Long-term results of total colonic agangliosis patients treated by preservation of the aganglionic right hemicolon and the ileo-cecal valve

Abstract

This long-term investigation of TCA patients treated according to Sauer's technique documented a good QoL despite a reduced BFS.

Long‐term outcome and quality of life in patients with total colonic aganglionosis in the Netherlands

“…In our population, 65% of the patients reported constipation, 47% reported faecal incontinence and 53% reported soiling. This is comparable with, or somewhat higher than, reports in other studies .The use of bowel management was reported by 18% of our patients and an adapted diet by 29%. Functional problems were reported more often in questionnaires than in medical records.…”

“…Health‐related QoL in patients with HD has been described in a limited number of studies, often assessed without the use of validated questionnaires . The only study reporting on QoL in patients with TCA, also reported good QoL . Disease‐specific QoL in our cohort was most influenced by frequent complaints of diarrhoea and other physical symptoms and has not been described previously in patients with TCA.…”

“…Children and adolescents with TCA report lower health-related QoL compared with normative data, especially in the physical domain. Health-related QoL in patients with HD has been described in a limited number of studies, often assessed without the use of validated questionnaires [11,[33][34][35][36][37][38][39]. The only study reporting on QoL in patients with TCA, also reported good QoL [11].…”

“…Eighteen (51%) of the 35 questionnaires were returned after a median (range) follow-up period of 11 (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19) years. Of these 18 patients, 10 (56%) were aged 4-11 years, four (22%) were aged 12-17 years and four (22%) were aged 18 years or older.…”

“…The primary treatment of HD is surgical resection of the aganglionic segment and reconstruction of the continuity of the intestinal tract [6][7][8][9][10]. Over the past decades, treatment has been improved and mortality decreased, but patients with TCA often experience functional complaints, such as soiling, faecal incontinence, severe motility problems and enterocolitis [3,[11][12][13][14][15][16][17][18][19]. It is unclear if these functional complaints result in decreased quality of life (QoL) [20].…”