先天性低位肛门直肠畸形120例临床分析

Clinical analysis of 120 cases of congenital low anorectal malformation

目的总结先天性低位肛门直肠畸形的临床特点,探讨准确诊断及治疗该病的方法。方法回顾性分析2013年1月1日至12月30日该院胃肠新生儿外科收治的120例先天性低位肛门直肠畸形患儿的临床资料,分析其临床表现、诊断方法、伴发畸形、治疗及预后,总结其临床特点。结果 (1)120例先天性低位肛门直肠畸形患儿分为肛门狭窄、肛门闭锁无瘘及肛门闭锁伴瘘3种类型,就诊时间分别为(1.00±1.47)、(102.60±155.20)、(317.60±368.41)d,且3种类型患儿就诊时间比较,差异均有统计学意义(P<0.05)。(2)其中有23例患儿术前行倒立位摄片及会阴部超声检查,测得直肠盲端距肛隐窝的距离为(19.83±16.21)、(9.39±3.65)mm,2种方式比较,差异有统计学意义(P<0.05)。(3)该组病例以伴发先天性心脏结构畸形最常见,其次为泌尿生殖系统畸形。先天性心脏结构畸形合并房间隔缺损(ASD)、动脉导管未闭(PDA)、室间隔缺损(VSD)发生率分别为57.3%(47/82)、45.1%(37/82)、9.8%(8/82),PDA与VSD、ASD与VSD的发病率比较,差异均有统计学意义(P<0.05),但ASD与PDA发病率比较,差异无统计学意义(P>0.05)。(4)120例患儿中110例行经会阴肛门成形术,且均治愈出院;64例患儿术后成功随访,术后无排便意识4例(6.2%),便秘14例(21.9%),粪污3例(4.7%);根据国内评分标准分类,59例(92.2%)为优,4例(6.2%)为良,1例(1.6%)为差。结论先天性低位肛门直肠畸形的就诊时间与其畸形类型有关,且经会阴肛门成形术为主要的手术方式,术后需要进行长期、规范随访,及时处理术后并发症是预防患儿术后排便功能障碍的关键。

Objective To summarize the clinical characteristics of congenital low anorectal malformation and explore the accurate and valuable methods for diagnosis and treatment. Methods The 120 cases of children with congenital low anorec- tal malformation were collected in our hospital, and were reviewed retrospectively to analyze and summarize the clinical manifesta- tions, diagnostic methods,associated anomalies,treatment and prognosis. Results （1）120 cases of children with congenital anorectal malformations were divided into 3 types： low anorectal malformation without fistula, anorectal malformation with fistula and anal stenosis. The age for diagnosis of them were （1.00＋1.47） days, （102.60＋155.20）days and（317.60：t：368.41）days respectively, and there were statistical differences among the three groups （P〈0.05）. （2） There were 23 cases whose distance be- tween the blind end of rectum to the anus were detected by the radiography and perineum ultrasound,and the distance were （19.83~16.21） mm and （9.39~3.65）mm. Through statistical analysis,there were statistical differences between the groups （P〈0.05）. （3） The cardiovascular system malf- ormation were the most common in these cases, follow by urogenital abnormalities. Morever, the incidence of cardiovascular system malf-ormation with ASD, PDA, and VSD were 57.3% （47/82）, 45.5% （37/82） and 9.75% （8/82）, respectively o By statistical analysis of their incidence,there was statistical differences between ASD and VSD （P〈0.05）, as well as PDA and VSD. However, there was no statistical difference of the incidence between ASD and PDA（P〉0.05 ）. （4）110 children who underwent transperineal anorectoplasty were cured after operation. 64 cases were followed up successfully after surgery. 4 cases（6.2%）didn＇t had defecate consciousness, 14 cases （21.9%） had constipation and 3 patients （4.7%）had inconti- nence. According to domestic criteria classification for bowel function, 59 cases （92.2%） were excellent, 4 cases （6.2%） were good and 1 case （1.6%） was poor. Conclusion Clinical manifestations of anorectal malformation were associated with the malformation type and clinic time. The transperineal anorectoplasty is the major surgery method ,and patients after the operation should carry on the long-term follow-up, and get treatment for postoperative complications timely, which is important for preventing the postopera- tive bowel dysfunction.