先天性巨结肠根治术后小肠结肠炎188例分析

目的 探讨先天性巨结肠根治术后小肠结肠炎发生的相关性,分析术后小肠结肠炎的高危因素.方法 收集2009 ～2012年先天性巨结肠及同源病根治手术治疗病例188例,根据病理诊断与手术切除范围不同分组,对比各组间术后小肠结肠炎发生率,分析术后小肠结肠炎发生的相关性.结果 先天性巨结肠与巨结肠同源病在术前小肠结肠炎发病率无明显差异（P＞0.05）,术后巨结肠同源病发生小肠结肠炎的几率明显高于先天性巨结肠（P＜0.05）;术前发生小肠结肠炎与术后发生小肠结肠炎明显相关（P＜0.05）;术中结肠切除范围和术后小肠结肠炎之间无明显相关（P＞0.05）;Soave与心形吻合方式对于术后小肠结肠炎的发生无明显相关（P＞0.05）;全结肠型巨结肠更易发小肠结肠炎.结论 术前小肠结肠炎、巨结肠同源病及全结肠型巨结肠仍然是影响术后小肠结肠炎发生的重要因素.     

经肛门巨结肠根治术后并发症及手术方式的改进

目的 减少经肛门Ⅰ期巨结肠根治术后的并发症。方法对27例先天性巨结肠经肛门Ⅰ期根治进行回顾分析，其中15例逐层梯度分离切出粘膜、环肌、纵肌，进入盆腔拖出肛门根治，长段型5例，普通型8例，短段型2例。12例单纯粘膜剥脱拖出(soave)肛门根治，长段型2例，普通型7例，短段型3例。结果出现并发症5例，其中单纯粘膜剥脱发生肌鞘感染l例，鞘内结肠假性梗阻1例，便秘2例；粘膜、肌层逐层梯度分离切除发生便秘1例；本组病例未发生小肠结肠炎。结论根据巨结肠类型，选择不同手术方式，粘膜、环肌、纵肌逐层梯度分离切除巨结肠根治术适应长段型和普通型病例，单纯粘膜剥脱巨结肠根治术适应短段型和超短型病例。     

先天性巨结肠术后发生小肠结肠炎的高危因素

目的：分析先天性巨结肠术后发生小肠结肠炎的高危因素。方法：1991年1月－1999年6月行先天性巨结肠根治手术155例,获随访141例,进行10个相关因素调查,包括非手术因素和手术因素。结果：34例发生不同程度小肠结肠炎（24％）,吻合口狭窄或瘘和肠梗阻是术后发生小肠结肠炎的高危因素,术前患小肠结肠炎是术后发生小肠结肠炎的另一危险因素。沁肠结肠炎患儿上呼吸道或肺部感染增加一倍。结论：术后小肠结肠炎是肠道梗阻和肠粘膜抵抗力降低共同作用的结果,避免和解除梗阻因素的同时改善患儿肠道粘膜功能是降低该并发症发生并使其疗效有显著性改善的新途径。     

先天性巨结肠术后小肠结肠炎的防治

目的探讨先大性巨结肠术后小肠结肠炎的预防和治疗。方法在应用各方血综合治疗基础上着重加强提高患儿免疫力方面的治疗。结果62例先大性巨结肠根治术后，发生小肠结肠炎的14例患儿均获治愈。结论住综合治疗基础上着重加强提高患儿免疫力方面的治疗，可减少先大性巨结肠术后小肠结肠炎的发病率及病死率.     

