Long-term Outcome of Hirschsprung Disease: Impact on Quality of Life and Social Condition at Adult Age

Abstract

BACKGROUND: 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. OBJECTIVE: This study aimed to assess the quality of life and social outcome of adult patients operated on for Hirschsprung disease during childhood. DESIGN: Patients operated on for Hirschsprung disease during childhood were identified and specific questionnaires were sent to them. SETTINGS: Data from 2 referral centers were used. PATIENTS: Patients who completed the questionnaires regarding quality of life and social condition were included. MAIN OUTCOME MEASURES: 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. RESULTS: 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. LIMITATIONS: This study had the limitations inherent to a retrospective study. CONCLUSION: 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.

Pediatric Colorectal Disorders

“…Adult outcomes in HD are still being elucidated. The main long-term concerns are fecal incontinence which is reported in around 15% and constipation in about 5% [12][13][14][15][16]. Interestingly, bowel quality of life does not seem to be compromised despite these long-term complications of the disease [13][14][15].…”

“…The main long-term concerns are fecal incontinence which is reported in around 15% and constipation in about 5% [12][13][14][15][16]. Interestingly, bowel quality of life does not seem to be compromised despite these long-term complications of the disease [13][14][15]. Concerns are higher in those who had longer-segment disease (i.e., standard rectosigmoid aganglionosis vs total colonic aganglionosis) [15].…”

“…Interestingly, bowel quality of life does not seem to be compromised despite these long-term complications of the disease [13][14][15]. Concerns are higher in those who had longer-segment disease (i.e., standard rectosigmoid aganglionosis vs total colonic aganglionosis) [15]. Also, functionality worsens with increasing age [13,15], highlighting the importance of care for the aging HD patient.…”

“…Concerns are higher in those who had longer-segment disease (i.e., standard rectosigmoid aganglionosis vs total colonic aganglionosis) [15]. Also, functionality worsens with increasing age [13,15], highlighting the importance of care for the aging HD patient. Sacral nerve stimulation is evolving as an adjunctive treatment in some selectively chosen patients.…”

Associations ofSLC6A20genetic polymorphisms with Hirschsprung’s disease in a Southern Chinese population

“…Persistent constipation, chronic abdominal distension, loss of appetite and failure to thrive are common symptoms in older children [5]. Timely surgical treatment to remove the affected bowel segment can save most patients’ lives, but obvious postoperative complications, such as fecal incontinence, constipation, repeated enterocolitis, nutrition disorder and so on [7], could bring the patients and their families severe economic burden and great psychological pressure [8], which might affect their quality of life in adulthood [9,10]. Even more, severe complications like HSCR-related short bowel syndrome could also kill the patients after surgery [11].…”