成人先天性巨结肠症的诊断和外科处理

目的 探讨成人先天性巨结肠症的诊断和外科处理。方法 对１９８５年４月￣２０００年２月收治的成人先天性巨结肠症６例的临床资料进行回顾性分析。结果 男４例,女２例,年龄１４￣４０岁。无神经节细胞段在乙状结肠远端和直肠１例,直肠５例。１例作结肠造瘘,５例作Ｉｋｅｄａ法手术,术后排便功能均优。结论 成人先天性结肠症的诊断主要依据便秘史,钡灌肠检查和／或肛门直肠测压。Ｉｋｅｄａ法手术是有效的手术治疗方法。     

成人先天性巨结肠症的诊断和外科治疗

目的　探讨成人先天性巨结肠症的临床特点和治疗方法。方法　回顾性分析 11例成人先天性巨结肠症的临床资料并随访 ,分析其临床特点和诊断方法 ,讨论手术方式。结果　男 7例 ,女 4例 ,年龄 14～ 4 7岁。 4例狭窄段位于直肠与乙状结肠交界处 ,3例位于直肠上段 ,4例位于直肠中下段。 3例追问到有胎便排出延迟病史 ,2误诊为乙状结肠冗长症 ,1例误诊为急性肠梗阻 ,1例死亡。 7例行改良Swenson手术 ,3例Soave手术 ,术后排便功能良好。结论　成人先天性巨结肠症临床表现不典型 ,容易误诊 ,诊断主要依据便秘史 ,特别是自幼有便秘史 ,钡灌肠、直肠肛管侧压和直肠粘膜活检可确诊 ,一期改良Swenson手术是有效的外科治疗方法之一。     

成人先天性巨结肠症的诊断及外科治疗

先天性巨结肠症是常见的肠道发育畸形之一,多于婴幼儿时期发病、就诊,迁延至成人者少见.成人先天性巨结肠症症状常不典型,易出现误诊,治疗过程亦有特殊之处.我院于1993～2003年间共收治15例成人先天性巨结肠症患者,其中13例行手术治疗,术后病理证实均符合先天性巨结肠症.本文对此组患者的诊疗进行回顾性分析,以探讨成人先天性巨结肠的临床表现及诊断、治疗方法.     

成人乙状结肠冗长症的外科治疗体会

在临床日常生活中以便秘为主诉的就诊者并不少见．但往往容易被忽略。近年来结肠冗长症所致的顽固性便秘引起了众多学者的注意,已进行大量的研究并取得了显著的效果。结肠冗长最多见于乙状结肠,约占63．4％。我院1992～2007年共收治11例乙状结肠冗长症病人并行乙状结肠及其系膜切除术,取得了较满意的效果,现报道如下。     

腹腔镜辅助Rehbein手术治疗成人先天性巨结肠3例报告

目的:探讨腹腔镜辅助Rehbein手术治疗成人先天性巨结肠的难点及对策。方法:回顾分析采用腹腔镜辅助Rehbein手术治疗成人先天性巨结肠3例患者的临床资料。结果:3例患者均取得较满意的疗效。结论:腹腔镜Rehbein手术更适合治疗成人巨结肠;手术最大的难点是患者结肠常有广泛严重的扩张,明显地增加了手术暴露及操作难度,应根据患者病情采取相应的措施。     

成人先天性巨结肠症的诊断和治疗

目的成人先天性巨结肠症的诊断和手术方法的探讨。方法对本院31例成人先天性巨结肠症的临床表现,诊断和手术方法,进行了回顾性分析。结果 29例行 Swenson 手术,1例 Duhamel 手术,术后并发吻合口瘘5例(16.7%)。早期吻合口狭窄2例,28例获得随访,完全恢复正常排便功能23例(78%),4例轻度排便失禁,1例完全排便失禁。结论成人巨结肠症手术后并发症较婴幼儿高。全结肠显著肥厚扩张时,保留回盲部的手术,术后并发吻合口瘘的发生率高;采用全结肠直肠切除回肠直肠末端吻合,并作预防性回肠造瘘术,可预防吻合口瘘的发生。     

