青少年巨结肠症患者在达到 18 岁或以上后的长期预后和生活质量——47 年单中心经验。

Long-term outcomes and the quality of life of Hirschsprung disease in adolescents who have reached 18 years or older--a 47-year single-institute experience.

机构信息

Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan.

出版信息

J Pediatr Surg. 2010 Dec;45(12):2398-402. doi: 10.1016/j.jpedsurg.2010.08.040.

DOI:10.1016/j.jpedsurg.2010.08.040

PMID:21129554

Abstract

BACKGROUND AND AIMS

Hirschsprung disease is considered to be curable when performing definitive surgery. However, the postoperative bowel function is not always satisfactory. The long-term postoperative function of bowel evacuation and the quality of life of such patients are considered to be important. In this study, we evaluated the general condition, bowel function, and social performance in adults who were older than 17 years who had suffered from Hirschsprung disease in childhood.

METHODS

From 1963 to 2009, 184 patients with Hirschsprung disease underwent definitive surgery, mostly Z-shaped anastomosis at Kyushu University Hospital. As a result, 146 (95.4%) of 153 of those patients survived and reached 17 years of age. Their present status and symptoms, anorectal functions, genitourinary functions, and social performance were evaluated during the clinical follow-up based on a questionnaire survey.

RESULTS

In our series, the evacuation score was rated as "excellent" (score of 7-8/8) in 66.7%, "good" (5-6/8) in 19.0%, "fair" (3-4/8) in 11.9%, and "poor" (0-2/8) in 2.4%. Therefore, 85.7% were considered to have a satisfactory bowel function. However, only 21.4% had a completely normal score of 8/8. Incontinence occurred in 16.7%, and soiling was present in 19.0% of the questionnaire respondents. The genitourinary function was considered to be within the reference range, and urinary problems were minimal. Among the respondents, 45.2% were married, and 68.4% of those married had children. The educational and professional careers of the respondents were successful.

CONCLUSIONS

In general, the bowel function was satisfactory. However, the ratio of patients with completely normal bowel function was low. Because incontinence and soiling impair the quality of life, pediatric surgeons should therefore continue trying to achieve a complete bowel function after definitive surgery for the treatment of Hirschsprung disease.

摘要

背景与目的

巨结肠症施行确定性手术后，被认为可以治愈。然而，术后的肠道功能并不总是令人满意。因此，长期的术后排便功能和这些患者的生活质量被认为是重要的。在这项研究中，我们评估了在童年患有巨结肠症且年龄大于 17 岁的成年人的一般情况、肠道功能和社会表现。

方法

1963 年至 2009 年，184 例巨结肠症患者在九州大学医院接受了确定性手术，主要是 Z 形吻合术。结果，153 例患者中有 153 例（95.4%）存活并达到 17 岁。通过问卷调查，对他们目前的状况和症状、肛肠功能、泌尿生殖功能和社会表现进行了临床随访评估。

结果

在我们的系列中，排便评分评定为“优秀”（7-8/8 分）占 66.7%，“良好”（5-6/8 分）占 19.0%，“中等”（3-4/8 分）占 11.9%，“差”（0-2/8 分）占 2.4%。因此，85.7%的患者被认为具有满意的肠道功能。然而，仅有 21.4%的患者获得完全正常的 8/8 分。失禁的发生率为 16.7%，问卷调查应答者中有 19.0%存在大便污染。泌尿生殖功能被认为在参考范围内，且排尿问题很少。应答者中 45.2%已婚，68.4%已婚者有子女。应答者的教育和职业生涯是成功的。