Clinique de Chirurgie Digestive et Endocrinienne (CCDE), Institut des Maladies de l'Appareil Digestif (IMAD), University Hospital of Nantes, Nantes, France.
EA 4725 SPHERE "methodS in Patient-centered outcomes & HEalth ResEarch," University of Nantes, Nantes, France.
Dis Colon Rectum. 2019 Jun;62(6):727-732. doi: 10.1097/DCR.0000000000001363.
Hirschsprung disease is a rare congenital disease typically requiring surgical treatment during childhood. Quality of life and social condition at adult age can be impaired by disease-specific sequelae.
This study aimed to assess the quality of life and social outcome of adult patients operated on for Hirschsprung disease during childhood.
Patients operated on for Hirschsprung disease during childhood were identified and specific questionnaires were sent to them.
Data from 2 referral centers were used.
Patients who completed the questionnaires regarding quality of life and social condition were included.
The Hirschsprung's Disease and Anorectal Malformations Quality of Life disease-specific questionnaire (8 dimensions explored; each scored from 0 to 100 maximum score) and a sociodemographic questionnaire were sent to identified patients. Sociodemographic data were compared with those of the French general population.
Thirty-four patients had Hirschsprung disease (men, 76%; mean age, 32 years) were included in the study. Mean total Hirschsprung's Disease and Anorectal Malformations Quality of Life score was 611 of 800 (maximum score 800). The 2 most impaired dimensions were "physical symptoms" and "diarrhea" (62.9/100 and 73.6/100). Fecal continence was only marginally affected (mean score, 89/100). Patients with Hirschsprung disease achieved better educational levels than the French general population. Parental and marital status did not differ between the 2 groups.
This study had the limitations inherent to a retrospective study.
The quality of life of adult patients with Hirschsprung disease sequelae is marginally impaired in this study. Despite the consequences of this congenital abnormality, the condition eventually achieved can be considered as satisfactory. See Video Abstract at http://links.lww.com/DCR/A917.
先天性巨结肠是一种罕见的疾病，通常需要在儿童时期进行手术治疗。疾病的特定后遗症可能会影响成年后的生活质量和社会状况。
本研究旨在评估儿童期接受巨结肠手术的成年患者的生活质量和社会结局。
确定儿童期接受巨结肠手术的患者，并向他们发送特定的问卷。
使用 2 个转诊中心的数据。
完成生活质量和社会状况问卷的患者被纳入研究。
采用先天性巨结肠和肛门直肠畸形生活质量问卷（8 个维度，每个维度评分范围为 0 至 100，最高 800 分）和社会人口学问卷评估患者。将社会人口学数据与法国一般人群进行比较。
本研究纳入了 34 例男性（76%）先天性巨结肠患者，平均年龄为 32 岁。平均总先天性巨结肠和肛门直肠畸形生活质量得分为 800 分中的 611 分（最高 800 分）。受影响最严重的 2 个维度是“躯体症状”和“腹泻”（62.9/100 和 73.6/100）。粪便控制仅轻度受损（平均得分为 89/100）。巨结肠患者的受教育程度高于法国一般人群。父母和婚姻状况在两组间无差异。
本研究存在回顾性研究固有的局限性。
本研究中，先天性巨结肠后遗症患者的生活质量仅略有受损。尽管存在这种先天性异常的后果，但最终的病情可以被认为是满意的。
观看视频摘要请访问 http://links.lww.com/DCR/A917。