经肛门Ⅰ期结肠拖出术治疗新生儿及婴幼儿先天性巨结肠

目的 介绍经肛门Ⅰ期结肠拖出术治疗新生儿及婴幼儿先天性巨结肠的初步经验。方法 患儿全麻后取截石位，扩肛后，牵开肛门。在直肠齿状线上0．5cm处游离直肠粘膜，形成粘膜管后，继续向近端游离，达腹膜返折后环形切断肌鞘一周，劈开肌鞘后壁，并切除部分肌条。进入腹腔后，直视下游离直肠、结肠的移行段和扩张段，妥善结扎系膜血管，达正常结肠后切断，拖出结肠浆肌层与保留肌鞘间断固定，结肠断端及直肠齿状线切缘缝合固定。结果 7例均顺利完成手术，手术时间90min-100min，出血约15ml～30ml，术中均未输血。7例均经病理证实为先天性巨结肠症，平均切除肠管13cm～25cm。1例于术后6d出现小肠结肠炎，治疗后好转，其余6例恢复顺利。术后2周开始扩肛，全部病例随访2个月—1年，每日排大便2～4次，无便秘复发、腹胀，无吻合口狭窄，无污粪，无肛周感染、肠管同缩等并发症。结论 经肛门Ⅰ期结肠拖出术治疗先天性巨结肠，具有手术创伤小、操作简单、术后恢复快、并发症少等优点，适用于新生儿及婴幼儿短段型及普通型先天性巨结肠的治疗。     

结肠造口术后单切口腹腔镜辅助Duhamel巨结肠根治术

先天性巨结肠症(Hirschsprung's discase,HD)是以结肠壁内神经节细胞缺如为特征的消化道发育畸形.对诊断明确的常见型HD可行Ⅰ期拖出根治术;但对于长段型HD因直肠灌洗效果不佳或并发小肠结肠炎需要采取分期手术,即在新生儿期先进行结肠造口缓解症状,待营养状况好转后再Ⅱ期根治手术.随着微创外科技术的进步和单切口腹腔镜手术的兴起,我们在常规腹腔镜巨结肠根治术的基础上,针对结肠造口术后患儿,利用原造口部位完成单切口腹腔镜辅助下Duhamel术治疗3例长段型HD,取得良好效果,报告如下.     

经肛门Soave巨结肠根治术的随访及并发症防治

目的 观察经肛门Soave巨结肠根治术的术后并发症及手术初步评价。方法 采用经肛门Soave巨结肠根治术60例，术后进行随访，其中最长随访1年，观察其术后并发症及排便功能。结果 术后合并小肠结肠炎4例，单纯性腹胀4例，吻合口瘘1例。术后并发症为16．7％。结论 经肛门巨结肠根治术具有手术创伤小、时间短、术后恢复快、术后护理简单、无手术疤痕及术后并发症少等优点。     

肠造口术后单切口腹腔镜辅助巨结肠根治术（Duhamel 术）

先天性巨结肠症（Hirschsprung′s discase,HD）是以肠管远端神经节细胞缺如为特征的消化道发育畸形。1886年首先由丹麦儿科医生 Harald Hirschs-prung 在临床描述而得名。自1948年 Swenson 采用病变结直肠切除矫治本病以来,最初手术因易发吻合口瘘而分三个阶段完成,即先行结肠造口、然后经肛门拖出手术保留临时的“保护性”结肠造口、最后再完成结肠造口关闭术;经过60余年的不断改进,在 Swenson 原创拖出术的基础上进化许多不同术式。随着微创手术技术的兴起,对常见型和较长段型的 HD 可以顺利完成一期拖出术而不必进行结肠造口。然而,针对超过结肠脾曲的长段型或全结肠型 HD,国际小儿内镜外科组织指南仍建议采取两期手术。即新生儿期因顽固便秘难以诊断且洗肠困难、甚至并发小肠结肠炎或肠穿孔不得以先行肠造口挽救生命,待3～6个月一般情况纠正后,再经肛门一次拖出完成手术的同时消除最后需要的肠关瘘。笔者针对肠造口术后 HD,利用原造口部位完成单切口腹腔镜辅助下改良 Duhamel 术治疗长段型或全结肠型 HD 取得良好效果,现介绍如下。     

