先天性巨结肠手术后患儿远期生活质量的研究
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摘要
目的:先天性巨结肠(Hirschsprung's disease, HD)是一种严重危害婴幼儿健康的先天性畸形,其发病率很高。由于新生儿HD保守治疗两年生存率极低,常通过手术治疗来挽救患儿的生命,但受治疗年龄、病变肠段范围、手术方法、随访时间等因素的影响,手术后远期仍存在手术并发症多和排便习惯与控制不良的问题,影响患儿的生长发育和心理健康,降低生活质量。本课题对所获得随访病例采用临床主观指标,结合直肠肛管测压客观检查方法,对HD术后患儿的生活质量、排便功能和生长发育进行评价,以期通过了解先天性巨结肠术后患儿的生活质量及其影响因素,为采取针对性康复干预措施提供依据。
     方法:2008年7月至2010年2月在山东省立医院复诊的先天性巨结肠根治术患儿73例,均详细填写随访一览表,行腹部查体、肛门指诊、肛管直肠测压。采用白玉作等编制的小儿肛门直肠术后生活质量评分标准,评价患儿生活质量;采用Heikkinen排便功能临床评分标准,评价患儿排便功能;采用WHO推荐NCHS-CDC-WHO(美国国家卫生统计中心-美国疾病控制中心-世界卫生组织)生长发育参考标准,评价患儿生长发育;研究一般资料、排便功能、肛管直肠测压结果、生长发育与生活质量的关系。建立数据库,应用SAS软件进行统计分析。
     结果:
     1.HD术后患儿生活质量评价:优49例(67.12%)、良19例(26.03%)、差5例(6.85%)。不同HD病变范围的患儿生活质量差异有统计学意义,等级相关分析显示,病变范围与生活质量成正相关关系。
     2.患儿排便功能评价及其与生活质量的关系:优33例(45.21%),无任何排便功能障碍;良32例(43.84%),有较轻的排便异常;一般7例(9.59%),有明显的排便功能异常;差1例(1.37%),排便功能严重障碍或完全失禁。排便功能评分与生活质量评分成正相关关系,HD术后患儿的肛管直肠压力变化也影响着生活质量的变化,等级相关分析显示,肛管静息压与生活质量也成正相关关系。逐步回归分析显示,排便功能与肛管静息压对生活质量有显著影响,排便功能对生活质量影响最大,排便功能障碍程度可作为生活质量水平的预测变量。
     3.患儿生长发育评价及其与生活质量的关系:生长发育正常57例(78.08%)、轻度营养不良8例(10.96%)、中度营养不良8例(10.96%)。不同的患儿生活质量差异有统计学意义,等级相关分析显示,生长发育状况与生活质量成正相关关系。
     结论:
     1.先天性巨结肠手术后,有部分患儿生活质量不同程度受损,尤其是病变范围为长段型和全结肠型的患儿。
     2.HD术后部分患儿存在不同程度的排便功能障碍。排便功能障碍对生活质量产生影响最大,障碍程度对生活质量水平具有预测作用;肛管直肠测压中测量肛管静息压有重要的意义,肛管静息压的恢复从侧面反映了生活质量的提高。医院、家庭、社会应该积极采取干预措施,减少排便功能障碍等不良因素对生活质量的影响。
     3.患儿的发育状况大多数良好,但也存在因营养状况差、发育不良而影响生活质量的状况。所以应长期监测患儿生长发育,给予针对性的营养治疗,改变其生活状态,提高生活质量。
Objective:Hirschsprung's disease is a sort of congenital malformations that threaten the health of children seriously. The disease has a high morbidity. After conservative treatments, neonatal with HD has an extremely low two-year survival rate, their lives have to be saved by surgical operation. But due to variations of age at operation, their ages, the length of aganglionosis colon, the types of operations and time of follow-up, the long-term postoperative effect is remained ascribed to complications and poor bowel evacuation habit. That will affect children's growth, development and mental health, and decrease their quality of life. This study evaluated the quality of life, defecation function, growth and development in children with HD through clinical subjective indexes and anorectal manometry postoperatively, so as to understand the status of quality of life and its affecting factors in children with HD after operation, and provide evidence for taking some measures to improve their quality of life.
