肛门直肠畸形合并先天性巨结肠

目的：探讨先天肛门直肠畸形（ＡＲＭ）合并先天性巨结肠（ＨＤ）的临床特点和诊治方法。方法：回顾性分析总结本院２０年来ＡＲＭ３２６例，ＨＤ３２０例，其中两种并存有３例，３例均经手术病理证实诊断，结论：ＡＲＭ作结肠造瘘术后，排便不畅应及时作结肠活检，随访ＡＲＭ术后患儿有腹胀，便秘，应进一步作钡灌肠和直肠粘膜乙酰胆碱酯酶检查，确诊后按巨结肠处理。     

一种新的手术方式——单纯经肛门手术治疗先天性巨结肠

目的 观察一期经肛门拖出术治疗先天性巨结肠患儿的经过及近期疗效。方法 6例年龄分别为15天、4、5、6、15个月及2岁的小儿接受了单纯经肛门治疗巨结肠的手术。诊断由术前钡灌肠及术中、术后的病理检查确定。2例并巨大粪石,麻醉下夹碎,温盐水灌洗清除。直肠粘膜自齿线上0.5cm起分离至腹膜返折处,移行段及乙状结肠上段经肛门内拖出,限制拖出的血管予电凝或结扎,将正常结肠与肛门齿线上粘膜吻合。结果 术后平均住院4天(2～7天),所有小儿每日有3～5次正常大便,无需药物及洗肠。结论 单纯经肛门直肠内手术的方式既安全又容易施行,而且术中对结肠系膜的游离范围最小。该法具有创伤少,术后恢复快,并发症少、住院时间短等优点,值得临床推广应用。     

新生儿先天性巨结肠的诊断与治疗

目的 总结新生儿先天性巨结肠的诊断及治疗经验，对改进的治疗方案进行评估。方法回顾1995年6月～2002年6月155例新生儿先天性巨结肠的诊断及治疗情况，并且对2000年5月前后不同的治疗方案进行比较。结果本组155例行X线钡剂灌肠、直肠测压及直肠黏膜活检确诊，107例行Ⅱ期根治术，48例新生儿期行I期根治术。147例治愈出院，术后发生肺炎25例，伤口裂开8例，8例死亡。在第一阶段107例中2例施行一期手术；第二阶段48例中46例施行一期手术。与第一阶段相比，第二阶段术后住院天数、并发症、死亡数明显下降。结论(1)新生儿先天性巨结肠的临床症状不典型，一般情况较好者尽可能先保守治疗，造瘘术只用做最后手段。对结肠穿孔者应常规在直肠上段、乙状结肠部位行组织活检。对于诊断有疑问的病例在手术中取多处活检是必要的。(2)不同类型的先天性巨结肠选择不同的手术方式是治疗各种类型先天性巨结肠的原则。对于大部分常见型病例，经肛门手术可以取得良好效果。     

经肛门改良Soave根治术治疗先天性巨结肠疗效观察

目的:观察经肛门Soave根治术治疗先天巨结肠术后排便及其它并发症情况。方法:自2001年9月～2003年9月对24例年龄20天～6个月的先天性巨结肠患儿行肛门Soave根治术,术后定期随访,随访时间3个月～2年,对患儿大便控制能力,粘连性肠梗阻,及小肠结肠炎等的发生率进行评价。结果:术后并发症有小肠结肠炎5例(21.8％),便秘1例(4％),污粪2例(8％)。结论:经肛门Soave根治术术后可获得良好的排便控制。     

经肛门手术治疗15例小儿先天性巨结肠症

目的对小儿先天性巨结肠症肛门手术治疗的适应证、方法、疗效和随访结果进行总结。方法采用经肛门直肠肌鞘内病变粘膜切除,保留直肠肌鞘6 cm左右,沿结肠壁处理肠系膜血管结肠拖出并在齿状线以上吻合。结果手术时间平均75 min(50~100 min),均于术后48 h内进食,平均术后住院6 d,随访2~18个月,患儿排便情况1~5次/d,无大便失禁和肛门狭窄(术后坚持扩肛6个月)。结论本术式简单,效果好、并发症少,值得临床推广。     