成人先天性巨结肠症的诊断和治疗

目的 成人先天性巨结肠症的诊断和手术方法的探讨。方法 对本院3例成人先天性巨结肠症的临床表现，诊断和手术方法，进行了回顾性分析。结果 29例行Swenson手术，1例Duhamel手术，术后并发吻合口瘘5例(16．7％)。早期吻合口狭窄2例．28例获得随访，完全恢复正常排便功能23例(78％)，4例轻度排便失禁．1例完全捧便失禁。结论 成人巨结肠症手术后并发症较婴幼儿高。全结肠显肥厚扩张时，保留回盲部的手术，术后并发吻合口瘘的发生率高；采用全结肠直肠切除回肠直肠末端吻合．并作预防性回肠造瘘术，可预防吻合口瘘的发生。     

腹腔镜辅助经肛门Soave拖出术治疗先天性巨结肠症及同类性疾病

目的 探讨腹腔镜辅助经肛门Soave手术治疗先天性巨结肠症及同类性疾病的可行性和效果.方法 2010年3月至2011年12月,对31例先天性巨结肠症及同类性疾病的患儿实施腹腔镜辅助经肛门Soave手术,作者改进这种技术,在经脐或经肛门腹腔镜监视下联合直肠肌鞘入路游离左半结肠或全部结肠,然后拖出体外完成直肠乙状结肠或次全结肠切除术. 结果 本组31例患儿均顺利完成手术,16例行直肠乙状结肠切除,15例次全结肠切除术,平均手术时间(117 ±13) min,切除肠段35 ～ 80 cm,术中估计出血5～20 ml,1例因小肠梗阻开腹探查.随访无吻合口狭窄和便秘复发,仅1例出现小肠结肠炎.结论 经脐或经肛门腹腔镜辅助Soave手术治疗先天性巨结肠及同类性疾病安全、有效,手术创伤更小.     

先天性巨结肠症内皮素B受体基因分析

目的　检测中国散发先天性巨结肠症 (HD )是否有EDNRB基因突变 ,以探讨EDNRB与HD发生的关系。方法　提取 3 4例中国散发性HD患儿手术切除的组织标本基因组DNA ,聚合酶链反应(PCR)扩增EDNRB基因第 3 ,4,6外显子 ,单链构象多态 (SSCP)分析上述外显子是否有突变。结果　13例短段型HD中 ,2例有EDNRB基因突变 ;常见型及长段型未见EDNRB基因突变。结论　中国散发短段型HD有EDNRB基因突变 ,提示EDNRB基因与先天性巨结肠症的发生有关。     

腹腔镜辅助改良Soave术治疗成人先天性巨结肠症

目的 评价腹腔镜辅助改良Soave术治疗成人先天性巨结肠症（HD）的临床价值.方法 回顾性分析华中科技大学同济医学院附属协和医院2005年3月至2009年12月间行腹腔镜辅助改良Soave术的28例术前诊断为成人HD患者的临床资料.结果 本组28例患者均成功实施了腹腔镜辅助改良Soave术,无中转开腹.手术时间135～185（165±12）min,术中出血量50～250 ml,无一例术中输血.术后病理诊断示：19例符合HD,9例符合先天性巨结肠类缘病.术后直肠肌鞘感染2例,肛门口轻度污粪3例,平均住院时间（17.5±1.0）d.术后随访无排粪失禁及便秘复发.结论 腹腔镜辅助改良Soave术治疗成人HD安全、有效.     

