Elsevier

Journal of Pediatric Surgery

Volume 45, Issue 12, December 2010, Pages 2398-2402
Journal of Pediatric Surgery

PAPS Papers
Long-term outcomes and the quality of life of Hirschsprung disease in adolescents who have reached 18 years or older—a 47-year single-institute experience

https://doi.org/10.1016/j.jpedsurg.2010.08.040Get rights and content

Abstract

Background and Aims

Hirschsprung disease is considered to be curable when performing definitive surgery. However, the postoperative bowel function is not always satisfactory. The long-term postoperative function of bowel evacuation and the quality of life of such patients are considered to be important. In this study, we evaluated the general condition, bowel function, and social performance in adults who were older than 17 years who had suffered from Hirschsprung disease in childhood.

Methods

From 1963 to 2009, 184 patients with Hirschsprung disease underwent definitive surgery, mostly Z-shaped anastomosis at Kyushu University Hospital. As a result, 146 (95.4%) of 153 of those patients survived and reached 17 years of age. Their present status and symptoms, anorectal functions, genitourinary functions, and social performance were evaluated during the clinical follow-up based on a questionnaire survey.

Results

In our series, the evacuation score was rated as “excellent” (score of 7-8/8) in 66.7%, “good” (5-6/8) in 19.0%, “fair” (3-4/8) in 11.9%, and “poor” (0-2/8) in 2.4%. Therefore, 85.7% were considered to have a satisfactory bowel function. However, only 21.4% had a completely normal score of 8/8. Incontinence occurred in 16.7%, and soiling was present in 19.0% of the questionnaire respondents. The genitourinary function was considered to be within the reference range, and urinary problems were minimal. Among the respondents, 45.2% were married, and 68.4% of those married had children. The educational and professional careers of the respondents were successful.

Conclusions

In general, the bowel function was satisfactory. However, the ratio of patients with completely normal bowel function was low. Because incontinence and soiling impair the quality of life, pediatric surgeons should therefore continue trying to achieve a complete bowel function after definitive surgery for the treatment of Hirschsprung disease.

Section snippets

Methods

From 1963 to 2009, 184 patients with Hirschsprung disease underwent definitive surgery mostly Z-shaped anastomosis4 at Kyushu University Hospital. As a result, 146 (95.4%) of 153 patients survived and reached 17 years of age. Five cases died of early postoperative complications, such as peritonitis, enterocolitis, and pneumonia, and 2 patients died in traffic accidents 13 and 17 years after operation, respectively. Their present status and symptoms, anorectal functions, genitourinary functions,

Background of patients

The 43 patients included 35 males and 7 females. The extent of aganglionosis and the number of patients affected were as follows: lower rectum, aganglionosis restricted to the lower rectum under peritoneal reflection (n = 2); sigmoid colon, aganglionosis extended to the sigmoid colon (n = 32); left-right colon, aganglionosis extended beyond the sigmoid colon, but did not reach the cecum (n = 4); total colon, aganglionosis was limited to the total colon and 30 cm of the terminal ileum (n = 3);

Discussion

Hirschsprung disease is one of the representative functional disorders in pediatric surgery and has been considered to be curable by anastomosis of the normal ganglionic intestine to the anal canal by means of definitive surgery.

In this study, we demonstrated the functional prognosis of Hirschsprung disease in adolescents. The bowel function of postoperative state in Hirschsprung disease in adolescents who reached 18 years or older was satisfactory. Regarding the social performance, they had

Acknowledgment

We thank Mr Brian Quinn for comments and help with the manuscript. This study was supported by Grants-in-Aid for Scientific Research from the Japan Society for the Promotion of Science and Kyushu University Interdisciplinary Programs in Education and Projects in Research Development (P&P).

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