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 3-year-old girl with Hirschsprung disease. The highest recurrence risk is in the sister of a girl with short segment Hirschsprung disease. The highest recurrence risk is in the brother of a girl with long segment Hirschsprung disease. The highest recurrence risk is in the sister of a boy with long segment Hirschsprung disease. The highest recurrence risk is in the brother of a boy with short segment Hirschsprung disease. The incidence of Hirschsprung disease is 1:5000; however, the overall recurrence risk in siblings is 4", given a relative risk of 200 compared to the general population. In isolated Hirschsprung disease, the length of the aganglionosis and sex of the children affects the risk of recurrence.
