时予以B超下心包穿刺同时输注冷沉淀控制了病情(因合并凝血功能障碍);气胸2例,准备出院复查时发现,无明显症状观察1周出院,1个月后复诊完全吸收.术后心脏射血分数改善显著由平均(58.4±2.3)分上升为(61.2±3.6)分,差异有统计学意义(P＜0.05).手术效果评价优良率100％.结论 Nuss手术治疗CHD术后漏斗胸安全可行,疗效满意.     

腔镜下腋、颈部入路胸锁乳突肌松解术矫正先天性肌性斜颈

Objective A transaxillary and transcervical endoscopy technique was developed for the release of the sternocleidomastoid muscle in congenital muscular torticollis. The efficacy of the op-eration was evaluated. Methods A total of 34 children with congenital torticollis were treated. The age ranged from 6 month to 14 years. A 5 nun trocar was inserted in the anterior axillary fold. A subcuta-neous tunnel perpendicular to the axis of the sternocleidomastoid was made, initially under direct vision and then endoscopically using a 5 mm endoscope. A subcutaneous space was established with CO2 in-flation at the pressure of 6 mm Hg. Another two 1.5～2 mm incisions were made besides sternocleido-mastoid for the introduction of mini forceps and electric knife, respectively. The sternocleidomastoid muscle and scalene were dissected out and divided with electrocautery. Lee's evaluation was carried out post-operatively. Results The operation was successfully completed under endoscope in all children. The mean operation time was 50 min ( range: 40～120 min). No injury of major nerve was encoun-tercel Haemorrage in one child required re-operation. Follow-up for 6 months to 3 years showed satis-factory cosmetic appearance and no recurrence. The results were classified as excellent in 88.2 %, good in 8.8%,and fair in 3.0%. The neck scars were not visible one month after surgery. Conclusions Transaxillary and transcervical endoscopic release of congenital torticollis is a safe and relatively quick procedure. It provides good access and good cosmetic outcomes. It avoids injury to neurovascular struc-tures.     

Notch配体、受体在小儿胆道畸形肝组织中的表达及意义

Objective To investigate the expressions of Notch ligands and its receptors in the liver tissues of the pediatric patients with bile duct malformations. Methods Twenty three patients including 12 patients with biliary atresia and 11 with choledochal cyst were enrolled in this study. The patients' liver specimens were harvested during surgery. Immunohistochemistry and real-time fluorescent quantitative RT-PCR were performed to examine the expression of Notch ligands and its receptors in the liver tissues. Nine health liver tissues served as controls. The clinical data of these patients were also collected and analyzed. Results The expression of Jag1 significantly increased in the proliferating ductules of the patients with biliary atresia. Jag2 was negative in the portal area of all patients. The mRNA of Jag1 of the patients with bile duct malformations was higher than that of controls (P＜0. 01). No difference of the mRNA of Jag2 was found between the bile duct malformation patients and the controls (P＞0. 05). The expression of Notch 1 and Notch 2 was mostly found in hepatocytes and bile ductules of the controls' and the choledochal cyst patients' liver tissues. No Notch 1 and Notch 2 expression was found in proliferating ductules of the biliary atresia patients. Notch 3 was expressed in the neovascularization and mesenchyme of the biliary atresia patients. There was no significant difference of Notch1 and Notch 2 mRNA between the biliary atresia patients, choledochal cyst patients and controls (P＞0. 05). Notch 3 mRNA of biliary atresia patients was higher than that of the controls (P＜0. 01). Notch 4 expression was undetectable in all patients. Conclusions The expression patterns of Notch ligands and its receptors are changed in the patients with biliary atresia, which may contribute to the pathogenesis of biliary atresia.     

腹腔镜辅助结肠次全切除术治疗先天性肠神经元发育不良症的对比研究

目的：对比研究先天性肠神经元发育不良症B型患儿行腹腔镜下结肠次全切除术、Soave术和Duhamel术的临床疗效。方法回顾性分析2005年3月至2013年3月接受腹腔镜结肠次全切除的B型肠神经元发育不良患儿的随访资料。其中接受腹腔镜辅助下Duhamel术（直肠后拖出术）28例,同期接受腹腔镜辅助下Soave术（直肠内拖出术）34例。入选的62例患儿诊断依据为临床表现、术前影像学结果、直肠黏膜活检、术中快检和术后病检报告。分别比较两组手术时间、术中出血量、术后住院时间、排便频率、术后并发症及排便功能方面有无差异。结果腹腔镜Duhamel术和腹腔镜Soave术在手术时间、术中出血量、术后住院时间方面比较,均无统计学意义（P＞0.05）。排便频率比较：腹腔镜下Duhamel术患儿术后2周内排便频率平均8次/d,明显少于腹腔镜下Soave术的15次（P＜0.05）;恢复到正常排便频率（1～2次/d）时间比较：腹腔镜下Duhamel术平均为（3.5±0.5）个月,腹腔镜下Soave术平均为（6.7±0.8）个月,差异有统计学意义（P＜0.05）。腹腔镜下Duhamel术患儿术后发生小肠结肠炎1例（3.6％）,污粪1例（3.6％）,粪石1例（3.6％）;腹腔镜下Soave术后发生小肠结肠炎4例（11.8％）,吻合口狭窄1例（2.9％）,便秘复发2例（5.8％）,污粪1例（2.9％）。肛周湿疹的发生率：Soave术组明显高于Duhamel术组（41.2％VS 7.2％）。58例术后获功能随访,平均随访时间为4年6个月（2个月至8年）。接受腹腔镜下Duhamel术的患儿其排便功能优良率为85.2％,腹腔镜下Soave术为87.0％。两组差异无统计学意义（P＞0.05）。结论行结肠次全切除的B型肠神经元发育不良患儿,腹腔镜下Duhamel术后小肠结肠炎的发生率低于Soave术式,腹腔镜下Duhamel术后近期排便频率少,且排便频率更快恢复正常,肛周湿疹发生率低。     

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.     