经肛门结肠拖出术治疗小儿先天性巨结肠--附57例报告

目的 探讨单纯经肛门结肠拖出术治疗小儿先天性巨结肠的可行性。方法 2001年7月—2002年5月，57例巨结肠患儿行经肛门结肠拖出术。结果 53例顺利完成单纯经肛门结肠拖出术，手术时间(1—2)小时，平均1．5小时，出血5m1—10m1。51例术后1天开始自行排便、进食，2例术后3天予开塞露灌肠后排便。4例长段型巨结肠，病变累及横结肠，在腹腔镜辅助下完成，手术时间(2—3．5)小时，平均3小时，术后(2—3)天开始排便、进食。随访(2—9)月，57例患儿排便功能均正常良好。结论 单纯经肛门结肠拖出巨结肠根治术治疗小儿先天性巨结肠可行，此术式损伤小、出血少、术后恢复快、住院时间短，术后排便功能恢复良好。     

经肛门Soave Ⅰ期拖出根治术治疗先天性巨结肠56例

1998年Forre[1]报道了经肛门SoaveⅠ期拖出根治术,近期效果满意。我科应用此术式治疗先天性巨结肠的新生儿56例,总结报道如下。     

先天性肛门闭锁超声定位诊断探讨

目的 探讨应用超声检查对先天性肛门闭锁进行定位诊断。方法 对25例先天性肛门闭锁患儿术前使用超声检查,测定直肠盲端与肛门隐窝皮肤之间的距离(P-P间距),并设立耻尾线(PC线),从而决定首次手术方式,并将超声检查结果与手术实际结果对比。结果 25例患儿均能准确地测得P-P间距,并能满意设立PC线,超声检查结果与手术基本相符。结论 超声检查应用于肛门闭锁术前定位诊断,具有准确率高、安全简便、可重复性强等优点,值得推广应用。     

肛门闭锁术后残留直肠尿道瘘的治疗

目的 :探讨肛门闭锁术后残留直肠尿道瘘的手术方式及疗效。方法 :8例本病患儿 ,根据病变情况分别采用直肠内直肠尿道瘘修补及经腹会阴联合术式行乙状结肠部分切除、直肠粘膜剥离、瘘管结扎、结肠经直肠肌鞘内拖出肛门成形术治疗。结果 :术后全部治愈出院 ,随访 1～ 6年无复发 ,疗效满意。结论 :上述两种术式较其他术式具有修补牢靠、术式简单、不易复发等优点 ,是目前修补直肠尿道瘘较理想术式之一。     

The association of imperforate anus and Hirschsprung's disease in siblings

The recognition of Hirschsprung's disease is often delayed in children with imperforate anus. Two siblings with imperforate anus who were born of consanguineous parents had persistent constipation after the repair of the anorectal malformation. Subsequently colon biopsy confirmed that each had long segment aganglionosis beginning at the splenic flexure. A suspicion that these two disorders may coexist is mandatory for early correct diagnosis.     

The management of end-stage renal disease in infants with imperforate anus

Genitourinary malformations are frequently associated with imperforate anus, and death from renal failure is reported in up to 6% of children with supralevator imperforate anus. In recent years, advances in renal transplantation and the management of end-stage renal disease (ESRD) have extended these therapies to infants in the first 2 years of life. In infants with imperforate anus and ESRD, it is unclear if the additional burdens of the anorectal malformation and its staged repair contraindicate dialysis and transplantation. This report describes our experience with three such infants and outlines an approach to their care, addressing the following key issues: the initial surgical management of the imperforate anus, the careful search for associated urinary tract and other malformations, the ESRD management, and the appropriate timing of the staged bowel reconstruction and renal transplantation. These cases confirm that such children may be successfully managed by dialysis and renal transplantation co-ordinated with bowel reconstruction; however, there remain the longterm risks of immunosuppression, bladder and bowel dysfunction, and associated congenital anomalies.     

High and intermediate imperforate anus: psychosocial consequences among school-aged children

Background/Purpose

Imperforate anus is an unusual malformation, which, even after surgical intervention, usually entails constipation and fecal incontinence. This study aimed to evaluate ongoing psychosocial effects of this birth defect in school-aged children.

Methods

Twenty-five children born with high and intermediate imperforate anus participated in the study, along with their parents and classroom teachers. One group of healthy children and 1 group of children with juvenile chronic arthritis, along with their parents, served as controls. Children and parents individually answered a questionnaire devised for this study. Parents filled out the Child Behavior Checklist and the children's teacher filled out the Teacher's Report Form.

