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     

Granular-cell tumorlike Schwann cell degeneration in the anal sphincter of an infant suspected of having ultrashort Hirschsprung's disease

A 19-month-old girl who had a history of constipation since birth and suspected cytomegalovirus (CMV) infection was admitted. Barium enema revealed no caliber change in the colon, anorectal manometry failed to show sphincter relaxation on rectal distension, and biopsied rectal specimens contained ganglion cells. She underwent rectal myectomy, after which her symptoms improved significantly. Morphologic studies, including electron micrographs of the muscle, demonstrated nerve plexuses with degenerative Schwann cells containing abundant eosinphilic cytoplasmic granules that resembled those of a granular-cell tumor. Granular-cell tumorlike change of the plexus is an unprecedented finding in any form of Hirschsprung's disease or its allied disorders, but appears to have been responsible for her symptoms. CMV as a causative agent is also discussed with a review of the literature.     

A Solitary Peutz-Jeghers-type Hamartomatous Polyp of the Rectum: Report of a Case and Review of the Literature

A solitary Peutz-Jeghers-type polyp of the rectum in a 64-year-oldJapanese man is reported. Barium enema and endoscopic examinationrevealed a solitary polypoid lesion in the rectum. The polypwas pedunculated, and measured 2 x 1.5 x 1.5 cm. The patienthad neither mucocutaneous pigmentation nor a family historyof gastrointestinal polyposis. Histopathologically, this polyphad an arborizing muscular network originating from the muscularismucosa, and was covered by well organized mucosa with epithelialhyperplasia. The smooth muscle bundles in the polyp were thickerthan those seen in Peutz-Jeghers syndrome, but their networkwas not as complex.     

维甲酸诱导大鼠肛门直肠畸形胚胎中RARmRNA表达的研究

目的探讨胚胎发育中维甲酸诱导的RARmRNA表达异常与肛门直肠畸形发生之间的相关性。方法利用全反维甲酸(ATRA)诱导大鼠产生肛门直肠畸形胚胎，用RT-PCR法测定妊娠11、12、13、14、15、16d时大鼠胚胎尾侧部分的RARmRNA表达模式并且与对照组中RARmRNA表达模式进行对比。结果实验组中80％胚胎具有肛门直肠畸形，对照组未见明显畸形。实验组中RARαmRNA表达水平在妊娠11、12、13、15d时低于对照组，表达曲线的高峰出现时间晚于对照组，实验组中RARβmRNA表达水平在妊娠12、14d时低于对照组，而RARγmRNA表达水平在实验组中与对照组中并无明显差异。结论ATRA可诱导胚胎发育过程中RARmRNA异常表达，它可能在妊娠11～13d这一尾侧区域发育最关键时段通过抑制RARα的表达启动并最终导致肛门直肠畸形及其伴发畸形的发生。     

高频电刀切除术治疗直肠大息肉72例临床分析

目的总结高频电刀切除直肠大息肉的经验。方法对2003-08~2005-08我院确诊的直肠大息肉72例患者进行高频电刀切除。结果56例1次性切除成功,16例经3~5次套切后全部成功切除。结论高频电刀切除直肠大息肉效果好,可以推广。     

婴幼儿经肛管结肠拖下术

目的 介绍一种经肛管肌鞘内结肠拖下术。方法 回顾性分析26例经肛管拖下术病例，在肛门上及直肠下段粘膜下分离，直肠壁有病变无法粘膜下游离者，亦应紧贴肠壁外解剖减少损伤的可能。拖下手术同时可行内括约肌重建。结果 所有病例无术中、术后严重并发症。近期随访效果良好。新生儿巨结肠手术可在60分钟左右完成。结论 该术式损伤小、近期效果好，适用于婴幼儿先天性巨结肠及某些肛门直肠畸形患儿。     