成人先天性巨结肠的诊断与外科治疗

目的探讨成人先天性巨结肠的诊断与外科治疗。方法回顾性分析1992年6月至2004年6月问收治的15例成人先天性巨结肠患者的临床资料。结果15例患者年龄17．54岁。人院前有9．5个月至50．0年(平均21．4年)的长期便秘、腹胀史；6例患者有急性腹痛史；均无脱水或营养不良表现。钡剂灌肠可见肠管狭窄段和扩张段。予以Soave手术2例，次全结肠切除加结肛吻合术13例；术后所有患者排便功能优良。结论自幼长期慢性便秘史和钡灌肠检查是诊断的主要依据；结肠次全切除加结肛吻合术是安全有效的手术方式。     

腹腔镜辅助改良Soave术治疗成人先天性巨结肠症

Objective To evaluate the clinical value of laparoscopy-assisted modified Soave procedure for Hirschsprung disease in adults.Methods Twenty-eight patients with a preoperative diagnosis of Hirschsprung disease underwent laparoscopy-assisted modified Soave procedure between March 2005 and December 2009.Clinical data were retrospectively analyzed.Results There were no conversions to open surgery.The mean operative time was (165±12) minutes (range:135-185 minutes).Estimated blood loss ranged from 50 to 250 ml,and no patients required intraoperative blood transfusion.Postoperative pathologic examination showed Hirschsprung diseases in 19 patients and Hirschsprung allied diseases in 9.Only two patients developed rectal cuff infection and three mild seepage.Other patients had no postoperative complications.The mean hospital stay was (17.5±1.0)days.No fecal incontinence or recurrent constipation occurred during follow-up.Conclusion Laparoscopyassisted modified Soave procedure is safe and effective for Hirschsprung disease.     

先天性巨结肠手术后排便造影检查的临床意义

目的评价排便造影检查在先天性巨结肠 (HD)术后随访的临床意义。方法对30例行HD根治手术治疗患儿行排便造影检查 ,并获得手术后的排便功能情况 ,行腹部和肛门指诊检查及肛管直肠测压检查。结果按照临床评分标准 ,本组 30例中“优者”4例 (13% ) ,即无任何排便异常者 ;“良好”2 1例 (70 % ) ,有较轻度的排便异常 ;“一般”5例 (17% ) ,有明显的排便异常 ;无“差”病例 ,即无排便严重障碍和完全失禁者。排便造影显示静息状态时直肠肛管角及直肠骶曲在“一般”组与对照组之间比较差异有显著意义 (P <0 0 5 )。“一般”组与对照组比较肛管长度缩短 (P <0 0 5 )。结论HD根治术后患儿排便功能良好 ,并随时间的延长排便功能可继续好转 ,排便控制建立在拖下结肠“直肠、乙状结肠化”的过程。术后大便储袋的形成、直肠肛管角和直肠骶尾曲在排便控制中起重要作用。排便造影比传统的钡灌肠更能确切地了解排便功能 ,对评价HD术后功能有重要价值。     

Early adult outcome of the Duhamel procedure for left-sided Hirschsprung disease--a prospective serial assessment study

Purpose

To assess both early adult functional outcome and change in long-term functional outcome over time after the Duhamel procedure (DP) for left-sided Hirschsprung disease (HSCR).

Methods

The study population consisted of 78 children (aged 19.9 ± 3.6 years) who previously underwent objective outcome assessment after DP was performed for HSCR during the period of 1980 to 1991. Inclusion criteria were previous evaluation of functional outcome and either rectosigmoid or left-sided HSCR. Outcome measures were assessed twice within the cohort, in 1997 and in 2005. The primary outcome measure was the Rintala (J Ped Surg. 1995;30:491-494) functional outcome score (FOS; maximum, 20). Controls consisted of 20 age-matched healthy children. Satisfactory functional score was defined as an FOS at or above the 10th percentile of controls (FOS, ≥17). Secondary outcome measures were the operation failure rate (defined by requirement for a stoma or major reoperative surgery), and enterocolitis rates (defined by intention to treat). Consecutive outcome scores were compared by paired t test. Data were expressed as mean ± SD, and P < .05 was considered significant.

