Grotesque Megacolon (Hirschsprung´s Disease)

Operative findings in a 3.9-year-old boy with a huge megacolon. The whole visible part of the sigmoid and the intraabdominal rectum are grotesquely dilated. It is a very large megacolon. The transitional zone lies deeply in the rectum and is not visible here. This case also belongs by definition to the classical type of congenital megacolon. This case report illustrates different aspects of Hirschsprung's disease: 1) In case of a long-standing disease the megacolon may become very large. 2) It points to an increasing decompensation of an otherwise normally innervated intestinal segment; therefore, a stasis of the intestinal content occurs all the more, and recurrent enterocolitis is promoted. 3) The pull-through operation becomes more difficult. 4) Part of the dilated intestinal segment must be removed because otherwise a continuous constipation is unavoidable. The latter is also true for ultrashort types which are often diagnosed only after years, and at this moment a sphinctermyectomy is not sufficient for the same reasons ( $$obst_21??££ compare patient with ultrashort type§§).