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目的探讨全消化道钡餐及钡滞留X线片在便秘、以肠神经元异常为代表疾病中的价值及临床意义。方法回顾性分析襄阳市中心医院2007年6月至2011年10月收治的36例疑为肠神经元异常性便秘病人的全消化道钡餐及钡滞留X线片资料。结果 30例为手术、手术后病检确诊为先天性巨结肠、巨结肠同源病及巨结肠并巨结肠同源病提供支持。6例未能诊断为肠神经元异常性疾病。结论全消化道钡餐及钡分时段滞留X线片,可为肠神经元异常便秘性疾病提供重要诊断参考,并具有胃肠传输功能与胃肠生理、病理形态兼顾优势。至少是对该类疾病诊断困难的一个补充。     

B型肠神经元发育异常症的诊断与手术治疗

目的：了解B型肠神经元发育异常症的诊断与手术治疗效果。方法：对45例该病患儿进行回顾性分析及远期随访。术前所有患儿均行钡灌肠X线检查，23例行下消化道动力学检测，17例行直肠粘膜活检加S100蛋白免疫组织化学染色。所有患儿均行病变肠段切除、结肠直肠吻合术。行多处全层活检手术标本。结果：本组中16例为单纯性B型肠神经元发育异常症，28例合并先天性巨结肠，1例合并肠神经节细胞减少。16例单纯性行钡灌肠X线检查时仅有4例可见确切的狭窄段，9例单纯性中有6例未出现直肠肛管抑制反射，17例活检中只有7例可获得提示性诊断。全层活检可准确诊断该病。术后3例发生小肠结肠炎患儿经保守治疗痊愈，1例发生闸门症候群而再次手术，其他患儿均能自解、自控大便。结论：全层活检是诊断该病的可靠方法。病变肠段切除、结肠直肠吻合术治疗本病可获得满意的疗效。     

全结肠一直肠型肠套叠诊治体会

目的：讨论全结肠一直型肠套叠的病因、诊断及治疗。方法：回顾7例全结肠一直肠型肠套叠病历资料。结果：7例中3例误诊。7例均手术治愈。结论：全结肠一直肠型肠套叠的病因及发病机理，除了其它类型肠套叠有相同的病因病机外，它还同时存在先天性肠旋转不良，其结肠系膜游离，未将结肠固定于后腹壁，使结肠活动度增大所致。诊断主要依据阵发性腹痛、呕吐、便血及腹部肿块，再结合B超、钡灌肠确诊不难。肛门指诊对本病有权重要的意义。发现套叠肠段由肛门突出，或指诊触及套叠肠段，或钡灌肠发现肠套位置较低，要考虑全结肠一直肠型肠套叠的可能。全结肠一直肠型肠套叠，套叠紧密，不易松懈复位，且多合并肠坏死，一旦确诊应手术治疗，可同时解决先天性肠旋转不良问题。非手术治疗无故。     

成年人先天性巨结肠同源病

先天性巨结肠同源病是在病变肠段内神经节细胞减少、未成熟、发育不全等。成年人先天性巨结肠同源病较少见。该病病史长，就医晚，多因巨结肠危象、肠梗阻、肠穿孔等急腹症就医。该病属于解剖性真性便秘，除了与假性便秘鉴别外，主要应与甲状腺功能低下的机能性巨结肠和特发性巨结肠相区别。该病多可手术治疗并可保留排便功能。本组提供5例报告，并予以讨论。     

全结肠-直肠型肠套叠诊治体会

目的:讨论全结肠-直型肠套叠的病因、诊断及治疗.方法:回顾7例全结肠-直肠型肠套叠病历资料.结果:7例中3例误诊.7例均手术治愈.结论:全结肠-直肠型肠套叠的病因及发病机理,除了其它类型肠套叠有相同的病因病机外,它还同时存在先天性肠旋转不良,其结肠系膜游离,未将结肠固定于后腹壁,使结肠活动度增大所致.诊断主要依据阵发性腹痛、呕吐、便血及腹部肿块,再结合B超、钡灌肠确诊不难.肛门指诊对本病有极重要的意义.发现套叠肠段由肛门突出,或指诊触及套叠肠段,或钡灌肠发现肠套位置较低,要考虑全结肠-直肠型肠套叠的可能.全结肠-直肠型肠套叠,套叠紧密,不易松懈复位,且多合并肠坏死,一旦确诊应手术治疗,可同时解决先天性肠旋转不良问题.非手术治疗无效.     