Results

According to test results, children with imperforate anus were happy and optimistic. They liked school better and reported better relationships with schoolmates than the other children. The index group reported statistically significantly more frequent constipation. According to parental responses, the imperforate-anus children suffered from fecal incontinence and odor, as well as constipation (P < .001). Index-group parents reported on the Child Behavior Checklist that their children had more emotional and behavioral problems. On the Teacher's Report Form, teachers reported few problems for the same children.

Conclusions

Patients with imperforate anus did not experience psychosocial impairment despite significant functional problems.     

Imperforate anus, malrotation, and Hirschsprung's disease with double zonal aganglionosis: an extremely rare combination

The author describes an extremely rare case of coexistence with imperforate anus, malrotation, and double zonal aganglionosis.A colostomy was performed on a 2-day-old male infant at the proximal sigmoid colon, because the finding of an invertgram revealed an intermediate type of imperforate anus. At the age of 1 month, a distal colostogram showed the low type without fistula. An upper gastrointestinal series revealed malrotation. At 4 months of age, Ladd's procedure and an anoplasty were done. The function of the colostomy was not good. At laparotomy, a narrowing terminal ileum was removed for being a suspected intestinal obstruction. Histologic findings of the removed ileum revealed aganglionosis. A rectal suction biopsy showed the positive finding of acetyl cholinesterase staining. Therefore, the patient was diagnosed with extensive aganglionosis. At 9 months of age, a 1-stage ileoendorectal pull-through with a right colon onlay patch was performed. Histologically, a skipped ganglionic bowel segment at the right colon, a double zonal aganglionosis, was found among the extensive aganglionosis. Although the ganglionic right colon was used for the colon patch, which was placed for antiperistaltic movement, postoperatively the bowel function was excellent.The author herein describes an extremely rare case of coexistence of imperforate anus, intestinal malrotation, and double zonal Hirschsprung's disease. A case with this association, to the author's knowledge, has not been reported previously in the literature.     

An analysis of ultrasound scanning as a guide in determination of "high" or "low" imperforate anus

The classic upside-down abdominal roentgenogram and the use of pelvic reference points have frequently been inaccurate in assessing the level of the distal pouch in patients with imperforate anus. The ultrasonic study described is different from that previously reported and allows more precise localization of the distal rectal pouch. A review of our experience allows us to make certain definitive statements about this diagnostic modality.     

Polyorchidism in a child with imperforate anus

Polyorchidism is a rare diagnosis. When recovered, it is frequently found in combination with other urologic pathologies. We report the case of a 14-month-old child with imperforate anus who was found to have polyorchia during repair of his inguinal hernia. Although cryptorchidism is not an uncommon finding in patients with imperforate anus, polyorchidism has never been reported. This is an unusual presentation of a rare entity.     

腹腔镜高位肛门闭锁一期成形术4例报告

目的 探讨腹腔镜辅助下高位肛门闭锁一期肛门成形术的可行性。方法先天性高位肛门闭锁患儿4例，男3例，年龄1天～3天；女1例，年龄3个月，为先天性-穴肛畸形。术中首先腹腔镜监视下，游离直肠，分离结扎断离直肠尿道瘘管；然后通过穿刺盆底肌肉中心形成隧道，将直肠从中脱出，与会阴皮肤吻合。结果4例均在腹腔镜辅助下一期完成肛门成形术，腹腔镜手术操作时间40分钟～90分钟，平均718分钟。出血量(5—20)ml，平均8．3ml。无需要输血，未出现术中并发症。所有病人术后恢复顺利，于术后11天去除尿管和膀胱造瘘管，无尿道瘘憩室发生，无切口感染。术后随访3月至1年，I例手术后1月出现肛门狭窄，于术后3月行狭窄段切除术治愈。另外3例无并发症发生，目前4例排便控制功能良好。结论腹腔镜铺助肛门一期成形术是治疗高位肛门闭锁的有效方法，手术打击小，处理直肠泌尿系瘘方便，辨认盆底肌中心准确，可免除患儿分期手术。     

Isolated imperforate anus in monozygotic twins: case report and implications

The authors report a case of isolated imperforate anus with perineal fistula in monozygotic twins. Only 4 other well-documented cases of monozygotic twins concordant for isolated anorectal malformation have been reported in the literature. In all these cases the defect occurred below the levator ani muscle. This case confirms previous reports on the incidence of low anorectal anomalies in monozygotic twins where the defect was isolated. A review of the literature relating to this condition in twins and siblings points toward low anomalies and more severe malformations having different genetic backgrounds.