Hirschsprung disease: lessons learned from the last 100 cases]

We analyize our experience in the management of the last consecutive 100 Hirschsprung's disease (HD) patients divided into two periods: 1992-1997 and 1998-2004, in order to find out differences in morbidity, mortality and outcome between them.During this period, 72 males and 28 females were treated. Twelve had family history and five suffered from Down's syndrome. Information about clinical onset, need of stomas, surgical procedures, continence, outcome and mortality was recorded. We compared the results between the two groups with non-parametrics stadistics test.50% of patients were symptomatic in the newborn period and 25% of them needed some surgical procedures. Seventy four patients suffered from rectosigmoid forms, fourteen colic forms and twelve were total colonic HD (7 with small bowel extension). Hystochemistry was diagnostic in 98%. Nursing was effective in 47 cases. Differences in the need of stomas were found between the two periods: 30% during the first period and 6% during the second one (p<0,05). Twenty percent (20) of the patients suffered from enterocolitis (with no differences between both groups), and 13 of them still had enterocolitis episodes in spite of stomas or pull-through procedures. We performed 49 Swenson, 29 Soave, 14 transanal and 2 Lester-Martin procedures. The median age at definitive operation was smaller in the last period when compared to the first (p< 0.05). We found good results on continence in 86%, with no relation with definitive surgical procedure nor with the period of time studied.The younger age at definitive treatment, the performance of stomas and the increase of transanal procedures were the principal differences between the two groups.