腹腔镜辅助下改良Swenson巨结肠根治术手术配合

先天性巨结肠是儿外科常见病。近年来 ,随着腹腔镜手术的发展 ,改变了以往的结肠造瘘、二期根治及造瘘的手术方式。 1 999年 1 1月至 2 0 0 1年 5月 ,我院行腹腔镜辅助下改良 Swenson巨结肠根治术(直肠肛管背侧纵切口鸡心领斜形吻合手术 ) 1 9例 ,取得良好效果。1　临床资料1 9例先天性巨结肠患儿 ,男 1 3例、女 6例 ,年龄 6～ 33个月。1 8例为普通型巨结肠 ,1例为长段型巨结肠。所有患儿经直肠粘膜活检、直肠肛管测压和钡剂灌肠确诊。腹腔内手术由腹腔镜系统及超声刀完成 ,会阴部手术采用改良 Swenson手术。手术时间为 ( 2 2 0± 5 0 ) mi…     

经脐单部位腹腔镜与传统开腹手术治疗巨结肠及同源病的临床疗效比较

目的比较经脐单部位腹腔镜(embryonic natural orifice transumbilical endoscopic surgery,ENOTES)与传统开腹手术行结肠次全切除术治疗长段型先天性巨结肠及巨结肠同源病的临床疗效。方法先天性巨结肠及巨结肠同源病患儿61例,其中长段型先天性巨结肠33例,巨结肠同源病28例。将上述61例患儿分为两组:经脐腹腔镜组及开腹手术组,比较两组患儿的手术时间、术中出血量、肠蠕动恢复时间及手术近、远期并发症,并作统计学分析。结果经脐单部位腹腔镜组较开腹组手术时间长[(24.60±44.50)min比(182.80±47.24)min,P0.05],经脐单部位腹腔镜组术后肠蠕动恢复更快[(21.32±2.55)h比(36.54±6.60)h,P0.05],两组术中出血量比较差异无统计学意义[(78.60±40.25)ml比(96.76±8.79)ml,P0.05],开腹组的术后近、远期并发症发生率均高于经脐腹腔镜组(P0.05)。结论经脐单部位腹腔镜较开腹次全结肠切除对患儿手术创伤小,术后肠蠕动恢复快,腹部伤口外形美观,术后并发症低。     

先天性巨结肠合伴肠神经分布异常（附4例报告）

目的：对先天性巨结肠合伴肠神经分布异常的现象行回顾性组织学观察。方法：１９９１年１月至１９９７年１２月１１１例先天性巨结肠根治术切除肠段标本，其远端病理证实为无神经节细胞症，拟对施出段即切除肠管近端亦作常规Ｈ．Ｅ染色病理学回顾性观察；且配合嗜银染色法及特异性神经元烯醇酶（ＮＳＥ）染色法。结果：１１１例先天性巨结肠中有４例合伴肠神经分布异常，其中３例合伴肠神经元性发育异常（ＩＮＤ），１例为神经节细胞     

成人巨结肠类缘病急诊手术14例

目的：探讨成人巨结肠类缘病急诊手术中的诊断及治疗方法。方法：14例成人巨结肠类缘病急诊手术患者,10例行术中快速冰冻病理检查,9例行病变肠段切除一期吻合、回肠造口术,4例行病变肠段切除、结肠造口术,1例行全结肠切除一期吻合术。结果：14例中12例术后恢复顺利,2例出现术后胃瘫。结论：成人巨结肠类缘病急诊手术行病变肠段切除一期吻合术是安全可行的,术中快速冰冻病理检查、肠管腹壁造口及术者经验对手术成功至关重要。     

