Department of Surgery, Division of Pediatric Surgery.
Department of Surgery, Anorectal Physiology Laboratory, University of Groningen, University Medical Center Groningen, Groningen.
J Pediatr Gastroenterol Nutr. 2022 Mar 1;74(3):348-354. doi: 10.1097/MPG.0000000000003355.
Knowledge on long-term outcomes in patients with Hirschsprung disease is progressing. Nevertheless, differences in outcomes according to aganglionic lengths are unclear. We compared long-term bowel function and generic quality of life in Hirschsprung patients with total colonic or long-segment versus rectosigmoid aganglionosis.
In this nationwide, cross-sectional study participants with proven Hirschsprung disease received the Defecation and Fecal Continence questionnaire, and the Child Health Questionnaire Child Form-87, or the WHO Quality of Life-100. We excluded deceased patients, patients who were younger than 8 years, lived abroad, had a permanent enterostomy, or were intellectually impaired.
The study population (n = 334) was operated for rectosigmoid (83.9%), long-segment (8.7%), or total colonic aganglionosis (7.5%). Fecal incontinence in general was not significantly different between the three groups, but liquid fecal incontinence was significantly associated with total colonic aganglionosis (odds ratio [OR] = 6.00, 95% confidence interval [CI] 2.07-17.38, P = 0.001). Regarding constipation, patients with total colonic or long-segment aganglionosis were less likely to suffer from constipation than the rectosigmoid group (OR = 0.21, 95% CI, 0.05-0.91, P = 0.038 and OR = 0.11, 95% CI, 0.01-0.83, P = 0.032). Quality of life was comparable between the three groups, except for a lower physical score in children with total colonic aganglionosis (P = 0.016).
Over time Hirschsprung patients with total colonic or long-segment aganglionosis do not suffer from worse fecal incontinence in general. A difference in stool consistency may underlie the association between liquid fecal incontinence and total colonic aganglionosis and constipation in patients with rectosigmoid aganglionosis. Despite these differences, generic quality of life is comparable on reaching adulthood.
关于先天性巨结肠患者长期预后的知识正在不断发展。然而，无神经节细胞肠段长度不同导致的结局差异尚不清楚。我们比较了全结肠型或长段型与直肠乙状结肠型先天性巨结肠患者的长期肠道功能和一般生活质量。
在这项全国性的横断面研究中，经证实患有先天性巨结肠的患者接受了排便和粪便控制问卷以及儿童健康问卷儿童 87 项（Child Health Questionnaire Child Form-87，CHQ-CF87）或世界卫生组织生活质量量表 100 项（WHO Quality of Life-100）。我们排除了死亡患者、年龄小于 8 岁的患者、居住在国外的患者、永久性肠造口术患者和智力障碍患者。
研究人群（n=334）接受手术的类型为直肠乙状结肠型（83.9%）、长段型（8.7%）或全结肠型无神经节细胞肠段（7.5%）。三组患者一般粪便失禁无显著差异，但液体粪便失禁与全结肠型无神经节细胞肠段显著相关（比值比[OR] = 6.00，95%置信区间[CI] 2.07-17.38，P=0.001）。在便秘方面，全结肠型或长段型无神经节细胞肠段患者发生便秘的可能性明显低于直肠乙状结肠型患者（OR=0.21，95%CI 0.05-0.91，P=0.038 和 OR=0.11，95%CI 0.01-0.83，P=0.032）。三组患者的生活质量相当，除了全结肠型无神经节细胞肠段患儿的身体评分较低（P=0.016）。
随着时间的推移，全结肠型或长段型无神经节细胞肠段先天性巨结肠患者一般不会出现更严重的粪便失禁。大便稠度的差异可能是直肠乙状结肠型无神经节细胞肠段患者液体粪便失禁和便秘与全结肠型无神经节细胞肠段相关的原因。尽管存在这些差异，但成年后一般生活质量相当。