经脐腹腔镜结肠拖出术治疗先天性巨结肠症

目的 介绍经脐腹腔镜下拖出术治疗先天性巨结肠的手术方法以及临床手术经验.方法 回顾2009年6月至9月,对9例先天性巨结肠患儿采用经脐腹腔镜下拖出术进行治疗.患儿平均年龄为31.9个月(年龄范围在1～99个月),平均体重为16.2 kg(体重范围在4.7～25 kg).患儿脐窝处分别置入3个5 mm trocar,在腹腔镜镜头监控下,使用特制弯曲手柄型腹腔镜操作杆分离相应肠系膜及血管.扩肛,分离直肠肌肉与黏膜,然后将病变肠管呈袖套式拖出肛门外切除,行结肠肛门心形吻合术.记录术前各项检查以及手术相关数据.对手术患儿进行随访,记录术后并发症的发生情况.结果 平均手术时间为155 min(时间范围在110～185 min),平均失血量为40 ml(失血范围在10～100 ml).没有患儿术中转为传统腹腔镜手术或者开腹手术.无一例患儿术中出现腹部血管、肠管、输尿管、输精管损伤.患儿术后3 d进流食,术后7 d可出院.在随访期间,详细记录患儿术后并发症和恢复情况:1例患儿术后16 d出现小肠结肠炎,经过抗感染等对症治疗后好转;其余患儿均未出现术后并发症(吻合口狭窄、吻合口漏、便秘、污粪、大便失禁、腹泻、小肠结肠炎).术毕脐窝稍红肿,术后30d患儿复诊未见明显手术瘢痕.结论 经脐腹腔镜拖出术来治疗先天性巨结肠具有简单易行、美观的优点,适用于长段型巨结肠患儿.     

先天性巨结肠二次手术的原因分析及手术方法探讨

目的探讨先天性巨结肠根治术后二次手术的原因、手术指征及手术方式。方法回顾性分析自2008年1月至2013年12月在本院行先天性巨结肠根治术后二次手术的12例患儿临床资料。结果二次手术原因中,先天性巨结肠根治术后反复腹胀,便秘10例,肠梗阻、肠穿孔Ⅰ例,直肠膀胱瘘1例。在10例反复腹胀、便秘患儿中,二次手术证实无神经节细胞段残留或肠神经元发育异常8例,吻合口狭窄2例。二次手术时10例行Soave术,1例行Rehbein术,1例行Martin术。无一例死亡,12例均能自主排便,大便次数1~4次/d(平均2次/d)。1例出现污粪,2例出现小肠结肠炎,经保守治疗后好转。结论无神经节细胞段残留是巨结肠根治术后二次手术的主要原因,依据术前检查及术中冰冻检查结果准确判断病变肠管的范围十分重要。Soave术是二次手术的优先选择,且效果良好。     

升结肠贴补回肠直肠心形吻合术治疗全结肠无神经节细胞症

目的探讨采用升结肠贴补回肠直肠心形吻合术治疗全结肠无神经节细胞症的临床效果。方法分析自2006年6月至2013年6月本院收治的15例新生儿严重腹胀、低位小肠梗阻和肠穿孔病例资料,一期仅行急诊回肠末端造瘘加结肠多处活检,术后经病理检查明确诊断为全结肠无神经节细胞症;6-12个月后,行二期升结肠贴补回肠直肠心形吻合术;记录术后并发症的发生情况,并记录随访期间排便情况、肛门测压情况和机体内环境情况。结果所有手术均获成功,平均住院时间10．5 d,平均出血量30 mL,术后出现小肠结肠炎2例;无肠吻合口瘘、切口感染、肛门狭窄等,无死亡病例;术后随访1-2年,平均随访1．2年,术后早期肛周有红肿、糜烂,6个月后基本消失;术后大便次数约6-9次／d,2年后大便次数约2-3次／d;粪便由稀水样转为软粪;1例出现污粪,无大便失禁;随访血电解质、血红蛋白、白蛋白、球蛋白均正常,生长发育良好,接近正常儿;发现巨结肠根治术后直肠静息压、肛管静息压均较术前明显减低（P ＜0.05）;1例出现直肠肛门抑制反射。结论升结肠贴补回肠直肠心形吻合术既保留了吸收功能较好的右半结肠,符合结肠生理,同时行回肠直肠心形吻合,吻合口呈心形斜面,口径宽大且不在同一平面上,无狭窄、盲袋及闸门综合征的发生,可获较好排便功能,是根治全结肠无神经节细胞症的一个有效而可行的术式。     

An abnormal distribution of C-kit positive cells in the normoganglionic segment can predict a poor clinical outcome in patients with Hirschsprung's disease.