巨结肠根治术后便秘复发的原因探讨

目的 探讨巨结肠根治术后便秘复发的原因,以提高手术效果.方法 总结1995年至2005年在我院行手术治疗的巨结肠病例392例,其中32例因便秘复发再次手术,分析复发病例的手术时间、方式、病理结果、肠炎发生情况及排便功能.结果 巨结肠根治术便秘复发率为4.5%,左半结肠切除术后复发率为16.0%(26/162),明显高于结肠次全切除术的复发率2.1%(4/189) (P<0.01).32例复发病例中,巨结肠同源病(HAD)及先天性巨结肠(HD)合并同源病共26例.复发的原因主要有近端HAD肠管切除不够或继发HAD改变,HD肠管切除不完全;远端切除不够,吻合口过高;吻合口狭窄、肛门狭窄及失弛缓等.复发病例的肠炎发生率明显高于无复发病例(P<0.05).结论 HAD较HD更易便秘复发,彻底切除近远端病变肠管,吻合口尽可能做到后壁距肛门缘1.5cm处,对小肠结肠炎早期诊断、早期治疗,术后置肛管以及术后复诊,一旦发现吻合口狭窄,及时扩肛等措施可降低巨结肠术后便秘的复发率.     

Evidence of secondary neuronal intestinal dysplasia in a rat model of chronic intestinal obstruction.

The etiology of neuronal intestinal dysplasia remains largely unknown. There is, however, supporting evidence of the existence of Hirschprung's disease or chronic intestinal obstruction associated with neuronal intestinal dysplasia. With the aim of investigating the possible development of neuronal intestinal dysplasia linked to chronic intestinal obstruction, we have examined the enteric nervous system response to long-term obstruction in a rat model. Three different surgical techniques were tested in Wistar male rats. In animals that survived longer than the cutoff chronic intestinal obstruction point (6 weeks), full-thickness biopsies and acetylcholinesterase (AChE), NADH, hematoxylin-eosin, and anti-S100 protein stainings were performed. The results of our model indicate that chronic intestinal obstruction induced different degrees of enteric nervous system dysplasia, including histological features of neuronal intestinal dysplasia. The relationship between chronic intestinal obstruction and anomalies of the enteric nervous system, including neuronal intestinal dysplasia, needs to be further studied.     

Complications of Barrett's esophagus: indications for esophageal resection with special reference to high-grade dysplasia

BACKGROUND: Columnar lined oesophagus (Barrett's oesophagus) can sometimes be associated with complications such as stricture, ulcer and dysplasia. In some selected cases there is an indication for oesophageal resection. METHODS: From 1983 to 1997, 12 patients underwent oesophagectomy for "complicated" Barrett's oesophagus. All patients had gastroesophageal reflux and Barrett metaplasia for many years. Ten of them were symptomatic, and pH-manometric alterations as well as alterations were noted at biliary scintigraphy. Ten patients had intestinal metaplasia. Two patients had previous antireflux operations. Four had a long (3-5 cm) and undilatable stricture. One was affected by a perforating ulcer. One patient had an indefinite grade dysplasia but endosonography revealed high suspicion of cancer. Six patients had a high-grade dysplasia. Operative technique consisted of a transhiatal oesophagectomy in nine cases and a laparotomic and right thoracotomic oesophagectomy (Ivor-Lewis) in two. RESULTS: There was no 30-day mortality; three post operative complications were observed. One of the four patients suffering from stricture died four years after intervention due to non-related causes; the other three are still living and regularly feed per os after 12, 9 and 7 years. The patient with ulcer is still living after 6 years and regularly feeding per os. The patient suffering from an indefinite grade dysplasia had an adenocarcinoma (stage IIa) on the operative specimen. The patient is still living after 2 years. Three patients operated for high-grade dysplasia had an adenocarcinoma on the specimen. Two patients (stage I) are living after 3 and 5 years. One patient (stage IIa) died after 19 months with recurrence. CONCLUSIONS: In case of non neoplastic "complicated" Barrett's oesophagus the indication for the oesophageal resection can be considered as the extreme useful therapy only after an accurate selection of patients. Especially in case of high-grade dysplasia, the great incidence of unexpected adenocarcinoma indicates oesophagectomy for patients who are suitable for surgery.     