对称性四足坐骨联体儿分离一例

联体A和联体B:出生日期2009年6月19日,产后诊断联体双胎.足月剖宫产,入院时体重5.0kg,呈对称性四足坐骨联体畸形,脐部及耻骨联合相连,背部两婴儿于骶尾部以软组织相连,形成体桥;背部体桥中间,见会阴为女性外观,呈泄殖腔畸形,两婴儿大小便均由该处排出.两婴染色体核型分析均为46,XX.两婴儿均有各自上下肢,可活动.     

腹腔镜下结肠切除、直肠内拖出术治疗巨结肠类缘病

目的探讨腹腔镜下结肠部分或次全切除、直肠内拖出术治疗巨结肠类缘病的可行性。方法1999年10月-2006年5月，对保守治疗无效或复发的38例先天性巨结肠类缘病（Hirschsprung’s disease-allied disorder，HAD）和先天性巨结肠（Hirschsprung’s disease，HD）合并HAD的患儿在腹腔镜下行结肠部分或次全结肠切除、直肠内拖出术。分别在脐缘、右上、右下、左中腹刺入4个5mm trocar，行结肠次全切除时，左侧需建立2个操作孔，分别在左上及左中下腹。腹腔镜下分别游离降结肠、横结肠、升结肠及回盲部侧腹膜，肛门手术按改良Soave方法。结果38例均在腹腔镜下完成手术。9例行左半结肠切除，手术时问110—180min，平均135min；29例行结肠次全切除并按Deloyers法将升结肠逆时针转位270。下拖，保留的升结肠长度7—13cm，平均11．5cm，手术时间140—220min，平均175min。术中出血15～70ml，平均35ml。病理诊断肠神经元性发育异常10例，神经节细胞减少症3例，神经节细胞未成熟症4例，未分类型9例，HD合并IND6例，HD合并HG2例，HD合并IGC4例。38例术后随访6个月一7年，平均3年5个月，29例次全结肠切除患儿3个月内每日大便4—18次，6—10个月后渐转为每日2～3次，9例左半结肠切除患儿术后6个月后每13大便1—2次，无吻合口狭窄，无便秘复发。结论腹腔镜下结肠部分或次全切除经肛门拖出根治术治疗HAD安全、有效、可行，手术创伤较小，但需要一定的腹腔镜操作经验。     

关于对1999及2004年相隔5年淄博市病残儿变化趋势的分析

目的了解淄博市1999年和2004年相隔5年后病残儿的变化趋势,为有效降低该地区出生缺陷发生率,制定干预方案.结果相隔5年系统发病率前3位顺序无变化,但发病率第3位的心血管系统疾病明显减少.遗传性疾病的人数所占比例明显减少.结论淄博市遗传病的发病率较非遗传病有下降趋势.     

小儿纵隔、颈纵隔型淋巴管瘤19例临床分析

目的 探讨小儿纵隔、颈纵隔型淋巴管瘤的诊断和治疗。方法 分析19例纵隔及颈纵隔型淋巴管瘤的临床资料，其中颈纵隔型14例，瘤体均深入锁骨或胸骨到达纵隔内，纵隔型5例，瘤体位于前上或后纵隔内。所有病例均由病理证实。结果 14例颈纵隔型中，2例系先行颈部病灶切除，术后发现纵隔病灶而再次经胸手术；1例先经胸行囊肿切除，术后再次行颈部囊肿切除；10例经颈胸联合切口手术；1例放弃手术。5例纵隔型均侧胸人路手术。完整切除3例，其他病例行囊肿揭顶引流，部分切除，残留囊壁石炭酸烧灼。术中发现左肺发育不良1例，术中损伤无名静脉1例，术后复发3例，无死亡病例。结论 颈纵隔部位的肿瘤一经诊断应立即手术，切口的选择应遵循充分暴露、避免损伤的原则；术中不宜强求完整切除，残留囊腔敞向胸膜腔可减少术后复发。     

腔镜下腋、颈部入路胸锁乳突肌松解术矫正先天性肌性斜颈

Objective A transaxillary and transcervical endoscopy technique was developed for the release of the sternocleidomastoid muscle in congenital muscular torticollis. The efficacy of the op-eration was evaluated. Methods A total of 34 children with congenital torticollis were treated. The age ranged from 6 month to 14 years. A 5 nun trocar was inserted in the anterior axillary fold. A subcuta-neous tunnel perpendicular to the axis of the sternocleidomastoid was made, initially under direct vision and then endoscopically using a 5 mm endoscope. A subcutaneous space was established with CO2 in-flation at the pressure of 6 mm Hg. Another two 1.5～2 mm incisions were made besides sternocleido-mastoid for the introduction of mini forceps and electric knife, respectively. The sternocleidomastoid muscle and scalene were dissected out and divided with electrocautery. Lee's evaluation was carried out post-operatively. Results The operation was successfully completed under endoscope in all children. The mean operation time was 50 min ( range: 40～120 min). No injury of major nerve was encoun-tercel Haemorrage in one child required re-operation. Follow-up for 6 months to 3 years showed satis-factory cosmetic appearance and no recurrence. The results were classified as excellent in 88.2 %, good in 8.8%,and fair in 3.0%. The neck scars were not visible one month after surgery. Conclusions Transaxillary and transcervical endoscopic release of congenital torticollis is a safe and relatively quick procedure. It provides good access and good cosmetic outcomes. It avoids injury to neurovascular struc-tures.