     Methods:73 cases that underwent operations for HD in the Department of Pediatric Surgery in Shandong Provincial Hospital were followed up. The study was undertaken from July 2008 to February 2010. And the clinical manifestations were recorded using the standard questionnaires in detail. Abdominal examination, anorectal manometry and anal examinations were applied. The standard quality of life scoring systems developed by Bai Yu-zuo was used to evaluate the quality of life. The Heikkinen standard qualitative clinical scoring systems were used to evaluate the defecation function. And the NCHS-CDC-WHO child growth standards were used to evaluate the growth and development. We further explored the relationship between the age of follow-up, the length of aganglionosis colon, defecation function, the results of anorectal manometry, growth and development and quality of life. S AS software was used in statistical analysis.
     Results:
     1. In 73 cases,49 (67.12%) of them were in excellent quality of life,19 (26.03%) in good,5 (6.85%) in poor. The differences of quality of life had statistical significance depending on the length of aganglionosis colon. Spearman's rank correlation analysis showed that the length of aganglionosis colon was correlated positively to the quality oflife.
     2. In 73 cases,33 (45.21%) of them were excellent in postoperative defecation function, and with normal bowel habit; 32 (43.84%) were good, and with minor abnormal bowel habit; 7 (9.59%) were fair, and with marked abnormal bowel habit; and 1 (1.37%) was poor and with serious abnormal bowel habit or defecation incontinence. The defecation function was correlated positively to the quality of life. There was statistical significance in postoperative anal rest pressure among different quality of life classes, and Spearman's rank correlation analysis showed that the postoperative anal rest pressure was correlated positively to the quality of life. Stepwise regression analysis indicated that both defecation function and anal rest pressure could predict the quality oflife level in children with HD, and the defecation dysfunction was the most important predictor of the quality oflife.
     3. In 73 cases,57 (78.08%) of them were in normal growth and development,8 (10.96%) in mild malnutrition,8 (10.96%) in moderate malnutrition. The differences of quality of life had statistical significance in children with different growth and development level. And Spearman's rank correlation analysis showed that the growth and development was correlated positively to the quality oflife.
     Conclusions:
     1.Some children with HD had some extent of quality of life deterioration after operation, especially in long and whole segment of aganglionosis colon.
     2.Some children with HD had some extent of defecation dysfunction after operation. The defecation dysfunction was the most important predictor of their quality of life. Anorectal manometry was also essential to predict the quality of life in children with HD, the recovery of anal rest pressure reflected the improvement of quality of life. Hospitals, families and society should take some positive measures to reduce the influence of harmful factors on quality of life such as defecation function.
     3. Most children with HD developed good, but some still had bad nutrition and development, which affected their quality of life badly. So we should take long-term growth condition monitoring, provide nutrition therapy to change their growth and development, further to improve their quality of life.
引文
[1]De Lorijn F, Boeckxstaens GE, Benninga MA. Symptomatology, pathophysiology, diagnostic work-up, and treatment of Hirschsprung disease in infancy and childhood[J]. Curr Gastroenterol Rep,2007,9(3):245-253.
    [2]张金哲,潘少川,黄澄如.实用小儿外科学(M).杭州:浙江科学技术出版社,2003:789—811.
    [3]杨烨,侯豫,赵新,等.先天性巨结肠肠壁Cx43蛋白和Cajal间质细胞的分布[J].中国当代儿科杂志,2009,11(3):213-216.
    [4]陈雷铃,胡廷泽.先天性巨结肠与肠神经系统.中华小儿外科杂志.2001,22(2):126-128.
    [5]Emison ES,McCallion AS,Kashuk CS.A common sex-dependent mutation in a RET enhancer underliea Hirschsprung disease risk[J]. Nature,2005,434 (7035):857-683.
    [6]Torre DL,Ortega-Salgado JA. Transanal endorectal pull-through for Hirschsprung's disease (J).J Pediatr Surg,1998,33(8):1283-1286.
    [7]Georgeson KE, MM Fuenfer, Hardin WD, et al. Primary laparoscpic pull-through for Hirschsprung's disease in infants and children (J). J Pediatr Surg,1995,30(7):1017-1022.