Ectopic class II major histocompatibility antigens in Hirschsprung's disease and neuronal intestinal dysplasia.

Although the etiology of Hirschsprung's disease and neuronal intestinal dysplasia remains obscure, both have histological abnormalities involving ganglion cells and neuronal elements. Searching for a common pathway that may inhibit normal maturation of neurogenic precursors, we examined the possible role of an immune mechanism in the maldevelopment of the enteric neural network. Six patients with Hirschsprung's disease were studied by comparing biopsy specimens from diseased colon with ones taken from proximal ganglionic colon in the same patients. These were similarly compared with colonic biopsy specimens from patients studied with chronic constipation or bowel removed at the time of operation for other disorders. Biopsies were taken from four other patients with neuronal intestinal dysplasia. Each was examined by hematoxylin & eosin staining, acetylcholinesterase histochemistry, and immunohistochemistry of major histocompatibility complex (MHC) class I and class II antigens. All rectal samples from Hirschsprung's disease patients exhibited elevated acetylcholinesterase histochemistry and absent ganglia to confirm the diagnosis. These findings were correlated with marked elevation of class II MHC in the aganglionic area, whereas the proximal normal ganglionic segments showed no elevation. Rectal biopsy specimens from patients with chronic constipation exhibited no such elevation. A similar elevation of class II MHC was detected in the mucosa and submucosa of all four patients with the rare neuronal intestinal dysplasia disorder whose diagnosis was confirmed by giant ganglia in Auerbach's plexuses, aberrant Meissner's ganglia in the lamina propria mucosa, and giant neurofibrils in the mucosa and submucosa. The correlation of elevated class II MHC in these two neuronal dysfunction disorders may indicate an underlying autoimmune mechanism as is seen in thyroiditis and insulin dependent diabetes mellitus.(ABSTRACT TRUNCATED AT 250 WORDS)     

Diagnostic difficulties in neuronal intestinal dysplasia and segmental colitis

An 11-month-old girl with a prolonged history of bloody, mucoid diarrhea is presented. Although the initial diagnosis given by the rectosigmoid biopsy obtained during laparotomy was neuronal intestinal dysplasia, accompanying findings including mixed inflammatory cell infiltration of the mucosa and submucosa with mucosal ulcerations suggested nonspecific colitis. The subsequent biopsy specimen that was obtained after performing colostomy and treating with broad-spectrum antibiotics and rectal irrigations showed improvement in the structure of ganglion cells and submucous and myenteric plexuses. Although the mucosal ulcerations and inflammatory reaction improved, the colonic stricture persisted, so the Duhamel procedure was performed, and the patient had an uneventful outcome. It is claimed that inflammatory disease of the rectosigmoid colon of unknown etiology and neuronal intestinal dysplasia have occurred together in the current case or that one disease might cause the other in time.     

Photodynamic Therapy (PDT) for Oesophageal Dysplasia and Early Carcinoma with mTHPC (m-Tetrahydroxyphenyl Chlorin): A Preliminary Study