Results

Operation failure occurred in 9 (11.5%) of 78. Consecutive FOSs were obtained in 40 (57%) of 69. A satisfactory functional score was observed in 23 (58%) of 40 adults as opposed to 33 (47%) of 70 children 8 years previously (P = .02). Satisfactory outcome (defined by satisfactory functional score and lack of enterostomy or major revision pull-through procedure) was observed in 23 (47%) of 49. Previously, this figure was 34 (44%) of 78. Individual paired FOSs showed a significant improvement with time (1997: 14.9 ± 4.1; 2005: 16.4 ± 2.8; P = .02).

Conclusions

At early adult follow-up, the operation failure rate has not changed from that of the same cohort 8 years earlier. However, a significant improvement in individual FOSs was demonstrated.     

Hirschsprung disease and fecal incontinence: diagnostic and management strategies

Purpose

Ideally, fecal incontinence after operative management for Hirschsprung disease should not occur. If it does, it presents a formidable challenge. The purpose of this study was to describe the causes of fecal incontinence and present our algorithm for its treatment.

Methods

We reviewed 68 patients with Hirschsprung disease and fecal incontinence referred to us after surgery at other institutions. Patients were evaluated by contrast enema and by an examination under anesthesia to look specifically for the integrity of the anal canal. They were designated as having a dilated colon and constipation or a nondilated colon and a tendency to diarrhea based on their stooling pattern and the appearance of the contrast enema. Medical management was started that included laxatives for those patients with a dilated colon and constipation. For those with a nondilated colon and tendency to diarrhea, the management included loperamide, pectin, and a special dietary regimen (constipating diet, 3 meals per day, and no snacks). Those patients who responded to medical management were retrospectively considered to have been pseudoincontinent. Those who did not respond were considered truly incontinent. The truly incontinent group was treated with enemas alone for those with a dilated colon, or enemas, loperamide, pectin, and a constipating diet for those with a nondilated colon and tendency to diarrhea.

Results

Fifty-six patients had true incontinence and 12 had pseudoincontinence. Of the true incontinent group, 27 had a dilated colon and 29 had a nondilated colon. Five of these patients had a damaged or absent anal canal (anastomosis at the anal skin) and all of them had true incontinence. In the dilated colon group with true incontinence, 23 (85%) patients were clean after treatment. In the nondilated colon group with true incontinence, 23 (79%) were successfully treated. All patients in the pseudoincontinent groups had no soiling after treatment. Of 55 in the truly incontinent group, 39 (70%) had had an endorectal (Soave type) pull-through.

Conclusion

Fecal incontinence after operative management of Hirschsprung disease represents a serious problem. Poor surgical technique may be a contributing factor in some of the cases. Successful management depends on the appropriate evaluation, which determines whether the incontinence is true or pseudo, and the type of colon the patient has. Each category can be well treated, leading most of the time to a clean child.     

成年人先天性巨结肠的诊治体会

目的探讨成年人先天性巨结肠的诊断及治疗方法。方法回顾性分析我院于1998年10月-2006年10月收治的成年人先天性巨结肠10例的临床资料。结果全组除1例由于多次腹部及肛门直肠手术史选用了保守治疗，效果理想外，其余均采用手术治愈。结论成年人先天性巨结肠误诊率高。诊断依据主要是追溯自幼便秘史，钡灌肠、全消化道钡餐X线透视及24、48h，甚至72h钡滞留X线摄片。诊断不能确定时加直肠黏膜活检及肛门直肠测压。有过多次手术史者，再手术时应视病情而定，并尽可能多地收集既往手术资料。强调治疗及手术方式应个体化。     

Reoperations in Hirschsprung disease

Background

We sought to identify causes of preventable complications related to operations for Hirschsprung disease.