    [8]Moore SW, Albertyn R, Cywes S. Clinical outcome and long-term quality of life after surgical correction of Hirschsprung's disease (J). J Pediatr Surg,1996, 31:1496-1502.
    [9]Gad El-Hak NA, El-Hemaly MM, Negm EH, et al.Functional outcome after Swenson's operation for Hirshsprung's disease (J).Saudi J Gastroenterol,2010, 16(1):30-34.
    [10]Bai YZ, Chen H, Hao J, et al. Long-term outcome and quality of life after the Swenson procedure for Hirschsprung's disease (J). J Pediatr Surg,2002, 37:639-642
    [11]陈新国,郭宗远,刘润玑,等.先天性巨结肠Duhamel术后远期功能评价(J). 中华小儿外科杂志,2005,26:60—64
    [12]王维林,郝晶,白玉作,等.先天性巨结肠Swenson改良术后排便功能与生活质量研究(J).中华小儿外科杂志,2003,24(2):129—132.
    [13]Saleh W, Rasheed K, Mohaidly MA, et al.Management of Hirschsprung's disease:A comparison of Soave's and Duhanmel's pull-through methods (J).Pediatr Surg Int,2004,8:590-593.
    [14]张金哲.小儿肛肠疾病治疗进展(J).临床小儿外科杂志.2007,6(2):1—2.
    [15]王维林.小儿排便障碍性疾病的诊断与治疗(M).北京:人民卫生出版社,2002,7:251-259.
    [16]Yanchar NL, Soucy P. Long-term outcome after Hirschsprung's disease:Patients' Perspectives (J).J Pediatr Surg,1999,34:1152-1160.
    [17]Niramis R, Watanatittan S, Anuntkosol M, et al. Quality of life of patients with Hirschsprung's disease at 5-20 years post pull-through operations (J). Eur J Pediatr Surg,2008,18(1):38-43.
    [18]Hartman EE, Oort FJ, Aronson DC, et al. Critical factors affecting quality of life of adult patients with anorectal malformations or Hirschsprung's disease (J). Am J Gastroenterol,2004,99:907-913.
    [19]DisethTH,Bjornland K.Bowel function,mental health,and psychosocial function in adolescents with Hirschsprung's disease (J). Arch Dis Child,1997,76:100-106.
    [20]Ludman L, Spitz L, Tsuji H, et al. Hirschsprung's disease:Functional and psychological follow up comparing total colonic and rectosigmoid aganglionsis (J).Arch Dis Child,2002,86:348-351.
    [21]Tohoku. Social adaptation of children with congenital fecal dysfunction:From the viewpoint of the mother-child relationship (J). J Exp Med,2005,206:117-124
    [22]Hartman EE, Oort FJ, Aronson DC. Explaining change in quality of life of children and adolescents with anorectal malformations or Hirschsprung disease (J).Pediatrics,2007,119(2):e374-383
    [23]Hartman EE, Oort FJ, Sprangers MA; Hanneman MJ, et al. Factors affecting quality of life of children and adolescents with anorectal malformations or Hirschsprung disease (J). J Pediatr Gastroenterol Nutr,2008,47 (4):463-471.
    [24]李正,王慧贞,吉士俊.实用小儿外科学(M).北京:人民卫生出版社,2001,1:797.
    [25]林霄,龙莉铃.先天性巨结肠研究进展[J].实用医技杂志,2009,16(5):367-369.
    [26]杜鹏,金先庆.儿童先天性巨结肠手术的治疗进展[J].重庆医学,2009,15(8):1967-1970.
    [27]高亚,李恭才,张宪生,等.Ⅰ期经肛门巨结肠根治术15例报告[J].中华小儿外科杂志,2001,22(1):21-23.
    [28]Ghirardo V, Betalli P, Mognato G, et al.Laparotomic versus laparoscopic Duhamel pull-through for Hirschsprung's disease in infants and children[J].J Laparoendosc Adv Surg Tech A.2007,17(1):119-123.
    [29]汤绍涛,周欣,阮庆兰,等.先天性巨结肠术后发生小肠结肠炎的高危因素[J].中华小儿外科杂志,2001,8(4):213.