出口梗阻性便秘手术的护理体会

目的：总结直肠前突及直肠黏膜脱垂患者手术护理体会，进一步提高患者的护理质量。方法．28例用手按压阴道后壁排出粪便，多数患者长期服用导泻药物以协助排便。结果：对本组38例直肠前突和直肠黏膜脱垂患者经过积极的手术治疗和实施护理措施，大便排出困难均明显得到改善，临床症状消失，减轻了患者的痛苦，取得了良好效果，33例患者每日排便1次，有3例为每日排便2次，2例每2—3天排便1次。避免了并发症，使患者尽快的康复。结论：对直肠前突和直肠黏膜脱垂患者实施的本护理措施是有效的，提高了临床治愈率。缩短了患者住院时间，使他们早日康复。     

EphB2在先天性肛门直肠畸形直肠末端表达的研究

目的 研究酪氨酸蛋白激酶受体--EphB2在先天性肛门直肠畸形直肠末端组织中的mRNA表达和蛋白表达的水平,探讨EphB2与先天性肛门直肠畸形发生的关系.方法 RT-PCR方法和Western蛋白印迹方法,检测31例不同类型先天性肛门直肠畸形直肠后壁末端、5例后天瘘及8例正常直肠后壁末端EphB2在蛋白水平和mRNA水平的表达情况,应用单因素方差分析比较正常组和畸形组、不同类型的畸形组之间EphB2表达水平的差异.结果 ①先天性肛门直肠畸形末端肠壁EphB2基因mRNA相对表达量明显低于正常对照组(0.99±0.11,P=0.00).不同类型的畸形组之间,高位组(0.43±0.07)和中位组(0.47±0.10)之间差异无统计学意义(P=0.33),但二者明显低于低位组(0.64±0.06,P=0.00).后天瘘组(0.97±0.09)与正常对照组比较差异没有统计学意义(P=0.63);②Western蛋白印迹结果显示,先天性肛门直肠畸形高位组(0.21±0.05)、中位组(0.24±0.04)和低位组蛋白表达水平均明显低于后天瘘组(0.51±0.05)和正常对照组(0.52±0.03,P=0.00).不同类型的畸形之间,高位组和中位组之间无明显差异(P=0.11),但二者明显低于低位组(P＜0.01).后天瘘组与正常对照组比较差异没有统计学意义(P=0.67).结论 EphB2在先天性肛门直肠畸形直肠末端低表达.EphB2的表达和肛门直肠畸形的发生有关,EphB2在先天性肛门直肠畸形的发生中可能具有重要作用.     

EphB2基因多态性与先天性肛门直肠畸形的相关性研究

目的 探讨EphB2受体基因第6外显子的-1395A/G多态性在辽宁地区汉族人群中的分布及其与先天性肛门直肠畸形(CAM)的关系.方法 采用PCR-RFLP方法,对65例CAM患儿和115名健康儿童EphB2基因-1395A/G多态位点进行基因型检测,用SHEsis在线统计软件分析等位基因频率、基因型频率及其组间差异.结果 EphB2受体基因第6外显子的-1395A/G多态A等位基因频率及AA基因型频率在CAM组(85.4%、75.4%)与正常对照组(17.0%、4.3%)间差异有显著统计学意义(P＜0.01).结论 EphB2受体基因第6外显子的-1395A/G多态与CAM存在相关性.     

肛门直肠畸形合并先天性巨结肠的诊治

目的 总结儿童先天性肛门直肠畸形(ARM)合并先天性巨结肠(HD)的临床特点,探讨其病因和适宜的诊治方法.方法 回顾性分析2004年1月至2008年4月6例ARM合并HD患儿的临床资料、诊治过程及预后情况.年龄1岁8个月～1I岁,平均4.1岁;男女比例1:5.所有病例于ARM矫正手术后有不同程度便秘、腹胀等症状,钡灌肠显示结直肠均有显著的扩张,仅有2例可见明确痉挛段和移行段.6例患儿直肠肛管测压直肠肛门抑制反射不能引出.2例采用经肛门Soave术式,4例采用经腹肛门Soave巨结肠根治术.结果 术后病理检查6例标本远端肠壁内均未见神经节细胞.免疫组化组织蛋白酶D:近段阳性,远端阴性.结论 对于ARM患儿,特别是对于ARM畸形矫正术后仍有便秘的患儿要警惕合并HD的可能.此外,ARM合并HD患儿往往同时存在有多种畸形,在胚胎发育过程中这些畸形的发生可能存在着一定的关联.