Methods

We reviewed the cases of 51 patients with Hirschsprung disease who underwent a primary procedure elsewhere, had a complication, and were referred for reoperation.

Results

Thirty-five patients had 1 failed operation, 10 had 2, and 6 had 3. Initial operations were Soave (20), Duhamel (15), Swenson (5), transanal endorectal (4), myectomy (3), unknown (3), and laparoscopic Swenson (1). Thirty-one patients presented with a stoma. Patients without a stoma (20) had fecal impaction (8), recurrent enterocolitis (6), and fecal incontinence (6). None had both enterocolitis and incontinence. Reoperation was performed posterior sagittally (40) or transanally (5). Indications included stricture (21), megarectal Duhamel pouches (12), fistulae (11 [8 rectocutaneous, 2 rectourethral, and 1 rectovaginal]), pouchitis (2), and retained aganglionic bowel (8). After reoperation, 14 were continent, 11 had a stoma (8 permanent), 6 had voluntary bowel movements but soiled occasionally, 6 received rectal irrigations to avoid enterocolitis, 6 were incontinent but clean with bowel management, and 2 were lost to follow-up.

Conclusion

Stricture, megarectal pouch, fistula, and retained aganglionic bowel are preventable complications. Enterocolitis is partially preventable but can occur after a technically correct procedure. Fecal incontinence is a preventable complication likely because of anal canal damage.     

肾上腺皮质增生类疾病的诊断及外科治疗(附180例报告)

目的 探讨肾上腺皮质增生类疾病的临床诊断及外科治疗方法. 方法 肾上腺皮质增生患者180例.男74例,女106例.年龄6～76岁,平均40岁.其中皮质醇增多症152例,醛固酮增多症28例.180例均行肾上腺手术治疗,术后病理检查均符合肾上腺皮质增生诊断.回顾分析患者临床表现特点、实验室检查结果、肾上腺CT影像特点及疗效. 结果 180例中库欣病(CD)107例,促肾上腺皮质激素(ACTH)非依赖性肾上腺大结节增生(AIMAH)22例,异位ACTH所致肾上腺皮质增生(EAAH)19例,原发性色素结节样肾上腺皮质增生(PPNAH)4例,特发性醛固酮增多症(IHA)28例.实验室检查前4组平均24 h尿游离皮质醇(24 h UFC)分别为287.6(95.2～535.7)、307.9(24.8～808.2)、852.5(102.5～3127.0)、564.3(243.8～1124.6)μg,皮质醇分泌节律消失比例分别为99%(102/103)、92%(11/12)、100%(17/17)、100%(4/4);AIMAH、EAAH、PPNAH组大、小剂量地塞米松抑制试验(DDST)均不被抑制,CD组大剂量DDST可被抑制.28例IHA患者中血钾降低(<3.5 mmol/L)17例,尿钾升高(>30 mmol/24 h)15例,肾素及醛固酮测定符合诊断.180例患者肾上腺CT检查均提示病变侧肾上腺体积增大.102例皮质醇增多症患者行单侧肾上腺切除,术后1周内CD、AIMAH、EAAH、PPNAH组平均24 h UFC分别为157.4(56.2～233.5)、117.9(22.5～418.5)、756.7(116.5～1137.0)、164.3(124.6～422.6)μg.21例IHA患者行单侧肾上腺病灶切除,术后血压恢复正常8例(38%),较术前下降13例(62%).57例行双侧肾上腺手术治疗(双侧全切39例,一侧全切一侧次全切16例,双侧次全切除2例),其中皮质醇增多症患者术后1周复查24 h UFC为12.8～98.5μg,平均77.6μg;IHA患者血压正常.106例(59%)随访4～158个月,平均32个月,库欣病症状及高血压症状均有改善. 结论 需要手术治疗的肾上腺皮质增生类疾病首选单侧肾上腺全切治疗,后期根据临床观察可选择对侧肾上腺次全切除或全切除,可获得较好治疗效果.