1987 年 23 巻 1 号 p. 7-17
Motility disorders of the gut in children have become a matter of increasing concern for the pediatric surgeon. Infantile hyper trophic pyloric stenosis is the most common disease requiring surgery in early infancy. While this entity was first described as early as 1888 by Harold Hirschsprung, its etiology and pathogenesis are still an enigma. Fortunately, its surgical treatment is simple and safe, which cannot be said of all other motility disorders of the infantile gut. Small-bowel dysmotility in atresia of the small bowel and in gastroschisis is not related to innervation disorders, but entirely secondary to damaged intestinal muscles caused by concomitant ischemia of the bowel wall. In contrast, the temporarily adynamic bowel of the prematurely born child, as well as Hirschsprung's disease and related disorders, are the result of anomalies of the intestinal innervation. Although aganglionosis has long been recognized as a congenital anomaly of the bowel, neuronal dysplasia(abnormal tissue development) of the gut is still a mystery to surgeons and physicians alike. With his pressure studies of the colon, Swenson first recognized Hirschsprung's disease for what it was. This led to the resection of the manometrically diagnosed abnormal colon, which was found to be aganglionic. Histological investigation of the bowel wall became the conclusive tool, replacing manometry, in the diagnosis of Hirschsprung's disease. Its reliability has become a point of discussion again, as the finding of either ganglionosis or aganglionosis is not the definite discriminating factor between normally and abnormally innervated bowel. Nor does it provide sufficient informing to explain the cause of all innervation disorders of the gut in infancy and childhood. Many hypotheses have been proposed. The enteric nervous system is still an enigma, although its origin is known, at least in birds. Why neural crest cells travel, along what paths, how they reach their destination, and what may go wrong during this migratory prcess are questions that must be answered before a full understanding and, consequently, proper treatment of neuronal dysplasia of the gut will be achieved.
