Hirschsprung's disease: an historical review.

Abstract

A historical review of Hirschsprung's disease is of relevance for several reasons. The historical events are revealing as to how clinical diseases are often slowly unravelled. In addition, many unsolved problems are highlighted. Firstly the exact cause is unknown. There is obviously an interaction between genetic and environmental factors, the nature of which is of interest to basic scientists as well as clinicians. Secondly the pathophysiological explanation for the functional obstruction, and especially its variability, is still incomplete. Much more needs to be known about normal gastrointestinal physiology before this question can be fully answered. Thirdly the technique and timing of operative correction remains inconclusive. Despite extensive postoperative assessment there is no one operation that is clear superior. Each have characteristic problems, but all share the main problem; the abnormal internal sphincter. It remains uncertain how much (if any) of the sphincter should be bypassed. As well the optimal timing of operation is uncertain, with some theoretical advantages being suggested for earlier operation. However, these advantages need to be balanced against possible technical problems. Nevertheless the aim of surgical correction should be the full attainment of normal faecal continence.