Sawicka Ewa
Klinika Chirurgii Dzieci i Młodziezy, Instytut Matki i Dziecka, ul. Kasprzaka 17a, 01-211 Warszawa, Poland.
Med Wieku Rozwoj. 2005 Oct-Dec;9(4):695-726.
Anorectal malformations constitute the most varied group of congenital defects. Constant challenge for the surgeon being engaged in this subject are the studies of new factors which could improve the functional results of treatment.
The aim of the study was the evaluation of late results after surgical treatment in children with anorectal anomalies. Assessment of the qualification criteria for the kind of surgical repair: one-stage (OSR) or stage repair (SR), age of the patient at the end of treatment and accompanying sacral bone defects were taken into consideration as the factors of functional prognosis.
93 children with different types of anorectal defects were operated on between 1990-2002 in Department of Pediatric Surgery in the Institute of Mother and Child. The evaluation was performed in 60 patients over 3 years old: in 30 after OSR (group I) and in 30 after SR proceeded by colostomy (group II). Anorectal malformations were classified according to Pena's division based on the place of fistula orifice. Most kinds of anorectal anomalies were operated on according to the principles of posterior sagital anorectoplasty (PSARP). In patients with congenital anal stenosis a modified cutback procedure was done. In long-term evaluation in every child precise diagnostics of sacral bone with definition of congenital sacral bone defects and sacral measurements were performed, in order to estimate their influence on the results of treatment of anorectal anomalies. In postoperative evaluation of functional results the following criteria were taken into consideration: physical examination, evaluation of fecal continence by using quantitative scoring method and manometric study. The control group included 20 children with constipation.
Sacral bone defects were diagnosed in 10% of the OSR group (group I) and in 73% of patients of the SR group (group II). Sacral measurements showed significant differences in SR group compared with OSR and control group. In clinical examination postoperative appearance of perineal and anal region depended on congenital development of perineal structure. In ST group of patients symptoms of 'flat perineum' were seen with various degree of muscles hypodevelopement, lack of perineal raphe and weakly marked anal dimple. Results of fecal continence in OSR group (group I) were defined as normal in 93% of patients and good in 7% of cases. In SR group (group II) the results were as following: normal in 41%, good in 17%, fair in 38% and poor in 4% of cases. The worst results were obtained in the patients with sacral defects in SR group. In manometric study there were no significant differences except for one parameter (anal resting pressure) between OSR group (group I) and the control group. In group II manometric study was different in three parameters: anal resting pressure, squeezing pressure and presence of internal sphincter reflex. The presence of sacral bone defects had the most important influence on manometric study results.
Functional results in the OSR group of patients were not different from the control group. Precise criteria for this kind of surgical treatment and lack of serious sacral defects were the most important factors for good prognosis. The results of late evaluation in SR group depended on coexisting sacral bone agenesis.
肛门直肠畸形是先天性缺陷中种类最多样的一组疾病。对于从事该领域的外科医生而言,持续面临的挑战是研究能够改善治疗功能效果的新因素。
本研究的目的是评估肛门直肠畸形患儿手术治疗后的远期效果。将手术修复方式(一期修复或分期修复)、治疗结束时患者的年龄以及伴随的骶骨缺损等作为功能预后的因素,对手术修复类型的资格标准进行评估。
1990年至2002年期间,在母婴研究所儿科外科对93例不同类型肛门直肠缺陷的患儿进行了手术。对60例3岁以上的患者进行了评估:30例接受一期修复(第一组),30例接受分期修复并行结肠造口术(第二组)。根据瘘口位置,按照佩纳分类法对肛门直肠畸形进行分类。大多数肛门直肠畸形病例按照后矢状位肛门直肠成形术(PSARP)的原则进行手术。对于先天性肛门狭窄的患者,采用改良的回切手术。在长期评估中,对每个患儿进行精确的骶骨诊断,明确先天性骶骨缺损和骶骨测量值,以评估其对肛门直肠畸形治疗结果的影响。在术后功能结果评估中,考虑以下标准:体格检查、采用定量评分法评估大便失禁情况以及测压研究。对照组包括20例便秘患儿。
第一组(一期修复组)10%的患者诊断出骶骨缺损,第二组(分期修复组)73%的患者诊断出骶骨缺损。与一期修复组和对照组相比,分期修复组的骶骨测量值存在显著差异。在临床检查中,会阴和肛门区域的术后外观取决于会阴结构的先天性发育情况。在分期修复组的患者中,可见不同程度肌肉发育不全、会阴缝缺失和肛门酒窝不明显的“扁平会阴”症状。一期修复组(第一组)93%的患者大便失禁结果被定义为正常,7%的病例为良好。在分期修复组(第二组),结果如下:41%为正常,17%为良好,38%为尚可,4%为较差。分期修复组中骶骨缺损患者的结果最差。在测压研究中,除一个参数(肛门静息压)外,一期修复组(第一组)与对照组之间无显著差异。在第二组中,测压研究在三个参数上有所不同:肛门静息压、挤压压和内括约肌反射的存在。骶骨缺损的存在对测压研究结果影响最大。
一期修复组患者的功能结果与对照组无差异。这种手术治疗的精确标准以及无严重骶骨缺损是良好预后的最重要因素。分期修复组的远期评估结果取决于并存的骶骨发育不全情况。