    [30]Menezes M, Puri P. Long-term outcome of patients with enterocolitis complicating Hirschsprung's disease[J]. Pediatr Surg Int.2006,22(4):316-318.
    [31]王忠荣,张敬杰,徐兵.幼兔灌肠与肠道细菌移位关系的实验研究[J].安徽医科大学学报,2004,39(1):40-42.
    [32]汤绍涛,刘春萍,阮庆兰,等.先天性巨结肠并发小肠结肠炎肠道免疫功能的变化[J].中华小儿外科杂志,1999,20(1):18-20.
    [33]崔清波,李昭铸,郭俊斌.先天性巨结肠根治术后并发症的防治[J].哈尔滨医科大学学报,2006,40(6):516-517.
    [34]温哲,张道荣,牛爱国,等.先天性巨结肠改良Duhamel术式治疗后远期生活质量评估及对策[J].中华小儿外科杂志,2001,22(5):271-273.
    [35]古立暖,刘桂海,陈新国,等.先天性巨结肠经肛门结肠拖出术后结肠形态及排便功能观察[J].中华小儿外科杂志,2003,24(5):475-476.
    [36]余世耀,施诚仁,励楚刚,等.先天性巨结肠经肛门一期拖出术的评估[J].上海第二医科大学学报2005,25(1):80-81.
    [37]Kubota M, Kamimura T, Suita S.External anal sphincter dysfunction and postoperative bowel habits of patients with Hirschsprung's disease[J].J Pediatr Surg,1997,32:22-24.
    [38]Bradlyn AS, Ritchey AK, Harris CV, et al.Quality of life research in pediatric oncology.Research metheds and barriers[J].Cancer,1996:78(6):1333-1339.
    [39]Catto-Smith AG, Trajanovska M, Taylor RG Long-term continence after surgery for Hirschsprung's disease (J).J Gastroenterol Hepatol,2007,22(12): 2273-2282.
    [40]Ruttenstock E,Puri P. A meta-analysis of clinical outcome in patients with total intestinal aganglionosis (J). Pediatr Surg Int,2009,25 (10):833-839.
    [41]Zhang SC,Bai YZ, Wang W,et al.Clinical outcome in children after transanal 1-stage endorectal pull-through operation for HD (J). J Pediatr Surg,2005, 40:1307-1311.
    [42]E1-Sawaf MI, Drongowski RA, Chamberlain JN, et al.Are the long-term results of the transanal pull-through equal to those of the transabdominal pull-through? A comparison of the 2 approaches for Hirschsprung's disease[J]. J Pediatr Surg, 2007,42(1):41-47.
    [43]Shu-Cheng Zhang, M.D., Yu-Zuo Bai, M.D., Wei Wang, M.D, et al.Long-term outcome, colonicmotility, and sphincter performance after Swenson's procedure for HirschsPrung's disease: single-center 2-decade experience with 346 cases[J].Am J Surg,2007,194(1):40-47.
    [44]Skaba R, Simsora M. Long-term results and quality of life in patients after surgery in childhood for Hirschsprung's disease by the Kasai method of colo(ileo) rectoplasty[J]. Rozhl Chir,2002,81:622-627.
    [45]Saleh W, Rasheed K, Mohaidly MA, et al. Management of Hirschsprung's disease:A comparison of Soave's and Duhamel's pull-through methods[J]. Pediatr Surg Int,2004,8:590-593.
    [46]郝晶,李旭、李心元.先天性巨结肠术后远期排便功能与生活质量[J].临床儿科杂志,2004,22(6):373-375.
    [47]周雪鸿,苏泽礼,单振潮,等.经肛门Ⅰ期先天性巨结肠根治术后排便功能及生活质量的研究[J].宁夏医学杂志,2008,30(10):871-873.
    [48]ZHANG Shu-Cheng, WANG Wei-Lin, BAI Yu-Zuo, et al. Evaluation of anorectal function after transanal one-stage endorectal pull through operation in children with Hirschsprung's disease[J]. Chin J Contemp Pediatr,2007,9(3):188-192.
    [49]陈新国,古立暖,刘桂海,等.先天性巨结肠手术后排便造影的临床观察[J].中华普通外科杂志,2003,18:616-619.