PURPOSE: The loss or decrease of interstitial cells of Cajal (ICCs) has been implicated in several disorders of human intestinal motility. We have encountered a few cases suffering from severe constipation or enterocolitis resulting in patient death after a definitive operation for HD, even though the normoganglionic intestine had been successfully pulled through. We investigated the distribution of ICCs using c-kit immunostaining in the normoganglionic segment and compared these findings with the clinical outcome after a definitive operation in each case. PATIENTS AND METHODS: The distributions of ICCs were investigated by using c-kit immunostaining in the normoganglionic segment in the resected bowel in 15 cases with HD. The distributions of protein gene product 9.5 (PGP 9.5) as a general neuronal marker and those of NADPH-diaphorase (NADPH-d) as a marker of nitric-oxide neurons were also examined. The numbers of ICCs and neurons were evaluated quantitatively. The histopathological results were compared with the clinical outcome after definitive operation in each case. RESULTS: C-kit immunoreactive cells showed a normal distribution in the normoganglionic segment in 13 cases, while they were markedly (less than 50% compared with the other cases) decreased in 2 cases. The distributions of PGP 9.5 and NADPH-d were almost the same in all cases. The bowel movements of 13 cases showing normal c-kit distribution were satisfactory. In contrast, the bowel movements were impaired in 2 cases with a decreased number of c-kit positive cells. One infant suffered from severe persistent constipation and thus had to undergo a resection of a dilated colon. The other infant died of sepsis due to postoperative enterocolitis and showed a markedly dilated colon. CONCLUSION: A decreased number of c-kit positive cells in the normoganglionic segment can thus allow us to predict a poor clinical outcome after definitive surgery, probably due to poor intestinal motility. Therefore examining the c-kit distribution in a resected bowel specimen in patients with HD should be mandatory in order to select the optimal postoperative treatment regimen for each case.     

Clinical risk factors of hirschsprung-associated enterocolitis. II: Postoperative enterocolitis

Among 302 Hirschsprung patients diagnosed between 1976 and 1993, 213 patients who had undergone definitive operations for Hirschsprung's disease (HD) were reviewed for the occurrence of postoperative Hirschsprung-associated enterocolitis (HAEC). The role of 42 parameters were analyzed. We detected 34 patients (14.0%) with postoperative HAEC with no mortality. In nine patients (26.5%) HAEC persisted postoperatively but no effect of preoperative HAEC on postoperative HAEC was detected. The length of the aganglionic segment did not have any effect on HAEC. Down's syndrome was not present in any of our patients with HAEC. Enterocolitis appeared after Swenson, Duhamel and Boley operations in 23, 10 and one patient, respectively. The incidence of HAEC was inversely proportional to age and weight at the time of definitive operation. The presence of colostomy before or during definitive operation had no effect on postoperative HAEC. Complications of definitive operations, including stricture, did not have any effect on HAEC.     

经肛门巨结肠根治手术方式的改进

目的 探讨改良经肛门巨结肠根治术的的临床疗效及安全性. 方法 回顾性分析本院自2006-2010年采用改良经肛门巨结肠根治术治疗的68例患儿临床资料. 结果 66例患儿顽固性便秘消失,营养改善,近期无吻合口漏,无腹腔及伤口感染,远期随访无便秘复发及直肠回缩,无吻合口狭窄、污裤及便失禁,2例再发便秘,病理检查确认为巨结肠类缘性疾病,3例有小肠结肠炎. 结论 改良经肛门先天性巨结肠根治术效果满意,术后并发症少,具有较高的临床实用性和安全性.     

Surgical treatment of necrotizing enterocolitis: when? how?

