ABSTRACT
This chapter explains the key principles in the management of complex pediatric colorectal diagnoses. It provides case-based presentations, radiographic images, operative images with multiple choice questions to test knowledge. The chapter presents a case study of a 6 years old male with total colonic Hirschsprung disease at birth. He has an ileostomy that has previously been revised, with loss of 30 cm of small bowel. Total colonic Hirschsprung disease patients can be challenging to manage following an ileoanal or ileo-Duhamel pull-through. These children should be managed initially with an ileostomy, until they are at an age when the ileostomy output is thickened, and this can be with the aid of loperamide, water-soluble fiber, and constipating foods. If the parents are highly motivated and dedicated to meticulous skin care, the surgeon may consider performing the operation earlier in life, but the risks of perineal skin breakdown should not be underestimated.
