Long-term outcomes of Hirschsprung's disease

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.