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Published on: April 26, 2019
Long-term outcomes of Hirschsprung's disease
Risto J Rintala1, Mikko P Pakarinen
1Section of Pediatric Surgery, Hospital for Children and Adolescents, University of Helsinki, Finland. risto.rintala@hus.fi
Insights
Hirschsprung's disease (HD) often leads to lifelong bowel dysfunction and psychosocial challenges, impacting adult quality of life. Specialized, long-term follow-up is crucial for managing these persistent issues.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Clinical Medicine
Background:
- Hirschsprung's disease (HD) surgical outcomes remain suboptimal despite advances.
- Functional deficits and psychosocial issues in childhood HD frequently persist into adulthood.
Purpose of the Study:
- To evaluate the long-term functional and psychosocial outcomes in adult patients with Hirschsprung's disease.
- To identify specific challenges and risks associated with adult HD.
- To emphasize the need for specialized, lifelong patient follow-up.
Main Methods:
- Review of prospective and cross-sectional studies on adult HD patients.
- Assessment of bowel function, quality of life, and psychosocial adjustment.
- Analysis of long-term sequelae and associated conditions.
Main Results:
- Adult HD patients commonly experience constipation and fecal soiling, with lower overall bowel function scores than controls.
- Quality of life may decline with age, though most adults maintain social functioning.
- Increased risk for certain cancers necessitates targeted screening programs.
Conclusions:
- Long-term complications of Hirschsprung's disease significantly affect adult patients.
- Lifelong, specialized medical follow-up is essential for managing adult HD.
- Early diagnosis and management can improve long-term outcomes and quality of life.
Abstract:
Despite significant developments in the understanding of the pathologic anatomy and physiology of Hirschsprung's disease (HD), the results of surgical therapy remain far from perfect. The functional defects and psychosocial difficulties that occur commonly in children with HD are passed on to adulthood in a significant proportion of patients. Recent prospective and adequately controlled cross-sectional studies reveal that constipation and fecal soiling are common late sequelae in adulthood. HD patients show uniformly lower scores of overall bowel function than healthy control subjects. Functional outcome and quality of life may be interrelated and deteriorate with increasing age. Despite these shortcomings, at adult age, most of the HD patients appear to be able to function as normal members of the society in terms of psychosocial, occupational, and recreational activities. Patients are at risk of developing related conditions, such as cancer, that require planning of specific screening programs. Many of the long-term problems that these patients experience are specific to HD. The follow-up of HD patients should be performed by medical personnel familiar with the disease, preferably in a specialized referral center, and the follow-up should continue beyond childhood.