Barrett's oesophagus is a premalignant condition in which stratified squamous type mucosa of the normal oesophagus is replaced by specialised intestinal type columnar mucosa. Oesophageal resection was previously considered to be the treatment of choice for high-grade dysplasia or superficial carcinoma arising in this columnar-lined mucosa. We treated four patients with Barrett's oesophagus and high-grade dysplasia, and one patient with superficial oesophageal carcinoma with photodynamic therapy (PDT) using an argon-pumped dye laser light (652 nm). PDT was also delivered using a xenon arc lamp (Paterson lamp, light 652 nm±15 nm) in two patients with Barrett's oesophagus and high-grade dysplasia. mTHPC (m-tetrahydroxyphenyl chlorin) 0.15 mg/kg was used as a photosensitiser in all the patients. We have been able to demonstrate the elimination of columnar-lined oesophageal mucosa, reduction in the length of the Barrett's segment or downgrading of the dysplasia in all of the patients. There is no evidence of recurrence in the patient who had oesophageal carcinoma, at 27 months follow-up. We conclude that mTHPC is useful as a photosensitiser for PDT in the management of Barrett's oesophagus with high-grade dysplasia or superficial carcinoma and the Paterson lamp is a potential alternative light source for PDT. Paper received 5 October 2000; accepted after revision 10 July 2001.     

Internal hernia and volvulus of the small bowel following liver transplantation

Internal herniation with volvulus of the small intestine is an uncommon, but potentially fatal, complication after liver transplantation. We present here four cases in which the herniation occurred around the Roux-en-Y loop used for the biliary reconstruction. One patient died due to intestinal and liver allograft necrosis; another lost almost the entire small intestine and has since undergone successful intestinal transplantation. Two patients survived following surgery that involved reduction of the hernia and closure of the mesenteric defect. Clinical diagnostic implications emphasize early diagnosis and appropriate operative intervention. Received: 2 May 1996 Received after revision: 7 October 1996 Accepted: 28 October 1996     

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目的 总结6例短肠综合征病人肠管倒置手术失败的教训，强调合理选择手术适应证的重要性和必要性，提出短肠综合征合理的治疗策略，方法 回顾性分析6例短肠综合征病人，均在外院接受肠管倒置手术，除1例为结肠倒置外，其余均为小肠倒置，倒置肠管长度从10～50cm不等，5例小肠倒置手术均在广泛肠切除的同时进行，术后1例出现吻合口瘘，4例出现肠梗阻。结果 1例病人因肝功能衰竭于术后5年死亡，1例病人放弃治疗，1例行肠瘘及倒置小肠切除治愈，2例行倒置肠管切除治愈，1例行倒置肠管复位治愈。治愈病人依靠普通饮食和部分肠内营养支持维持正常营养状况。结论 短肠综合征病人应首选肠康复治疗和小肠移植。只有极少数病人适宜进行肠倒置手术，手术时倒置肠管不应超过10cm，手术时机应在广泛肠切除2年以后进行．手术时应注意倒置肠管的血液供应，避免肠缺血。     

Impact of antireflux operation on columnar-lined esophagus

BACKGROUND: The effect of antireflux operation on the natural history of columnar-lined esophagus (CLE) is not fully understood. The aim of this study was to assess a single center's experience and review the literature on the impact of antireflux operation on CLE without high-grade dysplasia. STUDY DESIGN: The medical records of 26 patients with CLE but without high-grade dysplasia who underwent antireflux operation in our unit were retrospectively analyzed at longterm followup with detailed endoscopic investigation. Thirteen patients presented with intestinal metaplasia (6 had short segments, and 1 had preoperative laser ablation) and 13 without intestinal metaplasia.For the group of 13 patients presenting with intestinal metaplasia, the mean endoscopic followup was 74.7 months (median 46 months). Three of six with short-segment lesion and two of seven with circumferential involvement had complete regression of intestinal metaplasia (one after laser therapy). None had progression to dysplasia or carcinoma. RESULTS: For the group of 13 patients without intestinal metaplasia, mean endoscopic followup was 43.9 months (median 28 months). One had complete regression of CLE, and none developed intestinal metaplasia during surveillance. CONCLUSIONS: Our study suggests that antireflux operation can alter the natural history of CLE, allowing disease stabilization in a substantial proportion of patients. After antireflux operation, total regression of CLE is possible, but in an unpredictable manner.