摘要目的:总结运用腹腔镜辅助下肛门成形术(laparoscopic-assisted anorectal pull-through，LAARP)治疗先天性中、高位型肛门直肠畸形患儿在手术后的短期和长期预后，以及手术相关的经验和方法。方法:收集2001年8月至2019年6月在首都儿科研究所行LAARP术治疗的300例先天性肛门直肠畸形(congenial anorectal malformation，ARM)患儿的临床资料。其中，男294例，女6例；手术年龄为(3.1±0.4)个月，手术年龄范围为8 d至14岁(38例患儿手术年龄<28 d)；高位型174例，中位型126例；直肠膀胱瘘59例，直肠尿道前列腺部瘘99例，直肠尿道球部瘘136例，直肠前庭瘘3例，直肠阴道瘘3例。对79例患儿行传统3孔LAARP术，221例患儿行经脐单孔LAARP术；对38例新生儿行1期LAARP术，余262例患儿行3期LAARP术。结果:所有患儿均无中转开放手术的情况发生。平均手术时间为1.52 h，住院时间为(11.6±1.7) d。所有患儿均无术后伤口感染、尿道损伤、瘘管复发或排尿困难等并发症，术后逆行尿路造影检查提示：1.0%(3/300)的患儿发生尿道憩室，不伴尿路感染、排尿困难、尿道黏膜外翻和尿路结石等并发症；7.7%(23/300)伴直肠黏膜脱垂，需行二次手术治疗；1.0%(3/300)有肛门狭窄，于LAARP术后5个月再次行肛门成形术；0.7%(2/300)有肛门回缩，于LAARP术后5～6 d再次行手术治疗。男性患儿中的高位型和中位型ARM患儿在手术时间(1.78 h比1.95 h)、肛门狭窄发生率(1.9%比1.6%)和尿道憩室发生率(0.7%比1.7%)之间的差异没有统计学意义( P>0.05)。体表电刺激肛穴中心提示：高位型ARM患儿中有27.6%(48/174)能观察到强烈收缩，中位型ARM患儿中有90.5%(114/126)的患儿能观察到强烈收缩，说明这部分ARM患儿的外括约肌和纵肌是发育良好的，而其他收缩不明显且肛穴较浅患儿的外括约肌和纵肌发育不良。高位型ARM患儿的电刺激阳性率明显低于中位型患儿( P<0.001)，男女患儿的肛穴发育情况的差异无统计学意义( P>0.05)。术后随访时间范围为3个月至15年。随访3年以上的228例患儿的中期预后年龄为5.8岁(范围为3.0～18.0岁)，其中95.2%(217/228)的患儿有自发肠蠕动，88.6%(202/228)的患儿未出现污便或1级污便，10.1%(23/228)的患儿有2级污便，5.3%(12/228)的患儿有3级污便；13.2%(30/228)的患儿有1级便秘，11.0%(25/228)的患儿有2级便秘，无患儿伴发3级便秘(Krickenbeck评分)。 结论:腹腔镜对于肛门直肠成形术中游离和切除直肠、关闭瘘管、扩张横纹肌复合体隧道等操作有很大帮助，且LAARP术治疗先天性中、高位型肛门直肠畸形疗效较为确切，短期和长期预后良好。

abstractsObjective:To explore the short/long-term outcomes after laparoscopically assisted anorectal pull-through (LAARP) surgery in children with congenital middle/high-position anorectal malformation and summarize the relevant surgical experiences.Methods:Clinical data were reviewed for 300 children with congenial anorectal malformation (ARM) undergoing LAARP at Capital Institute of Pediatrics from August 2001 to June 2019. There were 294 boys and 6 girls with a mean operative age of (3.1±0.4) months (8 days to 14 years). And 38 cases were aged under 28 days. The clinical type was high (n=174) and median (n=126). There were rectocystic fistula (n=59), rectourethral prostatic fistula (n=99), rectourethral bulbar fistula (n=136), rectovestibular fistula (n=3) and rectovaginal fistula (n=3). Conventional three-incision(n=79) and single-incision (n=221) LAARP was performed. The stage of LAARP was Ⅰ (n=38) and Ⅲ (n=262).Results:None of them were converted into open surgery. The mean operative duration was 1.52 hours and the hospital stay (11.6±1.7) days. There was no onset of such postoperative complications as wound infection, urethral injury, fistula recurrence or dysuria. Postoperative retrograde urography showed that urethral diverticula occurred in 1.0%(3/300) of children, without urinary tract infection, dysuria, urethral mucosa ectrosion or urinary calculus. And 7.7%(23/300) were associated with rectal mucosal prolapse, requiring secondary surgery; 1.0%(3/300) had anal stenosis and underwent anoplasty again at 5 months after LAARP. Anal retraction was observed in 0.7% (2/300) and surgery was performed again at 5-6 days after LAARP. No significant difference existed in operative duration (1.78 vs 1.95 h), incidence of anal stricture (1.9% vs 1.6%) or incidence of urethral diverticulum (0.7% vs 1.7%) among boys with high/median ARM ( P>0.05). Surface electrical stimulation of central anal acupoint indicated that 27.6%(48/174) in children with high ARM and 90.5%(114/126) in children with type ARM displayed strong contraction. Thus this part of ARM with external sphincter and longitudinal muscle was well-developed. And indistinct and shallow contraction denoted dysplasia of external sphincter and longitudinal muscle. The positive rate of electrical stimulation was significantly lower in children with high ARM than that in those with median ARM ( P<0.001) and no statistical difference existed in anal acupoint development between boys and girls ( P>0.05). Postoperative follow-up ranged from 3 months to 15 years. The medium-term prognosis of 228 children followed up for >3 years was 5.8(3-18) years. And 95.2%(217/228) had spontaneous bowel movement, 88.6%(202/228) had no or grade 1 stool, 10.1% (23/228) grade 2 stool, and 5.3% (12/228) grade 3 stool; 13.2% (30/228) grade 1 constipation, 11.0% (25/228) grade 2 constipation and none grade 3 constipation (Krickenbeck score). Conclusions:LAARP is both safe and effective for children with intermediate/high ARMs during operations of rectum dissection, fistula closure and tunneling.