| 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.
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