    [50]Huang Y,Zheng S,Xiao X.Preliminary evaluation of anorectal manometry in diagnosing Hirschsprung's disease in neonates[J]. Pediatric Surg Int,2009,25(1):41-45.
    [51]江米足,周雪莲,徐珊,等.肛门直肠测压法在小儿先天性巨结肠诊断中的应用[J].浙江大学学报(医学版),2001,30:178-179.
    [52]Keshtgar AS, Ward HC, Clayden GS. Investigations for incontinence and constipation after surgery for HirschsprunKs disease in children[J]. Pediatr Surg Int,2003,19:4-8.
    [53]张树成,王维林,白玉作,等.经肛巨结肠根治术后肛肠功能评价[J].中华小儿外科杂志,2006,27(3):132-136.
    [54]Keshtgar AS,Ward HC,Clayden GS.Diagnosis and management of children with intractable constipation[J].Seminars inpediatric surgery,2004,13:300-309.
    [55]Zaslavsky C,Loening B.Anorectal manomeytic valution of children and adolescents postsurgery for Hirschsprung's disease[J].J Pediatr Surg,2003,38: 191-195.
    [56]Van Leeuwen K, Geiger JD, Barnett JL, et al. Stooling and manometric findings after primary pull-throughs in Hirschsprung's disease:Perineal versus abdominal approaches[J].J Pediatr Surg,2002,37(9):1321-1325.
    [57]WHO Multicentre Growth Reference Study Group:Assenssment of differences in linear growth among populatons in the WHO Multicentre Growth Reference Study. Acta Paediatr Suppl 2006; 450:56-65.
    [58]Berthold Koletzko, Peter Cooper,王卫平,等.临床儿科营养(M).北京:人民卫生出版社,2009,11:5-68.
    [59]何朝升,陈新国,古立暖,等.先天性巨结肠经肛拖出加内括约肌部分切除术后 排便功能研究[J].中国现代普通外科进展,2008,11(2):145-148.
    [60]Bai Yuzuo, Yuan Zhengwei, Wang Weilin, et al. Quality of life in children with fecal incontinence after surgically corrected anorectal malformation (J). J Pediatr Surg,2000,35:462-464.
    [61]Heikkinen M, Rintala R, Luukkonen Helsinki P. Long-term anal sphincter performance after surgery for Hirschsrung's disease (J). J Pediatr Surg, 1997,32(10):1443-1446.
    [62]WHO/The WHO Child Growth Standards [EB/OL]. http://www.who.int/child growth/en/.2006,2007.
    [63]孟广冻.泌尿外科症状学:吴阶平.泌尿外科(M).济南:山东科学技术出版社,2000,44.
    [64]薛兆英.男性性功能障碍:吴阶平.泌尿外科(M).济南.山东科学技术出版社,2000,688.
    [65]张虹,江布先,杨传民,等.先天性巨结肠根治术后并发症与术式的关系(J)山东医药,1999;39(14):3-4.
    [66]Obermayr F,Szavay P,Beschorner R,et al.Outcome of transanal endorectal pull-through in patients with Hirschsprung's disease (J).Eur J Pediatr Surg,2009,19(4):220-223.
    [67]Dronov AF,Smirnov AN,Zalikhin DV,et al.Video-assisted surgery of Hirschsprung's disease in children. Khirurgiia(Mosk),2009(6):50-53.
    [68]Mills JL,Konkin DE,Milner R,et al.Long-term bowel function and quality of life in children with Hirschsprung's disease (J). J Pediatr Surg,2008,43(5):899-905.
    [69]Levitt MA,Dickie B,Pena A.Evaluation and treatment of the patient with Hirschsprung's disease who is not doing well after a pull-through procedure (J). Semin Pediatr Surg,2010,19(2):146-153.
    [70]Peni Prato A,Gentilino V,Giunta C,et al. Hirschsprung disease:do risk factors of surgical outcome exist? (J) J Pediatr Surg,2008,43(4):612-619.
    [71]Taguchi T,Ieiri S. Hirschsprung's disease (J).Nippon Geka Gakkai Zasshi,2009,110(4):184-190.
    [72]Levitt M,Pena A.Update on pediatric faecal incontinence (J).Eur J Pediatr Surg,2009,19(1)1-9.

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