In 10 years (1981–1990) 28 out of 54 neonates (51.8%) with definite necrotizing enterocolitis (NEC) underwent surgery. Operation was performed at 13.5 ± 8.8 (range 3–38) days of life, after 1.7 ± 1.5 (range 1–6) days from the onset of symptoms. Aiming to perform laparotomy before the occurrence of perforation, surgery was liberally indicated in stage Ilia, according to Walsh-Kliegman. Explorative laparotomy (+peritoneal drainage in 2 cases) was performed in 4 patients with massive intestinal necrosis: all died within 3 days of surgery. In one neonate, only pneumatosis was present and resection was not considered mandatory. Intestinal resection and enterostomy was performed in 17 neonates, 5 of them with perforation; three developed an intestinal stenosis. Enterostomy was closed after 116.2 ± 61.8 days (range 26–193); 11 patients (64.7%) are long-term survivors. Intestinal resection and primary anastomosis was performed in 6 babies, 3 of them with perforation. Postoperatively, 2 dehiscences and 1 stenosis were recorded, but all children survived. In our opinion, resection followed by primary anastomosis seems to be the most satisfactory surgical option.     

改良Swenson术治疗先天性巨结肠

目的 探讨改良Swenson手术治疗先天性巨结肠的有效性、安全性。方法 对我院自1995年10月-2004年6月采用改良Swenson术治疗的52例（男50例，女2例，48例为常见型，4例为短段型）先天性巨结肠患儿进行回顾性分析。所有患儿均经病理明确诊断，术后常规肛门指检。随访40例、时间1～6年。对顽固性便秘、营养状况、吻合口狭窄、污裤及失禁等情况进行评价。结果 52例患儿顽固性便秘消失，营养改善。近期无吻合口漏，无腹腔及伤口感染，远期随访无便秘复发及直肠回缩，无吻合口狭窄、污裤及失禁。52例中仅2例有小肠结肠炎，占3．8％。结论改良Swenson术适用于常见型、短段型及长段型先天性巨结肠并且疗效满意，具有较高的实用性和安全性。     

Rectal involvement in necrotizing enterocolitis

Although necrotizing enterocolitis (NEC) is a common cause of neonatal intestinal disease, rectal involvement has not previously been reported. We describe five children with NEC involving the rectum. In four infants pancolorectal disease occurred; in the fifth the ascending colon was spared. A Swenson-type abdomino-perineal resection was performed in four cases. No definitive cause for the extension of the disease into the distal rectum was noted. There were no immediate postoperative complications. Long-term follow-up showed normal anorectal function and a normal-appearing perineum. Possible rectal involvement should be considered in all neonates with colonic NEC. Contrast studies visualizing the rectum are advised prior to closing colostomies performed in newborns with NEC. Ileorectal anastomosis after resection produces a satisfactory result. Offprint requests to: S. Z. Rubin     

Surgical strategies for necrotising enterocolitis: a survey of practice in the United Kingdom

BACKGROUND: Strategies for the surgical management of necrotising enterocolitis are various and controversial. OBJECTIVE: To characterise variation in surgical management of this disease across the United Kingdom. METHODS: Postal survey of 104 consultant paediatric surgeons with a 77% response rate. RESULTS: Duration of antibiotic treatment (median 10 days, range 6-14), time until the start of enteral feeding (median 10 days, range 4-21), and absolute indications for surgery all vary between surgeons. Peritoneal drainage is used by 95% of surgeons. Forty two percent use it in neonates of all weights, whereas 36% restrict its use to those <1000 g. Peritoneal drainage is used for stabilisation by 95% and as definitive treatment by 58%. At laparotomy, operative procedures include diverting jejunostomy, resection and stoma, resection with primary anastomosis, and "clip and drop". All procedures are used in infants of all weights except resection and primary anastomosis, which is used predominantly in larger infants (55% in <1000 g; 77% in >1000g; p=0.005). Infants may be considered too unwell for peritoneal drainage by 11% of surgeons compared with 90% for laparotomy (p<0.0001). CONCLUSIONS: There is considerable variation in surgical strategies for necrotising enterocolitis. Peritoneal drainage is used by most surgeons, with controversial indications and expectations. The use of resection and primary anastomosis is influenced by the weight of the neonate.