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Long-term Outcome of Hirschsprung Disease

Impact on Quality of Life and Social Condition at Adult Age

Drissi, Farouk M.D.1; Meurette, Guillaume M.D., Ph.D.1; Baayen, Corine Ph.D.2; Wyart, Vincent1; Cretolle, Célia M.D., Ph.D.3; Guinot, Audrey M.D.4; Podevin, Guillaume M.D., Ph.D.5; Lehur, Paul-Antoine M.D., Ph.D.1

Diseases of the Colon & Rectum: June 2019 - Volume 62 - Issue 6 - p 727–732
doi: 10.1097/DCR.0000000000001363
Original Contributions: Benign
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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.

1 Clinique de Chirurgie Digestive et Endocrinienne (CCDE), Institut des Maladies de l’Appareil Digestif (IMAD), University Hospital of Nantes, Nantes, France

2 EA 4725 SPHERE “methodS in Patient-centered outcomes & HEalth ResEarch,” University of Nantes, Nantes, France

3 Service de Chirurgie pédiatrique, Hôpital Necker-Enfants Malades, University of Paris-Descartes, Paris, France

4 Service de Chirurgie pédiatrique, University Hospital of Nantes, Nantes, France

5 Service de Chirurgie pédiatrique, University Hospital of Angers, Angers, France

Funding/Support: None reported.

Financial Disclosure: None reported.

Poster presentation at the meeting of the European Society of Coloproctology, Dublin, Ireland, September 23 to 25, 2015.

Correspondence: Guillaume Meurette, M.D., Ph.D., Clinique de Chirurgie Digestive et Endocrinienne (CCDE), Institut des Maladies de l’Appareil Digestif (IMAD), 1, place Alexis Ricordeau, CHU de Nantes, 44093 Nantes, France. E-mail: guillaume.meurette@chu-nantes.fr.

© 2019 The American Society of Colon and Rectal Surgeons