ABSTRACT
This chapter explains the key principles in the management of complex pediatric colorectal diagnoses. It provides case-based presentations, radiographic images, and operative images with multiple choice questions to test knowledge. The chapter presents a case study of a 6-year-old boy with known Hirschsprung disease (HD) and trisomy 21. He had a pull-through procedure as a newborn infant. The child describes obstructive symptoms. He has a long-standing history of abdominal distension and episodes of fecal incontinence, usually at night time, and on occasion passes large stools. He has had one episode of Hirschsprung-associated enterocolitis in the previous 12 months requiring hospitalization. His weight gain is suboptimal and his appetite is poor. The contrast enema shows the distal colon to be narrow and potentially externally constricted in the very terminal portion. This is most commonly secondary to a retained Soave cuff or could be due to a stricture.
